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Renal system complete

Renal system complete



Disorders of kidney and urinary tract are commonly seen in pediatric units as medical and surgical problems. Congenital malformations, neoplasms, infections, inflammations and progressive impairment ...

Disorders of kidney and urinary tract are commonly seen in pediatric units as medical and surgical problems. Congenital malformations, neoplasms, infections, inflammations and progressive impairment of renal functions are common conditions found in children.



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    Renal system complete Renal system complete Document Transcript

    • INTRODUCTION: Disorders of kidney and urinary tract are commonly seen in pediatric units as medical and surgical problems. Congenital malformations, neoplasms, infections, inflammations and progressive impairment of renal functions are common conditions found in children. NORMAL EMBRYOLOGY The Urinary system goes through three phases on its way to becoming fully functioning: 1. Pronephros 2. Mesonephros 3. Metanephros  Pronephros – Transient rudimentary and nonfunctioning system that begins in the fourth week of gestation (ie, day 22) and disappears by end of the fourth week (i.e, day 28). Degeneration of the pronephros is required for normal kidney development.  Mesonephros – Derived from the intermediate mesoderm by day 26 and by the fifth week develops into 20 paired tubules that produce small amounts of urine. The mesonephros ultimately fuses with the cloaca and contributes to the formation of the urinary bladder, and in the male, the genital system is derived from the mesonephric ducts and some tubules.  Metanephros – The metanephros, which is composed of the metanephric mesenchyme and ureteric bud epithelium (caudal portion of the mesonephric duct), is the last stage of renal development and forms the permanent kidney beginning at the fifth week of gestation. Starting from 4th week & end on 36 week of intra uterine life:     4 wks gestation : kidney start development 9 wks : first glomeruli , Bladder 36 wks: nephrogenesis ceases (1 million glomeruli in each kidney). Postnatal increase in the size of the kidney is due to enlargement of the Glomerular diameter & significant increase in tubular volume & length  Active period of nephrogenesis between 20-36wks, cease around 36 wks. DEVELOPMENT OF RENAL FUNCTION IN THE CHILD Renal function in an infant is subnormal by adult standards especially in the premature neonate, which put them into high risk to develop acute renal failure. Glomerular filtration begins between 9 to 12 weeks of gestation, initiating formation of urine. Fetal urine is the major component of amniotic fluid and significant 1
    • reduction of amniotic fluid (oligohydromnios) is usually associated with renal disease in the baby. It is expected the neonate produces urine and voids in 12 – 24 hours after birth. The GFR at birth is 10-20ml in the first 3 days but increases rapidly to 75-80ml by 8 weeks. The pH of urine of a newborn is inappropriately high due to the limited bicarbonate and sodium reabsorbtion. Prior to birth fetus does not require intact renal function because the mother provides homeostasis., therefore a child with bilateral agenesis will be able to survive for several days after birth. Renal function continues to improve during the first two years of life. At the end of this period, various parametres of renal function appraoch adult values, if corrected to a standard surface area. ANATOMY AND PHYSIOLOGY OF RENAL SYSTEM: The organs, tubes, muscles, and nerves that work together to create, store, and carry urine are the renal system. It is one of the excretory systems of the body. The renal system includes two kidneys, two ureters, the bladder, two sphincter muscles, and the urethra. The development starts from intra uterine period. 2 kidneys- which secrete urine The kidneys are situated retro peritoneally on each side of the vertebral column. Each kidney contains about one million nephrons, the functional units. A nephron consists of a glomerulus, proximal tubule, the thin limbs, the distal tubule and the collecting segment. 2 ureters- which convey the urine from kidneys to the urinary bladder 1 urinary bladder- where urine collects and is temporarily stored 1 urethra- through which the urine is discharged from the urinary bladder to the exterior. 2 sphincters- Internal: involuntary sphincter of smooth muscle External: skeletal muscle inhibits urination voluntarily until proper time 2
    • FUNCTIONS The regulation of fluid volume, blood pressure, excretion of metabolic waste products and drug metabolites are the primary functions of renal system. The kidneys are also responsible for conversion of vitamin D to its active form, serum pH regulation and synthesis of hormones such as erythropoietin and rennin. There are 3 functions of renal system. 1. Excretion & Elimination 2. Homeostatic regulation 3. Endocrine function 1. Excretion & Elimination: Removal of organic wastes products from body fluids (urea, creatinine, uric acid)  Excretion of excess electrolytes, nitrogenous wastes and organic acids  The maximal excretory rate is limited or established by their plasma concentrations and the rate of their filtration through the glomeruli.  The maximal amount of substance excreted in urine does not exceed the amount transferred through the glomeruli by ultrafiltration except in the case of those substances capable of being secreted by the tubular cells. 2. Homeostatic regulation: Water -Salt Balance Blood volume is associated with Salt volume. The greater the blood volume, the greater the blood pressure. Removing water lowers blood pressure. Regulate blood volume and blood pressure: Regulate plasma ion concentrations: by adjusting volume of water lost in sodium, potassium, and chloride ions (by urine controlling quantities lost in urine) releasing renin from the juxtra calcium ion levels glomerular apparatus 3
    • Acid - base Balance The kidneys control this by excreting H+ ions and reabsorbing HCO3 (bicarbonate). If plasma pH is low (acidic): H+ secretion in the urine If plasma pH is high (alkaline): and HCO3¯ H+ secretion in the urine and HCO3¯ reabsorption back to the plasma increases thus reabsorption back to the plasma decreases thus urine becomes more acidic, and the plasma more urine becomes more alkaline, and the plasma alkaline. more acidic. 3. Endocrine function: Kidneys have primary endocrine function since they produce hormones (erythropoietin, renin and prostaglandin).  Erythropoietin is secreted in response to lowered oxygen content in the blood. It acts on bone marrow, stimulating the production of red blood cells.  Renin the primary stimuli for renin release include reduction of renal perfusion pressure and hyponatremia. Renin release is also influenced by angiotensin II and ADH.  The kidneys are primarily responsible for producing vitamin D3  In addition, the kidneys are site of degradation for hormones such as insulin and aldosterone, The body takes nutrients from food and uses them to maintain all bodily functions including energy and self-repair. After the body has taken what it needs from the food, waste products are left behind in the blood and in the bowel. The renal system works with the lungs, skin, and intestines-all of which also excrete wastes-to keep the chemicals and water in the body balanced. The renal system removes urea from blood. Urea is carried in the bloodstream to the kidneys. The sensation to urinate becomes stronger as the bladder continues to fill and reaches its limit. At that point, nerves from the bladder send a message to the brain that the bladder is full, and the urge to empty your bladder intensifies. When the person urinates, the brain signals the bladder muscles to tighten, squeezing urine out of the bladder. At the same time, the brain signals the sphincter muscles to relax. As these muscles relax, urine exits the bladder through the urethra. When all the signals occur in the correct order, normal urination occurs. GLOMERULAR FILTRATION The glomerular filtration rate is approximately 20ml/min in full term newborns and 10 – 13 ml/min in infants born at 28-30 weeks gestation. It reaches its adult level by 12 – 24 months. Until then the kidneys are unable to fully maintain water balance and to filter solutes and medications out of the blood stream. Any fluid 4
    • that is not returned to the circulation becomes a component of urine. The daily urine production is approximately 1-3ml/kg/h for a neonate. RENAL PERFUSION The renal blood flow rate averages 20% to 25% of cardiac output each minute. Adequate blood flow to the kidneys is required to produce a sufficient glomerular filtration rate and urine production. When the sympathetic nervous system is stimulated, such as occurs in a stress response, both the afferent and efferent renal arterioles constrict, producing a decrease in blood flow. DIURESIS (MICTURITION) When bladder fills with 200 ml of urine, stretch receptors transmit impulses to the CNS and produce a reflex contraction of the bladder (PNS) RENAL SYSTEM IN A NUTSHELL It is the body‘s drainage system for removing wastes and extra water. The urinary system includes two kidneys, two ureters, a bladder, and a urethra. The kidneys are a pair of bean-shaped organs, each about the size of a fist and located below the ribs, one on each side of the spine, toward the middle of the back. Every minute, a person‘s kidneys filter about 3 ounces of blood, removing wastes and extra water. The wastes and extra water make up the 1 to 2 quarts of urine an adult produces each day. Children produce less urine each day; the amount produced depends on their age. The urine travels from the kidneys down two narrow tubes called the ureters. The urine is then stored in a balloon like organ called the bladder. Routinely, urine drains in only one direction—from the kidneys to the bladder. The bladder fills with urine until it is full enough to signal the need to urinate. In children, the bladder can hold about 2 ounces of urine plus 1 ounce for each year of age. For example, an 8-year-old‘s bladder can hold about 10 ounces of urine. When the bladder empties, a muscle called the sphincter relaxes and urine flows out of the body through a tube called the urethra at the bottom of the bladder. The opening of the urethra is at the end of the penis in boys and in front of the vagina in girls. 5
    • DIAGNOSTIC EVALUATION Diagnostic evaluation of the problems should include details of history of illness, clinical examination, blood tests, imaging of urinary tract and renal biopsy. There are only a few specific manifestations of renal diseases in infants and children. Therefore a careful family history of renal disease should always be obtained. HISTORY OF ILLNESS Past medical history - Mother‘s pregnancy history - Maternal polyhydramnios - Oligohydramnios - DM - HTN Neonatal history - Presence of single umbilical artery - Abdominal mass - Chromosome abnormality - Congenital malformation Present history - Burning micturition - Change in voiding pattern - Vaginal or urethral discharges - Poor growth - Weight gain - Trauma - Diabetes increases risk of UTI Assess output changes, voiding pattern changes, changes in urine color or pain Past history Past UTI, kidney trauma, stones, incontinence and leakage of urine Medications Childhood problems, alcohol and illicit drug use Nephrotoxicity Hypertension contributes to renal failure and nephropathy Family history Renal or cardiovascular disorders, diabetes, cancer or chronic illness - 6
    • PHYSICAL EXAMINATION A. INSPECTION What to test What is the procedure Observe whether child looks sick or well. Is child depressed, drowsy Height, weight,% charts Muscle bulk, subcutaneous fat, skin folds What if the interpretation Sick look suggests chronic renal failure, UTI, altered mental state suggests uremia. Short, thin stature suggests CRF. Obese, moon face suggests steroid effect. Loss of muscle mass, subcutaneous fat suggests CRF. Rickets General observation What to look for Wellness of the child Mental status Growth (short stature) Nutrition Renal rickets and chronic glomerular disease SLE Steroid therapy in nephritic syndrome Nephritis, nephritic syndrome Alports syndrome Dehydration Bony defects Face General examination Eyes Ear General examination Malar flush Hirsuitism Periorbital edema Anemia, cataract, icterus Lenticonus, deafness Dry tongue Uremic breath Neck Ribs Elevated jugular venous pressure Beading, harrison‘s sulcus CVS Cardiomegaly, congestive failure, pericardial effusion RS Distention Tachypnea, crepitations Tenderness on palpation Pleural effusion, pulmonary edema Peritonitis Kidneys Ballotable kidneys Enlarged kidneys Peritoneal free fluid Ascites – on percussion Nephritic syndrome, CRF Bladder Enlarged, urine retension Scrotum Scrotal edema Obstructive uropathy, bladder Nephritic syndrome testis Absence of testis Cryptorchidism Muscle bulk Poor muscle mass CRF Ankle edema Chest Mouth Uremia Pitting on pressure Nephrotic syndrome, acute nephritis Bony deformity Bowed legs, epiphyseal thickening heart Volume overload in CHF, glomerulonephritiss, CRF Renal rickets acute Chronic renal failure, anemia Abdomen neurogenic Genitalia Upper limbs and lower Renal osteodystrophy Foot slapping Peripheral neuropathy Elevated blood pressure AGN, CRF Gait Blood pressure 7
    • Palpation Check the status of the kidneys and urinary bladder. KIDNEY – with the child in supine position and the abdomen relaxed, place the palm of one hand posteriorly at the flank pushing the kidneys forward, while other hand is placed anteriorly below costal margin, pushing abdominal wall backward and upward. Kidneys is felt best in deep inspiration. The kidneys lie in the costovertebral area, the region bounded by the lumbar spine and the 12th rib on either side. To detect tenderness due to kidney inflammation, gently tap over the costovertebral area with a fist. This usually does not cause pain unless the underlying kidney is inflamed. BLADDER – the bladder can be palpated in the neonate and infants easily. In older children it is easily percussible when it is distended. The urinary bladder can be palpated just superior to the pubic symphysis. However, on the basis of palpation alone, urinary bladder enlargement can be difficult to distinguish from the presence of an abdominal mass. Abnormal findings: Kidney: Lump or mass: tumor Tenderness: infection Unequal size: hydronephrosis Bilateral enlargement: polycystic kidney disease Percussion: Bladder: Lump or mass: tumor or cyst Distention: retention or obstruction Percuss the abdomen for unusual dullness ( normal heard over the spleen at the right costal margin, over the kidneys, and 1 – 3 cm below the left costal margin) or flatness. A full bladder may yield dullness above the symphysis pubis. Tenderness in the flank may be detected by fist percussion. If CVA tenderness & pain are present, indicate a kidney infection or polycystic kidney disease. Abnormal findings: Kidney Percussion: Bladder percussion: Tenderness and pain indicates inflammation (glomerulonephritis or glomerulonephrosis) Dull sounds in a patient who has just voided indicates bladder dysfunction or infection 8
    • Auscultation: Listen for heart sounds – murmur may be present in the anemic child with renal disorder -elevated heart rate Auscultate blood pressure noting elevation or depression. Absence of bowel sounds may indicate peritonitis The abdominal aorta & renal arteries are auscultated for a bruit, which indicates impaired blood flow to the kidneys. Abnormal findings: Systolic Bruits Renal artery stenosis CLINICAL FEATURES OF RENAL DISEASE The primary symptoms of urinary system disorders are pain and changes in the frequency of urination. The nature and location of the pain can provide clues to the source of the problem.  Pain •Pain in the superior pubic region may be associated with urinary bladder disorders. •Pain in the superior lumbar region or the flank that radiates to the right upper quadrant or left upper quadrant can be caused by kidney infections such as glomerulonephritis, pyelonephritis, or kidney stones. •Dysuria may occur with cystitis and urinary obstructions.  Frequency of urination Individuals with urinary system disorders may urinate more or less frequently than usual and may produce normal or abnormal amounts of urine: •Incontinence It is an inability to control urination voluntarily, may involve periodic involuntary urination, or a continual, slow trickle of urine from the urethra. Incontinence may result from urinary bladder or urethral problems, damage or weakening of the muscles of the pelvic floor. Renal function and daily urinary volume are normal. •Urinary retention In this renal function is normal, at least initially, but urination does not occur. Urinary retention in males often results from prostatic enlargement and compression of the prostatic urethra. In both sexes, urinary retention may result from obstruction of the outlet of the urinary bladder, or from CNS damage.  Hematuria The presence of blood in the urine should be confirmed by the microscopic examination of urine. The colour may vary from frank red to shades of brown, described as tea or cola – coloured. It may be glomerular or 9
    • extra glomerular. Blood in the initial urine suggests urethral origin and terminal hematuria indicates bladder origin. If it is uniform throughout, then that is most often AGN. Hypercalciuria, clotting disorders, renal trauma, hemorrhagic cystitis, hemoglobinuria, methemoglobinuria and injestion of certain drugs such as rifapicn also leads to hematuria.  Edema GN manifests with facial puffiness and gross hematuria. Edema is turgid and does not pit readily on pressure. In nephrotic syndrome edema develops incidiously, starting with puffiness around the eyes and then involving the feet and legs. Edema is soft and easily pits on pressure.  Abnormalities of micturition A poor urinary stream in a male infant especially in the presence of a full bladder, suggests obstruction, most commonly due to posterior urethral valve. Crying during micturition and straining suggests obstruction. Retention of urine may be due to neurologic bladder or obstruction by stone or tumor. Persistent dribbling of urine indicates abnormal urethral insertion distal to bladder neck.  Oliguria It is the passage of insufficient volume to maintain homeostasis. (<500ml/24h/1.73m2; in infant it is <1ml/kg/hr). Decreased urine output is an important feature of renal disease. A cause such as gastroenteritis other conditions that lead to prenatal type of renal failure may be detected. It is an important feature of moderate or severe AGN and other conditions causing glomerular injury.  Polyuria It is the passage of excessive amount of urine (5-6 ml/kg/hr). Impairment of urinary concentration is an early feature of obstructive uropathy and other disorders characterized by tubulointerstitial lesions.  Fever A fever commonly develops when the urinary system is infected by pathogens. Urinary bladder infections (cystitis) often result in a low grade fever; kidney infections, such as pyelonephritis, usually produce very high fevers. 10
    • Diagnostic Evaluations – Flowchart Hematuria RBC deposits - ⁺ Hemoglobinuria, myoglobinuria KUB ultrasound Normal Abnormal,Hydronephrosis, Calculus,cyst, tumor Renal function, protienuria normal abnormal Family history Recurrent Present Hereditary nephritis (alport) Absent IgA nephropthy Renal biopsy LABORATORY EXAMINATION URINE EXAMINATION The urine examination should be fresh and relatively concentrated. A midstream urine specimen is generally adequate. In infants, a specimen obtained by supra pubic or transurethral catheterization is preferred for culture. Dipstick method is now widely used to test for proteinuria and many other constituents. Composite 11
    • strips that measure pH, glucosuria, hematuria, leucocytouria and bacteriuria are available and useful as screening tests. Microscopic examination- a fresh centrifuged specimen should be examined. Red cell casts – glomerular inflammation Clumping of neutrophills – acute pyelonephritis - A 24 hour urine collection is difficult but such procedure is resorted when actually needed. E.g. calcium, phosphate, creatinine, magnesium, oxalate. - Spot urine samples are sufficient for nephrotic syndrome and various forms of GN, protein / creatinine ratios tests. URINALYSIS The normal characteristics of urine: Color- pigment is urochrome Yellow color due to metabolic breakdown of hemoglobin (by bile or bile pigments) Beets or rhubarb- might give a urine pink or smoky color Vitamins- vitamin C- bright yellow Infection- cloudy Water: specific gravity = 1g/liter; Urine: specific gravity = 1.001 to 1.030 Pyelonephritus- urine has high s.g.; form kidney stones Diabetes insipidus- urine has low s.g.; drinks excessive water; injury or tumor in pituitary Odor- normal is ammonia-like diabetes mellitus- smells fruity or acetone like due to elevated ketone levels diabetes insupidus- yucky, asparagus pH - range 4.5-8 average 6.0 specific gravity– more than 1.0; ~1.001-1.003 vegetarian diet- urine is alkaline protein rich and wheat diet- urine is acidic 12
    • Abnormal Constitutes of Urine Glucose - when present in urine condition called glycosuria (nonpathological) Indicative of: • Excessive carbohydrate intake • Stress • Diabetes mellitus Albumin-abnormal in urine; it‘s a very large molecule, too large to pass through glomerular membrane > abnormal increase in permeability of membrane Albuminuria- nonpathological conditions- excessive exertion, pregnancy, overabundant protein intake-leads to physiologic albuminuria Pathological condition- kidney trauma due to blows, heavy metals, bacterial toxin Ketone bodies- normal in urine but in small amounts Ketonuria- find during starvation, using fat stores Ketonuria is couples w/a finding of glycosuria-- which is usually diagnosed as diabetes mellitus RBC- hematuria HemoglobinHemoglobinuria- due to fragmentation or hemolysis of RBC; conditions: hemolytic anemia, transfusion reaction, burns or renal disease Bile pigmentsBilirubinuria (bile pigment in urine)- liver pathology such as hepatitis or cirrhosis WBCPyuria- urinary tract infection; indicates inflammation of urinary tract Casts- hardened cell fragments, cylindrical, flushed out of urinary tract WBC casts- pyelonephritus RBC casts- glomerulonephritus Fatty casts- renal damage 13
    • BLOOD TESTS Normal range of blood urea level is 20-40 mg/dl. The levels increases in case of reduced renal perfusion, increased tissue break down, trauma, gastrointestinal bleeding and use of corticosteroids and tetracycline. Serum albumin is reduced in patients with heavy proteinuria, occasionally below 1.5g/dl. It is typically present in children with nephrotic syndrome and hypercholesterolemia. IMAGING STUDIES Expert ultrasonography is the initial modality in most renal disorders. Radionuclide examinations have clear indications particularly in the investigation of renal and urinary tract anomalies, obstructive uropathy, urinary tract infections and renovascular hypertension. Imaging Of The Urinary Tract  Micturing cystourethrogram (MCU) It is necessary for the diagnosis and evaluation of severity of vesico ureteric reflux and the detection of abnormalities of bladder and urethra. The contrast agent is introduced into the bladder through a catheter or directly by supra pubic puncture. Films are taken when the child is voiding.  Intra venous pylogram (IVP) The functional anatomy of the urinary system can be examined by administering a radiopaque compound that will enter the urine which permits the creation of an intravenous pyelogram, by taking an X-ray of the kidneys. This procedure permits detection of unusual kidney, ureter, or bladder structures and masses.  Ultrasonography It gives excellent information about the anatomical aspects. It is ideal for children as it is painless and requires no sedation or a contrast agent. It can be carried out even at bedside and repeated a required. In some interventions also ultra sound guidance is used. Considerable expertisation is required for interpretation of findings.  Radionuclide imaging This in non invasive, highly sensitive and expose the patient to a much smaller amount of radiation. The techniques include: a) Renal perfusion study (renography) It monitors the arrival, uptake and elimination of a radiopharmaceutical by the kidney. b) Renal static imaging It gives a two dimentional depiction of the concentration and distribution of the radionuclide. c) Clearance studies It accurately assesses the individual kidney function. d) Direct radionuclide cystography 14
    • It is more reliable for detecting VUR as the radiation dose to patient is greatly reduced compared to MCU. It provides a visual representation of the rate of bladder emptying, residual urine volume and evidence of VUR. RENAL BIOPSY Usually percutaneous specimen ix obtained s by inserting a needle through the skin and into the kidney. The sample of the kidney is then microscopically examined. It is usually not necessary in uncomplicated cases. Indications of renal biopsy: i. Nephrotic syndrome Secondary Suspected Corticosteroid non minimal lesion Congenital ii. Acute nephrotic syndrome Unresolving or progressive GN Associated systemic features iii. Acute renal failure Prolonged anuria Undetermined causes iv. Recurrent gross hematuria v. Persistent proteinuria vi. Hereditory nephropathies vii. Interstitial nephritis CONGENITAL ABNORMALITIES Congenital anomalies of the kidney and urinary tract constitute approximately 20 to 30 percent of all anomalies identified in the prenatal period. Defects can be bilateral or unilateral, and different defects often coexist in an individual child. CLASSIFICATIONS 1) a. b. c. Dysgenesis of the kidney Renal agenesis(absent kidney) Renal hypoplasia Renal dysplasia 15
    • 2) a. b. c. d. Abnormalities in shape & position: Ectopic kidney Fusion anomalies horseshoe kidney crossed fused ectopia 3) a. b. c. d. Abnormalities of the collecting system: Hydronephrosis Bladder extrophy PUV Patent urachus 1. DYSGENESIS OF THE KIDNEY a)Renal agenesis Renal agenesis is the name given to a congenital absence or under development of one or both kidneys. The kidneys develop between the 5th and 12th week of fetal life, and by the 13th week they are normally producing urine. When the embryonic kidney cells fail to develop, the result is called renal agenesis. It is due to failure of ureteric bud formation or mesenchymal blastoma differentiation of final mesenchymal condensation. ETIOLOGY - Usually there is no family history of renal agenesis, but in 20-36% of cases, there is a genetic cause. The risk of recurrence in future pregnancies is 3% unless one parent has unilateral renal agenesis, in which case the risk is about 15%. Women with uncontrolled diabetes in pregnancy may deliver a baby with bilateral renal agenesis. TYPES i. ii. Unilateral Bilateral 1.UNILATERAL Unilateral renal agenesis is much more common, but is not usually of any major health consequence, as long as the other kidney is healthy. It is associated with an increased incidence of mullerian duct abnormalities which are abnormalities of the development of the female reproductive tract and can be a cause of infertility. Children with this condition are advised to approach contact sports with caution. INCIDENCE Unilateral renal agenesis occurs in 1 of 1000-2000 live births. 16
    • CLINICAL MANIFESTATIONS no other symptoms at all. premature birth. low-set ears (This is because the ears and kidneys are formed at the same time in fetal development) The ureters may also be abnormal 2.BILATERAL Bilateral renal agenesis is the uncommon and serious failure of both a fetus' kidneys to develop during gestation. Bilateral renal agenesis with associated malformations is known as Potters Syndrome. This absence of kidneys causes oligohydramnios, which can place extra pressure on the developing baby and cause further malformations. The condition is frequently, but not always the result of a genetic disorder, and is more common in infants born to one or more parents with a malformed or absent kidney. When this condition is not compatible with survival; in fact, 40% of babies with bilateral renal agenesis will be stillborn, and if born alive, the baby will live only a few hours. Prior to birth fetus does not require intact renal function because the mother provides homeostasis., therefore a child with bilateral agenesis will be able to survive for several days after birth. INCIDENCE Bilateral renal agenesis occurs in 1 of 4500 live births and is usually found in boys. CLINICAL MANIFESTATIONS They may have a number of unique characteristics: dry loose skin wide-set eyes prominent folds at the inner corner of each eye sharp nose large low-set ears with lack of ear cartilage underdeveloped lungs absent urinary bladder anal atresia esophageal atresia unusual genitals The lack of amniotic fluid causes some of the problems (undeveloped lungs, sharp nose, clubbed feet) DIAGNOSIS It is often detected on fetal ultrasound because there will be a lack of amniotic fluid (called oligohydramnios). It is detected by US at 12th wk of gestation. 17
    • TREATMENT a. Short-term treatment Bilateral renal agenesis is fatal. If one kidney is present (unilateral renal agenesis) the child will develop normally. Many times the absence of a kidney is detected only incidentally when an older child or adult has an abdominal x-ray for some other reason. The remaining kidney, if properly functioning, can very effectively remove the wastes from the blood and keep the body entirely healthy. Once detected, families where renal agenesis has occurred will be offered genetic counseling because of the possibility of recurrence in future pregnancies. b. Long-term treatment Once diagnosed, children with one kidney (solitary kidney) will be encouraged to protect the remaining kidney from infection or injury. Periodic examinations of the kidney and prompt treatment of any urinary tract infection is required. These children may be counseled to avoid contact sports where the kidney could be injured. NURSING MANAGEMENT Protecting the kidney function is very important. Sometimes children will be prescribed a low dose of an antibiotic to take once a day to prevent kidney infection and damage. Blood pressure should be carefully monitored and elevations treated. Dialysis or kidney transplant are the only options to treat children whose solitary kidney has ceased to function. COMPLICATIONS frequent urinary tract infections high blood pressure kidney stones reflux hydronephrosis b)Renal hypoplasia This may appear as one small kidney with the other one larger. It occurs due to the partial development of kidney. Small kidneys also have small arteries and are associated with hypertension requiring nephrectomy. 18
    • c)Renal dysplasia Multicystic dysplastic kidney is a condition that results from the malformation of the kidney during fetal development. The kidney consists of irregular cysts of varying sizes and has no function. It is the most common type of renal cystic disease, and it is one of the most common causes of an abdominal mass in infants. TYPES I. II. Bilateral Unilateral INCIDENCE the disease is found to be bilateral in 19% to 34% of cases. CLINICAL MANIFESTATIONS Those with bilateral disease often have other severe deformities. In bilateral cases, the newborn has the classic abnormal facies Oligohydramnios Characteristic of Potter's syndrome Contralateral ureteropelvic junction Hypertension (Malignant transformation to Wilm's tumor has been reported) DIAGNOSIS It is usually diagnosed by ultrasound examination antenatally. Mean age at the time of antenatal diagnosis is about 28 weeks, with a range of 21 to 35 weeks. TREATMENT It is not treatable. The patient is observed periodically for the first few years to ensure the healthy kidney is functioning properly and that the unhealthy kidney is not causing adverse effects. In case of renal hypertension or malignant transformation, the unhealthy kidney is removed entirely CONSERVATIVE MANAGEMENT 1. cysts < 5cm , high chance of involution, or cause no problems. 2. reviewed annually for: - BP - urinary protein. - US for cysts involution, of MCDK. growth of contra-lateral kidney. Up to 2yrs of age then at 5yrs of age if normal. 19
    • Nephrectomy: 1- no involution by 2 yrs of age. 2- HTN 3- infections COMPLICATIONS Malignancy: Wilm's‘ tumor, adenocarcinoma& embryonic carcinoma. HTN: cured by nephrectomy. Infection, bleeding into, or rupture of cysts if large. 2. ABNORMALITIES IN SHAPE & POSITION: a)Ectopic kidey Renal ectopia or ectopic kidney describes a kidney that is not located in its usual position. It results from the kidney failing to ascend from its origin in the true pelvis or from a superiorly ascended kidney located in the thorax. b)Fusion anomalies 1. Horse Shoe Kidney It develops when the lower poles of the kidneys are fused in the midline due to fusion of ureteric buds during fetal development. These kidneys are more prone to develop wilms tumour than general. Diagnosis could be done with IVP. Surgery is indicated when uncontrolled urinary infections result in pyelonephritis. 2.Crossed fused ectopia After horseshoe kidney, crossed fused ectopia of the kidneys is the most frequent fusion abnormality of the urinary tract. In this abnormality, both the kidneys are located on the same side with two separate ureters arising from the respective kidneys. The ureter arising from the crossed over kidney travels back to the opposite side and inserts in the bladder. This congenital anomaly is the result of the abnormal development of the ureteric bud and metanephric blastema during the fourth to eighth weeks of gestation. 20
    • 3. ABNORMALITIES OF COLLECTING SYSYTEM a) Hydronephrosis It is the dilatation of the renal pelvis which may be found as unilateral or bilateral. It may be due to obstruction of urine flow in the distal urinary tract or reflux of urine up the ipsilateral ureter or due to bladder neck obstruction or urethral obstruction. ETIOLOGY Uretero Pelvic Junction Obstruction Vesico Urethral Reflux Megaureter Ureterocoele PUV CLINICAL MANIFESTATIONS urinary infections large abdominal mass abdominal pain failure to thrive anemia hypertension Hematuria renal failure. DIAGNOSIS - Antenatal US ( 18-20 WKS) - severity of antenatal US - Unilateral vs. bilateral - Renal parenchyma thin - Bladder - Amniotic fluid Post natal Physical exam: Abdominal mass, palpable bladder USG IVP MCU diuretic isotope renography MANAGEMENT Surgical removal or pyloplasty is done and in case of complication nephrectomy or percutaneous nephrostomy is indicated. 21
    • b) Posterior urethral valve (PUV) It is the most frequent cause of distal urinary tract obstruction. The valves are found usually at the point of junction of posterior urethra with anterior urethra. CLINICAL MANIFESTATION - dribbling of urine abnormal urine stream palpable bladder recurrent urinary tract infections vomiting failure to thrive pulmonary hypoplasia. Poor urinary stream Voiding dysfunction. Urosepsis. DIAGNOSIS - US suggestive at < 24 wks gestation MCU USG Endoscopy MANAGEMENT - It could be done by urinary catheterization Defenitive management is by transurethral destruction of valvular leaflet by balloon catheter. In some cases temporary urinary diversion is done. NURSING MANAGEMENT - correction of electrolytes. Treatment of sepsis. Resp.distress Temporary relieve of pressure c) Exstrophy of bladder (ectopia vesicae) In this the lower portion of abdominal wall and the anterior wall of the bladder are missing, so that the bladder is everted through the opening and may found on the lower abdomen just above the symphysis pubis, with continuous passage of urine to outside. It occurs as a result of altered, not arrested embryogenesis. INCIDENCE: -It is the most common congenital anomaly of lower urinary and genital tracts. 22
    • -It occurs in 1 in 30,000 to 40,000 live births. -it occurs frequently in males than in females. CLINICAL MANIFESTATION: This condition is diagnosed on inspection at birth. -Urinary dribbling through defect -skin excoriation -infection & ulceration of bladder mucosa -ambigous genetalia -wadling unsteady gait -UTI -Growth failure DIAGNOSTIC EXAMINATION: - physical examination -cystoscopy -X-ray -USG -IVP -urodynamic testing MANAGEMENT: -surgical closure of bladder within 48 hours -urinary diversion -complete correction in stages by reconstruction -orthopedic surgery in case of musculoskeletal problems (should be done by school age) 23
    • NURSING MANAGEMENT: Supporting nursing care is important before and after reconstructive surgery to prevent complications. Pre-operative care:-Protection of bladder area from infections and trauma -avoid irritating clothing over exposed bladder -position by back or side -humidifying with wet gauze -Preparation of parents and child for surgery Post-operative care:-close monitoring of child‘s condition -special attention to urinary catheter, drainage -teaching the parents regarding follow up care, complications and prevention d)Patent urachus The urachus is a remnant of allantois, a channel between the bladder and the umbilicus (belly button) where urine initially drains in the fetus during the 1st trimester of pregnancy. The channel of the urachus usually seals off and obliterates around the 12thweek of gestation and all that is left is a small fibrous cord between the bladder and umbilicus called the median umbilical ligament. A patent urachus occurs when the urachus did not seal off and there is a connection between the bladder and the umbilicus. A patent urachus can cause varying amounts of clear urine to leak at the umbilicus. If the urachus remains open all the way to the bladder, there is the danger that bacteria will enter the bladder through the open tube and cause infection. For this reason, the patent urachus of the infant must be removed TREATMENT A surgical incision is made in the baby's abdomen and the patent urachus is removed, then the opening to the bladder is closed. OTHER: Uretero pelvic junction stenosis It is the narrowing of the ureter at the junction between the ureter and renal pelvis of the kidney. It produces blockage of urine drainage from the kidney (similar to the waist in an hourglass). It produces increased backpressure on the kidney and can cause impaired kidney function and ultimately long term potential 24
    • damage to the kidney itself. It is found as a cause of hydronephrosis. It can be associated with ectopic or horse shoe kidney. INCIDENCE Ureteropelvic junction stenosis and obstruction is the most common cause of kidney blockage or obstruction in children. It is the most common site of obstruction in the upper urinary tract It occurs nearly 1 in 500 to 1:1250 live births. ETIOLOGY The two main causes of PUJ obstruction are: intrinsic muscular defect causing impaired peristalsis and urine drainage extrinsic obstruction caused by an aberrant or accessory vascular stalk leading to the lower pole of the kidney and crossing anteriorly to the PUJ or upper ureter. CLINICAL MANIFESTATIONS recurrent renal colic o flank or abdominal pain o nausea o vomiting flank mass without symptoms often associated with UTI upper abdominal pain DIAGNOSIS Prenatal ultrasonography USG IVP Renal scan Renal function test MANAGEMENT Surgery; pyloplasty is indicated to remove obstruction and to avoid complications. The indications for conservative or surgical therapy of PUJ obstruction are still developing. PUJ obstruction by crossing renal vessels is essential in choosing the appropriate surgical approach.  Pyeloplasty, is a surgical procedure to correct the obstruction. - If the obstruction is diagnosed during pregnancy or in the early newborn period, if possible, the health team typically tries to wait until the child is approximately 3 months of age to perform the surgery. This allows the child to grow and develop and mature to minimize any risk of anesthetic complications. 25
    • - After 3 months of age, the anesthetic risk has dropped and will remain minimal throughout the remainder of the child's life until older adult age. Conversely, surgical correction at that time still allows the kidney to recover from the blockage and ultimately grow and develop normally during the important first years of life and then into adulthood. - The surgery takes approximately 2-3 hours under a general anesthetia. An incision (cut) will be made, usually on the child‘s side. A catheter (small tube) will be inserted through the urethra into the bladder during surgery to drain the urine. The obstructed part of the kidney/ureter is removed and then the remaining parts joined. The wound will be closed with dissolvable stitches and sometimes paper tapes (steristrips) are also applied. A few children will have a small stent left in place which goes between the kidney and incision site. This stent can be used if necessary to help drain urine or, if needed, to insert dye during an x-ray. It can be removed with ease after drainage has stopped. Epispadiasis It is the congenital abnormal urethral opening on the dorsal aspect of penis. Urethra is displaced dorsally due to the abnormal development of the infraumbilical wall and upper wall of the urethra. It is associated with extrophy of bladder and ambiguous genetalia. TYPES: In male child In female child Anterior with normal continence Bifid clitoris with no incontinence of urine Posterior epispadiasis Subsymphyseal with incontinence of urine Male infants have short and broad penis with dorsal A cleft extends along the roof or entire urethra, curvature. involving the bladder neck. DIAGNOSIS: -diagnosed at birth itself -IVP -MCU -vesicourethral reflux -bladder capacity MANAGEMENT: Surgical correction 1st stage - it is done about 1.5 to 2 years of age for penile lengthening, elongation of urethral strip and chordee 26
    • correction. 2nd stage – it is done atleast 6 months after 1st stage urethral reconstruction 3rd stage – it is done about 3 - 4 years of age for bladder neck reconstruction and correction of VUR. Cytoplasty can be done to enhance the bladder capacity after 2 – 3 years of 3rd stage operation. Supportive nursing care should emphasize on prevention of infection. Hypospadiasis It is a congenital abnormal urethral opening on the ventral aspect of the penis. Undescended testes, inguinal hernia or upper urinary tract anomalies may be associated with hypospadiasis. It may be found in females as urethral opening in the vagina with dribbling of urine. INCIDENCE: It is a commonest malformation in a male child occurs 1-3 Occurs in 1-3 males per 1000 live births Close relatives of the patients are most likely to have compared to other population TYPES: It can be classified depending upon the site of urethral meatus. Anterior Middle Posterior 65-70% 10-15% 20% May be found as glandular or Penile shaft hypospadiasis coronal or on distal penile shaft. May be found on proximal penile shaft or as penoscrotal, scrotal or perineal type DIAGNOSIS: -Mostly observed at birth -observe for any abnormal voiding pattern - observe for inability of the boy to stand to urinate, he must sit to void. MANAGMENT: -surgical reconstruction to obtain straight penis at erection, to form urethral meatus at the tip of glans penis. -meatotomy is done at any age after birth. -chordee correction and advancement of prepuce can be done at 2 – 3 years of age. 27
    • -urethroplasty is done 3 – 4 months after chordee correction. (Surgical correction should be completed before admission to school.) SURGICAL COMPLICATIONS Surgical complication rates depend on the chosen procedure, and include urethrocutaneous fistula, meatal stenosis, urethral strictures, urethral diverticula, complete breakdown, skin necrosis, residual or recurrent curvature and hypospadiac cripple. Phimosis Phimosis refers to the narrow opening of the prepuse that prevents it being drawn back over the glans penis. The inability to retract the prepuse after the age of 3 years should be considered as true phimosis. It also can be acquired by the inflammation of glans or prepuse. It can predispose UTI. The term may also refer to clitoral phimosis in women, whereby the clitoral hood cannot be retracted, limiting exposure of the glans clitoridis. TYPES Different authors calssify it differently o Pathological o Physical o Physiological ETIOLOGY - balanitis (inflammation of the glans penis) Preputial stenosis or narrowness that prevents retraction, by fusion of the foreskin with the glans penis in children unusual masturbation practices secondary to chronic inflammation repeated catheterization forcible foreskin retraction Untreated diabetics due to the presence of glucose in their urine giving rise to infection in the foreskin CLINICAL MANIFESTATIONS - inability to retract the foreskin during routine cleaning or bathing - ballooning of the prepuce during urination - painful erections - Hematuria - recurrent urinary tract infections - preputial pain 28
    • - weakened urinary stream  Physical Phimosis, the foreskin cannot be retracted proximally over the glans penis.  Physiologic phimosis, the preputial orifice is unscarred and healthy appearing.  Pathologic phimosis, a contracted white fibrous ring may be visible around the preputial orifice MANAGEMENT: Phimosis in infancy needs to be treated only if it is causing obvious problems such as urinary discomfort or obstruction. If phimosis in older children or adults is not causing acute and severe problems, nonsurgical measures may be effective. 1.Non surgical methods include:  Steroid therapy Application of topical steroid cream, such as betamethasone, for 4–6 weeks to the narrow part of the foreskin is relatively simple, less expensive than surgical treatments and highly effective.  Manual stretching Stretching of the foreskin can be accomplished manually, with balloons or with other tools. Skin that is under tension expands by growing additional cells. A permanent increase in size occurs by gentle stretching over a period of time. The treatment is non-traumatic and non-destructive. Manual stretching may be carried out without the aid of a medical doctor. The tissue expansion promotes the growth of new skin cells to permanently expand the narrow preputial ring that prevents retraction. 2.Surgical methods It range from the complete removal of the foreskin to more minor operations to relieve foreskin tightness:  Circumcision It is the excision of the foreskin of the glans penis is choice of operative intervention don to treat phimosis. Other measure is the use of betamethasone cream to the narrowed preputial skin for two times daily for 4 weeks. This treatment usually becomes successful as the foreskin becomes soft, elastic and can be retracted gently and gradually.  Dorsal slit (superincision) Dorsal slit is a single incision along the upper length of the foreskin from the tip to the corona, exposing the glans without removing any tissue.  Ventral slit (subterincision) It is an incision along the lower length of the foreskin from the tip of the frenulum to the base of the glans, removing the frenulum in the process. Often used when frenulum breve occurs alongside the phimosis.  Preputioplasty It is in which a limited dorsal slit with transverse closure is made along the constricting band of skin can be an effective alternative to circumcision. It has the advantage of only limited pain and a short time of healing relative to circumcision, and avoids cosmetic effects. 29
    • PROGNOSIS The most acute complication is paraphimosis. In this condition, the glans is swollen and painful, and the foreskin is immobilized by the swelling in a partially retracted position. The proximal penis is flaccid. Paraphimosis It may develop in phimotic child. It is an uncommon medical condition where the foreskin becomes trapped behind the glans penis, and cannot be reduced (that is, pulled back to its normal flaccid position covering the glans penis). It is the retraction of a phimotic foreskin, behind coronal sulcus forming a tight constricting ring around the glans. The foreskin is retracted behind the glans penis and cannot be replaced to its normal position. CLINICAL MANIFESTATIONS painful, swollen glans penis in the uncircumcised or partially circumcised patient irritability Flaccidity of the penile shaft proximal to the area of paraphimosis is seen Erythematous and edematous glans The glans penis is initially its normal pink hue and soft to palpation. As necrosis develops, the color changes to blue or black and the glans becomes firm to palpation. ETIOLOGY The foreskin may be retracted during penile examination, penile cleaning, urethral catheterization, or cystoscopy; if the foreskin is left retracted for a long period, some of the foreskin tissue may become edematous (swollen with fluid), which makes subsequent reduction of the foreskin difficult. PREVENTION o Paraphimosis can be avoided by bringing the foreskin back into its reduced position after retraction is no longer necessary o Phimosis is a risk factor for paraphimosis; physiologic phimosis resolves naturally as a child matures, but it may be advisable to treat pathologic phimosis via long-term stretching or elective surgical techniques MANAGEMENT  Manual manipulation of the swollen foreskin tissue This involves compressing the glans and moving the foreskin back to its normal position, perhaps with the aid of a lubricant, cold compression, and local anesthesia as necessary.  Dorsal slit  Circumcision  The Dundee technique 30
    • It entails placing multiple punctures in the swollen foreskin with a fine needle, and then expressing the edema fluid by manual pressure. Prune belly syndrome ( Eagle-Barrett syndrome, Triad syndrome) It is a rare, genetic birth defect characterized by a triad of symptoms. 1.Deficiency or absence of anterior abdominal wall musculature. 2.Bilateral cryptorchidism 3.Ureter, bladder,& urethral abnormalities( megacystis, Megaureter, 2°dysplasia) The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the abdomens of those with the disorder. Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome, Obrinsky Syndrome, Frohlich Syndrome, or Triad Syndrome INCIDENCE About 1 in 40,000 births About 97% of those affected are male SYMPTOMS A partial or complete lack of abdominal muscles. There may be wrinkly folds of skin covering the abdomen. Undescended testicles in males Urinary tract abnormality such as unusually large ureters, distended bladder, accumulation and backflow of urine from the bladder to the ureters and the kidneys Frequent urinary tract infections due to the inability to properly expel urine. Later in life, a common symptom is post-ejaculatory discomfort. Most likely a bladder spasm, it lasts about two hours. DIAGNOSIS Via ultrasound while a child is still in-utero. sAn abnormally large abdominal cavity resembling that of an obese person is the key indicator, as the abdomen swells with the pressure of accumulated urine. In young children, frequent urinary tract infections Blood tests to check kidney function Voiding cystourethrogram Orthopedic evaluation 31
    • TREATMENT The type of treatment depends on the severity of the symptoms.  Vesicostomy Vesicostomy allows the bladder to drain through a small hole in the abdomen, thus helping to prevent urinary tract infections. Similarly, consistent self catheterization, often several times per day, can be an effective approach to preventing infections. A more drastic procedure is a surgical "remodeling" of the abdominal wall and urinary tract. Boys may have an orchiopexy, which moves the testicles to their proper place in the scrotum. Even with treatment, many patients experience renal failure. COMPLICATIONS distending and enlarging of internal organs such as the bladder and intestines Surgery is often required but will not return the organs to a normal size. Bladder reductions have shown that the bladder will again stretch to its previous size due to lack of muscle. Also many complications can come from enlarged/malformed kidneys which warrant the child to go on dialysis or require a kidney transplant. With proper treatment long healthy lives are possible. Musculoskeletal abnormalities include pectus excavatum, scoliosis, and congenital dislocations including the hip. CRYPTORCHIDISM (UNDESCENDED TESTIS) It is the absence of one or both testes from the scrotum. It is the most common birth defect regarding male genitalia. In unique cases, cryptorchidism can develop later in life, often as late as young adulthood. Cryptorchidism is distinct from monorchism the condition of having only one testicle. INCIDENCE - About 3% of full-term and 30% of premature infant boys are born with at least one undescended testis. By the age of 1 year the incidence decreases to less than 1% and does not change thereafter. Frequency 3.4 % in term boys FACTORS RESPONSIBLE FOR DESCENT - Initiated by-androgens Prompted by-differential growth Permitted by-lengthening of ductus Fascilitated by-gubernaculum TYPES 1.undescended testes In this type testis neither resides nor can be manipulated into the scrotum 32
    • - Abdominal – proximal to the internal inguinal ring Canalicular – between the internal and external inguinal rings Ectopic – outside the normal pathways of descent between the abdominal cavity and the scrotum 2.Retractile testes It can be manipulated into scrotum where it remains without tension 3.Anorchia Absence of testis CAUSES AND RISK FACTORS In most full-term infant boys with cryptorchidism but no other genital abnormalities, a cause cannot be found, making this a common, sporadic, idiopathic birth defect. A combination of genetics, maternal health and other environmental factors may disrupt the hormones and physical changes that influence the development of the testicles. - Endocrine abnormalities affecting hypothalamic pituitary testicular axis Denervation of genitofemoral nerve Traction of gubernaculum Abnormal development of epididymis Premature birth Congenital hernia Low birth weight endocrine disruptors that interfere with normal fetal hormone balance (pesticides) Diabetes and obesity in the mother exposure to regular alcohol consumption during Family history of undescended testicle or other problems of genital development congenital malformation syndromes (Down syndrome, Prader-Willi syndrome) In vitro Fertilization use of cosmetics by the mother pre-eclampsia CLINICAL MANIFESTATIONS - Nonpalpabe testes (either one or both) Retractile testes can be milked/ ushed back into scrotum DIAGNOSIS - Physical examnination Softer testes Not well developed rugae 33
    • Cremasteric Reflex In normal males, as the cremaster muscle relaxes or contracts, the testis moves lower or higher ("retracts") in the scrotum. This reflex is elicited by lightly stroking the superior and medial (inner) part of the thigh regardless of the direction of stroke. The normal response is an immediate contraction of the cremaster muscle that pulls up the testis on the side stroked (and only on that side). Various maneuvers using a crosslegged position, soaping the examiner's fingers, or examining in a warm bath Pelvic ultrasound / MRI locate the testes while confirming absence of a uterus. karyotype confirm or exclude forms of dysgenetic primary hypogonadism, such as Klinefelter syndrome or mixed gonadal dysgenesis. Hormone levels especially gonadotropins and AMH can help confirm that there are hormonally functional testes worth attempting to rescue, as can stimulation with a few injections of human chorionic gonadotropin to elicit a rise of the testosterone level. Abdominal laproscopy TREATMENT The primary management of cryptorchidism is watchful waiting, due to the high likelihood of self-resolution. Where this fails surgery is indicated. Refractile testis can be manipulated into scrotum by milking / pushing back into scrotum. Hormone therapy With lutenizing hormone releasing spray(nasal spray) and HCG injection(10 injections over 5 weeks is common). orchiopexy it is effective if inguinal testes have not descended after 4–6 months. It could be performed between 1 2 years of age. Surgical repair is done to o Prevent damage to undescended testicles by exposure to heat thus maintaining future fertility o Avoid trauma & torsion o Decrease incidence of tumour formation o Prevent cosmetic handicap An incision is made over the inguinal canal. The testis with accompanying cord structure and blood supply is exposed, partially separated from the surrounding tissues, and brought into the scrotum. It is sutured to 34
    • the scrotal tissue or enclosed in a "subdartos pouch." The associated passage back into the inguinal canal, an inguinal hernia, is closed to prevent re-ascent. HYDROCELE It is defined as a collection of fluid within the tunica vaginalis of the testis. It is the presence of fluid in the processus vaginalis and is result of the same developmental process as an inguinal hernia. INCIDENCE 10 – 60/1,000 newborn full term babies TYPES 1. Congenital - Communicating (―vogbreuk‖) It is one in which processes vaginalis remains open and into which peritoneal fluid may be forced by intra abdominal pressure and gravity. - Infantile tunica & processes vaginalis distended upto internal ring but sac has no connection with peritoneal cavity - Interstitial - Cord smooth,oval swelling associated with spermatic cord 2. Primary - Idiopathic (aetiology not known) - Imbalance between the fluid secretion and absorption of the tunica vaginalis 3. Secondary - Infection - Trauma - Tumor - Abnormalities in inguinal lymph nodes CLINICAL MANIFESTATION - scrotal mass that transilluminates testis not palpable fluctuant can get above swelling testicular sensation can be elicited CAUSES - Incomplete closure of the processus vaginalis from the peritoneum Residual peritoneal fluid that has yet to be reabsorbed after processus closure 35
    • - In older boys it is due to abnormal absorption or secretion secondary to another pathologic process(Trauma,Ischemia,Infection) Secondary to intrascrotal or intra-abdominal pathology Filariasis may produce hydrocele in infected boys and men Hydrocele may be seen following ipsilateral renal transplantation secondary to testicular torsion or incarcerated/strangulated hernia secondary to testicular cancer RISK FACTORS - Premature and low-birth-weight infants Indirect inguinal hernia Primary testicular/intrascrotal pathology Trauma Surgery Increased intra-abdominal pressure Lymphatic obstruction Ventriculoperitoneal shunt Peritoneal dialysis Bladder exstrophy MANAGEMENT Surgical: Lords placation The hydrocele is opened with a small skin incision without further preparation. The hydrocele sac is reduced (plicated) by suture Von Bergmann's technique Partial resection of the hydrocele sac, leaving a margin of 1–2 cm. Care is taken not to injure testicular vessels, epididymis or ductus deferens. The edge of the hydrocele sac is oversewn for haemostasis Winkelmann's or Jaboulay's technique Same as von Bergmann's technique but the edges are sewn together behind the spermatic cord COMPLICATIONS - infection pyocele,hematocele infertility atrophy of testis herniation of hydrocele sac (rare) rupture (rare) 36
    • AMBIGUOUS GENETALIA (Hermaphroditism) Ambiguous genitalia is a congenital defect in which the external genitalia - penis or vulva - of the child do not have the typical appearance of either a male or female. The genitalia usually show a combination of male and female characteristics. Normal Genetalia & Reproductive Organ Develoment: During conception, the mother of a child contributes an X chromosome and the father contributes an X or Y chromosome. If the father contributes an X chromosome, then a genetically female fetus (XX) will develop and if he contributes a Y chromosome, a genetically male fetus (XY) will be formed. In the early stages of fetal life, both male and female fetuses are identical. At a certain stage - about 8 weeks for an XY fetus and 12 weeks for an XX fetus - the changes that cause them to differentiate into male and female respectively, occur. These changes occur in the same fetal tissue in both types of fetuses. Cases of ambiguous genitalia occur when there is a disorder in the process of differentiation of this fetal tissue. In humans, biological sex is determined by five factors present at birth: - the number and type of sex chromosomes; the type of gonads—ovaries or testicles; the sex hormones, the internal reproductive anatomy (such as the uterus in females), and the external genitalia. People whose five characteristics are not either all typically male or all typically female are intersexed. ETIOLOGY A failure or abnormality in any of the steps of genetalia and reproductive development can lead to abnormal development. Abnormal gender determination Chromosome abnormalities result in disturbance of development. Abnormal differentiation of gonads When induction of the bipotential gonad fails, gender differentiation proceeds in the direction of the female phenotype, regardless of karyotype. Abnormal differentiation of ductal systems Biological inactivity of androgenic male organizer substances or insensitivity of ductal tissue to the action of these substances results in a persistent female duct system, which leads to the presence of a uterus and uterine tubes. Abnormal secretion of testicular androgen Complete failure of male hormone secretion produces female external genetalia in a genetic male. Partial or incomplete masculination with ambiguity of the external genetalia. 37
    • TYPES  True Hermaphroditism: Ovaries and testicles are present and the external genitalia are not clearly male or female. This condition is very rare.  Female Pseudohermaphroditism The child has ovaries and a penis-like structure (usually due to clitoromegaly).  Male Pseudohermaphroditism The child has undescended testicles and external female genitalia.  Mixed Gonadal Dysgenesis Genetalia vary greatly, but in those who appear predominately female, the dysplastic testis may cause musculination at puberty.  Congenital Adrenal Hyperplasia Inherited deficiency of adrenal corticosteroid hormones. Female - In this certain forms of this condition, the adrenal glands of the fetus produce excessive amounts of androgens (male hormones) which cause the female genitalia to look male. Male -Certain forms of this condition cause reduced male hormone production which allows female-looking genitalia to develop. TREATMENT Goal: To enable the affected child to grow into a well adjusted, psychosocially stable person who is able to identify with the assigned gender and is content with the same. 1.Counselling: Having ambiguous genitalia causes a lot of emotional distress for the individual. 2. Determination of the Individual's Sex: This will include - Physical examination of the external genitalia by a physician - Chromosomal analysis to determine genetic sex - Pelvic ultrasound to check for the presence of reproductive organs e.g. undescended testes, ovaries, uterus etc. - Ability or potential of the individual to actually belong or adapt to either of the sexes. 3. Reconstructive Surgery: To make the genitalia look more natural according to the chosen gender and to promote sexual function. 4. Hormone Therapy If the condition which caused the ambiguous genitalia is not very severe, hormone replacement therapy could be used. Hormone therapy is used to promote development of secondary sexual characteristics during puberty and sometimes, throughout life. 38
    • INGUINAL HERNIA A hernia occurs when an intestinal part of the body, such as an organ, pushes through a weakness in the muscle or surrounding tissue wall. The muscles are usually strong but sometimes fails to kep organs in place resulting hernia. An inguinal hernia is a lump or protrusion of abdominal-cavity contents through the inguinal canal. It occurs in the groin region and may extend to scrotum in male children. Inguinal canal In male inguinal canal transmit the spermatic cord, ilioinguinal nerve & genital branch of genitofemoral nerve. In female round ligament replace the spermatic cord. INCIDENCE - It accounts 80% of childhood hernias More common in male children 10-20/1000 live births. 30% occurring in preterm infants ETIOLOGY - an opening in the muscle wall does not close as it should before birth which leaves a weak area in the belly muscle. Pressure on that area can cause tissue to push through and bulge out. Overweight Lifting Coughing Straining TYPES Direct inguinal hernia: It occur medial to the inferior epigastric vessels when abdominal contents herniated through a weak spot in the fascia of the posterior wall of the inguinal canal which is formed by the tranvalis fascia. Indirect inguinal hernia It occur when abdominal contents protrude through the deep inguinal ring, lateral to the inferior epigastric vessels, this may be caused by failure of embryonic closure of the processus vaginalis. Other types Reducible hernia It can be pushed back into abdomen by manual pressure into it. Irreducible hernia It cannot be pushed back into abdomen by manual pressure into it. 39
    • - - Obstructed In this lumen of herniated part of intestine is obstructed but the blood supply to hernia sac is intact Incarcerated hernia In this adhesions develop between the wall of hernia sac and wall of intestine. Strangulated hernia In this the blood supply of the sac is cut off, thus, leading to ischemia. The lumen of the intestine may be patent or not. PATHOPHYSIOLOGY In males the testis are initially located in the abdomen. Around the seventh month of gestation, the testis migrate down into the scrotum via a passage, the inguinal canal. This canal begins to close before birth and is usually completely fused or shut by the 1st year of life. If this canal does not close completely and the muscles in the wall of the abdomen do not cover the opening well enough a hernia may develop. Descending testes Migrate from abdomen to scrotum during development of urinary and reproductive organs Large sized inguinal canal transmits testicles and accommodates spermatic cord Weak posterior wall of inguinal canal and shutter mechanism Lower intra abdominal pressure Hernia formation CLINICAL MANIFESTATIONS - Swelling/ visible lump / bulge in the groin or scrotum Appears when the child lifting heavy weights, coughing, bending, straining, or laughing Smooth and soft swelling Swelling increase while crying, coughing or straining may be painful sometime bulge without pain swelling and a feeling of heaviness, tugging, or burning in the area of the hernia symptoms may get better when child lie down 40
    • - Sudden pain Nausea & vomiting Bloating DIAGNOSIS - History collection Physical examination USG Urine routine MANAGEMENT Conservative No medical recommendation for inguinal hernia but elective surgery is recommended Making truss with non intrusive flat pads to hold the hernia securely during all activities Surgical management It is called hernia repair. It is not recommended in minimally symptomatic hernia. There are three different approaches to pediatric hernias.  Open repair – This involves making an incision just below the belt line and dissecting down to the hole in the muscle layer. This hernia is closed with stitches. The deeper tissues and skin are then sewn together with dissolvable sutures that are hidden under the skin so that there are no stitches to be removed.  Open repair with laparoscopic exploration – This is the same as the open repair except that before closing the hernia hole, a small camera (about 3 mm in diameter) is passed into the abdomen to examine the opposite groin from the inside. If a hernia is detected, a matching incision is made on the opposite side to allow repair of the second hernia.  Laparoscopic repair – In this approach the hernia is closed using laparoscopic techniques. A 3-mm or 5-mm camera is inserted through the umbilicus and additional instruments are introduced through needle holes to perform the hernia closure. There a number of different laparoscopic hernia repair techniques in children (eversion technique, intracorporeal suturing technique, single stitch technique) that a can use depending upon the particular patient. NURSING MANAGEMENT OF THE CHILD WITH UROLOGIC SURGERY Basic pre-operative care to be provided with special attention for the followings: Promoting understanding of parents about the planned surgical interventions by explanations according to level of understanding. Preparing for necessary diagnostic procedures Involving the parents in child‘s care Promoting normal urinary functions by monitoring intake and output, care of urinary catheter and drainage tube, encouraging adequate fluid intake and hygienic measures and observing signs of urinary infections. 41
    • Preventing infections by aseptic precautions, hand washing practices, taking care of wound, administering prescribed medications, I/V fluid therapy, monitoring features of infections and vital signs etc. Providing comfort by rest, sleep, comfortable positioning operated side with support, administering analgesics, antispasmodics and organizing diversions and recreation by toys and play. Providing adequate nutrition to promote healing and prevent infection along with adequate fluid to promote urinary function. Teaching the parents about related care, prevention of infections and complications, importance of fluid intake, diet and follow up. Available support services and facilities. URINARY TRACT INFECTIONS A UTI is an infection in the urinary tract. Infections are caused by microbes—organisms too small to be seen without a microscope—including fungi, viruses, and bacteria. Bacteria are the most common cause of UTIs. Normally, bacteria that enter the urinary tract are rapidly removed by the body before they cause symptoms. However, sometimes bacteria overcome the body‘s natural defenses and cause infection. An infection in the urethra is called urethritis. A bladder infection is called cystitis. Bacteria may travel up the ureters to multiply and infect the kidneys. A kidney infection is called pyelonephritis. INCIDENCE 3-8% of girls and 1-2% of boys develop a UTI during childhood. Peak incidence is between 2 – 6 years of age. CLASSIFICATION Various terms used to describe urinary tract disorders:           Bacteriuria – presence of bacteria in urine Asymptomatic bacteriuria – significant bacteriuria with no evidence of clinical infection Symptomatic bacteriuria – accompanied by physical signs Recurrent UTI – repeated episodes of bacteriuria Persistent UTI – persistence of bacteriuria despite antibiotic Febrile UTI – accompanied by fever, other physical signs of urinary infection Urethritis - inflammation of urethra Cystitis - inflammation of the bladder Pyelonephritis – inflammation of upper urinary tract and kidneys Urosepsis – febrile UTI coexisting with systemic signs of bacterial illness; blood culture reveals presence of urinary pathogen. ETIOLOGY Escherichia coli – 80% of cases Gram negative enteric organisms are frequently implicated. These are found in anal and perineal region Other organisms include Proteus, pseudomonas, klebsiella, staphylococcus aureus, haemophilus etc. 42
    • Anatomic and physical factors Structure of lower urinary tract accounts to bacteriuria in females Short urethra in young girls (2cm) & in mature women (4cm) provides pathway for organism to invade Closure of urethra in the end of micturition may lead to return of bacteria to bladder Longer male urethra and prostatic secretions inhibit entry of pathogens in males Urinary stasis The act of completely and repeatedly emptying of bladder flushes away the organisms but the incomplete emptying result from reflux , anatomic abnormalities, dysfunction of voiding mechanism, extrinsic ureteral compression caused by constipation etc leads to UTI. Altered urine and bladder chemistry The mechanical and chemical properties of urine and bladder mucosa maintain urinary sterility. Normally urine is acidic. An alkaline medium is favored by the pathogens. A urine pH of about 5 hampers the bacterial multiplication. RISK CATEGORY - - Throughout childhood, the risk of having a UTI is 2 percent for boys and 8 percent for girls. Having an anomaly of the urinary tract increases the risk of a UTI. o Vesicoureteral reflux o Urinary obstruction o Dysfunctional voiding Boys who are younger than 6 months sold who are not circumcised are at greater risk for a UTI than circumcised boys the same age. PATHOPHYSIOLOGY Cause : UVR, catheterization , postponement of voiding, DM, low fliud intake etc bacteria ascends the urethra Lining of urinary tract becomes inflammed Micturition reflex triggered Urgency, frequency, burning hematuria, irritability, failure to thrive, pyuria DIAGNOSTIC EVALUATION A urine sample will be collected and examined. The way urine is collected depends on the child‘s age: - If the child is not yet toilet trained, the health care provider may place a plastic collection bag over the child‘s genital area. The bag will be sealed to the skin with an adhesive strip. If this method is used, the bag should be removed right after the child has urinated, and the urine sample should be processed immediately. Because bacteria from the skin can contaminate this sample, the methods listed below are more accurate. 43
    • - -      A health care provider may need to pass a small tube called a catheter into the urethra of an infant. Urine will drain directly from the bladder into a clean container. Sometimes the best way to collect a urine sample from an infant is by placing a needle directly into the bladder through the skin of the lower abdomen. Getting urine through a catheter or needle will ensure that the urine collected does not contain bacteria from the skin. An older child may be asked to urinate into a container. The sample needs to come as directly into the container as possible to avoid picking up bacteria from the skin or rectal area. Urine culture First morning urine specimen Clean catch mid stream specimen Collection bag Supra pubic aspiration Bladder catheterization Urinalysis - Increased number of RBC - Nitrate test positive - Significant bacteriuria Renal ultrasound - GU tract anatomy - Pelvi calceal dilatation - Hydronephrosis - Renal scarring Dipstick tests USG VCUG - GU anatomy IVP THERAPEUTIC MANAGEMENT Goals -eliminate current infection -Identify contributing factors and reduce risk of occurrence -prevent systemic spread of infection -preserve renal function PHARMACOLOGIC MANAGEMENT antibiotic therapy based on: identification of pathogen history of antibiotic use location of infection 44
    •  - Antibiotics amoxicillin – 20-40mg/kg in 3 doses cefixime – 8mg/kg in 2 doses Cephalexin – 50-100mg/kg in 4 doses Gentamycin – 1-4mg/kg single dose Trimethoprim – 6-12mg/kg & 30-60mg/kg in 2 doses Cefotaxim – 100mg/kg in 3 divided doses Ciprofloxacin – 20-30mg/kg in 2 divided doses   - Antimicrobial drugs (sometimes it will not be effective due to resistance of organism) Anti-infective agents penicillin sulfonamide cephalosporin nitrofurantoin SURGICAL MANAGEMENT For primary reflux or bladder neck obstruction, surgical correction is needed to avoid recurrence. NURSING MANAGEMENT - Careful history taking regarding voiding habits, stooling pattern Caution the parents in suspected cases Collect appropriate specimen Checking diaper half hourly for straining, dripping of small amounts of urine. Explanation of procedure according to their age Administer proper dosage of medications Increase the fluid intake COMPLICATIONS Most UTIs are not serious, but some infections can lead to serious problems, such as kidney infections. - Chronic kidney infections Infections that recur or last a long time can cause permanent damage, including kidney scars, poor kidney growth, poor kidney function, high blood pressure, and other problems. - Some acute kidney infections infections that develop suddenly can be life threatening, especially if the bacteria enter the bloodstream, a condition called septicemia. PREVENTION - Simple hygienic habits should be followed Practice habit of voiding soon as they feel the urge Adolescent girls are advised to urinate soon after an intercourse 45
    • - Reinforce parents and older children the importance of compliance Circumcision in males Plenty of oral fluids Treatment of constipation, pinworms GLOMERULAR DISEASE Many diseases affect kidney function by attacking the glomeruli, the tiny units within the kidney where blood is cleaned. The glomerulus may be injured by several mechanisms, but it has only a limited number of histopathologic responses; different disease states can produce similar microscopic changes. Glomerular injury may be a result of genetic, immunologic, perfusion, or coagulation disorders. TYPES The three basic types of glomerular disease include: focal nephritic, diffuse nephritic, and nephrotic. Focal nephritic — The key feature of focal nephritic disease is blood in the urine (hematuria) without significant impairment of kidney function or proteinuria. A person with focal glomerulonephritis may not have any symptoms and their condition may go unnoticed until blood and protein are found during a routine urinalysis. The following conditions may cause focal glomerulonephritis.  Mild postinfectious glomerulonephritis, IgA nephropathy, thin basement membrane disease, hereditary nephritis(Alport syndrome), Henoch-Schönlein purpura (IgA vasculitis), mesangial proliferative glomerulonephritis Diffuse nephritic — Persons with diffuse nephritic disease have hematuria with impaired kidney function and proteinuria. This may be a severe form of focal nephritic disease for some people, or may be caused by a bodywide disease. Urinalysis may also show high levels of protein, and patients may have edema (swelling in the lower legs) or high blood pressure. The following conditions may cause diffuse glomerulonephritis.  Less than 15 years old – Postinfectious glomerulonephritis, membranoproliferative glomerulonephritis Nephrotic syndrome — People with nephrotic syndrome generally have protein in the urine (proteinuria) but little to no blood in the urine. Kidney function may worsen as nephrotic syndrome progresses. The following conditions may cause nephrotic syndrome.  Less than 15 years old – Minimal change disease, focal segmental glomerulosclerosis, mesangial proliferative glomerulonephritis 46
    • Nephrotic Syndrome Nephrotic syndrome, a manifestation of glomerular disease, characterized by nephrotic range proteinuria and the triad of clinical findings associated with massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. (Nephrotic range proteinuria - protein excretion of > 40 mg/m2/hr or a first morning protein: creatinine ratio of >2-3: 1) INCIDENCE: The annual incidence is 2-3 cases per 100,000 children per year in most Western countries and higher in underdeveloped countries resulting predominantly from malaria. Miniml change nephrotic syndrome constitutes 80% of nephrotic syndrome cases. ETIOLOGY: Most children with nephrotic syndrome have a form of primary or idiopathic nephrotic syndrome. a) Idiopathic nephrotic syndrome include minimal change disease (the most common), focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, membranous nephropathy and diffuse mesangial proliferation b) Associate with glomerular damage (systemic lupus erythematosus, lymphoma ,leukemia and infections) c) Hereditary proteinuria syndromes are caused by mutations in genes that encode critical protein components of the glomerular filtration apparatus. Such as Alport syndrome, sickle cell anemia. TYPES: 1. Idiopathic Nephrotic syndrome It is the primary disease which also known as childhood nephrosis, or minimal change nephrotic syndrome. Approximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndrome. Idiopathic nephrotic syndrome is associated with primary glomerular disease without evidence of a specific systemic cause. Idiopathic nephrotic syndrome includes multiple histologic types: minimal change disease, mesangial proliferation, focal segmental glomerulosclerosis (FSGS), membranous nephropathy, and membranoproliferative glomerulonephritis. 2. Secondary Nephrotic Syndrome It is a secondary disorder that occurs as a clinical manifestation after or in assosciation with glomerular damage. It occurs secondary to systemic diseases such as systemic lupus erythematosus, Henoch-Schönlein purpura, malignancy (lymphoma and leukemia), and infections (hepatitis, HIV, and malaria). Secondary nephrotic syndrome should be suspected in patients >8 yr and those with hypertension, hematuria, renal dysfunction, extrarenal symptoms (rash, arthralgias, fever), or depressed serum complement levels. Nephrotic syndrome has also developed during therapy with numerous drugs and chemicals. The histologic picture can resemble membranous glomerulopathy (penicillamine, captopril, gold, nonsteroidal antiinflammatory drugs, mercury compounds), MCNS (probenecid, ethosuximide, methimazole, lithium), or proliferative glomerulonephritis (procainamide, chlorpropamide, phenytoin, trimethadione, paramethadione). 3. Congenital Nephrotic Syndrome It is inherited as autosomal recessive disorder. Congenital nephrotic syndrome is defined as nephrotic syndrome manifesting at birth or within the first 3 months of life. Congenital nephrotic syndrome may be classified as primary or as secondary to a number of etiologies. 47
    • a) Primary congenital nephrotic syndrome is due to a variety of syndromes inherited as autosomal recessive disorders. Affected infants most commonly present at birth with edema due to massive proteinuria, and they are typically delivered with an enlarged placenta (>25% of the infant‘s weight). Severe hypoalbuminemia, hyperlipidemia, and hypogammaglobulinemia result from loss of filtering selectivity at the glomerular filtration barrier. Prenatal diagnosis can be made by the presence of elevated maternal and amniotic α-fetoprotein levels. b) Secondary congenital nephrotic syndrome can be occurred from underlying causes such as syphilis. PATHOPHYSIOLOGY - In nephrotic syndrome, type III hypersensitivity reaction occurs in which the immune complex precipitated in the tissue. - Activation of the complement system also stimulates vaksoaktive amines (including histamine) and this substance causes retraction of endothelial cells thus increasing vascular permeability. - Changes in membrane glomerolus, causing increased permeability, allowing the proteins (especially albumin) out through the urine (proteinurine). - Decreased oncotic pressure causing albumin moves from intra vascular space into interstitiel. - Transfer of proteins to the interstitial cavity causing lipoproteinemia. - It stimulates the liver to compensate by increasing the production of lipoproteins and increased concentrations of blood fats (hyperlipidemia). - When the liver is not able to compensate for damage in fat and protein metabolism. - Transfer of protein exit the vascular system, causing fluid to move into the space plasma interstitisel resulting edema and hypovolemia. - Decrease in vascular volume stimulates renin angiotensin system, which allows the secretion of aldosterone and antidiuretic hormone (ADH). - Aldosterone stimulates increased reabsorsi distal tubules of the sodium and water, leading to increased edema. 48
    • metabolic, biochemical, physiochemical or immune mediated disturbances basement membrane of glomeruli permeable to proteins loss of albumin in urine through the membrane hypoalbuminemia decrease in colloidal osmotic pressure in capillaries vascular hydrostatic pressure exceeds the pull of colloidal osmotic pressure fluid accumulation in interstitial space abdominal cavity (edema) (ascitis) Hypovolemia Decreased renal blood flow increased secretion of ADH and aldosterone Rennin release Na+ and water reabsorption Vasoconstriction EDEMA increased hydrostatic pressure CLINICAL MANIFESTATIONS Weight gain Puffiness on face (facial edema) o Especially around the eyes o Apparent on arising the morning o Subsides during the day Abdominal swelling (ascitis) Pleural effusion Labial or scrotal swelling Edema of intestinal mucosal which result in: o Diarrhea o Anorexia o Poor intestinal absorption Ankle / leg swelling Irritability Easily fatigued Lethargic 49
    • BP normal or slightly decreased Susceptible to infections Urine alterations o Decreased volume o Frothy DIAGNOSTIC EVALUATION History collection o weight gain, anorexia, irritability, less active Physical examination o Clinical manifestations Urine dipstick test for protienuria Blood tests –presence of casts, RBC o Serum protein low concentration o Reduced albumin o GFR normal or high o Hb normal or elevated o Elevated platelet count Renal biopsy if not respond to steroid treatment THERAPEUTIC MANAGEMENT Goals  Reduce excrtion of urinary protein  Reduce fluid retension  Preventing infection  Minimize complications related to treatment DIETARY MANAGEMENT - Low salt diet - Fluid restriction PHARMACOLOGICAL MANAGEMENT - Diuretic therapy for temporary relief from edema - Infections are treated with appropriate antibiotics - Corticosteroids for MCNS Prednisolone – 2mg/kg body weight/day in one or two divide doses - Relapse is treated with high dose steroid therapy - For children who do not respond to steroid therapy, immune - suppressants are given. STEROID THERAPY  Extended APN schedule o 60mg/m2/day as a single dose for 6 weeks o If remission is present , then 40 mg/m2/every other day for next 6 weeks o If remission is maintained, taper steroids in EOD in 2 weeks  Definition of response 50
    • - Remission No albumin on three consecutive early morning urine samples Relapse 2+ or more albumin on 3 consecutive early morning urine samples Frequent relapse 3 or more relapses in 6 months or 4 or more relapses in 1 year Steroid resistance No remission after 8 weeks of adequate daily steroids Steroid dependence Relapse within 15 days of stopping steroids after inducing remission or on tapering, on more than 2 occasions NURSING MANAGEMENT a. Focus Assessment       Urinary System (oliguric, urine retention, proteinurin and urine discoloration). Fluid and electrolyte balance (excess fluid, edema, ascites, weight gain, dehydration) Circulation (increased blood pressure) Neurology (decreased level of consciousness due to dehydration) Breathing (shortness of breath, tachypnea) Mobility (redness, malaise) b. Nursing Diagnosis 1. 2. 3. 4. Impaired Urinary Elimination related to Na and water retention. Excess Fluid Volume related to edema Imbalanced Nutrition Less Than Body Requirements related to damage protein metabolism Ineffective Breathing Pattern related to suppression of the diaphragm due to ascites c. Nursing interventions 1. Administer medications, such as diuretics, antibiotics, and corticosteroids as ordered. 2. Ask dietitian to plan a low-sodium diet with moderate amounts of protein. 3. Provide meticulous skin care to combat the edema that usually occurs with nephrotic syndrome. 4. Encourage activity and exercise and provide antiembolismstockings as ordered. 5. Frequently check the patient‘s urine for protein, indicated by frothy appearance. 6. Monitor and document the location and charater of edema. 7. Measure blood pressure while the patient is in s supine position and standing. 8. Monitor intake and output hourly. 9. Assess the patient‘s response to prescribed medications. 10. Stress the importance of adhering to the special diet 51
    • COMPLICATIONS 1. Due to drugs 2. Due to the disease 1.Due to drugs Toxicity of the following drugs may occur Furosemide, siranolactone Steroids Cyclophosphamide Levamisole Anticoagulants 2.Due to the disease Edema Biochemical hypothyroidism Hypocalcemic tetany Anemia Hypercoagulable states Acute renal failure Infection - Children in relapse have increased susceptibility to bacterial infections because of urinary losses of immunoglobulins, defective cell-mediated immunity, their immunosuppressive therapy, malnutrition, and edema or ascites acting as a potential culture medium. - Spontaneous bacterial peritonitis is a common infection, although sepsis, pneumonia, cellulitis, and urinary tract infections may also be seen. Thromboembolic events - Both arterial and venous thromboses may be seen, including renal vein thrombosis, pulmonary embolus, sagittal sinus thrombosis, and thrombosis of indwelling arterial and venous catheters. - To minimize the risk of thromboembolic complications, aggressive use of diuretics and the use of indwelling catheters should be avoided if possible. Cardiovascular disease - Hyperlipidemia, may be a risk factor for cardiovascular disease; myocardial infarction is a rare complication in children. Steroid therapy - Complications incude weight gain, ounding of face, increased appetite, hirsuitism, growth retardation, cataracts, HTN, gastro intestinal bleeding, infection and hyperglycemia. IMMUNISATIONS The children with NS should receive: - 23- serotype pneumococcal vaccine - 7-valent conjugate pneumococcal vaccine - routine childhood immunization schedule( for child is in remission and off daily prednisone therapy) 52
    • - - Live virus vaccines should not be administered to children who are receiving daily or alternate-day high-dose steroids (≥2 mg/kg/day of prednisone or its equivalent, or ≥20 mg/day if the child weighs >10 kg). Vaccines can be administered after corticosteroid therapy has been discontinued to nephrotic children in relapse if exposed to varicella, should receive varicella-zoster immunoglobulin (1 dose ≤96 hours after significant exposure) Influenza vaccine should be given on a yearly basis PROGNOSIS Ultimate recovery in most cases is good. It is a self timing disease. In children who receives steroid therapy the tendency to relapse decreases with time. With early detection and treatment, the membrane damage could be minimized. About 80% of affected children have favorable prognosis. Hemolytic-Uremic Syndrome Hemolytic-uremic syndrome (HUS) is one of the most common causes of community-acquired acute kidney failure in young children. It is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency . CLINICAL DESCRIPTION Hemolytic uremic syndrome (HUS) is characterized by the acute onset of microangiopathic hemolytic anemia, renal injury, and a low platelet count. Most cases of HUS occur after an acute gastrointestinal illness (usually diarrheal). INCIDENCE - Occurs in infants and small children between the ages of 6 months and 5 years. CASE CLASSIFICATION Probable • An acute illness diagnosed as HUS or TTP that meets the laboratory criteria in a patient who does not have a clear history of acute or bloody diarrhea in the preceding 3 wk • An acute illness diagnosed as HUS or TTP that (a) has onset within 3 wk after onset of an acute or bloody diarrhea and (b) meets the laboratory criteria except that microangiopathic changes are not confirmed. Confirmed • An acute illness diagnosed as HUS or TTP that both meets the laboratory criteria and began within 3 wk after onset of an episode of acute or bloody diarrhea 53
    • CLASSIFICATION OF HEMOLYTIC UREMIC SYNDROME  Infection Induced Verotoxin-producing Escherichia coli Shiga toxin-producing Shigella dysentereriae type 1 Neuraminidase-producing Streptococcus pneumoniae Human immunodeficiency virus  Genetic von Willebrand factor-cleaving protease (ADAMTS 13) deficiency Complement factor H (or related proteins) deficiency or mutation Membrane cofactor protein (MCP) mutations Thrombomodulin mutations Complement factor I mutations Vitamin B12 metabolism defects Familial autosomal recessive of undefined etiology Familial autosomal dominant of undefined etiology Sporadic, recurrent, undefined etiology without diarrhea prodrome  Other Diseases Associated With Microvascular Injury Systemic lupus erythematosus Antiphospholipid antibody syndrome Following bone marrow transplantation Malignant hypertension Primary glomerulopathy HELLP (hemolytic anemia, elevated liver enzymes, low platelet count) syndrome  Medication-Induced Calcineurin inhibitors (cyclosporine, tacrolimus) Cytotoxic, chemotherapy agents (mitomycin C, cisplatin, gemcitabine) Clopidogrel and ticlopidine Quinine SUBGROUPS 1st – associated with a diarrheal prodrome ( D+ / typical HUS) 2nd – not associated with antecedent diarrhea ( D- / atypical HUS) 54
    • ETIOLOGY - Rickettsia - Bacterial toxins (e-coli, salmonella, pneumococci) - Chemicals - Viruses (coxsackie virus, echovirus, adenovirus) - Usually transmitted by undercooked meat or unpasteurized milk or apple cider - HUS outbreaks have also been associated with municipal water supply; petting farms; swimming in contaminated ponds and consuming cheese, lettuce, or raw spinach contaminated with toxin PATHOPHYSIOLOGY Primary injury in endothelial lining of small glomerular arterioles Deposits of platelets and fibrin clots Swelling in the glomerular arterioles RBC damage resulted by the attempt to move through occluded blood vessels Spleen removes the damage Results in hemolytic anemia Result in characteristic thrombocytopenia CLINICAL MANIFESTATIONS History of a prodromal disease (gastroenteritis or an upper respiratory infection) Acquired hemolytic anemia Sudden onset of hemolysis Thrombocytopenia Renal injury Central nervous system symptoms DIAGNOSTIC EVALUATION History collectuion Clinical examination: - Triad of anemia, thrombocytopenia and renal failure 55
    • Laboratory examination: Urine - proteinuria, hematuria, urinary cast presence Blood - Elevated blood urea, nitrogen, creatinine - Low hemobglobin, hematocrit - High reticulocyte count THERAPEUTIC MANAGEMENT - early recognition of the disease - monitoring for potential complications - meticulous supportive care. careful management of fluid and electrolytes correction of volume deficit control of hypertension early institution of dialysis if the patient becomes anuric or significantly oliguric Red cell transfusions are usually required because hemolysis can be brisk and recurrent until the active phase of the disease has resolved. - Blood transfusion Fresh, washed packed cells for anemic child with caution to prevent circulatory overload Fresh frozen plasma and plasma pherisis PROGNOSIS The acute prognosis, with careful supportive care, for diarrhea associated HUS (D+) has <5% mortality in most major medical centers. Half of the patients require dialysis support during the acute phase of the disease. Most recover renal function completely, but of surviving patients, 5% remain dependent on dialysis, and up to 20-30% are left with some level of chronic renal insufficiency. The recovery rate is about 95%, but residual renal impairment ranges from 10% to 50% in various cases. Immunoglobulin A Nephropathy (Berger Nephropathy) IgA nephropathy is the most common chronic glomerular disease. The disease derives its name from deposits of Immunoglobulin A (IgA) in a granular pattern in the mesangium a region of the renal glomerulus. It is characterized by a predominance of IgA immunoglobulin within mesangial glomerular deposits in the absence of systemic disease (e.g., symptomatic systemic lupus erythematosus or Henoch-Schönlein purpura). INCIDENCE It is seen more often in male than in female patients. -is often benign in childhood in comparison to that of adults. 56
    • -is an uncommon cause of end-stage renal failure during childhood. CLINICAL MANIFESTATIONS Gross hematuria associated with loin pain. Proteinuria often <1000 mg/24 hr. Mild to moderate hypertension. Normal serum levels of C3 DIAGNOSIS History collection Physical examination (clinical features) Laboratorical investiagationss Renal biopsy TREATMENT - The primary treatment is appropriate blood pressure control - Fish oil, which contains anti-inflammatory omega-3 polyunsaturated fatty acids - Immunosuppressive therapy with corticosteroids - Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists are effective in reducing proteinuria and retarding the rate of disease progression. - Kidney transplantation PROGNOSIS Although IgA nephropathy does not lead to significant kidney damage in most children, progressive disease develops in 20-30% of patients 15-20 yr after disease onset. Therefore, most children with IgA nephropathy do not display progressive renal dysfunction until adulthood, prompting the need for careful long-term follow-up. Poor prognostic indicators at presentation or followup include persistent hypertension, diminished renal function, and heavy or prolonged proteinuria. Alport Syndrome (hereditary nephritis) AS, hereditary nephritis, is a genetically heterogeneous disease caused by mutations in the genes coding for type IV collagen, a major component of basement membranes. These genetic alterations are associated with marked variability in clinical presentation, natural history, and histologic abnormalities. GENETICS Approximately 85% of patients have X-linked disease caused by a mutation. Autosomal recessive forms of AS are caused by mutations in the COL4A3 and COL4A4 genes on chromosome 2 encoding the α3 and α4 57
    • chains, respectively, of type IV collagen. An autosomal dominant form of AS linked to the COL4A3-COL4A4 gene locus occurs in 5% of cases. CLINICAL MANIFESTATIONS - Asymptomatic microscopic Hematuria - Single or recurrent episodes of gross hematuria commonly occurring 1-2 days after an upper respiratory infection. - Proteinuria - Bilateral sensorineural hearing loss - Ocular abnormalities - Leiomyomatosis of the esophagus, tracheobronchial tree, and female genitals in association with platelet abnormalities is rare. DIAGNOSIS - Family history - Screening urinalysis of first-degree relatives - Audiogram, - Ophthalmologic examination - Diagnostic renal biopsy - Mutation screening or linkage analysis is not readily available for routine clinical use. - Prenatal diagnosis is available for families with members who have X-linked AS and who carry an identified mutation. TREATMENT - No specific therapy is available to treat AS - Angiotensin converting enzyme inhibitors can slow the rate of progression - Careful management of renal failure complications such as hypertension, anemia, and electrolyte imbalance is critical. - Patients with ESRD are treated with dialysis and kidney transplantation. PROGNOSIS - The risk of progressive renal dysfunction leading to end-stage renal disease (ESRD) is highest among hemizygotes and autosomal recessive homozygotes. Risk factors for progression are gross hematuria during childhood, nephrotic syndrome, and prominent GBM thickening. 58
    • Acute Glomerulo Nephritis AGN is an immune mediated inflammatory disease of the capillary loops in the renal glomeruli. AGN may be a primary event or a manifestation of a systemic disorder that can range from minimal to severe. INCIDENCE -Acute post streptococcal glomerulo nephritis (APGN) is the most common of post infectious renal disease in childhood. -It is common in early school age children. -And male female ratio is 2:1. ETIOLOGY It is an immune complex disease that occurs after an antecedent streptococcal infection with certain strains of group A β-hemolytic streptococcal infection. Acute post streptococcal glomerulonephritis is the most common. A latent period of 10 to 21 days occurs for the onset of clinical manifestations. PHASES Phase 1 – edema and oliguria present Phase 2 – edema reduces and urine output increases PATHOPHYSIOLOGY Immune complexes are deposited in the glomerular basement membrane. The glomeruli becomes edematous ad infiltrated with polymorphonuclear leukocytes, which occlude the capillary lumen. The resulting decrease in plasma filtration results in an excessive accumulation of water and retention of sodium that expands plasma and interstitial fluid volumes, leading to circulatory congestion and edema. Excess rennin may also be produced. Streptococcal infection Production of antigen antibody complex in glomerular loops Inflammatory reaction Proliferation and swelling of endothelial cells Diminish the amount of glomerular filtrate and allow the passage of blood cells and protein in the filtrate Sodium and water retention Damage of glomerular membrane Progressive renal failure 59
    • CLINICAL MANIFESTATIONS Sore throat/pyoderma/scabies/impetigo Oliguria Edema Periorbital puffiness Pedal edema Rapid weight gain Hypertension Circulatory congestion Hematuria Proteinuria Fever Headache Nausea and vomiting Anorexia Abdominal pain Malaise Hypertension DIAGNOSIS - History collection Affected children are in good helath typically until streptococcal infection occurs. Sometimes there will be history of only mild cold. An average latent period is about 10 days. - Physical examination Edema is relatively moderate. - Urine examination specific gravity, reduced total amount of urine, microscopic examination for red cells, WBC, pus cells, epithelial cells, granular cast - Blood examination increased level of urea, creatinine, ESR, decreased Hb, hyponatremia, hyperkalemia, reduced C3(serum complement) in early stages. - Throat swab culture Streptococci from culture of the pharynx - Chest X-ray 60
    • Cardiac enlargement, pulmonary congestion, pleural effusion. COMPLICATIONS - CCF - Acyte renal failure - Hypertensive encephalopathy - Persistent hypertension - Anemia - Growth failure - Chronic glomerulonephritis MANAGEMENT Therapeutic management includes general supportive measures and early recognition and treatment of complications. -Bedrest for weeks till urine got free from RBC -Diet management Protein restricted Restricted salt and fluid Freely allow carbohydrate containing food -Daily weight recording to assess increase and decrease edema - Regular measurement of vital signs, body weight and input and output is essential in order to monitor the progress of the disease and to detect complications. -symptomatic management administration of antibiotic Antihypertensive drugs Tranquilisers for convulsions and encephalopathy Diuretics are not indicated since edema is rarely massive & gradually disappears with the return of renal function. For pulmonary edema IV frusemide (2.4 mg/kg) is given. -dialysis may be needed in renal failure and severe electrolyte imbalance -management of complications CCF Hypertensive encephalopathy Respiratory support 61
    • NURSING MANAGEMENT Careful assessment of diseases status Regular monitoring of vital signs Maintain input and output Children with restricted fluid intake and those who does not have much edema should be observed for signs of dehydration if he lost weight. Administer antibiotics Promote rest, sleep and comfortable position Skin care for edematous part Dietary management PROGNOSIS Almost all children correctly diagnosed as having APSGN recover completely and specific immunity is conferred, so that subsequent recurrences are uncommon. A few of these children have been reported to develop chronic disease, but most of these cases are now believed to be different glomerular diseases misdiagnosed as post streptococcal disease. Chronic Glomerulo Nephritis It is not a single disease , it comprises advanced stages of several forms of GN. It is an advanced irreversible impairment of renal function with or without symptoms. It may develop as a primary disease or may occur in SLE or drug induced nephropathies. CLINICAL MANIFESTATIONS It may remain asymptomatic edema Severe hypertension Hematuria Nocturia Persistent anemia Bone pain Bony deformities Failure to thrive 62
    • DIAGNOSIS - Physical examination Growth retardation Long standing hypertension Bony changes - Urine examination - Blood examination anemia, increase urea, creatinine, low GFR - Urine analysis protein, RBC, cast - USG shrunken kidneys MANAGEMENT It can be done with steroid therapy and other immune suppressive drugs. Anti hypertensive drugs and antibiotics are useful for symptomatic measurement. URINARY LITHIASIS A kidney stone is a solid concretion or crystal aggregation formed in the kidneys from dietary minerals in the urine. Kidney stones (nephrolithiasis or urolithiasis or renal calculus) develop when a collection of minerals or other material form a small "stone." The stone can cause pain, block the flow of urine, and rarely cause long-term kidney problems if it is not recognized and treated promptly. INCIDENCE Stones are less common in children than in adults. Approximately 7% of urinary calculi occur in children less than 16 years of age. About 80% of those with kidney stones are men. ETIOLOGY There are wide geographic variations with relation to climate, diet and socio economic factors. Most children who develop kidney stones have an underlying condition that increases their risk of stones, although some children develop a stone for unknown reasons. 1. 2. 3. 4. High conc. of metabolic products in glomerular filtrate Changes in urine pH Urinary stagnation Deficiency of stone-forming inhibitors in urine 63
    • 1. High conc. of metabolic products in glomerular filtrate is due to: o Low urinary volume (with normal renal function) due to restricted fluid intake o Increased fluid loss from the body o Increased excretion of metabolic products forming stones o High plasma volume (high filtrate level) o Low tubular reabsorption from filtrate 2. Changes in urine pH due to: o Bacterial infection o Precipitation of salts at different pH 3. Urinary stagnation is due to: o Obstruction of urinary flow 4. Deficiency of stone-forming inhibitors: o Citrate, pyrophosphate, glycoproteins inhibit growth of calcium phosphate and calcium oxalate crystals o In type I renal tubular acidosis, hypocitraturia leads to renal stones STONE FORMATION A kidney stone usually forms when substances that are normally found in the urine, such as calcium, oxalate, cystine, or uric acid, are at high levels. In some children, stones can also form if these substances are at normal levels. The substances form crystals, which become anchored in the kidney and gradually increase in size, forming a kidney stone. Stones that are very small (less than 5 millimeters (0.2 inches)) can usually pass on their own, while larger stones usually require treatment. A kidney stone moves through the urinary tract and, if it is small enough, it will be passed in the urine. A larger stone can become stuck within the urinary tract, causing pain and sometimes blocking the flow of urine. CLINICAL MANIFESTATIONS      Abdominal or flank pain (renal colic) Gross or microscopic hematuria Nausea or vomiting Needing to rush to the bathroom to urinate Young children may not have any symptoms, and the kidney stone is found when an imaging test (like an X-ray) is done for another reason. DIAGNOSIS - - - Plain abdominal x-ray ( to know the size and nature) Radio opaque – struvite, Radiolucent – cystine, uric acid calculi Noncontrast spiral CT scan Number and location of calculi Hydronephrosis Plain radiograph of abdomen and pelvis. 64
    • - Renal USG Growth or diminished size or movement of calculus TYPES Urinary stones are typically classified by their location in the kidney (nephrolithiasis), ureter (ureterolithiasis), or bladder (cystolithiasis), or by their chemical composition (calcium-containing, struvite, uric acid, or other compounds) Calcium stones ( calcium oxalate & calcium phosphate) - Hypercalciuria - Hyperuricosuria - hyperoxaluria - Heterozygous cystinuria - Hypocitruria - Renal tubular acidosis Uric acid stones - Hyperuricosuria - Myeloproliferative disorders - Inflammatory bowel disease - After chemotherapy Struvite stones (magnesium ammonium phosphate) - UTI - Foreign body - Urinary stasis Cystine stones - Cystinuria Indinavir stones Nephrocalcinosis 1. Calcium salt stones - 80% of kidney stones contain calcium - The type of salt depends on a. Urine pH b. Availability of oxalate - General appearance: c. White, hard, radioopaque d. Calcium PO4: staghorn in renal pelvis (large) e. Calcium oxalate: present in ureter (small) 65
    • Causes of calcium salt stones:  Hypercalciuria: - Increased urinary calcium excretion Men: > 7.5 mmols/day Women > 6.2 mmols/day May or may not be due to hypercalcemia  Hyperoxaluria: - Causes the formation of calcium oxalates without hypercalciuria Diet rich in oxalates Increased oxalate absorption in fat malabsorption  Primary hyperoxaluria: - Due to inborn errors Urinary oxalate excretion: > 400 mmols/day Treatment: - Treatment of primary causes such as infection, hypercalcemia, hyperoxaluria Oxalate-restricted diet Increased fluid intake Acidification of urine (by dietary changes) Calcium salt stones are formed in alkaline urine 2. Uric Acid Stones - About 8% of renal stones contain uric acid - May be associated with hyperuricemia (with or without gout) - Form in acidic urine - General appearance: a. Small, friable, yellowish b. May form staghorn c. Radiolucent (plain x-rays cannot detect) d. Visualized by ultrasound or IVP Treatment: e. Purine-restricted diet f. Alkalinization of urine (by dietary changes) g. Increased fluid intake 3. Struvite stones (magnesium ammonium phosphate) - About 10% of all renal stones contain Mg amm. PO4 66
    • - Also called struvite kidney stones Associated with chronic urinary tract infection a. Microorganisms (such as from Proteus genus) that metabolize urea into ammonia b. Causing urine pH to become alkaline and stone formation - Commonly associated with staghorn calculi 75% of staghorn stones are of struvite type Treatment: c. Treatment of infection d. Urine acidification e. Increased fluid intake 4. Cystine stones - A rare type of kidney stone - Due to homozygous cystinuria - Form in acidic urine - Soluble in alkaline urine - Faint radio-opaque Treatment: - Increased fluid intake - Alkalinization of urine (by dietary changes) - Penicillamine (binds to cysteine to form a compound more soluble than cystine) 5. Indinavir stones - Indinavir sulfate is a protease inhibitor used in the treatment of HIV and 12% of medicine is excreted through urine after each dose - 4 % aquire symptomatic nephrolitiasis - Urine often contains crystals (starburst, rectangular, fan shaped) Treatment: - Dissolution therapy by urine acidification with ammonium chloride/ascorbic acid 6. Nephrocalcinosis - Refers to calcium deposition within the renal tissue Cause: - Furosemide administration Hyperparathyroidism Cortical necrosis Renal candidiasis RISK FACTORS Certain factors can increase a child's risk of developing kidney stones. 67
    • History of kidney stones Children who have had a kidney stone in the past have the highest risk of developing a stone in the future. Preventive measures can decrease the risk of developing a stone in the future. Less water intake The amount of fluids a child drinks directly affects the amount of urine the body makes. Drinking a small amount of fluids means that the kidneys make a small amount of urine, which increases the concentration of stone-forming substances in the urine. Drinking more fluids can reduce the risk of recurrent stones. Ketogenic diet Diets that include a very small amount of carbohydrates, called ketogenic diets, can increase the risk of developing kidney stones. Ketogenic diets are sometimes used to treat seizure disorders. Urinary tract abnormalities Congenital abnormalities in the kidneys, ureters, or bladder can increase the risk of developing a kidney stone. Medicines Some medicines increase the risk of forming crystals in the urine. These include furosemide (Lasix), acetazolamide (Diamox), and allopurinol (Aloprim, Zyloprim). Inherited disorders Several uncommon inherited disorders can increase a child's risk of developing kidney stones. MANAGEMENT HOME If the stone is small, pain is manageable, and the child is otherwise healthy, it is often possible to treat the stone at home. Stones smaller than 5 millimeters (0.2 inches) often pass on their own without treatment. Pain management should be done with the help of analgesics. The child should also drink more fluids than usual to help flush the stone out. HOSPITAL In some cases, the child will need to be hospitalized for treatment. The two most common reasons for hospitalization are that:   The stone is blocking the urinary tract, preventing the normal flow of urine. If the blockage is not treated quickly, it can cause permanent damage to the kidneys. The child's pain cannot be controlled because it is severe or because the child is vomiting. - Analgesia Management of pain often requires intravenous administration of NSAIDs or opioids.[1] Orally administered medications are often effective for less severe discomfort. - Expulsion therapy The use of medications to speed the spontaneous passage of ureteral calculi is referred to as medical expulsive therapy. Several agents, including alpha adrenergic blockers (such as tamsulosin) and calcium 68
    • channel blockers (such as nifedipine), have been found to be effective. A combination of tamsulosin and a corticosteroid may be better than tamsulosin alone. These treatments also appear to be a useful adjunct to lithotripsy. - Shock wave lithotripsy Shock wave lithotripsy is the treatment of choice for kidney stone in many children. Lithotripsy is done by directing a high-energy shock wave toward the stone. The energy causes the stone to break into fragments that can be passed. The procedure takes about 20 minutes. Some, although not all, children are given anesthesia to prevent movement during the treatment. The success of lithotripsy depends, in part, on the size of the stone; larger stones are more difficult to break up and sometimes need more than one treatment. It can take three months after lithotripsy for all of the stone fragments to pass. - Percutaneous nephrolithotomy Large stones or stones that do not break up with lithotripsy will require a minimally invasive surgical procedure to remove the stone. During the procedure, small instruments are passed through the skin (percutaneously) into the kidney to remove the stone. The child is given anesthesia before the procedure to prevent pain. - Ureteroscopy Ureteroscopy is a procedure that can be done if the stone is in the middle and lower portion of the ureter. The doctor passes a small instrument through the urethra and bladder, into the ureter. The instrument contains a camera and other instruments, which allows the doctor to see the stone. The stone can be removed or broken up into smaller pieces that can pass more easily. PREVENTION Children who develop a kidney stone have a significant chance of developing stones in the future. Studies have estimated the chances to be between 30 and 65 percent. A number of steps can decrease the chances of developing another stone. Blood and urine tests After a child has had a kidney stone, blood and urine tests are performed to identify factors that can increase the risk of future stones. Testing is not done until the child is at home, walking and playing normally, eating a normal diet, and has finished any treatment for urinary tract infection. Stone testing If the stone was passed and saved, it should be analyzed to determine the type of stone. Based on what the stone is made of, one or more treatments might help to reduce the risk of future stones. 69
    • Drink more fluids Drinking more fluids can help to decrease the risk of forming all types of kidney stones. The goal is to increase the amount of urine that flows through the kidneys and ureters and to lower the concentration of substances that promote stone formation.     Infants – 750 mL or more (25 ounces or three cups) Children younger than five years of age – 1000 mL or more (33 ounces or four cups) Children between five and ten years of age – 1500 mL or more (50 ounces or six cups) Children greater than 10 years of age – 2000 mL or more (66 ounces or eight cups) Monitoring After a first kidney stone, the child's doctor or nurse might recommend an imaging test (like ultrasound) to monitor for new stones. This is especially important for children who are at high risk of kidney stones. RENAL FAILURE 1.Acute Renal Failure ARF develops when renal function is diminished to the point where body fluid homeostasis can no longer be maintained. It is the severe deterioration of renal function, manifested as sudden reduction of urine excretion as oliguria or anuria leading to fluid and electrolyte imbalance and accumulation of metabolic nitrogenous wastes and other biochemical products. ARF is an acute and potentially reversible irritability of the kidneys to perform their normal functions to maintain homeostasis. INCIDENCE Variable ETIOLOGY The causes are divided into three groups: Prerenal factors include shock, congestive heart failure, volume depletion from vomiting/diarrhea or diabetic acidosis. The most common cause of decreased renal perfusion in children is dehydration. In prerenal ARF urine osmolality is high, urine and specific gravity is less than 1.020. Although renal perfusion is greatly decreased, renal tubular function is normal. Children may exhibit nonspecific symptoms such as fever, dehydration and tachycardia. Intrarenal ARF results from injury to the kidney itself. This type of ARF may be caused by HUS, nephrotoxins such as contrast dye, ingestion of poison, glomerulonephritis, acute tubular necrosis, severe infections, liver failure and chemotherapy, resulting in large amounts of calcium and uric acid 70
    • excretion. Because of glomerular damage sodium cannot be conserved and urine cannot be concentrated. The child may present with nausea/vomiting, hypertension and oliguria. Postrenal or obstructive ARF results from urinary tract outflow obstruction by renal calculi, tumours, trauma resulting in hematoma, neurogenic bladder or structural abnormalities such as ureterovesical junction stricture. Newborn delayed voiding after birth, electrolyte imbalance, a poor urinary stream and/or abdominal mass suggests a structural abnormality and should be investigated promptly. Urine osmolality and sodium levels are usually unaffected. PHASES 1. Initial Although renal damage is occurring, the child may be asymptomatic 2. Oliguric Less than 1ml/kg/hr of urine is produced. This stage usually lasts from <10 days to several weeks. Impaired glomerular filtration causes solute and water reabsorption, urine output declines and serum waste products cannot be removed. As the child becomes symptomatic, uremia develops. Neurotoxicity from uremia causes an altered mental status and altered peripheral sensation. Electrolyte imbalance causes dysrhythmias, water retension may lead to congestive heart failure and hypertension and metabolic acidosis may develop because of the kidney‘s inability to excrete hydrogen ions. In addition to anemia caused by decreased erythropoietin production, blood loss may be caused by gastrointestinal bleeding secondary to platelet and protein anticoagulant dysfunction. 3. Diuretic phase It lasts days to weeks, there is a gradual return of renal function due to cellular regeneration and healing. Excessive urine output leading to dehydration and electrolyte imbalance may occur during this stage as a result of incompetent tubular transport of water and solutes. 4. Recovery phase It may take several months. If left untreated ARF can result in fluid overload, electrolyte imbalance, metabolic acidosis, uremia and coma. Can be treated by supportive care, including volume restoration, diuretic administration and dialysis. CLINICAL MANIFESTAIONS Depend upon underlying causes and duration Severe oliguria/ Anuria Child may be markably well / extremely sick Nausea / Vomiting Lethargy 71
    • Dehydration Acidotic breathing Altered consciousness Irregular cardiac rate, rhythm Edema Hypertension DIAGNOSIS Careful history taking Vomiting, diarrhea, fever, other renal disease Laboratory investigations Anemia, raised serum creatinine level, blood urea, electrolytes, pH, bicarbonate and complete blood count, reduced C3, Urine examination Protienuria, Hematuria, presence of casts USG Structural abnormalies, calculi IVP Acute tubular necrosis Radionuclide studies Evaluate GFR, renal blood flow Renal biopsy Ultimate cause PATHOPHYSILOGY Due to the different etiological factors, renal hypoperfusion and renal outflow obstruction will occur. It leads to impairment of renal function and renal failure. Marked reduction in GFR and renal blood flow due to vasoconstriction results in sodium and fliud retention which leads to edema. HTN may develop due to rennin – angiotensin mechanism caused by arteriolar constriction, increased circulatory overload and sodium retention. Metabolic acidosis may develop from failure of excretion of hydrogen ions due to reduced functions od nephron and hypercatabolic states, which lead to increased production of acids. Hyperkalemia may develop due to disturbed sodium and potassium exchange in the distal tubules and increased extra cellular potassium level resulting from damage tissue cells, RBCs and hypercatabolic states. 72
    • Due to etiologic factors Renal vasoconstriction arteriolar constriction GFR HTN Na & H2O retention circulatory overload & Na+ retention Edema function of nephrons damaged tissue cells & RBCs failure of excretion Of H2 ions disturbed Na+ & K+ exchange production of acids metabolic acidosis extra cellular K+ overload hyperkalemia TREATMENT Medical treatment o Fluid and dietary restrictions o Use of diuretics o Maintain Electrolytes o May need dialysis to jump start renal function o May need to stimulate production of urine with IV fluids, Dopomine, diuretics, etc. o Hemodialysis - Subclavian approach - Femoral approach - Peritoneal dialysis - Continous renal replacement therapy (CRRT) - Does not require dialysate Nursing interventions - Monitor I/O, including all body fluids - Monitor lab results - Watch hyperkalemia symptoms: malaise, anorexia, parenthesia, or muscle weakness, EKG changes - Watch for hyperglycemia or hypoglycemia if receiving TPN or insulin infusions - Maintain nutrition - Safety measures - Mouth care - Daily weights - Assess for signs of heart failure 73
    • - Skin integrity problems PROGNOSIS Mortality rate of ARF is about 20 to 40% which is influenced by the cause and duration of renal failure with severity of pathological changes. Poor prognosis is related to associated sepsis, HUS, prolonged anemia, cardiac failure, hepatic failure and respiratory failure with delayed initiation of treatment. CHRONIC KIDNEY DISEASE 2. Chronic Renal Failure It is a permanent irreversible destruction of nephron leading to severe deterioration of renal function, finally resulting to end stage renal disease. ETIOLOGY Cause below 5 years of age is mostly congenital anomalies After 5 that is acquired glomerular disease, hereditary disease o o o o Glomerular disease Congenital anomalies Obstructive uropathy Miscellaneous CLINICAL MANIFESTATIONS Renal - Hyponaturmia - Dry mouth - Poor skin turgor - Confusion, salt overload, accumulation of K with muscle weakness - Fluid overload and metabolic acidosis - Proteinuria, glycosuria Cardiovascular - Hypertension - Arrythmias - Pericardial effusion - CHF - Peripheral edema Neurological - Burning, pain, and itching, parestnesia - Motor nerve dysfunction - Muscle cramping - Shortened memory span 74
    • - Apathy - Drowsy, confused, seizures, coma, EEG changes GI - Stomatitis - Ulcers - Pancreatitis - Uremic fetor - Vomiting - consitpation Respiratory - chance of infection - Pulmonary edema - Pleural friction rub and effusion - Dyspnea Endocrine - Stunted growth in children - Amenorrhea - Male impotence - Increased aldosterone secretion - Impaired glucose levels R/T impaired CHO metabolism - Thyroid and parathyroid abnormalities - Hemopoietic - Anemia - Decrease in RBC survival time - Blood loss from dialysis and GI bleed - Platelet deficits - Bleeding and clotting disorders – purpura and hemorrhage from body orifices , ecchymoses Skeletal - Muscle and bone pain - Bone demineralization - Pathological fractures - Blood vessel calcifications in myocardium, joints, eyes, and brain Skin - Yellow-bronze skin with pallor - Puritus - Purpura - Uremic frost - Thin, brittle nails - Dry, brittle hair, and may have color changes and alopecia 75
    • Early symptoms Weakness Anorexia Nausea Failure to thrive Unexplained anemia Osteodystrophy Growth failure Late manifestations Gastrointestinal bleeding Pericarditis Congestive cardiac failure Altered sensorium Indications of poor prognosis Convulsions Coma Cardiomyopathy DIAGNOSIS Blood examination Decreased hematocrit, Hb%, Na+, Ca++, HCO-3, increased K+ & phosphorus Renal function test Gradual increase in BUN, uric acid & creatinine Urinalysis Variation in specific gravity, increased urine creatinine, change in total urine output X-Ray Chest, hands, knees, pelvis, spine to detect bony defect ECG, IVP, MCU, radio nuclide imaging Extent of complications Other abnormal findings - Metabolic acidosis - Fluid imbalance - Insulin resistance - Immunoligical problems PATHOPHYSIOLOGY In the early stage of disease child remains asymptomatic. Advance renal damage will occur only in late stages. Increased numbers of neurons are destructed at various degrees and a few remain intact but hypertrophied and functional. This less number of nephrons are enough to make sufficient adjustments in fluid and electrolyte balance. As the disease progress to end stage severe reduction in number of nephrons occur and the kidney will not b able to maintain fliud and electrolyte balance. The accumulatin of various substances in blood result in complications STAGES OF CRF I. II. Diminished Renal Reserve Normal BUN, and serum creatinine absence of symptoms Renal Insufficiency 76
    • GFR is about 25% of normal, BUN Creatinine levels increased Renal Failure GFR <25% of normal increasing symptoms ESRD or Uremia GFR < 5-10% normal, creatinine clearance <5-10 ml/min resulting in a cumulative effect III. IV. COMPLICATIONS - Azotemia Metabolic acidosis Electrolyte imbalance CCF HTN Severe anemia Growth retardation Delayed or absent sexual maturation MANAGEMENT - Conservative management - Correction of reversible component of renal dysfunction - Preservation of renal function - Treatment of metabolic and psycho-social problems - Optimization of growth - Preparation for treatment of ESRD - Treat for infection, accelerated hypertension, CCF, obstruction of urine flow - to improve renal function Dietary therapy Low protein diet Severe protein restriction may produce protein calorie malnutrition Diet should consist of 100 percent RDA for calories Protein should be of high biological value and should comprise 6 – 10 % of all calories Salt restriction in patients with hypertension and fluid overload Patients with salt losing nephropathy should take a liberal amount of salt and water If the GFR falls below 10ml/min/1.73m2, potassium intake should be restricted.(hyperkalemia may develop) Vit D is essential to raise the serum calcium and suppress parathormone secretion. Dialysis Renal transplatation NURSING MANAGEMENT Frequent monitoring o Hydration and output o Cardiovascular function 77
    • o Respiratory status o Electrolytes o Nutrition Mental status o Emotional well being Ensure proper medication regimen Skin care Bleeding problems Care of the shunt Education to client and family 3. End Stage Renal Disease ESRD represents the state in which a patient‘s renal dysfunction has progressed to the point at which homeostasis and survival can no longer be sustained with native kidney function and maximal medical management. At this point, renal replacement therapy (dialysis or renal transplantation) becomes necessary. The ultimate goal for children with ESRD is successful kidney transplantation because it provides the most normal lifestyle and possibility for rehabilitation for the child and family. RENAL REPLACEMENT THERAPIES Dialysis It is a treatment modality used to manage ESRD. Dialysis is the diffusion of solute molecules through a semipermiable membrane, passing from higher concentration to that of lower concentration. It is the process of separating colloids and crystalline substances in solution by the difference in their rate of diffusion through a semi permeable membrane. The purpose of dialysis is to remove endogenous and exogenous toxins and to maintain fluid electrolyte and acid- base balance till the renal function recovers. It is a substitute for some excretory functions of kidneys but does not replace the endocrine and metabolic functions. GENERAL PRINCIPAL: Movement of fluid and molecules across a semi permeable membrane from one compartment to another INDICATIONS a) b) c) d) Uremic symptoms with neurologic abnormalities Persistent hyperkalemia, above 6.5 mEq/L Blood urea level more than 150 mg./dl Severe acidosis, pH less than 7.2, TCO2 less than 10-12 mEq/L 78
    • e) Hyperphosphatemia f) Pulmonary edema and CCF METHODS OF DIALYSIS 1. Hemodialysis 2. Peritoneal dialysis Continuous ambulatory peritoneal dialysis Automated peritoneal dialysis (Continuous cycling peritoneal dialysis) 3. Continuous renal replacement therapies Continuous venovenous hemofiltration Continuous venovenous hemodialysis Continuous venovenous hemodiafiltration 1.Hemodialysis Pediatric hemodialysis is referred to extracorporeal renal replacement therapy in children under the age of 15 years. The neonates, infants and smaller children have special requirements for dialysis. Pediatric dialysis program could start even from a neonate who is less than 1 kg. The treatment requirements almost are similar to adults but there are certain differences as: 1. Renal replacement therapies 2. Growth and development 3. Psychological demands. SPECIFIC CONSIDERATIONS: The choice of dialyser, extracorporeal circuit volume and blood flow rates requires specific consideration.  The extracorporeal circuit The amount of blood occupied by the blood vessels and the dialyser should not exceed more than 10% of the total blood volume of the child. If the child is severely anemic ( Hb < 5-6g/dL) the extracorporeal circuit volume should not exceed 7%. The total blood volume can be calculated by multiplying the child‘s weight by 80mls.  Blood lines and dialysers The surface area of the dialyser should not exceed the body surface area (BSA) of the child. BSA (Mostellar equation) = Multiply the child‘s height by its weight Divide the result by 3600 Determine the square root of result 2 i.e., SA(m )= √([Ht in cms x Wt in kgs]÷3600)  Blood flow rate It is determined by the child‘s size, blood volume, blood pressure and tolerance for dialysis. -Blood flow rate should not exceed 2.5 x weight (kg) ÷ 100 TREATMENT PARAMETERS: 79
    •  Fluid replacement for hypotension should not exceed a maximum of 10ml/kg body weight and may given in divided doses.  Ultra filtration should not exceed 5% of total body weight for each dialysis session.  Clearance rates for urea should not exceed 3 mls/min/kg body weight with 1.5 to 2ml/min/kg body weight for extremely uremic children. VASCULAR ACCESS: Hemodialysis requires the creation of a vascular access and the use of special dialysis equipment – the hemodialyzer (artificial kidney). Vascular access are of the three types: 1. Fistulas Arterio vascular fistula is an access in which a vein and artery are connected surgically. The preferred site is the radial artery and a forearm vein that produces dilation and thickening of superficial vessels of the forearm to provide easy access for repeated venipuncture. 2. Grafts Subcutaneous (internal) arteriovenous graft is a synthetic prosthetic graft for circulatory access made by the anastomosis of artery and vein. 3. External vascular access For this percutaneous catheters are inserted in the femoral, subclavian or internal jugular veins. A central catheter into internal jugular vein is more permanent form. COMPLICATIONS - more prone to cardiovascular instability due to small blood volume. Excessive weight loss Hypotension Hypothermia in infants Dialysis equilibrium syndrome: Fluid removal and decrease in BUN during hemodilaysis cause changes in blood osmolarity. These changes trigger a fluid shift from the vascular compartment into the cells. In the brain, this can cause cerebral edema, resulting in increase intracranial pressure and visible signs of decreasing level of consciousness. Symptoms: Sudden onset of headache, nausea and vomiting, nervousness, muscle twitching, palpitation, disorientation and seizures Treatment: Hypertonic saline, Normal saline if immediate treatment for disequilibrium is not provided it leads to: Convulsions Coma Cardiac arrhythmia ADVANTAGES suited for children who do not have someone in family to perform home peritoneal dialysis easy for those who live near dialysis center achieves rapid correction of fluid and electrolyte abnormality 80
    • DISADVANTAGES associated to the rapid change, it can cause muscle cramping and hypotension school absence during dialysis strict fluid and dietary restrictions boredom for child during the session 2. Peritoneal dialysis The difficulty in finding a vascular access and maintaining adequate blood flow for hemodialysis makes peritoneal dialysis as a preferred choice. Peritoneal dialysis is a technique that employs the patient‘s peritoneal membrane as a dialyzer. Excess body water is removed by an osmotic gradient created by the high dextrose concentration in the dialysate; wastes are removed by diffusion from the peritoneal capillaries into the dialysate. Because peritoneal dialysis is not as efficient as hemodialysis, it must be performed daily rather than 3 times weekly as in hemodialysis. Children and adolescents typically have three 3- to 4-hr sessions per week during which fluid and solute wastes are removed. The peritoneal dialysis exchange procedure consists of 3 steps/ phases (exchanges): Infusion : A sterile, dialysis solution flows into your peritoneal cavity by gravity via a catheter or tube that has been surgically placed into the abdomen. The filling takes about 10 minutes. Once the filling is complete, the catheter is shut so that it does not leak. Dwell : The lining of the peritoneal cavity called the peritoneum acts as a natural filter. It lets the waste products and excess fluids in the blood filter through into the dialysis solution, while holding back important substances that the body needs. The length of time varies from 3 - 6 hours. While the solution is in the body you can move about. Drain : The dialysis solution containing the wastes is drained again by gravity from your body through the catheter into an empty bag. This takes about 10-20 minutes. A bag containing sterile dialysis solution replaces the bag containing waste products. The whole process is then repeated. Each of these replacements is called a ' Bag Exchange'. TYPES 1. Continuous ambulatory peritoneal dialysis It is the most commonly used method of peritoneal dialysis. The filtration process occurs most hours of the day. The exchange usually take about 3 minutes 3-4 times a day and only require a solution bag with tubing attached to it that connects to the child‘s blood stream. It gives freedom. 2. Automated Peritoneal Dialysis (Continuous cycling peritoneal dialysis) Continuous Cyclic Peritoneal Dialysis 81
    •  Continuous regimen means that the dialysis solution is present in the peritoneal cavity continuously, with the exception of short significant periods between exchange.  It uses duel lumen catheterization, i.e., 2 catheters, one for inflow and other for outflow. Intermittent Peritoneal Dialysis  It means the dialysis sessions are performed several times a week.  This technique uses one catheter for inflow and outflow. Flow is interrupted after both inflow and outflow during exchange. Nocturnal intermittent peritoneal dialysis It is a form of intermittent peritoneal dialysis which is performed every night. For an efficient dialysis a total time upto 12 hours is required. So this method helps dialysis become more efficient and to reduce frequency of dialysis and increase the resting time. PROCEDURE The abdomen is cleaned in preparation for surgery, and a catheter is surgically inserted with one end in the abdomen and the other protruding from the skin. Before each infusion the catheter must be cleaned, and flow into and out of the abdomen tested. The warmed solution is allowed to enter the peritoneal cavity by gravity and remains a variable length of time (usually 10-15 minutes) according to the rate of solute removal and glucose absorption in individual patients. The total volume is referred to as dwell while the fluid itself is referred to as dialysate. The dwell can be as much as 2.5 litres, and medication can also be added to the fluid immediately before infusion. The dwell remains in the abdomen and waste products diffuse across the peritoneum from the underlying blood vessels. After a variable period of time depending on the treatment (usually 4–6 hours), the fluid is removed and replaced with fresh fluid. RISKS HTN & other cardiac complications Seizure Obstructed catheter Dialysate leakage Hyperglycemia Increased triglyceride levels Increased protein loss Parental stress and burnout ADVANTAGES Ability to perform dialysis treatment at home Technically easier than hemodialysis, especially in infants Ability to live a greater distance from medical center Freedom to attend school and after-school activities Less-restrictive diet Less expensive than hemodialysis Independence (adolescents) 82
    • DISADVANTAGES Catheter malfunction Catheter-related infections (peritonitis, exit site) Impaired appetite (due to full peritoneal cavity) Negative body image Caregiver burnout 3.Continuous Renal Replacement Therapies It is useful in patients with unstable hemodynamic condition, sepsis etc. it is an extra corporeal therapy I which fluid and electrolytes are continuously removed from blood using a special pump-driven machine. It continuously pass patient‘s blood across a highly permeable filter. Continuous venovenous hemofiltration Large amount of fluid moves by pressure across the filter bringing with it by convection other molecules such as urea, creatinine, uric acid and phosphorus. It is replaced with desirable electrolyte composition similar to blood. Continuous venovenous hemodialysis It utilizes the principle of diffusion by circulating dialysate in a countercurrent direction on the ultra filtrate side of the membrane, no replacement fluid is used. Continuous venovenous hemodiafiltration It employs both the replacement fluid and dialysate, offering the most effective solute removal of all forms of renal replacement therapies. ADVANTAGES Hemodynamic stability – Avoid hypotension complicating hemodialysis – Avoid swings in intravascular volume Easy to regulate fluid volume – Volume removal is continuous – Adjust fluid removal rate on an hourly basis Customize replacement solutions Lack of need of specialized support staff 83
    • DISADVANTAGES Lack of rapid fluid and solute removal – GFR equivalent of 5 - 20 ml/min – Limited role in overdose setting SLED – Developing role Filter clotting – Take down the entire system RENAL TRANSPLANTATION Kidney transplantation is recognized as the optimal therapy for children with end-stage renal disease (ESRD). Even though renal transplantation has been conducted from 1954, children were not considered as suitable candidates until 1960s. The psychological, physiological and social difficulties of children (side effects of immune suppression, technical difficulties etc.) were successfully addressed and it is now universally accepted to provide maximum opportunity for normal growth and development and the best quality of life. Vigilant medication administration is required after transplantation to prevent organ rejection. The child may achieve 40 – 80 % renal function with the transplant and demonstrate improved growth, enhanceded cognitive development, and improved psychosocial development and quality of life. INCIDENCE AND ETIOLOGY Congenital, hereditary, and cystic diseases are the cause in more than 52% of children 0 to 4 yr of age, Glomerulonephritis, focal segmental glomerulosclerosis account for 38% of cases in 10 to 19 yr of age. Structural disease (49%) Various forms of glomerulonephritis (14%) Focal segmental glomerulosclerosis (12%) INDICATIONS  Almost all children with ESRD  renal replacement therapy DONOR SELECTION 1. Living related donor Living related donor is preferred for a pediatric patient because it allows transplantation to be planned when the child ad donor is in optimal health. The suitable donors include parents, siblings, grandparents, uncles and aunts. This method accounts more survival rate than from the cadaveric donors. It also allows a immune conditioning regimen to be commenced during pre-transplant week. (mycophenolate mofetil and prednisolone). It allows for a final cross match. 84
    • 2. Cadaveric donor A cadaveric transplant is placed when there is no availability of a related living donor. A cadaver is a patient declared brain dead who had previously given consent for organ donation. After permission for donation is granted, the kidneys are removed and stored until a recipient has been selected. PREPARATION FOR TRANSPLATATION  A pre–emptive transplantation i.e.; transplantation occurs prior to dialysis is preferred for children.  Clinical and laboratory evaluation of kidney donor and recipient Blood Type Testing The recipient and donor should have either the same blood type or compatible ones. If the recipient blood type is A Donor blood type must be A or O If the recipient blood type is B Donor blood type must be B or O If the recipient blood type is O Donor blood type must be O If the recipient blood type is AB Donor blood type can be A, B, AB, or O The AB blood type is the easiest to match because that individual accepts all other blood types. Blood type O is the hardest to match. Although people with blood type O can donate to all types, they can only receive kidneys from blood type O donors. Tissue Typing It is a blood test for human leukocyte antigens (HLA), is called tissue typing. To receive a kidney where recipient's markers and the donor's markers all are the same is a "perfect match" kidney. Perfect match transplants have the best chance of working for many years. Most perfect match kidney transplants come from siblings. Crossmatch crossmatch is done to ensure the recipient does not have pre-formed antibodies to the donor . Serology Testing is also done for viruses, such as HIV (human immunodeficiency virus), hepatitis, and CMV (cytomegalovirus) to select the proper preventive medications after transplant. CONTRAINDICATIONS Pre-existing metastatic malignancy or HIV Patients with remission of malignancy off maintenance treatment for a minimum of 2 yr may be reconsidered on an individual basis for transplantation, with close post-transplantation surveillance Patients with autoimmune diseases resulting in ESRD are candidates for transplantation after a period of immunologic quiescence of the primary disease for a period of at least 1 year before transplantation. Severe neurologic dysfunction 85
    • SPECIAL CONSIDERATIONS - - - - Dialysis may be required for a period before transplantation to optimize nutritional and metabolic conditions, to achieve an appropriate size in small children or to keep a patient stable until a suitable donor is available For young infants, a recipient may need to weigh at least 8-10 kg to minimize the risk for vascular thrombosis and to accommodate an adult-sized kidney. This can require a period of dialysis support until the child is at least 12 to 18 mo of age. It would be best match the recipient with an appropriately sized cadaveric kidney. But it increases the chance of vascular thrombosis. Sibling donors of same size are best suitable. Transplantation with an adult-sized kidney has been successful in children who weighed <10 kg or were <6 months of age. For children weighing 20kg or more, the kidney is placed extra peritoneally. Intraperitoneal approach is required for those weighing less than 20 kg and receiving adult kidney. If the donor is less than 2 years, the kidneys are transplanted ‗en-block‘, together with donor aorta and venacava. Many surgeons prefer to remove the appendix at the time of transplantation as it will be difficult to differentiate between appendicitis and tenderness that accompanies acute rejection. PROCEDURE The transplant surgery is performed under general anesthesia. The operation usually takes 2-4 hours. This type of operation is a heterotopic transplant meaning the kidney is placed in a different location than the existing kidneys. (Liver and heart transplants are orthotopic transplants, in which the diseased organ is removed and the transplanted organ is placed in the same location.) The kidney transplant is placed in the front (anterior) part of the lower abdomen, in the pelvis. The original kidneys are not usually removed unless they are causing severe problems such as uncontrollable high blood pressure, frequent kidney infections, or are greatly enlarged. The artery that carries blood to the kidney and the vein that carries blood away is surgically connected to the artery and vein already existing in the pelvis of the recipient. The ureter, or tube, that carries urine from the kidney is connected to the bladder. Recovery in the hospital is usually 3-7 days. POST TRANSPLANTATION COMPLICATIONS  Rejection is a major problem - Hyperacute rejection: occurs within minutes to hours after transplantation Renal vessels thrombosis occurs and the kidney dies There is no treatment and the transplanted kidney is removed - Acute Rejection: occurs 4 days to 4 months after transplantation It is not uncommon to have at least one rejection episode Episodes are usually reversible with additional immunosuppressive therapy (Corticosteroids, muromonab-CD3, ALG, or ATG) Signs: increasing serum creatinine, elevated BUN, fever, wt. gain, decrease output, increasing BP, tenderness over the transplanted kidneys - Chronic Rejection: occurs over months or years and is irreversible. 86
    • - The kidney is infiltrated with large numbers of T and B cells characteristic of an ongoing, low grade immunological mediated injury Gradual occlusion renal blood vessels Signs: proteinuria, HTN, increase serum creatinine levels Supportive treatment, difficult to manage Replace on transplant list Infection Hypertension Malignancies (lip, skin, lymphomas, cervical) Recurrence of renal disease Retroperioneal bleed Arterial stenosis Urine leakage NURSING MANAGEMENT Immediate post operative management (Preventing rejection and promoting renal function) - Administer immuno suppressants accurately on time.  Medication that prevent rejection  Cyclosporine  Tacrolimus  Azathioprine  Mycophenolate mofetil  Prednisone  Antithymocyte Ig (ATGAM)  Sirolimus - Focus on achieving graft functioning Early mobilization Monitor closely for few days with attention to fluid and electrolyte replacement Assess for rejection and infections. Care should be given to  central venous catheter for fluid replacement and central venous pressure measurement  arterial line for blood pressure monitoring blood taking.  Indwelling urine catheter to measure intake and output  Drain tube to collect leakage from anastomoses 87
    • - Routine monitoring includes vital signs, central venous pressure and urine output. Fluid balance and central venous pressure should be monitored to ensure appropriate renal perfusion RENAL TUBULAR ACIDOSIS (RTA) INTRODUCTION Lungs and Kidneys are responsible for Normal acid base balance Alveolar ventilation removes CO2 Kidneys reabsorb filtered Bicarbonate and excrete a daily quantity of Hydrogen ion equal to that produced by the metabolism of dietary proteins. Hydrogen ions are excreted primarily by enhancing the excretion of ammonium ions in the urine Renal tubular acidosis: A group of disorders characterized by an inability of the kidney to reabsorb bicarbonate or secrete hydrogen ions, resulting in hyperchloremic, normal anion gap acidosis. DEFINITION The term "renal tubular acidosis" (RTA) refers to a group of disorders in which, despite a relatively well-preserved glomerular filtration rate, metabolic acidosis develops because of defects in the ability of the renal tubules to perform the normal functions required to maintain acid-base balance. INCIDENCE Predominant age: All ages Predominant sex: Male > Female (with regard to type II RTA with isolated defect in bicarbonate reabsorption) TYPES Distal or type 1 RTA Proximal or type 2 RTA Hypoaldosteronism or type 4 RTA There is a transiently severe form of distal RTA in infants, the term type 3 or mixed RTA is now most often applied to a rare autosomal recessive syndrome with features of both distal and proximal RTA.  Acidosis - the tendency for RTA to lower the blood's pH  Acidemia - when the blood pH is below normal (7.35) 88
    • Type 1-Distal RTA Distal RTA (dRTA) is the classical form of RTA. Inability of the distal tubule to acidify the urine. Due to impaired hydrogen ion secretion, increased backleak of secreted hydrogen ions, or impaired sodium reabsorption (causing less negative potential in the lumen and hence less hydrogen/potassium secretion). Urine pH >5.5. Pathophysiology: - failure of acid secretion by the alpha intercalated cells of the cortical collecting duct of the distal nephron. it leads to an inability to acidify the urine to a pH of less than 5.3 renal excretion is the primary means of eliminating acid from the body, there is consequently a tendency towards acidemia There is an inability to excrete H+ while K+ cannot be reabsorbed, leading to acidemia (as H+ builds up in the body) and hypokalemia (as K+ cannot be reabsorbed). Since calcium stones demonstrate a proclivity for deposition at higher pHs (alkaline), the substance of the kidney develops stones bilaterally; this does not occur in the other RTA types. Type 2-Proximal RTA Type II (proximal) RTA: Defect of the proximal tubule in bicarbonate (HCO3) reabsorption. HCO3 fully reabsorbed only when plasma HCO3 concentration <15–16 mEq/L (compared with normal threshold of 24 mEq/L). Urine pH <5.5 unless plasma HCO3 above reabsorptive threshold. Pathophysiology: - Proximal RTA (pRTA) is caused by a failure of the proximal tubular cells to reabsorb filtered bicarbonate from the urine, leading to urinary bicarbonate wasting and subsequent acidemia. The distal intercalated cells function normally, so the acidemia is less severe than dRTA and the urine can sacidify to a pH of less than 5.3. occasionally be present as a solitary defect it is usually associated with a more generalized dysfunction of the proximal tubular cells called Fanconi's syndrome, in which there is also phosphaturia, glycosuria, aminoaciduria, uricosuria, and tubular proteinuria. The principal feature of Fanconi's syndrome is bone demineralization (osteomalacia or rickets) due to phosphate wasting. Type 3 RTA-Combined proximal and distal RTA Extremely rare autosomal recessive syndrome with features of both type I and type II. This term is no longer used. In some patients, there are features of both dRTA and pRTA. This form of RTA has also been referred to as juvenile RTA. 89
    • - Combined dRTA and pRTA is also observed as the result of inherited carbonic anhydrase II deficiency. Mutations in the gene encoding this enzyme give rise to an autosomal recessive syndrome of osteopetrosis, renal tubular acidosis, cerebral calcification, and mental retardation. Type 4 RTA Due to aldosterone resistance or deficiency that results in hyperkalemia. - It is not actually a tubular disorder at all nor does it have a clinical syndrome similar to the other types of RTA described above. It was included in the classification of renal tubular acidoses as it is associated with a mild (normal anion gap) metabolic acidosis due to a physiological reduction in proximal tubular ammonium excretion (impaired ammoniagenesis), which is secondary to hypoaldosteronism, and results in a decrease in urine buffering capacity. Its cardinal feature is hyperkalemia, and measured urinary acidificsation is normal, hence it is often called hyperkalemic RTA or tubular hyperkalemia. Urine pH usually <5.5. RISK FACTORS Genetics Type I RTA: Autosomal dominant or recessive. May occur in association with other genetic diseases (e.g., Ehlers-Danlos syndrome, hereditary elliptocytosis, or sickle cell nephropathy). The autosomal recessive form is associated with sensorineural deafness. Type II RTA: Autosomal dominant form is rare. Autosomal recessive form is associated with ophthalmologic abnormalities and mental retardation. Occurs in Fanconi syndrome, which is associated with several genetic diseases (e.g., cystinosis, Wilson disease, tyrosinemia, hereditary fructose intolerance, Lowe syndrome, galactosemia, glycogen storage disease, and metachromatic leukodystrophy). Type IV RTA: Some cases familial, such as pseudohypoaldosteronism type I (autosomal dominant) ETIOLOGY Type I RTA: Genetic: Autosomal dominant, autosomal recessive associated with sensorineural deafness Sporadic Ehlers-Danlos syndrome Autoimmune diseases: Sjögren syndrome, rheumatoid arthritis (RA), systemic lupus erythematosus Hematologic diseases: Sickle cell disease, hereditary elliptocytosis 90
    • Medications: Amphotericin B, lithium, ifosfamide, foscarnet, analgesics, K+-sparing diuretics (amiloride, triamterene), trimethoprim Toxins: Toluene, glue Hypercalciuria, diseases causing nephrocalcinosis Vitamin D intoxication Medullary cystic disease Glycogenosis type III Fabry disease Wilson disease Hypergammaglobulinemic syndrome Obstructive uropathy Chronic pyelonephritis Chronic renal transplant rejection Leprosy Hepatic cirrhosis Malnutrition Type II RTA: Diseases associated with Fanconi syndrome Sporadic Multiple myeloma and other dysproteinemic states Amyloidosis Heavy-metal poisoning (e.g., cadmium, lead, mercury, copper) Medications: Acetazolamide, sulfanilamide, ifosfamide, outdated tetracycline, topiramate Autoimmune disease Interstitial renal disease Nephrotic syndrome Congenital heart disease Defects in calcium metabolism (hyperparathyroidism) Type IV RTA: Medications: Nonsteroidal anti-inflammatory drugs, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, heparin/LMW heparin, calcineurin inhibitors (tacrolimus, cyclosporine) (1) Diabetic nephropathy Obstructive nephropathy Nephrosclerosis due to hypertension Tubulointerstitial nephropathies Primary adrenal insufficiency Pseudohypoaldosteronism (end-organ resistance to aldosterone) 91
    • Gordon syndrome Sickle cell nephropathy CLINICAL MANIFESTATIONS - - - Distal (Type I) RTA non-anion gap metabolic acidosis growth failure nephrocalcinosis hypercalciuria Patients with isolated, sporadic, or inherited proximal RTA (Type II) growth failure in the 1st year of life polyuria dehydration anorexia vomiting constipation hypotonia Patients with primary Fanconi syndrome will have additional symptoms secondary to phosphate wasting such as rickets. Those with systemic diseases will present with additional signs and symptoms specific to their underlying disease Hyperkalemic (Type IV) RTA with growth failure in the first few years of life Polyuria Dehydration Rarely, with life-threatening hyperkalemia Patients with obstructive uropathies may present acutely with signs and symptoms of pyelonephritis, o Fever o Vomiting o foul-smelling urine hyperkalemic non-anion gap metabolic acidosis Alkaline or acidic urine Elevated urine sodium levels & inappropriately low urine potassium levels reflect the absence of aldosterone effect. DIAGNOSIS - History collection • Often asymptomatic (particularly type IV) • Failure to thrive in children • Anorexia, nausea/vomiting 92
    • • Weakness or polyuria (due to hypokalemia) • Rickets in children • Osteomalacia in adults • Constipation • Polydipsia Diagnostic Tests • Electrolytes reveal hyperchloremic metabolic acidosis. Plasma anion gap normal (anion gap = Na - [Cl + HCO3]). Normal values (in mEq/L): Neonates ≤16; infants/children ≤14–16; adolescents/adults 8 ± 4). Hypokalemia or normokalemia: Type I (if due to impaired distal H+ secretion or increased H+ backleak), type II Hyperkalemia: Type IV, type I (if due to impaired distal Na+ reabsorption) Plasma HCO3 (in untreated RTA): Type I: <15 mEq/L; type II: 12–20 mEq/L; type IV: >17 mEq/L Blood urea nitrogen and creatinine usually normal (rules out renal failure as cause of acidosis) Urine pH: Inappropriately alkaline (pH >5.5) despite metabolic acidosis in type I or in type II when HCO3 above reabsorptive threshold (15–16 mEq/L) Urine culture: Rule out urinary tract infection (UTI) with urea-splitting organism (may elevate pH) and chronic infection Urine anion gap (urine Na+, K+, and Cl- on random urine): Reflects unmeasured urine anions, so inversely related to urine NH4+ (or acid) excretion. Positive urine anion gap in an acidemic patient indicates impaired renal acid excretion. Results tend to be: o Negative in HCO3 losses due to diarrhea, or UTI caused by urea-splitting organisms o Negative in other extrarenal causes of normal anion gap metabolic acidosis o Variable in type II RTA o Positive in type I RTA, type IV RTA (3)[C] o Positive in impaired acid excretion due to renal failure Urine calcium: o o • High in type I Typically normal in type II A renal ultrasound - to identify underlying structural abnormalities such as obstructive uropathies as well as to determine the presence of nephrocalcinosis. 93
    • TREATMENT  correction of the acidemia with oral sodium bicarbonate, sodium citrate or potassium citrate. This will correct the acidemia and reverse bone demineralization  Hypokalemia and urinary stone formation and nephrocalcinosis can be treated with potassium citrate tablets which not only replace potassium but also inhibit calcium excretion and thus do not exacerbate stone disease as sodium bicarbonate or citrate may do  Patients with proximal RTA often require large quantities of bicarbonate, up to 20 mEq/kg/24 hr in the form of sodium bicarbonate or sodium citrate solution (Bicitra or Shohl's solution). Also we have( polycitra solution) which same as bictra with adding of potassium citrate.  The base requirement for distal RTAs is generally in the range of 2-4 mEq/kg/24 hr, although patient requirements may vary .  Patients with Fanconi syndrome generally require phosphate supplementation .  Patients with distal RTA should be monitored for the development of hypercalciuria. Those with ssymptomatic hypercalciuria (e.g., recurrent episodes of gross hematuria), nephrocalcinosis, or nephrolithiasis may require thiazide diuretics to decrease urine calcium excretion.  Patients with type IV RTA may require chronic treatment for hyperkalemia with sodium-potassium exchange resin  Rickets may be present in primary renal tubular acidosis (RTA), particularly in type II or proximal RTA. Administration of sufficient bicarbonate to reverse acidosis stops bone dissolution and the hypercalciuria that is common in distal RTA. Proximal RTA is treated with both bicarbonate and oral phosphate supplements to heal bone disease. Doses of phosphate similar to those used in familial hypophosphatemia or Fanconi syndrome should be used . Vitamin D is needed to offset the secondary hyperparathyroidism that complicates oral phosphate therapy  The mainstay of therapy in all forms of RTA is bicarbonate replacement . PROGNOSIS Depends on associated disease, otherwise good with therapy Transient forms of all types of RTA may occur. COMPLICATIONS Nephrocalcinosis, nephrolithiasis (type I) Hypercalciuria (type I) Hypokalemia (type I, type II if given bicarbonate) Hyperkalemia (type IV, some causes of type I) Osteomalacia (type II due to phosphate wasting) 94
    • VESICO URETERAL REFLUX VUR implies the passage of urine into the ureter and kidney during micturition. Normally the long submucosal and intra vascular segment of the ureter at the ureterovesical junction closes when bladder contracts, effectively preventing VUR. So it is an abnormal retrograde flow of bladder urine into the ureters. Urine normally travels from the kidneys via the ureters to the bladder. In vesicoureteral reflux the direction of urine flow is reversed. INCIDENCE       VUR is present in more than 10% of the population. In children without urinary tract infections 17.2-18.5% have VUR. In those with urinary tract infections the incidence may be as high as 70%. Younger children are more prone to VUR. It decreases to 15% by the age of 12. It is more common in males ante-natally, in later life there is a female preponderance with 85% of cases. ETIOLOGY - Vesicoureteral reflux may present before birth as prenatal hydronephrosis, an abnormal widening of the ureter or with a urinary tract infection or acute pyelonephritis. It is associated with recurrent UTI CLINICAL MANIFESTATIONS - Newborns may be lethargic with faltering growth UTI - If the child is with UTI, he may have: - pyrexia - dysuria - frequent urination - malodorous urine - Chills - Vomiting - feeling that the bladder does not empty completely DIAGNOSTIC EVALUATION - - History Collection & Physical Examination inheritance urinary tract infection Nuclear cystogram for subsequent evaluations as there is less exposure to radiation Fluoroscopic voiding cysto urethrogram Grading the initial work up Ultrasonic cystography Abdominal ultrasound 95
    • - Urethral dialation urine culture to check for a UTI Ultrasound of the kidneys to find out the size and shape of the kidneys. It can't detect reflux. TYPES Primary VUR Primary VUR is present at birth. It is caused by a defect in the development of the valve at the end of the tube that carries urine from the kidneys to the bladder (ureter). This is the most common type of VUR and is usually detected shortly after birth. Insufficient submucosal length of the ureter relative to its diameter causes inadequacy of the valvular mechanism. This is precipitated by a congenital defect/lack of longitudinal muscle of the intravesical ureter resulting in an ureterovesicular junction (UVJ) anomaly. Secondary VUR Secondary VUR occurs when an obstruction in the bladder or urethra causes urine to flow backward into the kidneys. Secondary VUR can occur at any age and can be caused by surgery, injury, a pattern of emptying the bladder that's not normal, or a past infection that puts pressure on the bladder. It is more common in children who have other birth defects, such as spina bifida. In this category the valvular mechanism is intact and healthy to start with but becomes overwhelmed by raised vesicular pressures associated with obstruction, which distorts the ureterovesical junction. The obstructions may be anatomical or functional. The severity is graded using the International Classification of I to V based on the appearance of the urinary tract on contrast voidig cysto urethrogram. The more the reflux the higher the rate of injury. Grade I - reflux into non-dilated ureter Grade II - reflux into the renal pelvis and calyces without dilatation Grade III - mild/moderate dilatation of the ureter, renal pelvis and calyces with minimal blunting of the fornices Grade IV – dilation of the renal pelvis and calyces with moderate ureteral tortuosity Grade V – gross dilatation of the ureter, pelvis and calyces; ureteral tortuosity; loss of papillary impressions TREATMENT - The goal of treatment is to minimize infections,renal injury and other complications of reflux. In newborn nd infants , prophylactic antibiotics In lder children, bowel and bladder management 96
    • - (sulfamethaxole – trimethoprim, Cephalosporins, nitrofurantoin) Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics. Medical management is recommended in children with Grade I-III VUR as most cases will resolve spontaneously. When medical management fails to prevent recurrent urinary tract infections, or if the kidneys show progressive renal scaring then surgical interventions may be necessary. Endoscopic injection Deflux is a gel that is used in endoscopic injections to treat Vesicoureteral Reflux. It is the material that the surgeon injects around the ureteral opening to create a valve function and stop urine from flowing back up the ureter. Deflux consists of two types of sugar-based molecules called dextranomer and hyaluronic acid. Both substances are well-known from previous uses in medicine. Both materials are also biocompatible, which means that they do not cause significant reactions within the body. In fact, hyaluronic acid is produced and found naturally within the body. Surgical Management A surgical approach is necessary in cases where a breakthrough infection results despite prophylaxis, or there is non-compliance with the prophylaxis. There are three types of surgical procedure available for the treatment of VUR: endoscopic (STING/HIT procedures) laparoscopic open procedures (Cohen procedure, Leadbetter-Politano procedure). Follow up - Annual evaluation Blood pressure, weight, height, VCU, urine culture 97