1. URO NEPHROLOGY PATHOLOGIES

4. URINE NORMAL RESULTS Urinalysis requires a fresh sample in a clean container done within 2 hours of collection. If is going to be delayed, needs refrigeration, avoiding the decomposition of the sample Normal urine is a clear straw-colored liquid, with a slight odor. It is affected by diet, concentration, Ds, drugs, and Physical activity. It contains some crystals, a small number of cells from the tissues that line the bladder, and transparent (hyaline) casts. Normal urine does not contain sugars, yeast cells, protein, ketones, bacteria, or parasitic organisms. pH The pH of normal urine is 4.5–8.0. average is 6.0 Acidic urine is associated with xanthine, cystine, uric acid, calcium oxalate stones Alkaline urine is associated with calcium carbonate, calcium phosphate, magnesium phosphate stones. Increased Ph ↑ than 7 (alkaline) Decrease Ph ↓ 7 (more acid) Resp/Met alkalosis Resp/Metabolic acidosis Vegan diet Diabetes Mellitus Vomiting Starvation Diuretics Diarrhea UTI Diet high in meat

5. Abnormal results: CLOUDINESS: Urine may be cloudy because it contains red or white blood cells, bacteria, fat, mucus, or pus from a bladder or kidney infection. ODOR The aromatic odor of normal urine is caused by presence of volatile acid. Foul-smelling urine is a common symptom of urinary-tract infection. Fruity odor/sweet smell of acetone: DM, starvation, dehydration, ketones. Some foods (asparagus), and certain antibiotics can affect the urine odor BLOOD AND TISSUE CELLS Red blood cells in the urine can be due: vigorous exercise or exposure to toxic chemicals. Bloody urine can also be a sign of bleeding in the G/U tract as a result of systemic bleeding disorders, kidney diseases, bacterial infections, obstructions in the urinary tract, traumatic injuries, and tumors. A high number of white blood cells in the urine is a symptom of UTI A large number of cells from tissue lining (epithelial cells) indicate damage to the small tubes that carry material into and out of the kidneys.

6. SPECIFIC GRAVITY (N-1005/1035) Reflects the concentrating capability of kidneys, and hydration status of patient Low specific gravity (below 1.005), more dilute the urine: associated with diabetes insipidus, ATN, pyelonephritis, AGN, overhydration, renal failure (decrease water reabsorption) High specific gravity (above 1.035), more concentrated the urine: patients who are in shock, suffer from nephrotic syndrome, acute GN, CHF, water restriction/dehydration (kidneys reabsorbing water, urine is concentrated), excessive sweating, diarrhea, fever. PROTEIN Proteinuria can be a symptom of kidney stones, inflammation of the kidneys, degenerative kidney disease, or multiple tumors. Findings of more than 1+ protein with SG under 1015  abnormal protein excretion CRYSTALS Some appear in normal urine, such as calcium oxalate or uric acid crystal A large number of calcium oxalate crystals, is a sign of abnormally high levels of calcium in the blood. Other crystals, including tyrosine, leucine, and cholesterol, are abnormal. NITRITES: They are found when nitrate reducing bacteria are present in large numbers. A negative test, does not rule a UTI.

7. BLOOD AND TISSUE CELLS Red blood cells in the urine can be due: vigorous exercise or exposure to toxic chemicals. Presence of more than 5 RBC/HPF in a bloody urine can also be a sign of bleeding in the G/U tract as a result of systemic bleeding disorders, kidney diseases, bacterial infections, obstructions in the urinary tract, traumatic injuries, and tumors. A high number of white blood cells in urine is a symptom of kidney/UTI disease A large number of cells from tissue lining (epithelial cells) indicate damage to the small tubes that carry material into and out of the kidneys. CASTS Formed when protein and other materials settle in the kidney tubules and collecting ducts. Casts are dislodged by normal urine flow. A large number of white blood or red blood cast cells in a urine specimen is a sign of kidney disease. LEUKOCYTES ESTERASE: Produced by the granulocytic blood cells during an infection Presence of 5 or more leukocytes (WBC) per high power field may indicate UTI, interstitial cystitis, calculous disease, and glomerulonephritis.

8. SUGARS A high level of glucose and other sugars in the urine (glycosuria) is often a symptom of diabetes mellitus. Glycosuria can also be caused by advanced kidney disease, impaired tubular reabsorption, shock, cancer of the pancreas, Cushing. KETONES The presence of abnormally high numbers of ketones in the urine (ketonuria) usually results from uncontrolled diabetes mellitus. Ketonuria can also be caused by prolonged starvation, or decreased CH intake, Etho, fever UROBILINOGEN Finding urobilinogen in the urine may be a symptom of liver disease caused by the formation of fibrous tissue, medications that damage the liver, or obstructive jaundice. BUN: Blood Urea Nitrogen, is a general indicator of renal function. Is influenced by diet, catabolic state of patient CREATININE: Is a more sensitive indicator of renal function than BUN. Is influenced by muscle mass of patient

9. Urine Color Range from pale yellow (diluted) to amber yellow (concentrated), due to the pigment urochrome. The color indicates the concentration of urine and varies with the specific gravity. Abnormal Urine Colors Red Color: Hematuria/Myoglobinuria: Brownish red color. muscle damage, vigorous exercice Dark Red Urine: bleeding from kidneys Bright Red Urine: bleeding in lower urinary tract Pink diaper syndrome: pinkish color on diaper 2ary to urate crystals in neonates Foods: Berries, beets, food coloring Drugs: Dilantin, phenothiazines, Orange: Pyridium, Rifampin, Urate Crystals Green: Food coloring, Pseudomonas infection Brown/Black: Hemoglobin, Bilirubin, Flagyl, Nitrofurantoin Dark Yellow: Bile pigments (urobilinogen or bilirubin), ↑ Carotene in diet Blue: Methilene blue, Defect in the tryotophan absorption Milky/Cloudy Color: Pus/Bacteria in urine. Also large amount of fat foods

10. MALE GENITALIA: Testes descends into well-formed, pigmented and rugated scrotum, or are in the inguinal canal Urethral normal opening: At center of shaft. Hypospadia / Epyspadia Phymosis Hydrocele Paraphymosis Neonatal cryptorchidism. Balanitis Testicular Torsion (swollen, bluish) In the first week, the urine can assume a red brick color represented by urates, staining the diapers as blood. They disappear in water, being a differential diagnosis with hematuria

11. Phimosis/Paraphimosis Phimosis: Inability to retract the prepuce (foreskin). Normal in children under 3 years. In older males, phimosis may be due to the inflammation at the tip of the foreskin Paraphimosis: Inability to reduce the foreskin due to venous congestion of the foreskin. Can progress to arterial compromise and gangrene S/S: Phimosis can cause urinary retention due to pain or obstruction of the urethra TX: Phimosis: Steroid cream applied to foreskin to loosen the phimotic ring. In chronic phimosis- Circumcision Paraphimosis: Lubrication and compression of the foreskin and glans. In refractory cases, a vertical incision of the ligating band is done.

13. PARAPHYMOSIS

14. BALANOPOSTHITIS-BALANITIS (balanitis, of the glans; posthitis, of the prepuce) Balanitis is an inflammation of the foreskin and head of the penis. Balanitis is usually caused by poor hygiene in uncircumcised men. The inflammation can be due to a bacterial, fungal, or viral infection, caustic soaps, or failure to properly rinse soap off while bathing. Symptoms: redness of foreskin or penis, rashes on the head of the penis, foul-smelling discharge, painful penis and foreskin Treatment: Tx depends on the cause of the balanitis. Infectious balanitis may be treated with ATB or ointments, good hygiene. Circumcision-severe case Complications: Chronic inflammation or infection can: Scar and narrow the opening of the penis (urethral stricture) Painful retraction of the foreskin to expose the tip of the penis( phimosis) Difficulty to reposition the foreskin to cover the head of the penis (PP); swelling can affect the blood supply to the tip of the penis Prevention: Good hygiene

15. BALANITIS

16. HYDROCELE Accumulation of peritoneal fluid in the tunica vaginalis of scrotum, due to obliteration of processus vaginalis At about 28 weeks of gestation, the testicles descend from the developing baby's abdominal cavity into the scrotum. A sac (processus vaginalis) accompanies each testicle, allowing fluid to surround them. In most cases, the sac closes and the fluid is absorbed. If the fluid remains after the sac closes, the condition is known as a noncommunicating hydrocele . Because the sac is closed, fluid can't flow back into the abdomen. Usually the fluid gets absorbed within a year. In some cases, however, the sac remains open. With this condition, known as communicating hydrocele , the sac can change size or, if the scrotal sac is compressed, fluid can flow back into the abdomen. S/S: Non tender swelling of scrotum, round and smooth Distal testicular shadow can be seen by transillumination R/O: Hernia. Hydroceles transilluminates and does not reduce. Hernia are reducible TX: Spontaneous regression in 90% of cases. Surgery- Hydrocelectomy, only after the first year of life, particularly if is communicating hydrocele or inguinal hernia is present at the same time

18. HYDROCELE

21. HYPOSPADIAS Is when the urethral meatus is located below and proximal to its normal position The ventral foreskin is lacking while the dorsal portion has a hood aspect The ventral urethral meatus- in most cases opens on or adjacent to the glans penis, but the opening can be on the shaft or in the perineum. Chordee - is a ventral curvature of the penis shaft, most apparent on erection. Patients with hypospadia should not be circumcised, because the foreskin is necessary for later repair. The ideal age is between 18 and 24 months.

24. HYPOSPADIAS

26. HYPOSPADIAS

28. Epispadias.

29. URINARY TRACT INFECTION Is an infection from the bladder until the kidneys, occurring when bacteria enter the opening of the urethra and multiply in the urinary tract, where can lead to a significant damage Types: Urethritis: Infection of the urethra Cystitis: Bladder infection is by far the most common UTI. Pyelonephritis: Inf. of renal parenchyma and renal pelvis, due to ascending infection from bladder, is due to G- Etiology : Most common: E. coli 75-90%% Less common: Klebsiella sp, Proteus mirabilis, Enterococcus or other G- Uncommonly: staph saprophyticus

33. LAB: Urinalysis: Screening  Dipstick (Nitrite, Leukocyte esterase) Microscopy Presence of nitrite most specific, because bacteria convert nitrate to nitrite in bladder, best detected in morning sample. (nitrate splitting bacteria, specially G-) Leukocytes (leukocyte esterase) dipstick test - Detects esterases released from broken down Leukocytes- pyuria. Urinalysis-5 and up WBC/HPF (bacteria on Gram stain/high power field Pyelonephritis: Leukocytosis with left shift. WBC, WBC casts, RBC, pyuria, Urine Culture + Urine culture: *Midstream urine sample-positive over 100.000 colonies *Catheterized specimen-positive over 10.000 colonies *Urine culture via suprapubic tap (sterile)- Positive  1.000 col. *If positive for UTI, check BUN/Creatinine and BP  renal function *Urine Culture: sterile collection is the Gold Standard for Dx

34. IMAGING: *Renal U/S- Noninvasive evaluation of structural defects. It shows kidney size/shape, but it poorly show ureters,provides no information on function. Diagnose urolithiasis, hydronephrosis, hydroureter, and bladder distention replacing the intravenous pyelogram (IVP) in many cases. *DMSA Renal scan: This study frequently uses technetium Tc 99m dimercaptosuccinic acid (DMSA). Detects tubular damage and scarring and shows the kidney outline, but it does not show the collecting system. *Voiding Cystourethrogram (VCUG): Shows urethral and bladder anatomy and detects vesico-ureteral reflux (VUR). Girls < 5 years with febrile UTI, or males with 2 separate UTI After first positive culture UTI: Renal Ultrasound VCUG initially, later nuclear cystogram Renal Scan: DMSA (R/O scarring, even if negative VUR) Mag – 3 (obstruction)

36. Common Antimicrobial Drugs Used in Pediatric Urinary Tract Infections Drug Dosage and Interval Parenteral Therapy Ampicillin 100 mg/kg/d 12 h (< 1 week) q 6-8 h (> 1 week) Ceftriaxone * 25-50 mg/kg/d q 12-24 h Cefotaxime 150 mg/kg/d q 6-8 h Gentamicin 5 mg/kg/d q 12 h (< 1 week) 7.5 mg/kg/d q 8 h (> 1 week) Oral Therapy Amoxicillin † 45 mg/kg/d q 8 h Augmentin 45 mg/kg/d q 12 h Trimethoprim/ Sulfamethoxazole (Septra ) 8 mg/kg/d TMP, 40 mg/kg/d SMX q 12 h 40/200mg/5 ml suspension Cephalexin (Keflex) 25-50 mg/kg/d q 6 h 125/250 mg suspension/5 ml Cefixime (Suprax) 8 mg/kg/d or 4mg/kg q 12 h 100 mg suspension/5 ml * Should not be used in neonates because of potential biliary pseudolithiasis. If cocci are present in urinary sediment, ampicillin should be added until culture and sensitivities are available.

37. HEMATURIA Define as abnormal number of RBC in urine with more than 3 rbc-hpf. Origin: Sign/symptom Upper tract Lower tract Pain CVA/none suprapubic Dysuria Blood Clots Absent. Only + Common with trauma Cellular cast Common Absent Proteinuria > 150mg/dl Common Absent RBC Distorted Normal

38. CLINICAL AIDS: DISTINGUISHING THE ORIGIN OF GROSS HEMATURIA Test for: Glomerular ExtraRenal * Urine color Brown, tea-colored Present RBC Casts Present Absent Red cell Morphology Dysmorphic/distorted Normal RBC shape Urine Stream Bloody More bloody at initial (distal urethral origin) or at end suggesting trigonitis Proteinuria >2+ NO Is divided in: pre-renal: proteinuria Renal: presence of RBC cast (GN), proteinuria Postrenal: absence of RBC cast and proteinuria: UTI, tumors, trauma, stones Evaluation of Hematuria: U/A with CX, BUN/Creatinine, Complements, ANA, IgA, Renal Biopsy Renal/bladder U/S- Whenever gross hematuria

40. VESICO-URETHERAL REFLUX Is the passage of urine from the bladder to the renal pelvis, secondary to an incompetent valvular mechanism at the uretero-vesical junction Epidemiology: Family factors- 34% found to have VUR. Classification: REFLUX: Grade I- Into a non-dilated distal ureter Grade II- Into the upper collecting system, with normal calyceal and no dilatation Grade III- Mildly dilated/tortuous ureter, with mild blunting of calyceal Grade IV- Grossly dilated ureter, moderate dilatation/tortuosity of ureter. Grade V- Massive reflux with gross ureteral dilatation and tortuosity of ureter S/S: UTI sx, HTN, Abdominal mass secondary, Hydronephrosis, FTT, Enuresis LAB: Voiding cystourethrogram (VCUG)- Gold standard Renal U/S- detects hydronephrosis DMSA renal scan- detects renal scarring DMSA: Dimercaptosuccinic acid

42. TX: Incidence of renal scarring, is proportional to the amount of reflux Most VUR resolves spontaneously grade I-II-III Grade IV-V unlikely to resolve spontaneously  Surgery Deflux- Dextranomer/ Hyaluronic acid copolimer injection- Resolve 88-67% of Grade I-II-III-IV ATB prophylaxis (amoxycillin-2 months) Monitor by VCUG every 12-18 months, urine cultures.

47. GLOMERULONEPHRITIS ( APSGN ) Renal Immune complex disease caused by Group A-B hemolytic streptococcus types 12/49 after 1-3 weeks of a skin/throat infection. characterized by HEMATURIA, PROTEINURIA, HTN, OLIGURIA, EDEMA ( sodium/water retention ) Classification: * Low serum complement level- Systemic diseases -autoimmune disease: systemic Lupus erythematosus * Low serum complement level- Renal diseases -Acute poststreptococcal GN (APSGN) -Membranoproliferative GN types 1, 2, 3 * Normal serum complement level: Renal diseases -IgA nephropathy

49. IgA NEPHROPATHY (Berger’s Disease): Most common glomerular disease worldwide. IgA deposits in the Mesangial layer More common in boys, white>black Hematuria (Berger Ds), mild proteinuria, 10% HTN. Associated with URI No efficient treatment. Daily dose of 4 gr of fish oil-fatty acid (Omega-3) with 400 U/day Vit-E: retard renal function loss. ACE inhibition used in any renal ds where proteinuria and HTN is present Immunosuppressive tx with prednisone and Azathioprine has shown promise in uncontrolled trials and long term treatment (1-2 y) ESRD: 5-10% progress to renal failure in children/adolescents, 40% in adulthood C3 COMPLEMENT is normal

50. Goodspasture Syndrome: Rapidly, progressive disease, presents with anti-GBM antibodies (Glomerular Basement Membrane) in the glomeruli. Affects the lungs and kidneys, often with pulmonary hemorrhage. Presents hemoptysis and nephritic syndrome Tx: Therapy with steroids, cyclophosphamide and azathioprine alone or in combination with plasmapheresis to remove circulating immune complexes. Wegener’s Granulomatosis: Is a systemic vasculitis characterized by nephritis and pulmonary or sinus disease. Antineutrophil cytoplasmic antibody ( ANCA ) may be pathogenic in this Glomerulomephritis. It is a useful marker of disease activity Rapid progression: pulse methylprednisolone is indicated Most cases: prednisone plus cyclophosphamide for 1-2 years

51. LAB: Throat culture positive for B-hemolytic Streptococcus. Urine microscopy: crenated RBC’s , RBC cast., WBC, proteinuria Chemistry: Shows degree of renal failure- Elevation of BUN/ Creatinine Clearance / K Complement C-3 serum level- LOW ASO titer, Streptozyme test or Anti-DNase B: positive and elevated titers in patients with APSGN ESR/CRP: Elevated ECG: assess hyperkalemia CXR: Check pulmonary edema and cardiac size. Renal U/S: to assess size and parenchyma texture RPGN: Rapidly progressing glomerulonephritis: Anti-GBM/ANCA Anti-GBM: Goodpasture Syndrome ANCA: Wegener’s granulomatosis

52. TX: * Diet- restricted Sodium, Fluids, Potassium *Loop Diuretics: Furosemide- Lasix. For volume, BP and K control *Potassium lowering agents: Kayexalate, Insulin *Anti-hypertensive agents: *Vasodilators such as CCB- Nifedipine, lasix *IV hydralazyne, Na nitroprussiate: for severe hypertension treatment * ATB - Penicillin *Immunosuppressive agents: Prednisone, cyclophosphamide  Treat all except APSGN

56. NEPHROSIS: Is a glomerular disorder characterized by: Heavy proteinuria, hypoproteinemia, hypercholesterolemia (hyperlipidemia), Anasarca (Edema) Etiology: Primary or idiopathic(90%)- Minimal Change Nephrotic Syndrome (85%) -Focal segmental glomerulosclerosis (10%) -Mesangial proliferation (5%) Secondary cases(10%) -Associated with systemic ds: SLE, Henoch-Shonlein purpura, IgA GN -Associated with infections/Allergen induced (bee stings, snake bites) -Drug induced ( Penicillamine, Gold, NSAID’S) Other: < 1 year: cong. infection, syphilis, congenital Nephrotic syndrome >10 years: Membrano-proliferative GN or membranous GN is common

58. TX: *Diet- restrict salt and fluids, Rest *Corticoids: Prednisone 2mg/kg/d PO 4 weeks. Later 1,5 mg/kg/d PO 4 wk. Proteinuria is normal  Taper Prednisone for 3-6 months. *After 4 weeks of Steroid Tx or 2 relapses in 6 months  proteinuria still high  Steroid Resistant  renal biopsy  cytotoxic drugs (Clorambucil, Cyclophosphamide) or Immunosuppressants such as Cyclosporine) *Diuretics: Furosemide (Lasix), Spironolactone *ATB- PCN prophylaxis *Pneumovax vaccine

59. Minimal change disease in a 2-year-old boy with nephrotic syndrome. Eyelid edema in any child should prompt the performance of urinalysis

63. SCROTAL SWELLING

64. Proteinuria- Is the presence of protein in the urine. Normal less than 150mg/ 24hours Primary glomerular disorders Tubular: Tubule damage prevents protein reabsorption: Ischemic Tubular Injury, Reflux nephropathy, Interstitial nephritis Glomerular: Damage: results ↑ permeability of glomerular capillary wall Minimal Change Ds.- mc in school age Membranoproliferative GN Membranous GN Messangial proliferative GN Henoch-Shonlein IgA nephropathy SLE Non-pathologic: Orthostatic, Febrile, Exercise proteinuria

65. Secondary disorder: Hereditary: IDDM, Alport Autoimmune: SLE, Goodpasture’s, Wegener’s Infectious: Endocarditis, Heroin, Gold, mercury Neoplastic: Lymphoma, leukemia, Myeloma Types of proteinuria: Overload: Because of high levels in blood stream (Bence-Jones protein) Orthostatic: proteinuria only when erect, none when laying down Tubular: when is generated by renal tubules Glomerular: Glomerular leak of protein S/S: Proteinuria, HTN, Edema, Ascitis, Purpura, Suprapubic/CVA pain LAB: Urine colormetric dipstick: measure urine protein levels: 1+=30mg/dl 2++=100mg/dl 3+++=300mg/dl 4++++=2000mg/dl 24 hour collection of urine protein excretion BUN, creatinine, total protein, albumin, cholesterol Urine culture ASO titer Creatinine clearance Urine sediment C3, C4, ANA

67. ENURESIS: Involuntary discharge of urine after the age of expected bladder control, between 5-6 years. Epidemiology: Diurnal (small group) more common in females Nocturnal (majority of cases-Bedwetting) 2-3X more in males Urinary Control: Between 3-4 years Primary enuresis: Continence never achieved (never dry) smaller bladder cap. 2 y., less psychological Sx. Secondary enuresis: Incontinence recurs after a dry period of 3-6M . More in females. More psychological Sx. Primary nocturnal enuresis: 20% at 5 years, 10% at 7/8 years, 5% at age 10 years, 1% adults Primary diurnal enuresis: 1% 7-12 years Enuresis Impact: Causes significant child emotional distress, and lower self esteem Embarrassment, Reluctance in participating in overnight activities Cause the family anger and frustration

69. LAB: Urinalysis (glucose), urine culture and microscopic exam  R/O: DM, UTI, Diabetes insipidus If there is Hx of UTI: Renal U/S, Voiding Cystourethrogram If suggestive of neurologic dysfunction: Urodynamic studies TX: Education, Reassurance, Avoid punishment, praise, start chart Decrease fluids at night. Empty bladder before sleeping Desmopressin (DDAVP): ADH- Not to give under 6 years old Decrease overnight urine volume Can be given intranasal. Imipramine: useful, 65-67% of improvement

71. WILMS TUMOR (Nephroblastoma) Malignant tumor of the kidney in the pediatric age. Epidemiology: 5% of all childhood cancer 50% occurs under 3 years old Peak age: 2-4 years Higher incidence in Black female children Associated to congenital anomalies Genetics: 15-20% are hereditary in origin Familial cases are often bilateral and occurring at earlier age S/S: Fever, anorexia, hematuria, abdominal pain/distention, anemia, HTN- 60%, due to obstruction of renal art. P.E.: Abd. mass extending from flank toward midline abd. Is unilateral. Associated with aniridia, cryptorchidism, horseshoe kidney Metastasis to: Lung, liver, bone, brain

72. LAB: CBC, Lytes, Urine analysis (micro/macro hematuria), liver/kidney profile Biopsy and staging of tumor (prognosis depend on staging and Bx.) Imaging: U/S of abdomen CT of abdomen Chest X-Ray: R/O chest metastasis Dx: CT and Bx TX: Surgery- Nephrectomy, sometimes followed by Chemo and Radiation Radiation: for stages III/IV or pulmonary metastasis Chemo: Stages I/II- Vincristine / actinomycin D, every 3 weeks for 6 M Stages III/IV- Vincristine, AMD, doxorubicin for 6-15 months.

73. STAGING: Stage I- Tu. restricted to one kidney and completely resected. Renal capsule intact Stage II- Tumor extends beyond the kidney but is completely excised Stage III- Residual nonhematogenous tumor is confined to the Abd. Stage IV- Hematogenous spread to lungs and liver Stage V- Bilateral disease Prognosis: Favorable: Stage I/II- more than 90% cured Age at presentation less than 24 months Tumor weight less than 250g Poor: Anaplastic pathology Lymph node involvement Distant metastasis

77. Displacement of left renal calyces by Tu.