Males with Alport syndrome commonly develop end-stage renal failure in the 2nd or 3rd decade of life, occasionally in association with hearing loss. Females usually have a normal life span and only subclinical hearing loss.
Idiopathic Familial Benign Haematuria
All investigations normal
Urine test of the parents and siblings
An excellent prognosis, but long-term follow-up is required to exclude Alport syndrome
RGH, persistent microscopic hematuria, or dysuria in the absence of stone formation
Hypercalciuria (without hypercalcemia)
Diagnosis: 24-hr urinary calcium excretion exceeding 4 mg/kg, urine calcium to creatinine ratio (mg/mg)
Hypercalciuria may lead to nephrolithiasis
RX: Oral thiazide
Uncommon in childhood and a rare cause of haematuria.
The most common cause of nephrotic syndrome in adults.
Associated with systemic lupus erythematosus, cancer, gold or penicillamine therapy, and syphilis and hepatitis B virus infections.
Nephritis with rapid progression to end-stage renal failure
Causes : poststreptococcal, lupus, membranoproliferative, and the glomerulonephritides of Goodpasture disease, anaphylactoid purpura, and other forms of vasculitis
Acute renal failure, often after an acute nephritic or nephrotic episode
Diagnosis: Renal biopsy
Paediatric Nephrology Emergency
Acute Renal Failure
Develops when renal function is diminished to the point at which body fluid homeostasis can no longer be maintained.
Oliguria (daily urine volume less than 400 ml/m2) is common, the urine volume may approximate normal.
Nonoliguric renal failure : in certain types of acute renal failure (aminoglycoside nephrotoxicity).
Hypovolemia, hypotension, hypoxia
Acute tubular necrosis
Acute interstitial nephritis
Localized intravascular coagulation
Obstructive uropathy, stone, blood clot
Diminished urine output
Oedema (salt and water overload)
Hypertension, vomiting, and lethargy (uremic encephalopathy).
Complications of acute renal failure: volume overload with congestive heart failure and pulmonary edema, arrhythmias, gastrointestinal bleeding due to stress ulcers or gastritis, seizures, coma, and behavioral change
Life threatening: GIT bleed, pericarditis and encephalopathy
Investigation: CBC, urea and electrolytes, PO4, Ca, blood gases, C3, US kidneys, urine electrolytes ( Na and creatinine), fractional excretion of Na (less than 1% in hypovalaemia)
Hyperkalemia: no potassium-containing fluid, foods, or medications until adequate renal function is re-established
> 7 mEq/L (mmol/L): Nebulised salbutamul, IV Calcium gluconate, sodium bicarbonate, ca resonium, glucose and insulin
Moderate acidosis is common in renal failure: Na bicarbonate
Hypocalcemia and hyperphosphataemia: Ca binders (Ca carbonate).
The primary disease process (nifedipine, diazoxide, sodium nitroprusside or labetalol as a continuous intravenous infusion is indicated for hypertensive crises).
Expansion of the extracellular fluid volume (salt and water restriction is critical).
Indications for dialysis: fluid overload, and congestive heart failure, electrolyte abnormalities (especially hyperkalemia), central nervous system disturbances, hypertension.
In general, recovery of function is likely following renal failure resulting from prerenal causes, the hemolytic-uremic syndrome, acute tubular necrosis, acute interstitial nephritis, or uric acid nephropathy.
On the other hand, recovery of renal function is unusual when renal failure results from most types of rapidly progressive glomerulonephritis, bilateral renal vein thrombosis, or bilateral cortical necrosis.
Chronic Renal Failure
In children under 5 yr of age is commonly the result of anatomic abnormalities (hypoplasia, dysplasia, obstruction, malformations)
After 5 yr of age acquired glomerular diseases (glomerulonephritis, hemolytic-uremic syndrome) or hereditary disorders (Alport syndrome, cystic disease) predominate
UT malformation + Glomerulonephritis + Pyelonephritis….. > 50% of causes
BUN and creatinine (nitrogen accumulation and level of renal function).
hypocalcemia, hyperphosphatemia, osteodystrophy.
High of intact parathyroid hormone levels.
MANAGAMENT depends upon the degree of renal insufficiency (CRD) STAGE of CKD GFR ML/MINUTE/1.73M 2 FEATURES 1 : chronic changes with normal GFR 90-120 None 2 :Impaired RF (mild) 60-89 None 3 : Moderate 30-59 None, short stature, PTH 4 : Severe (pre-terminal) 15-29 Acidosis, anaemia, BP, lethargy, etc 5 :ESRF < 15% Dialysis or RX
Diet in chronic renal failure.
Water and electrolyte management in chronic renal failure (fluid, K, Na).
Acidosis in chronic renal failure.
Anemia in chronic renal failure.
Hypertension in chronic renal failure.
Drug dosage in chronic renal failure.
End-stage Renal Failure
Dialysis is generally initiated when the patient's GFR < 15 ml/1.73m2/minute
Continuous ambulatory peritoneal dialysis (CAPD)
Continuous cyclic peritoneal dialysis (CCPD)
Peritoneal cavity- has a semipermeable membrane that surrounds intestine and other organs in abdominal cavity.
CAPD : dialysate flows in, dwells, then drain; this process is repeated 4-5 times