Which one of the following hereditary tubulo-interstitial kidney diseases shows Autosomal Recessive pattern of inheritance?
Nephronophthisis
Nephronophthisis is the classic autosomal recessive hereditary tubulo-interstitial kidney disease and the most common genetic cause of end-stage renal disease in children. In contrast, medullary cystic kidney disease (types 1 and 2) and juvenile hyperuricaemic nephropathy (now grouped as ADTKD) are autosomal dominant.
Which of the following statements are correct regarding anti-glomerular basement membrane disease?
1. It is a rare autoimmune disease which presents as rapidly progressive glomerulonephritis.
2. Antibodies are formed against $α3$ chain of type 4 collagen GBM.
3. The $α3$ chain is expressed in basement membrane of glomerulus and the retina.
4. The disease may be associated with retinal hemorrhage when it is known as Goodpasture syndrome.
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Which of the following are poor prognostic indictators in glomerular disease?
1. Female sex
2. Hypertension
3. Persistent and severe proteinuria
4. Elevated creatinine levels at presentation
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Which of the following are indications for a renal biopsy?
1. Chronic kidney disease of uncertain etiology
2. Nephritic syndrome
3. Uncontrolled severe hypertension
4. Renal transplant dysfunction
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Which of the following drugs are effective in preventing uric acid nephropathy in patients receiving chemotherapy?
1. Probenecid
2. Forced saline diuresis
3. Rasburicase
4. Acetazolamide
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