DISEASES OF THE LIVER AND PANCREAS

Sodium wasting and tubular acidosis . Consistent with initial injury to the distal tubules and collecting ducts. Progresses to terminal renal failure in 5 to 10 years. High index of suspicion in children or adolescents with otherwise unexplained chronic renal failure, a positive family history, and chronic tubulointerstitial nephritis on biopsy. ................
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