Abstract
A case of medullary cystic disease of the kidney with uremia and salt-wasting, treated by nephrectomy and renal transplantation at the age of 14 years, was studied by light microscopy and scanning and transmission electron microscopy. The results confirm previous findings of excessive basement membrane production around renal tubules and cysts, free communication between cysts and nondilated tubules, and a corticomedullary inflammatory infiltrate unlike that of pyelonephritis. The epithelial cells lining the cysts were not typical of those of any one particular segment of the nephron. A finding not previously reported is the presence of numerous intracytoplasmic vesicles and dilated intercellular spaces among the cells lining the cysts, suggesting that abnormally active fluid transport plays a role in the salt-losing syndrome of this disorder.
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