medullary cystic disease


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medullary cystic disease

a chronic familial disease of the kidney, characterized by the slow onset of uremia. The disease appears in young children or adolescents, who pass large volumes of dilute urine with greater than normal amounts of sodium. Hemodialysis is the usual treatment for the disease as the uremia progresses and becomes severe. See also uremia.

medullary cystic disease

Familial juvenile nephrophthisis, Senior-Loken syndrome Nephrology A group of AD and, less commonly AR, renal diseases characterized by renal cysts located at the corticomedullary junction in a background of scarring; MCD presents as functional tubular defects and Fanconi syndrome associated with azotemia, uremia and high-output renal failure 3-5 yrs after onset Clinical Retarded growth, renal osteodystrophy, tapetoretinal degeneration Lab Salt wasting, ↓ Na+, acidosis with hyperchloremia