The renal lesion in congenital chloride diarrhea.

Congenital chloride diarrhea is an inherited defect of active intestinal Cl- transport which results in a large wastage of electrolytes and water. The effects of this disease and of replacement therapy on renal histology, function, growth, and the renin-angiotensin-aldosterone system were studied in 18 patients. When the patients were given KCl supplement, histologic findings included juxtaglomerular hyperplasia hyalinized glomeruli, calcifications, and arteriolar changes. Renal function and growth were reduced, and the hormonal activities were high. These abnormalities were evidently due to chronic dehydration. The dehydration could be corrected by increasing the dose of KCl, but only the addition of NaCl corrected the hyperaldosteronism. Adequate replacement therapy prevented the renal involvement.