Proton-Pump Inhibition of Gastric Chloride Secretion in Congenital Chloridorrhea

Abstract

In 1945 Gamble et al.1 and Darrow2 reported on two infants with severe congenital diarrhea, in back-to-back articles with identical titles. Both infants had very high stool chloride concentrations, low urinary chloride excretion, hypochloremia, metabolic alkalosis, and hypokalemia. Gamble et al. concluded that the disease was caused by abnormal secretion of chloride into the intestine, and they named it congenital alkalosis with diarrhea. Darrow believed that the primary defect was intestinal chloride malabsorption, but he gave much of the credit for characterizing the disorder to Gamble et al. and used the name they had chosen. Subsequent studies supported Darrow's belief . . .