Proton-Pump Inhibition of Gastric Chloride Secretion in Congenital Chloridorrhea

• Published in: The New England journal of medicine Vol. 336; no. 2; pp. 106 - 109
• Main Authors: Aichbichler, Berendt W, Zerr, Charles H, Santa Ana, Carol A, Porter, Jack L, Fordtran, John S
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 09.01.1997
• Subjects: Adult; Biological and medical sciences; Chlorides - analysis; Chlorides - metabolism; Chronic Disease; Diarrhea; Diarrhea - complications; Diarrhea - congenital; Diarrhea - drug therapy; Digestive system; Feces - chemistry; Gastric Mucosa - secretion; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Hypokalemia - drug therapy; Hypokalemia - etiology; Intestinal Absorption; Male; Medical disorders; Medical procedures; Medical research; Medical sciences; Omeprazole - pharmacology; Omeprazole - therapeutic use; Other diseases. Semiology; Proton Pump Inhibitors; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Human; Hydroelectrolytic balance disorder; Diarrhea; Male; Congenital disease; Inorganic element; Proton pump inhibitor; Case study; Metabolic disorder; Treatment; Chlorides; Digestive diseases; Intestinal disease; Adult; Hypokaliemia
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJM199701093360205

In 1945 Gamble et al. 1 and Darrow 2 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 . . .