Familial juvenile polyposis of the stomach.

Innumerable polyps of the stomach were recognized in a 13-yr old girl. She had no extragastric polyps on roentgenographic and endoscopic studies. Her elder brother received a subtotal gastrectomy because of gastric polyposis at 14 yr of age. Their mother died of gastric cancer at 37 yr of age. Only these three subjects in this family had unusual brown hair and were low in normal intelligence. Polyps produced chronic and severe loss of both blood and protein, which resolved after a subtotal gastrectomy at 18 yr of age. Macroscopic and histologic observations of polyps in the resected stomach confirmed the diagnosis of juvenile polyposis. Both siblings are now in good health. Classification of this hereditary syndrome as a newly recognized entity, familial juvenile polyposis of the stomach, is proposed.