Cystic fibrosis of the pancreas is a fatal disease of infants and children characterized by a familial tendency, the celiac syndrome, and atypical bronchopneumonia. Prior to 1938 cystic fibrosis was confused with celiac disease but is now known to be one of a number of conditions which produce the symptom complex of the celiac syndrome. This case is reported because of the rareness of the condition.
A schoolgirl, aged 9, was admitted to the Cleveland Clinic Hospital on September 27, 1943.
She was hospitalized elsewhere at 1 year of age for malnutrition and again at the age of 6, when she had a chronic tracheobronchitis with a temperature of 104 F. A tentative diagnosis of celiac syndrome and pancreatic infantilism was made at that time. During the interval between admissions she averaged four stools a day but had eight large, foul, fatty stools when she included fats in her diet. She had a protuberant abdomen and moderate generalized adenopathy. The lungs were clear to percussion and auscultation. Laboratory examination revealed 4,210,000 erythrocytes and 69 per cent hemoglobin. The glucose tolerance curve was normal except for a rather low fasting sugar. Roentgenologic examination revealed pronounced increase in density of the right hilar shadow in the chest, opaque maxillary sinuses, and clouded ethmoid sinuses.
Between her sixth and ninth years the patient averaged one to two stools daily but had constipation and pain just above the umbilicus. Her physician gave her liver injections and recommended a high protein, high carbohydrate. . .