THE CHIEF pathologic changes of fibrocystic disease or fibrosis of the pancreas, according to Andersen1 and others2 are (1) replacement of the acini of the pancreas by epithelium-lined cysts embedded in fibrous tissues but without changes of the islets of Langerhans; (2) bronchitis, bronchiectasis, abscesses, and bronchopneumonia of the lungs; (3) symptoms of vitamin-A deficiency in children who die within the first year of life, and, occasionally, (4) atresia of the small bowel or the cystic or pancreatic ducts. The cause of the pancreatic lesion is not known, but probably it is present at birth.
The symptoms of these patients are comparable to those observed in dogs following ligation of the pancreatic duct and can be ascribed3 to the absence of the external secretion of the pancreas. The symptoms of the disorder in infants and children are related to the gastrointestinal tract and to the respiratory system.
HIRSCH EF, CARBONARO L, BIGGS AD, PHILLIPS FL. POSTPRANDIAL HYPOLIPEMIA OF PANCREATIC FIBROCYSTIC DISEASE: A Diagnostic Test. AMA Am J Dis Child. 1953;86(6):721–725. doi:10.1001/archpedi.1953.02050080736003
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