• The characteristic lesions of fibrocystic disease in the pancreas involve the exocrine structures, and far-reaching nutritional disturbances follow the loss of the external pancreatic secretion. The endocrine structures remain intact, and the islands of Langerhans continue to produce internal secretion.
A study of 325 cases showed that the disease is really systemic; it is not limited to the pancreas but involves exocrine glands in many other parts of the body. Focal necrosis with biliary obstruction is seen in the liver, the sweat glands secrete abnormally high concentrations of electrolytes, and in nearly all cases there is pulmonary disease characterized by abnormal production of mucus and obstruction of bronchi.
There were 145 deaths in this series, and 130 were ascribed to the pulmonary involvement. The abnormally high losses of sodium chloride in the sweat during hot weather may also be fatal. Progress in the diagnosis and treatment of this important disease depends on greater recognition of its systemic character and its importance in the pathogenesis of meconium ileus, chronic pulmonary disease, cirrhosis of the liver, and portal hypertension in children.
di Sant'Agnese PA. FIBROCYSTIC DISEASE OF THE PANCREAS, A GENERALIZED DISEASE OF EXOCRINE GLANDS. JAMA. 1956;160(10):846–853. doi:10.1001/jama.1956.02960450028007
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