A CASE OF CYSTIC FIBROSIS OF THE PANCREAS ASSOCIATED WITH CHRONIC PULMONARY DISEASE AND CIRRHOSIS OF THE LIVER

Abstract

A case report of a n-yr.-old boy who had steatorrhea since shortly after birth, chronic cough and dyspnoea on effort since the age of 10 yrs., and death at the age of 17 yrs. as a result of bronchopneumonia and lung abscesses due to infection by Staphylococcus aureus hemolyticus and Streptococcus hemolyticus. Post-mortem examination revealed almost complete replacement of the acinar elements of the pancreas by fat and fibrous tissue, pulmonary emphysema, chronic pulmonary suppuration with squamous metaplasia of the bronchial epithelium, bronchopneumonia and cirrhosis of the liver. It is suggested that the cirrhosis of the liver may have resulted from prolonged fatty infiltration of the liver which, in turn, may be an effect of the loss of the exocrine secretion of the pancreas. The common association of pulmonary suppuration and emphysema with cystic fibrosis of the pancreas has not been explained. S. Farber has postulated that in this disease the body secretions are too viscid, and that tenacious secretions in the bronchi may lead to partial obstruction, rendering them prone to infection.