PEDIATRICS Vol. 4 No. 1 July 1949, pp. 53-69
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THE PATHOGENESIS OF FIBROCYSTIC DISEASE OF THE PANCREAS

A Study of 36 Cases with Special Reference to the Pulmonary Lesions

WOLF W. ZUELZER M.D.1 and WILLIAM A. NEWTON JR. M.D.1

1 The Department of Laboratories, Children's Hospital of Michigan, and the Departments of Pediatrics and Pathology, Wayne University College of Medicine, Detroit, Mich.

Pathologic studies and clinical data from 36 fatal cases of fibrocystic disease of the pancreas support the concept that the basic pathologic process consists in an anomaly of secretory function and frequently involves other glandular structures besides the pancreas—notably the respiratory tract, the intestinal tract and the biliary system.

The development of fixed organic lesions in the affected organs was found to be variable and to depend to some extent on the time factor. Extrapancreatic lesions were rare and inconspicuous in infants dying in the newborn period, whereas, in patients who had survived the newborn period, involvement of the respiratory tract was constant and changes in other systems were commonly encountered. Variations in the intensity of the pancreatic changes suggested the possibility of a postnatal onset of the process in some cases.

Inspissation of abnormally viscid meconium in the small intestine was the sole demonstrable cause of complete intestinal obstruction in four of the five patients dying with meconium ileus in the newborn period. It was shown however that inspissation of meconium as part of the disease process can lead to secondary obliteration and organic atresia of the intestine in utero. Evidence was presented to show that the atresia of the cystic duct frequently encountered in older infants is due to a similar process of secondary obliteration.

A detailed study of the pulmonary lesions encountered in 28 patients with respiratory symptoms indicated that the original pathologic process in the lungs is analogous to that in the pancreas and consists in an accumulation of viscid secretion in the air passages. The resulting state of respiratory obstruction is an important component of the disease process which by itself can lead to death. This point was illustrated by a representative case history. The respiratory tract, being, in contrast to the pancreas, accessible to bacterial invaders, usually becomes the seat of secondary infection which is perpetuated in the presence of inadequate drainage from the plugged air passages and leads to progressive inflammatory changes.

No evidence for a primary nutritional factor in the genesis of the pulmonary changes was found. The conclusion was reached that nutritional deficiencies play a secondary though clinically important part in the evolution of the disease process.

Submitted on October 7, 1949




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