THE PATHOGENESIS OF FIBROCYSTIC DISEASE OF THE PANCREAS

作者：Wolf W. Zuelzer, William A. Newton · 发表于：PEDIATRICS · 年份：1949 · DOI：10.1542/peds.4.1.53 · 被引用次数：137 · 研究领域：Pancreatitis Pathology and Treatment、Pediatric Hepatobiliary Diseases and Treatments、Intestinal Malrotation and Obstruction Disorders

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 ...