Pathologic lesions in cystic fibrosis of the pancreas

Abstract

<h2>Summary</h2> The autopsy findings of twenty case examples of fibrocystic disease of the pancreas are reviewed, and the pertinent data is recorded in chart form. A short résumé of the literature on this disease is included with an emphasis on the desirability of using the term celiac syndrome instead of the designation of celiac disease. There were twenty cases of fibrocystic disease of the pancreas encountered in 363 autopsies from a total of 10,775 admissions to the Pediatric Service of the University of California Hospital. It is noted that there is no apparent correlation between the microscopic appearance of the pancreas and the severity of the clinical syndrome. This suggests that factors of absorption, enteric insufficiency and anomalous function, etc., contribute to the development of the clinical picture. Therapeutic efforts have been consistently disappointing, even in the clinically early cases, and autopsies of patients treated by the newest regimen have presented essentially the same morphological lesions as in untreated cases. This is a disease often involving many glandular organs, besides the pancreas and lungs. Liver anomalies, salivary gland lesions, and cystic changes in t the intestinal mucous membrane may be encountered. Simple secondary bacterial infection must be a factor in the progress of the disease, especially in the lungs. A more fundamental factor, however, is probably at work. Several possibilities exist, but current studies directed toward the elucidation of the chemical and enzymatic details of mucin production seem especially pertinent.