Генетически детерминированная сочетанная патология поджелудочной железы: муковисцидоз, панкреатит, диабет

Abstract

In this article, we report a case of cystic fibrosis (CF) with untypical manifestations, including obstructive pancreatitis with relative pancreatic insufficiency, complicated by pancreatolithiasis and type 1 diabetes mellitus (DM1). Interestingly, the patient had almost none clinical manifestations of CF in the lower respiratory tract. The first manifestation was abdominal pain associated with chronic obstructive pancreatitis. The patient had only laboratory manifestations of DM1 without any clinical signs. DM1 treatment led to an increase in the child's body weight and increased external respiration, which allowed an operation for pancreatitis. We removed calculi and performed longitudinal pancreaticojejunostomy. The surgery alleviated pain and increased the external secretion of the pancreas. Thus, this case demonstrates difficulties associated with CF diagnosis, as well as effectiveness and feasibility of surgical treatment for obstructive pancreatitis, even in cases when it is believed to be an inherited and incurable disease. Key words: cystic fibrosis, pancreatitis, pancreatic insufficiency, pancreatolithiasis, type 1 diabetes mellitus