Congenital concomitant pathology of the pancreas: cystic fibrosis, pancreatitis, and diabetes

E. Kondratyeva, A. Razumovsky, A. Smirnov, V. V. Kholostova
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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
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胰腺的先天性伴随病理:囊性纤维化、胰腺炎和糖尿病
在本文中,我们报告一例囊性纤维化(CF)的非典型表现,包括梗阻性胰腺炎伴相对胰功能不全,并发胰石症和1型糖尿病(DM1)。有趣的是,患者几乎没有下呼吸道CF的临床表现。首先表现为腹痛并伴有慢性阻塞性胰腺炎。患者仅有DM1的实验室表现,无临床体征。DM1治疗导致孩子体重增加,外部呼吸增加,这使得胰腺炎手术成为可能。我们切除了结石并进行了纵向胰空肠吻合术。手术减轻了疼痛,增加了胰腺的分泌。因此,该病例显示了CF诊断的困难,以及手术治疗阻塞性胰腺炎的有效性和可行性,即使在被认为是遗传性和不治之症的病例中也是如此。关键词:囊性纤维化,胰腺炎,胰腺功能不全,胰石症,1型糖尿病
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来源期刊
Voprosy Prakticheskoi Pediatrii
Voprosy Prakticheskoi Pediatrii Medicine-Pediatrics, Perinatology and Child Health
CiteScore
1.20
自引率
0.00%
发文量
50
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