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儿童慢性胰腺炎并发胰胸膜瘘:一例报告及文献复习

Pancreaticopleural fistula in children with chronic pancreatitis: a case report and literature review.

作者信息

Zhang Jia-Yu, Deng Zhao-Hui, Gong Biao

机构信息

Department of Pediatric Digestive Diseases, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China.

Department of Digestive Diseases, Shanghai Shuguang Hospital, Shanghai University of Chinese Medicine, Shanghai, 201203, China.

出版信息

BMC Pediatr. 2020 Jun 3;20(1):274. doi: 10.1186/s12887-020-02174-x.

Abstract

BACKGROUND

Pancreaticopleural fistula (PPF) is a very rare and critical complication of pancreatitis in children. The majority of publications relevant to PPF are case reports. No pooled analyses of PPF cases are available. Little is known about the pathogenesis and optimal therapeutic schedule. The purpose of this study was to identify the pathogenesis and optimal therapeutic schedule of PPF in children.

CASE PRESENTATION

The patient was a 13-year-old girl who suffered from intermittent chest tightness and dyspnea for more than 3 months; she was found to have chronic pancreatitis complicated by PPF. The genetic screening revealed SPINK1 mutation. She was treated with endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic retrograde pancreatic drainage (ERPD); her symptoms improved dramatically after the procedures.

CONCLUSIONS

PPF is a rare pancreatic complication in children and causes significant pulmonary symptoms that can be misdiagnosed frequently. PPF in children is mainly associated with chronic pancreatitis (CP); therefore, we highlight the importance of genetic testing. Endoscopic treatment is recommended when conservative treatment is ineffective.

摘要

背景

胰胸膜瘘(PPF)是儿童胰腺炎一种非常罕见且严重的并发症。大多数与PPF相关的出版物都是病例报告。目前尚无PPF病例的汇总分析。关于其发病机制和最佳治疗方案知之甚少。本研究的目的是确定儿童PPF的发病机制和最佳治疗方案。

病例介绍

该患者是一名13岁女孩,间歇性胸闷、呼吸困难3个多月;被发现患有慢性胰腺炎并发PPF。基因筛查显示SPINK1突变。她接受了内镜逆行胰胆管造影(ERCP)和内镜逆行胰管引流(ERPD)治疗;治疗后症状明显改善。

结论

PPF是儿童罕见的胰腺并发症,可导致严重的肺部症状,常被误诊。儿童PPF主要与慢性胰腺炎(CP)相关;因此,我们强调基因检测的重要性。保守治疗无效时建议内镜治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8563/7268358/97a6d6020823/12887_2020_2174_Fig1_HTML.jpg

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