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Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants

机译:在慢性胰腺炎的儿童中胰腺癌瘘,含有复合棉塞1型变种

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Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication. A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of SPINK1. We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches. PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy.
机译:胰腺炎瘘(PPF)是慢性胰腺炎(CP)的罕见并发症,其需要在患有胸腔积液的患者中患有患者的临床怀疑的高指标。通过放射性成像从胰腺导管从胰腺导管的瘘管暗成这种并发症的确认。一个9岁的男孩,呈现出巨大的右胸腔积液,第二次呈现为ppf,来自涉及Spink1的两个突变的基因突变的CP并发症。通过内窥镜胰管支架在保守治疗方法失败后,我们成功地管理了这种情况。 PPF是所有年龄段的罕见但严重的CP。儿童PPF的诊断需要高指数的临床怀疑,应考虑在存在胰腺病理的大规模胸腔积液的差异诊断中。高水平的胸腔流体淀粉酶和放射性成像的结果当患者有症状在PPF的诊断中发挥基本作用。目前,磁共振胆管胆管扫描(MRCP)是首选的成像模式。内镜治疗和手术是不响应保守治疗的患者的治疗选择。

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