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黏附性小肠梗阻作为家族性地中海热的一种具有挑战性的并发症:基于病例的综述。

Adhesive small-bowel obstruction as a challenging complication of familial Mediterranean fever: A case-based review.

机构信息

Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.

Department of Pediatric Surgery, Gazi University Faculty of Medicine, Ankara, Turkey.

出版信息

Int J Rheum Dis. 2024 Jan;27(1):e14867. doi: 10.1111/1756-185X.14867. Epub 2023 Aug 14.

Abstract

Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self-limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis. Physicians are generally aware of other complications associated with FMF but reports on peritoneal adhesions and intestinal obstruction in English-language literature are inadequate to increase clinicians' awareness. Therefore, it is crucial to meticulously evaluate FMF patients presenting with abdominal pain and ileus because these symptoms could be due to adhesive small-bowel obstruction (ASBO). Furthermore, patients presenting with ASBO without a history of abdominal surgery should also be thoroughly evaluated, especially as it could be an initial presentation for an autoinflammatory disease. Herein, we present a pediatric case of FMF with the M694V homozygous mutation, complicated by ASBO while under colchicine treatment. Additionally, we provide a comprehensive review of the available literature on ASBO in FMF.

摘要

家族性地中海热(FMF)是最常见的遗传性自身炎症性疾病,其特征是反复发作和自限性发热和浆膜炎。反复发作的浆膜炎偶尔会导致腹膜粘连,这可能导致机械性肠梗阻。腹痛和呕吐等症状可能类似于典型的 FMF 发作,导致误诊和严重的发病率,包括绞窄和肠坏死。医生通常了解与 FMF 相关的其他并发症,但英语文献中关于腹膜粘连和肠梗阻的报道不足以提高临床医生的认识。因此,仔细评估出现腹痛和肠梗阻的 FMF 患者至关重要,因为这些症状可能是由于粘连性小肠梗阻(ASBO)引起的。此外,没有腹部手术史但出现 ASBO 的患者也应进行彻底评估,尤其是因为这可能是自身炎症性疾病的初始表现。本文介绍了一例儿童 FMF 病例,该病例携带 M694V 纯合突变,在秋水仙碱治疗期间并发 ASBO。此外,我们还对 FMF 中 ASBO 的现有文献进行了全面回顾。

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