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粘连性小肠梗阻合并家族性地中海热:病例系列及文献综述

Adhesive small bowel obstruction with familial Mediterranean fever, case series and literature review.

作者信息

El-Mefleh Naser, Nakwan Aya

机构信息

Department of Pediatric Surgery, National Hospital, Aleppo, Syria; Department of Pediatric Surgery, Deva Hospital, Aleppo, Syria; Faculty of Medicine, Aleppo University, Aleppo. Syria.

Faculty of Medicine, Aleppo University, Aleppo. Syria.

出版信息

Int J Surg Case Rep. 2025 Aug 19;135:111823. doi: 10.1016/j.ijscr.2025.111823.

Abstract

INTRODUCTION AND IMPORTANCE

Familial Mediterranean Fever (FMF) manifests in 90 % of patients as recurrent episodes of peritoneal inflammation, mimicking an acute surgical abdomen. These attacks typically resolve spontaneously within 72 h. However, recurrent peritonitis can lead to primary intraperitoneal adhesions even in the absence of prior surgery-a rare but serious complication of FMF that may result in bowel strangulation or volvulus. Early recognition is crucial to prevent life-threatening complications.

CASE PRESENTATION

We present three pediatric cases of FMF complicated by primary adhesive small bowel obstruction (PASBO). Two patients had a prior FMF diagnosis, while the third was diagnosed postoperatively based on surgical findings.

CLINICAL DISCUSSION

All three patients required surgical intervention for PASBO, with varying degrees of adhesion severity. Early recognition of PASBO in FMF patients is essential to prevent complications such as bowel necrosis.

CONCLUSION

PASBO should be considered in FMF patients with persistent obstructive symptoms or atypical abdominal pain. Conversely, PASBO in patients with a history of recurrent abdominal pain should raise suspicion for undiagnosed FMF. Increased awareness among pediatric surgeons, gastroenterologists, and pediatricians is critical for timely intervention.

摘要

引言与重要性

家族性地中海热(FMF)在90%的患者中表现为反复的腹膜炎发作,类似于急腹症。这些发作通常在72小时内自行缓解。然而,即使没有既往手术史,反复的腹膜炎也可能导致原发性腹腔内粘连——这是FMF一种罕见但严重的并发症,可能导致肠绞窄或肠扭转。早期识别对于预防危及生命的并发症至关重要。

病例报告

我们报告3例FMF并发原发性粘连性小肠梗阻(PASBO)的儿科病例。2例患者既往已诊断为FMF,而第3例根据手术发现术后确诊。

临床讨论

所有3例患者均因PASBO需要手术干预,粘连严重程度各不相同。FMF患者中早期识别PASBO对于预防肠坏死等并发症至关重要。

结论

对于有持续梗阻症状或非典型腹痛的FMF患者,应考虑PASBO。相反,有反复腹痛病史的患者出现PASBO应怀疑未诊断的FMF。小儿外科医生、胃肠病学家和儿科医生提高认识对于及时干预至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb7f/12397809/fa615b63c873/gr1.jpg

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