A distinct case of an 8-year-old female with cyclic neutropenia presenting with C. septicum abdominal sepsis and myonecrosis requiring a bowel resection and leg fasciotomy.

1 例 8 岁女性周期性中性粒细胞减少症 ， 表现为败血症腹壁杆菌败血症和肌坏死 ， 需要进行肠切除和腿部筋膜切开术。

• 发表时间: 2023 Sep
• 来源期刊: J Surg Case Rep
• DOI: 10.1093/jscr/rjad512
• PMID: 37727224
• 文章类型: Case Reports
• 作者列表: Anzinger H,Cadili L,Li A,Barclay A,Hayashi AH

关键词: Clostridium septicum; cyclic neutropenia; myonecrosis; pediatric surgery; pediatrics; sepsis

Abstract

Clostridium septicum is a very rare cause of severe spontaneous pediatric enterocolitis and is often associated with underlying malignancy or immunocompromise. Likewise, cyclic neutropenia is a rare congenital immunodeficiency that is characterized by cyclical periods of neutropenia, often with more severe symptoms in the pediatric population. Here, we present a unique case of spontaneous C. septicum enterocolitis, sepsis, and myonecrosis in a child with undiagnosed cyclic neutropenia. Early recognition of pediatric sepsis, frequent reevaluation and identification of rapidly progressive infection, and early surgical intervention are critical for the effective management of a rare and severe infection.

摘要

败血梭状芽胞杆菌是一种非常罕见的严重自发性小儿小肠结肠炎的病因，常与潜在的恶性肿瘤或免疫功能受损有关。同样，周期性中性粒细胞减少症是一种罕见的先天性免疫缺陷，其特征是周期性的中性粒细胞减少症，通常在儿科人群中症状更严重。这里,我们介绍了一个独特的自发性败血症小肠结肠炎病例，脓毒症,以及未确诊的周期性中性粒细胞减少症患儿的心肌坏死。早期认识到小儿脓毒症，频繁的重新评估和快速进行性感染的识别，早期手术干预对于有效治疗罕见和严重感染至关重要。