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儿童以难治性腹泻和低白蛋白血症为首发表现的朗格汉斯细胞组织细胞增生症:两例报告及文献复习。

Langerhans cell histiocytosis in children with refractory diarrhoea and hypoalbuminaemia as the initial presentation: two case reports and a literature review.

机构信息

Division of Pediatric Gastroenterology and Nutrition, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, No. 1665, Kongjiang Rd, Shanghai, China.

Shanghai Institute for Pediatric Research, Shanghai, China.

出版信息

BMC Pediatr. 2024 Mar 22;24(1):203. doi: 10.1186/s12887-024-04612-6.

Abstract

Langerhans cell histiocytosis (LCH) involving the gastrointestinal tract is a rare condition for which clinical experience is limited. We describe the cases of two patients who initially presented with chronic diarrhoea, hypoproteinaemia, and intermittent fever. These findings suggest that in cases of refractory diarrhoea accompanied by recurrent hypoalbuminaemia, especially with abdominal rash, LCH should be considered. Gastrointestinal endoscopy, biopsy, and imaging studies are essential for obtaining a definitive diagnosis. This approach might be helpful for the early recognition of gastrointestinal tract involvement in LCH.

摘要

郎格汉斯细胞组织细胞增生症(LCH)累及胃肠道的情况较为罕见,临床经验有限。我们描述了两名最初表现为慢性腹泻、低蛋白血症和间歇性发热的患者的病例。这些发现表明,在伴有复发性低白蛋白血症的难治性腹泻的情况下,特别是伴有腹部皮疹的情况下,应考虑 LCH。胃肠道内镜检查、活检和影像学研究对于明确诊断至关重要。这种方法可能有助于早期识别 LCH 中胃肠道受累。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5de9/10958846/fc5a037508a6/12887_2024_4612_Fig1_HTML.jpg

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