摘要
回顾性分析1例5岁7月龄原发性小肠淋巴管扩张症(PIL)患儿的临床表现、实验室指标、内镜病理及淋巴核素显像特征,并随访治疗归转。患儿以腹泻、腹胀、手足搐搦起病。实验室检查示重度低白蛋白血症(16.4 g·L^(-1))、低IgG(2.32 g·L^(-1))、淋巴细胞减少(0.82×10^(9)/L)及低钙低镁血症,影像学检查提示多浆膜腔积液,胃肠镜检查见十二指肠及回肠末端黏膜弥漫性白色雪花样物质,病理示淋巴管扩张伴炎细胞浸润。特征性发现^(99) Tc^(m)-DX淋巴核素显像提示左静脉角持续显影,提示胸导管出口梗阻。经低脂高蛋白、高MCT饮食联合白蛋白输注等治疗,7天后白蛋白升至26.8 g·L^(-1),症状显著缓解。儿童原发性小肠淋巴管扩张症的主要临床表现为腹泻、腹胀、水肿、低蛋白血症、电解质紊乱;淋巴核素显像明确了胸导管梗阻这一关键病理机制,为该病的病因诊断提供了可靠的影像学依据。
A retrospective analysis of the clinical manifestations,laboratory indicators,endoscopic pathology,and lymphoscintigraphy characteristics of a 5-year-7-month-old child with Primary Intestinal Lymphangiectasia(PIL)was conducted,along with a follow-up of treatment outcomes.The child presented with symptoms of diarrhea,abdominal bloating,and tetany in hands and feet.Laboratory tests revealed severe hypoalbuminemia(16.4 g·L^(-1)),low IgG(2.32 g·L^(-1)),lymphocytopenia(0.82×10^(9)/L),and hypocalcemia with hypomagnesemia.Imaging studies indicated multiple serous effusions,and gastrointestinal endoscopy showed diffuse white,snowflake-like substances on the mucosa of the duodenum and terminal ileum.Pathology demonstrated lymphatic dilation with inflammatory cell infiltration.Characteristic findings from ^(99)Tc^(m)-DX lymphoscintigraphy indicated persistent imaging of the left venous angle,suggesting obstruction at the thoracic duct outlet.Treatment with a low-fat,high-protein,high-medium-chain triglyceride(MCT)diet combined with albumin infusion led to a significant increase in albumin levels to 26.8 g·L^(-1) after 7 days,with a marked alleviation of symptoms.The main clinical manifestations of primary intestinal lymphangiectasia in children include diarrhea,abdominal bloating,edema,hypoproteinemia,and electrolyte disturbances.Lymphoscintigraphy confirmed the key pathological mechanism of thoracic duct obstruction,providing a reliable imaging basis for the etiological diagnosis of the disease.
作者
方辉
杨翠翠
孙宁宁
周杰新
赵晓峰
方莹
FANG Hui;YANG Cuicui;SUN Ningning;ZHOU Jiexin;ZHAO Xiaofeng;FANG Ying(Department of Pediatrics,Fuyang People's Hospital,Fuyang 236000,Anhui,China;Department of Gastroenterology,Xi′an Children′s Hospital,Xi′an 710003,Shanxi,China)
出处
《临床儿科杂志》
北大核心
2026年第3期248-251,共4页
Journal of Clinical Pediatrics
基金
市重点研发项目临床转化专项(No.FYZDYF2023LCYX027)。
