儿童小肠淋巴管扩张症6例临床分析及营养干预后随访

Clinical analysis and follow-up of intestinal lymphangiectasia in six children receiving medium chain triglycerides therapy

目的探讨儿童小肠淋巴管扩张症的临床表现、诊治特点。方法对2009年11月至2011年8月首都儿科研究所附属儿童医院收治的6例小肠淋巴管扩张症患儿的临床表现、实验室检查、内镜、病理、诊治经过进行回顾性总结分析。结果 6例中男4例,女2例。年龄5个月至10岁。病程15 d至3年。主要临床表现有腹泻、水肿、腹胀、发热、腹腔积液,3例患儿出现生长发育落后,1例患儿病程中出现抽搐,6例患儿淋巴细胞绝对数均偏低,均存在低白蛋白血症,血IgG降低5例。6例患儿均行胃镜检查,其中5例在十二指肠有特征性白色粟粒样改变,6例病理检查显示患儿十二指肠黏膜组织均存在淋巴管扩张,3例行淋巴管造影支持诊断。其中5例予以中链三酰甘油(MCT)饮食后,临床症状明显缓解。结论小肠淋巴管扩张症在儿童中多以腹泻和水肿为主要表现,综合典型临床表现、实验室检查及内镜特点结合病理可明确诊断,通过特殊的饮食可控制疾病,生长发育同正常同龄儿。

Objective To explore the clinical features, diagnostic approaches and management of intestinal ]ympbangi- ectasia（IL） in children. Methods A retrospective analysis of the clinical manifestation, laboratory test data, endoscop- ic and pathological findings, and treatment was performed in 6 children diagnosed with IL in Capital Institute of Pediat- rics from Nov 2009 to Aug 2011. Results Four of the eases were boys and two were girls, and the range of age was from 5 months to 10 years. The duration of IL was from 15 days to 3 years. The main clinical manifestations included diar- rhea, edema, abdominal distention, fever and ascites. Three children growed up slowly, and convulsion was found in 1 child. The lymphocyte count in blood decreased in 6 children; Hypoproteinemia was found in all children , 5 children with IgG loss. Gastroscopy examination were performed in all 6 children; 5 cases revealed characteristic whitish miliary changes. Pathological observation revealed the presence of the dilated lymphatic vessels of the small intestine mucosa in 6 children; 3 cases were diagnosed through the lymphogram. Clinical symptoms were relieved obviously in 5 cases receiv- ing MCT therapy. Conclusion The main clinical manifestations of IL in children are diarrhea and edema. The typical clinical manifestation, laboratory test data, endoscopic and pathological findings suggest the diagnosis of IL definitely. Special dietry is important in the treatment of the IL children. Growth and development are the the same with the normal age children.