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目的提高对儿童小肠淋巴管扩张症(IL)的认识。方法对2例IL患儿的临床表现、诊断、治疗及预后等临床资料进行分析。2例患儿除行血液、尿液、腹部B超等检查外,均接受电子小肠镜检查,并取小肠黏膜行病理活检。结果例1,9岁发病,以腹泻、水肿起病。WBC5.80×109L-1,L0.15,清蛋白17g.L-1,IgG2.83g.L-1,IgA0.61g.L-1。小肠镜检查发现自回盲瓣至回肠黏膜弥散性白色结节样隆起,绒毛欠清晰,病理提示黏膜淋巴管扩张症,考虑原发性IL。饮食对症治疗有效,随访情况良好。例2,12岁发病,以双下肢、颜面及眼睑水肿起病。WBC5.60×109L-1,L0.216,清蛋白24g.L-1,IgG1.83g.L-1,IgA0.58g.L-1。小肠镜检查发现空回肠交界以下小肠黏膜散在结节,不平,颜色偏白,病理结果提示部分绒毛顶端可见扩张的淋巴管,考虑先天性心脏病和手术引起的继发性IL。饮食对症治疗效果不理想。结论对于慢性腹泻或反复水肿伴低蛋白血症的患儿要警惕IL的可能,尽早行内镜及病理活检检查以明确诊断,并尽量寻找病因,给予有效治疗,以改善预后。
Objective To improve the awareness of childhood intestinal lymphangiectasis (IL). Methods The clinical data of 2 cases of IL children with clinical manifestations, diagnosis, treatment and prognosis were analyzed. In addition to the 2 cases of children with blood, urine, abdominal ultrasound B, etc., are receiving electronic enteroscopy, and small intestinal mucosa biopsy. The results of 1,9-year-old onset, with diarrhea, edema onset. WBC5.80 109L-1, L0.15, albumin 17g.L-1, IgG 2.83g.L-1, IgA 0.61g.L-1. Small bowel examination found that the ileocecal valve to the ileum mucosa diffuse white nodular lumps, villi less clear pathology prompted mucosal lymphangieasis, consider the primary IL. Symptomatic treatment of diet effective, follow-up in good condition. Case 2, 12-year-old onset, with both lower extremities, facial and eyelid edema onset. WBC5.60 109L-1, L0.216, albumin 24g.L-1, IgG 1.83g.L-1, IgA0.58g.L-1. Small intestine examination found the junction of the ileum small intestine mucosa scattered nodules, uneven, color white, pathological findings suggest that the top of the villi can be seen dilated lymphatic vessels, congenital heart disease and surgery to consider secondary IL. Symptomatic treatment of diet is not satisfactory. Conclusion For children with chronic diarrhea or recurrent edema and hypoalbuminemia, we should be alert to the possibility of IL. Endoscopy and biopsy should be performed as soon as possible to confirm the diagnosis and try our best to find out the cause of the disease, so as to improve the prognosis.