Objective: To analyze the clinical manifestations, diagnosis, treatment and prognosis of intestinal lymphangiectasia (IL) in children in order to improve the skills of diagnosis and treatment of IL. Method: Clinical manifestations, laboratory findings, gastroscopic findings, histopathological examinations and lymphatic radionuclide imaging assessments were analyzed retrospectively among 47 IL patients who were hospitalized in the Gastroenterology Department of Beijing Children's Hospital Affiliated to Capital Medical University from June 2007 to December 2015. All patients were followed up by telephone. According to the various causes, the patients were divided into the primary intestinal lymphangiectasia (PIL) group and secondary IL group, and their clinical manifestations were compared by t test, Rank sum test or Chi-square test. Result: In 47 IL patients, there were 38 children (81%) younger than 3 years old. There were 43 PIL patients (91%) and 4 secondary IL patients (9%). Between PIL and secondary IL, there were statistical differences in serum albumin (t=-3.950, P<0.005) , globulin(t=-2.850, P=0.007), age of onset(U=27.000, P=0.024), age at diagnosis(U=29.000, P=0.030) and course of disease(U=26.500, P=0.023), whereas there were no statistical differences in lymphocyte count, IgG, lymphatic radionuclide imaging, histopathology and gender(all P>0.05). Edema (44 cases, 94%), diarrhea (42 cases, 89%), accompanied with infection (35 cases, 74%) and ascites (30 cases, 64%) were the main clinical manifestations. In 47 IL patients, 45 patients were done gastroscopy and histopathological examinations, and there were 31 patients' histopathological examinations(69%) were positive. Forty patients were done lymphatic radionuclide imaging, and there was evidence of protein losing from gut via lymphatic radionuclide imaging in 39 patients(98%). Among 47 patients, 35 patients (74%) were followed up, 32 patients had good prognosis, 2 patient failed to show evidence of improvement, 1 patient died and no patient experienced a relapse till the end of the follow-up. In 35 patients, 28 patients were treated with medium chain triglycerides (MCT) dietary therapy, 26 patients showed improvement in symptoms, and 2 patients had no improvement. Among 35 patients with follow-up, there were 6 patients received surgical treatment, and their symptoms were improved. Conclusion: PIL are the majority of IL in children younger than 3 years old. The main clinical manifestations are edema, diarrhea, accompanied with infection and ascites. For the patients without the evidence of lymphangiectasia from duodenum histopathological examination, further consideration of lymphatic radionuclide imaging, clinical manifestations, and laboratory studies are needed to make a final diagnosis. MCT dietary therapy is the cornerstone of IL medical management.
目的： 分析儿童小肠淋巴管扩张症（IL）的临床表现、诊断、治疗及预后，以提高对儿童IL的诊疗水平。 方法： 对2007年6月至2015年12月在首都医科大学附属北京儿童医院消化科诊断为IL的47例患儿的临床表现、实验室检查、胃镜、组织病理学、淋巴核素显像进行回顾性分析，并对出院后诊疗情况进行随访。按病因分为原发组和继发组，应用t检验、秩和检验或χ(2)检验比较两组的临床表现。 结果： 在47例IL中，<3岁的患儿38例，占81%；原发IL43例（91%），继发IL4例（9%）。原发IL与继发IL相比，血清白蛋白（t=-3.950, P<0.005），球蛋白(t=-2.850, P=0.007)，发病年龄(U=27.000, P=0.024)，诊断时年龄（U=29.000, P=0.030），病程(U=26.500, P=0.023)差异有统计学意义，而淋巴细胞绝对值计数、IgG、淋巴核素显像阳性比例、病理组织学阳性比例及男女分布差异无统计学意义（P均> 0.05）。IL的临床表现主要有水肿44例（94%）、腹泻42例（89%）、合并感染35例（74%）及腹腔积液30例（64%）。47例IL中45例行胃镜及组织病理学，其中31例（69%）组织病理学阳性；40例行淋巴核素显像检查，39例（98%）有阳性表现。47例患儿随访到35例（74%），其中32例患儿预后良好，2例症状无明显缓解，1例死亡，至随访结束无复发病例。28例患儿出院后继续予高中链脂肪酸（MCT）饮食治疗，26例症状明显缓解，2例症状无明显好转。35例中有6例进行了手术治疗，症状均缓解。 结论： 儿童IL以<3岁原发患儿为主。以腹泻、水肿、合并感染及腹腔积液多见。对于十二指肠组织病理学未发现有淋巴管扩张的患儿，需结合核素显像及临床表现、实验室检查做出综合判断。IL多以长期高MCT饮食保守治疗为主。.
Keywords: Child; Follow-up studies; Lymphangiectasis, intestinal.