摘要
目的探讨小肠淋巴管扩张症的临床表现,诊断、治疗及预后等。方法2例患儿除一般的血清学、尿液、超声心动图、腹部B超和腹部CT等检查外,均接受胃镜和(或)小肠镜检查并取黏膜行病理活检。结果例1,5岁发病,以反复水肿起病,小肠镜检查发现空肠、回肠弥漫性白色粟米样改变,病理提示肠黏膜固有膜内及黏膜肌层内可见到较多扩张淋巴管,淋巴管内皮细胞增生不明显。例2,5月龄发病,以水肿、腹泻起病,胃镜检查发现十二指肠黏膜弥漫性白色粟米样改变,正常绒毛结构消失,病理提示个别绒毛黏膜内可见轻度扩张淋巴管。2例患儿均除外引起小肠淋巴管扩张的继发性因素,结合起病年龄小,考虑原发性小肠淋巴管扩张症,其中例1诊断为小肠弥漫性淋巴管扩张。这2例患儿通过中链三酰甘油乳剂治疗后,随访症状明显缓解。结论小肠淋巴管扩张症在儿童中可能并不少见。对于反复低蛋白血症、反复水肿或慢性腹泻的患儿要警惕原发性小肠淋巴管扩张症,应尽早行内镜及病理活检,并通过特殊的饮食控制疾病。
Objective To explore the clinical features,diagnostic approaches and management of congenital intestinal lymphangiectasia through a study of two cases and a review of the literatures.Methods The two patients studied,in addition to regular serological studies,urinalysis,echocardiogram,abdominal ultrasonogram and abdominal CT scan,underwent gastroscopy and/or double balloon enteroscopy（DBE） and small intestine mucosa biopsies were taken for pathological studies.Results Case 1：5-year-old female,presenting with recurrent edema,was diagnosed as suffering from hypoalbuminemia not due to renal,cardiac and hepatic causes after comprehensive workup including serological studies,urinalysis,echocardiogram,abdominal ultrasonogram and liver biopsy.Under gastroscopy,small white dots were seen in the duodenal mucosa.Retrograde DBE was later performed.Throughout the ileum and jejunum,diffuse,generalized white-yellowish nodular changes resembling a cobble-stone appearance were seen.Normal intestinal villous morphology was lost,and patchy areas of superficial hemorrhage were also observed.Pathological observation revealed the presence of dilated lymphatic vessels without evidence of endothelial hyperplasia in the lamina propria and muscularis mucosae,and diagnosis of intestinal lymphangiectasia was made.Case 2：6-month-old female baby presenting with edema and diarrhea and hypoalbuminemia not due to extra-intestinal causes.Gastroscopy examination revealed diffuse whitish military changes in the duodenal mucosa,with patchy superficial mucosal hemorrhage and loss of normal villous appearance.Pathological observation revealed dilatation of the lymphatic vessels in the lamina propria and interstitial edema in individual villi,and intestinal lymphangiectasia was suggested.Considering the young age of the patients,the diagnosis of primary intestinal lymphangiectasia was made.In both patients,dietary treatment with medium-chain triglyceride supplementation（plain MCT or emulsion） to substitute for long-chain fat was initiated.D
出处
《中国循证儿科杂志》
CSCD
2007年第6期434-437,共4页
Chinese Journal of Evidence Based Pediatrics
