Bujanover Y, Liebman W M, Goodman J R, Thaler M M
Digestion. 1981;21(2):107-14. doi: 10.1159/000198550.
A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common iliac lymph nodes. Abdominal CT scan revealed a possible periaortic mass. Small intestinal biopsies demonstrated normal villous architecture with dilated lacteals, and electron microscopy revealed enterocytes with normal as well as blunted microville, enlarged, dilated lacteals and intercellular vacuoles. An exploratory laparatomy was performed and revealed no masses but dilated serosal lymphatic vessels. Medical treatment, including marked restriction of long chain triglyceride intake, resulted in loss of peripheral edema, weight stabilization, and normal activity.
一名患有原发性肠淋巴管扩张症的16岁男孩出现了持续6周的外周水肿。实验室和影像学检查包括绝对淋巴细胞减少、低白蛋白血症、脂肪泻、粪便51铬白蛋白值异常、小肠皱襞水肿、肠系膜淋巴管扩张，以及双侧髂外和髂总淋巴结充盈缺损。腹部CT扫描显示主动脉周围可能有肿块。小肠活检显示绒毛结构正常但乳糜管扩张，电子显微镜检查显示肠上皮细胞微绒毛正常以及变钝，乳糜管增大、扩张，细胞间有液泡。进行了剖腹探查术，未发现肿块，但发现浆膜淋巴管扩张。包括严格限制长链甘油三酯摄入在内的医学治疗导致外周水肿消退、体重稳定且活动正常。
