Tift W L, Lloyd J K
Arch Dis Child. 1975 Apr;50(4):269-76. doi: 10.1136/adc.50.4.269.
The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from dietary treatment is provided by symptomatic relief while on the diet, clinical relapse upon relaxation of t,e regimen, and improvement in growth rates. In most patients the underlying lymphatic defect, and thus the need for dietary treatment, appears to be permanent.
本文描述了6例原发性肠淋巴管扩张症患儿的临床病程,这些患儿接受低脂中链甘油三酯补充饮食治疗3至8年(4例治疗时间超过5年)。尽管实验室检查结果表明乳糜漏仍在持续,但饮食治疗带来的长期益处体现在饮食期间症状缓解、饮食方案放宽后临床复发以及生长速率改善。在大多数患者中,潜在的淋巴缺陷以及饮食治疗的必要性似乎是永久性的。