Desai A P, Guvenc B H, Carachi R
Department of Pediatric Surgery, King's College Hospital, London, United Kingdom.
Eur J Pediatr Surg. 2009 Aug;19(4):241-5. doi: 10.1055/s-0029-1216389. Epub 2009 May 15.
Primary intestinal lymphangiectasia is an uncommon congenital anomaly. It is an intrinsic abnormality of the intestinal lymphatics system. Over the years, various treatment options such as diuretics, albumin transfusions and a medium chain triglycerides (MCT) diet as well as surgical options such as resection of isolated segments and peritoneal-venous shunts have been used. An MCT diet, which is a low fat, high protein diet, is increasingly used in the management of this anomaly.
The aim was to review the evidence for medium chain triglycerides as a therapeutic option in patients with primary intestinal lymphangiectasia.
A literature search was performed and individual case details were extracted. We found 55 cases, of which 3 were from our own institute. The cases were divided in 2 groups: Group A (n=27) consisted of patients treated with MCT, and Group B (n=28) consisted patients not treated with MCT. Cases were analysed for symptomatic response to MCT as well as mortality.
17 of 27 cases (63%) treated with MCT had complete resolution of symptoms while only 10 of 28 (35.7%) patients in group B showed complete resolution. Mortality for Group A was 1 out of 27 (3.7%), while mortality in group B was 5 of 28 (17.85%) patients.
We conclude that, although an MCT diet is not completely curative in all cases, it does improve the symptoms of primary intestinal lymphangiectasia and reduces mortality. Hence it is a valid option in the paediatric age group.
原发性肠淋巴管扩张症是一种罕见的先天性异常。它是肠道淋巴系统的一种内在异常。多年来，已经采用了各种治疗方法，如利尿剂、白蛋白输注和中链甘油三酯（MCT）饮食，以及手术方法，如孤立节段切除术和腹膜静脉分流术。MCT饮食是一种低脂、高蛋白饮食，越来越多地用于这种异常情况的管理。
目的是综述中链甘油三酯作为原发性肠淋巴管扩张症患者治疗选择的证据。
进行文献检索并提取个体病例细节。我们发现了55例病例，其中3例来自我们自己的研究所。病例分为两组：A组（n = 27）由接受MCT治疗的患者组成，B组（n = 28）由未接受MCT治疗的患者组成。分析病例对MCT的症状反应以及死亡率。
接受MCT治疗的27例病例中有17例（63%）症状完全缓解，而B组28例患者中只有10例（35.7%）症状完全缓解。A组死亡率为27例中的1例（3.7%），而B组死亡率为28例中的5例（17.85%）。
我们得出结论，虽然MCT饮食并非对所有病例都能完全治愈，但它确实能改善原发性肠淋巴管扩张症的症状并降低死亡率。因此，它是儿科年龄组的一种有效选择。
