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原发性肠淋巴管扩张症婴儿的营养治疗及效果评估:病例报告

Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports.

作者信息

Li Suyun, Liu Xiaoqian, He Yuan, Li Qianyu, Ji Linlin, Shen Wenbin, Tong Guansheng

机构信息

Department of Nutrition, Beijing Shijitan Hospital, Capital Medical University Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University Department of Nuclear Medicine, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

出版信息

Medicine (Baltimore). 2017 Dec;96(51):e9240. doi: 10.1097/MD.0000000000009240.

DOI:10.1097/MD.0000000000009240
PMID:29390480
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5758182/
Abstract

RATIONALE

Intestinal lymphangiectasia (IL) is a rare enteropathy involving the expansion and rupture of intestinal lymphatic channels. Although several reports have studied cases of primary IL (PIL), this condition is very rare, and is even less commonly encountered in infants. This study aimed to investigate the nutritional therapy and effect assessment of chylous reflux disorder caused by PIL in infants.

PATIENT CONCERNS

Infantile patients were enrolled in the Affiliated Beijing Shijitan Hospital of the Capital Medical University between January 2012 and March 2014. The minimum age of onset was 4 months and the maximum age of onset was 16 months, with an average age of 4.9 months.

DIAGNOSES

All children were inpatient who had been diagnosed with chylous reflux syndrome (chylothorax and/or chylic abdomen) caused by PIL.

INTERVENTIONS

Retrospective analysis and individualized nutrition therapy of these cases were carried out. Finally, nutritional therapy and prognosis of PIL were assessed and summarized.

OUTCOMES

All the children survived, showed improvement in the serum total protein, albumin, and HGB levels after nutritional therapy. After comprehensive nutritional therapy, we were able to achieve diarrhea control for all the 9 patients, and after treatment, the children passed soft, yellow stools 1 to 2 times/d. After treatment, the height and weight of all patients increased to within the normal ranges of the World Health Organization standard chart. The mean serum albumin level reached 41.3 g/L. All nutrition-related indicators were found to have significant improvement compared with the baseline levels.

LESSONS

The results revealed that nutritional therapy for the 9 children with PIL was effective, and it may be able to improve the clinical syndromes and symptoms of children with PIL and promote recovery.

摘要

理由

肠淋巴管扩张症(IL)是一种罕见的肠病,涉及肠道淋巴管的扩张和破裂。尽管有几份报告研究了原发性IL(PIL)病例,但这种情况非常罕见,在婴儿中更不常见。本研究旨在探讨婴儿PIL所致乳糜反流性疾病的营养治疗及效果评估。

患者情况

2012年1月至2014年3月,首都医科大学附属北京世纪坛医院收治了婴儿患者。发病最小年龄为4个月,最大年龄为16个月,平均年龄为4.9个月。

诊断

所有儿童均为住院患者,已被诊断为由PIL引起的乳糜反流综合征(乳糜胸和/或乳糜腹)。

干预措施

对这些病例进行回顾性分析和个体化营养治疗。最后,对PIL的营养治疗和预后进行评估和总结。

结果

所有儿童均存活,营养治疗后血清总蛋白、白蛋白和血红蛋白水平有所改善。经过综合营养治疗,9例患者均实现了腹泻控制,治疗后患儿每天排1至2次软黄便。治疗后,所有患者的身高和体重均增加至世界卫生组织标准图表的正常范围内。平均血清白蛋白水平达到41.3g/L。与基线水平相比,所有营养相关指标均有显著改善。

经验教训

结果显示,对9例PIL患儿的营养治疗是有效的,它可能能够改善PIL患儿的临床综合征和症状,并促进康复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b98/5758182/09f0618feca2/medi-96-e9240-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b98/5758182/41c0e9264773/medi-96-e9240-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b98/5758182/09f0618feca2/medi-96-e9240-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b98/5758182/41c0e9264773/medi-96-e9240-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b98/5758182/09f0618feca2/medi-96-e9240-g003.jpg

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