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先天性乳糜性腹水:一种采用中链甘油三酯饮食和奥曲肽治疗的婴儿腹水罕见病因。

Congenital Chylous Ascites: A Rare Cause of Infantile Ascites Treated With MCT-Based Diet and Octreotide.

作者信息

Rashid Rafia, Shafi Ahmed Syed, Mahmud Salahuddin

机构信息

From the Department of Paediatric Gastroenterology, Hepatology & Nutrition, Dhaka Shishu (Children) Hospital, Dhaka, Bangladesh.

出版信息

JPGN Rep. 2021 Dec 10;3(1):e149. doi: 10.1097/PG9.0000000000000149. eCollection 2022 Feb.

Abstract

Congenital chylous ascites (CCAs) are a rare disease that results from the accumulation of chylomicron-rich lymphatic fluid within the peritoneal cavity due to maldevelopment of the intra-abdominal lymphatic system. Medium-chain triglyceride (MCT)-based diet, total parenteral nutrition (TPN), and repeated paracentesis are considered supportive management for CCA. Cases unresponsive to conservative treatment usually require surgical intervention. We report a case of CCA in a premature neonate treated successfully with intravenous infusion of octreotide (synthetic somatostatin analog), after failing to respond to supportive therapies. Due to the lack of standards in diagnosis and treatment, this disease constitutes a medical challenge, and individual therapy seems to be noteworthy.

摘要

先天性乳糜腹水(CCAs)是一种罕见疾病,由于腹腔内淋巴系统发育不良,富含乳糜微粒的淋巴液在腹腔内积聚所致。基于中链甘油三酯(MCT)的饮食、全胃肠外营养(TPN)和反复腹腔穿刺术被认为是CCA的支持性治疗方法。对保守治疗无反应的病例通常需要手术干预。我们报告了1例早产新生儿先天性乳糜腹水,在对支持性治疗无反应后,通过静脉输注奥曲肽(合成生长抑素类似物)成功治愈。由于缺乏诊断和治疗标准,这种疾病构成了一项医学挑战,个体化治疗似乎值得关注。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc95/10158395/b0e23a42bd0a/pg9-3-e149-g001.jpg

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