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依维莫司治疗难治性婴儿乳糜性腹水

Refractory Infantyl Chylous Ascites Treatment by Everolimus.

作者信息

Baran Masallah, Kahveci Sinem, Aksoy Betul, Cagan Appak Yeliz, Dogan Gizem, Orsdemir Hortu Hacer, Koyluoglu Gokhan

机构信息

Izmir City Hospital; Izmir Katip Celebi University Faculty of Medicine, Department of Pediatric Gastroenterology, Hepatology and Nutrition, Izmir, Türkiye.

Izmir City Hospital, Clinic of Pediatric Gastroenterology, Hepatology and Nutrition, Izmir, Türkiye.

出版信息

Medeni Med J. 2025 Jun 26;40(2):110-113. doi: 10.4274/MMJ.galenos.2025.82284.

Abstract

The accumulation of lymphatic fluid in the abdomen is defined as chylous ascites. Different causes play a role in the etiology of the disease. Congenital anomalies are the most common cause in pediatric patients. A high protein, low fat diet rich in medium-chain fatty acids should be planned. The first-line treatment is dietary management and is total parenteral nutrition, and the first preferred medical agent is somatostatin. In patients who do not respond to standard treatments, surgical treatment or new limited alternative medical agents are applied. A six-month-old girl presented with a complaint of abdominal swelling was diagnosed with chylous acid. The patient was put on the standard nutritional therapy and somatostatin treatment but did not respond to these treatments. The chylous ascites was controlled with everolimus treatment.

摘要

腹腔内淋巴液的积聚被定义为乳糜性腹水。不同病因在该疾病的发病机制中起作用。先天性异常是儿科患者中最常见的病因。应规划富含中链脂肪酸的高蛋白、低脂饮食。一线治疗是饮食管理和全胃肠外营养,首选的药物是生长抑素。对标准治疗无反应的患者,采用手术治疗或新的有限替代药物。一名6个月大的女童因腹部肿胀就诊,被诊断为乳糜性腹水。该患者接受了标准营养治疗和生长抑素治疗,但对这些治疗无反应。依维莫司治疗控制了乳糜性腹水。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/58bf/12203448/04e4d887b73e/MedeniMedJ-40-2-110-figure-1.jpg

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