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A girl with protein-losing enteropathy during a ketogenic diet: a case report.
BMC Pediatrics ( IF 2.4 ) Pub Date : 2020-03-03 , DOI: 10.1186/s12887-020-1991-8
Juan Wang 1 , Li Jiang 1 , Min Cheng 1
Affiliation  

BACKGROUND A ketogenic diet (KD) is an effective treatment for intractable epilepsy in children. Protein-losing enteropathy (PLE) is a rarely reported but serious complication of KDs. CASE PRESENTATION A 3-month-old female patient presented with PLE while following a KD as treatment for intractable epilepsy. She also had genovariation of the STXBP1 gene. The patient suffered from general edema and hypoalbuminemia but no diarrhea. Esophagogastroduodenoscopy (EDG) revealed lymphatic ectasia in the lamina propria. We diagnosed her with intestinal lymphangiectasia, and after decreasing the KD ratio from 4:1 to 1.05:1, we successfully controlled her edema and hypoalbuminemia. As of now, the convulsions and hypsarrhythmia have disappeared, and the seizure-free state has lasted for 20 months. CONCLUSIONS PLE may be managed by decreasing the ketogenic ratio rather than discontinuing a KD since for some patients, a KD is the only effective therapy available at present.

中文翻译:

生酮饮食期间患有蛋白质丢失性肠病的女孩:一例病例报告。

背景技术生酮饮食(KD)是治疗儿童顽固性癫痫的有效方法。蛋白质丢失性肠病(PLE)是KD的罕见报道,但严重并发症。病例介绍一名3个月大的女性患者在接受KD治疗顽固性癫痫时出现PLE。她还患有STXBP1基因的基因变异。该患者患有一般性水肿和低白蛋白血症,但没有腹泻。食管胃十二指肠镜检查(EDG)显示固有层的淋巴结扩张。我们诊断出她患有肠道淋巴管扩张症,将KD比例从4:1降低至1.05:1后,我们成功地控制了她的水肿和低白蛋白血症。截至目前,惊厥和心律失常已消失,无癫痫发作状态持续了20个月。
更新日期:2020-03-03
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