A girl with protein-losing enteropathy during a ketogenic di
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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. They diagnosed her with intestinal lymphangiectasia, and after decreasing the KD ratio from 4:1 to 1.05:1,

They treated her with a KD with a 2:1 ratio of fat to nonfat according to the milk formula and monitored her blood ketones regularly. Multiple AEDs were also maintained at the same doses. After 1 week of the KD, because her blood ketone levels were very low (average 1.5 mmol/L) and the seizures continued, they gradually increased the KD ratio to 4:1. Subsequently, the seizure frequency decreased. At the end of the first month on the KD, a seizure-free state was reached

In conclusion 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.

Source: https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-020-1991-8
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