Protein-Losing Enteropathy as a Complication of the Ketogenic Diet

Ahn, Won Kee; Park, Soyoung; Kim, Heung Dong

doi:10.3349/ymj.2017.58.4.891

Cited by 5 publications

(3 citation statements)

References 12 publications

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“…A ketogenic diet did demonstrate some success at controlling refractory epileptic spasms in patients with DS, but the sample size in the literature is small (10 reports) 50,59,60 . Overall, 8 out of 10 patients responded to the treatment, with half of them achieving complete seizure control.…”

Section: Resultsmentioning

confidence: 99%

Epileptic spasms in individuals with Down syndrome: A review of the current literature

2020

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Epilepsy can occur in individuals with Down syndrome (DS), with epileptic spasms representing the most frequent seizure type in this population. Epileptic spasms can have devastating consequences on the development of individuals with the condition. This review sought to explore the lifetime prevalence and underlying mechanism of epileptic spasms in this population. We also aimed to review the response rate to various treatments, the relapse rate, and the development of subsequent epilepsy or autism in this population. A comprehensive literature search was conducted for articles discussing the lifetime prevalence, diagnosis, treatment, outcomes, or underlying etiology of epileptic spasms in animal models or individuals with DS. According to available literature, the global clinic‐based lifetime prevalence of epilepsy in individuals with DS ranged from 1.6% to 23.1%, with epileptic spasms representing 6.7%‐66.7% of these cases. Response rate to treatment with adrenocorticotropic hormone/corticosteroids was highest (81%) and has the most literature supporting its use, with other regimens, including vigabatrin and other antiepileptic drugs, having lower response rates. Epileptic spasms occur more frequently in children with DS than in the general population, though more studies are needed to determine the true lifetime prevalence of epileptic spasms in this population. Generally, children with DS and epileptic spasms tend to be more responsive to treatment and have better outcomes than children with epileptic spasms of unknown etiology (ie, without DS), in terms of response and relapse rates as well as the development of intractable epilepsy (eg, Lennox‐Gastaut syndrome).

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Section: Resultsmentioning

confidence: 99%

Epileptic spasms in individuals with Down syndrome: A review of the current literature

2020

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“…Although hypoproteinemia is one of the presentations of PLE, it is also much more common than PLE [1,2]. There are two published case reports for PLE initiated by a KD [4,5], in both of which the patients discontinued the KD. Our patient is the first to not discontinue a KD when suffering from PLE.…”

Section: Discussionmentioning

confidence: 99%

“…The main mechanisms of PLE are mucosal injury and lymphatic abnormalities [3], and lymphangiectasia is suspected to be a mechanism of PLE [4,5]. Intestinal lymphangiectasia has been reported in children following a high-fat diet [3] and may cause the leakage of lymphatic fluids rich in albumin and other proteins into the GI tract.…”

Section: Discussionmentioning

confidence: 99%

A girl with protein-losing enteropathy during a ketogenic diet: a case report

2020

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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.

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Protein-Losing Enteropathy as a Complication of the Ketogenic Diet

Cited by 5 publications

References 12 publications

Epileptic spasms in individuals with Down syndrome: A review of the current literature

Epileptic spasms in individuals with Down syndrome: A review of the current literature

A girl with protein-losing enteropathy during a ketogenic diet: a case report

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