Ketogenic diet in pyruvate dehydrogenase complex deficiency: short- and long-term outcomes.

ABSTRACT:

Objectives

Our aime was to study the short- and long-term effects of ketogenic diet on the disease course and disease-related outcomes in patients with pyruvate dehydrogenase complex deficiency, the metabolic factors implicated in treatment outcomes, and potential safety and compliance issues.

Methods

Pediatric patients diagnosed with pyruvate dehydrogenase complex deficiency in Sweden and treated with ketogenic diet were evaluated. Study assessments at specific time points included developmental and neurocognitive testing, patient log books, and investigator and parental questionnaires. A systematic literature review was also performed.

Results

Nineteen patients were assessed, the majority having prenatal disease onset. Patients were treated with ketogenic diet for a

SUBMITTER: Sofou K 

PROVIDER: S-EPMC5306430 | biostudies-literature | 2017 Mar

REPOSITORIES: biostudies-literature