Long‐term ketogenic diet therapy improves mitochondrial encephalopathy with lactic acidosis and stroke‐like episodes (<scp>MELAS</scp>): A case report

He, Fang; Ye, Lingqi; Miao, Pu; Zhou, Jiong; Ding, Yao; Wang, Shuang

doi:10.1111/cns.14274

Cited by 4 publications

(1 citation statement)

References 9 publications

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“…There are reports indicating that patients with MELAS syndrome benefited from the KD diet in a manner analogous to that described above [45]. Notably, Fang He et al [40] disclose the example of a patient who is now 20 years old but was diagnosed with mitochondrial encephalopathy when he was only 9 years old. Before the KD was started, none of the other therapeutic techniques that were tried helped avoid seizures and a general deterioration in the patient's condition.…”

Section: Nutritional Approaches In Primary Mitochondrial Diseasesmentioning

confidence: 88%

Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence

Luca,

Pădureț,

Țarcă

et al. 2023

Nutrients

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Inborn errors of metabolism (IMDs) are a group of inherited diseases that manifest themselves through a myriad of signs and symptoms, including structural or functional cardiovascular damage. The therapy of these diseases is currently based on enzyme-replacement therapy, chaperone therapy or the administration of supplements and the establishment of personalized dietary plans. Starting from the major signs identified by the pediatric cardiologist that can indicate the presence of such a metabolic disease—cardiomyopathies, conduction disorders or valvular dysplasias—we tried to paint the portrait of dietary interventions that can improve the course of patients with mitochondrial diseases or lysosomal abnormalities. The choice of the two categories of inborn errors of metabolism is not accidental and reflects the experience and concern of the authors regarding the management of patients with such diagnoses. A ketogenic diet offers promising results in selected cases, although, to date, studies have failed to bring enough evidence to support generalized recommendations. Other diets have been successfully utilized in patients with IMDs, but their specific effect on the cardiac phenotype and function is not yet fully understood. Significant prospective studies are necessary in order to understand and establish which diet best suits every patient depending on the inherited metabolic disorder. The most suitable imagistic monitoring method for the impact of different diets on the cardiovascular system is still under debate, with no protocols yet available. Echocardiography is readily available in most hospital settings and brings important information regarding the impact of diets on the left ventricular parameters. Cardiac MRI (magnetic resonance imaging) could better characterize the cardiac tissue and bring forth both functional and structural information.

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Section: Nutritional Approaches In Primary Mitochondrial Diseasesmentioning

confidence: 88%