Recent case reports showed improvement of cardiomyopathy following a ketogenic diet. Two siblings girl and boy, 7- and 5-year-old, both affected with GSD IIIa, developed severe and rapidly worsening left ventricular hypertrophy in the first years of life, while treated with frequent diurnal and nocturnal hyperproteic meals followed by orally administered uncooked cornstarch. A diet rich in fats as well as proteins and poor in carbohydrates could be a beneficial therapeutic choice for GSD III with cardiomyopathy. Future research is needed to confirm the beneficial effect of this treatment and to design treatment strategies with the aim to provide alternative source of energy and prevent glycogen accumulation. Clinically patients show fasting hypoglycemia, hyperlipidemia, growth delay, enlargement of liver and both skeletal muscles, and heart involvement. Cardiomyopathy with left ventricular hypertrophy is a relatively common finding although with variable severity and progression. It may be associated with potential risk of serious arrhythmia and symptomatic heart failure Austin et al. Functionally these patients have only partial glycogenolysis, while glycolysis and gluconeogenesis are preserved. Standard treatment consists of frequent diurnal and nocturnal hyperproteic meals followed by orally administered uncooked cornstarch, with the aim to maintain normal blood glucose Kishnani et al. These measures are effective in maintaining the metabolic control although growth retardation, liver, and cardiac and muscular complications may still occur in the long-term follow-up even in well-controlled patients Kishnani et al. Recent reports regarding one infant and one adult patient showed an improvement of cardiomyopathy following a ketogenic diet Valayannopoulos et al.
The main limitation of this clinical report is that it is not a left study; however each child served as its own control. Kop, 1 and William C. Loss of cardiac carnitine palmitoyltransferase 2 results in rapamycin-resistant, acetylation-independent hypertrophy, Journal of Biological Chemistry Giuseppe Vallar for his useful suggestions ketogenic Fondazione And e Luisa Mariani for vwntricular generous financial diet to our clinical activity. Jul 20, hypertrophy December 8, Ventricular C.
With so much information circulating the internet, we wanted to set the record straight on a handful of common misconceptions we often hear. These are the top five Keto myths The high amounts of fat consumed on the Keto diet leads to heart disease. You won’t get your necessary intake of vegetables or fruits nutrient deficiencies. The keto diet has not been around long enough to support any real health claims. SHOP Keto? Keto Mythbusters. Additionally, consider the fact that obesity is linked to hypertension and an enlarged left ventricle left ventricular hypertrophy, increasing the risk for heart failure. It has been proven time and time again that the keto diet can lead to significant weight loss especially for obese, at-risk people. Dropping hundreds of pounds in these cases can assist in reducing the risk of heart disease as well.