Ketogenic diet using medical nutrition in infants: the experience of a Russian center

Résumé fourni par la source

Background. The aim of this study is to evaluate the efficacy and safety of initiating a classical ketogenic diet in infants with epileptic encephalopathies using the specialized formula. Materials and methods. A single-center prospective observational study included 14 infants with drug-resistant epileptic seizures. A genetic etiology was confirmed in 9 patients, including 2 children with glucose transporter 1 deficiency syndrome. The ketogenic diet was initiated in a hospital setting with gradual replacement of feedings with a specialized ketogenic formula used off-label outside the approved age. The dietary energy value and the ketogenic ratio were tailored individually. The follow-up period was 6 to 12 months. Changes in seizure frequency, diet tolerability, blood ketone levels, acid-base status, and physical development of the children were evaluated. Results. Seizure freedom was achieved in 2 children with GLUT1 syndrome (14.3 %). A 75-99% reduction in seizure frequency was observed in 4 patients (28.6%), and a 50-74% decrease in 6 children (42.9%). Two patients (14.3%) demonstrated a reduction in seizure frequency of less than 50%. Thus, a clinically meaningful response, defined as a reduction in seizure frequency of at least 50%, was achieved in 12 out of 14 children (85.7%). No serious adverse events or diet discontinuation due to intolerance were reported. Some patients developed transient metabolic acidosis, which resolved following ketogenic ratio adjustment and anticonvulsant therapy modification. No negative trends in physical development were observed. Conclusion. Early initiation of a ketogenic diet in infants with epileptic encephalopathies was associated with a significant reduction in seizure frequency and satisfactory tolerability. The use of specialized nutrition support facilitates adherence to the calculated ketogenic ratio and dietary management in infants. Safe implementation of nutrition therapy requires an inpatient initiation, individualized nutritional formulas, and routine clinical and laboratory monitoring.

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