The goal of this observational study is to learn about the clinical and nutritional effectiveness of ketogenic diet (KD) in pediatric patients with genetic, neurological or metabolic conditions requiring KD. The main question\[s\] it aims to answer are: * does KD support adequate growth? * does KD improve clinical symptoms? * how does KD impact quality of life? Participants will be followed up as per clinical practice
Scientific and clinical interest in KD is growing with a progressive increase in its therapeutic indications, ie recent studies show beneficial effects of KD in other diseases like migraine, autism spectrum disorder and brain tumors. However, so far, the majority of results on KD effects have been collected in patients with drug-resistant epilepsy proving its effectiveness on clinical outcome (e.g. reducing epileptic seizures), frequently leaving out the nutritional assessment and the impact on quality of life. Nonetheless, these factors are indeed essential to ensure safety and efficacy of KD treatment, especially when dealing with children and in perspective of the progressive expansion of the target population that can benefit from this nutritional therapy. The present multicenter study aims to analyse these poorly explored areas, by evaluating, in a cohort of pediatrics patient with indication to KD therapy, not only the KD effectiveness on the course of the underlying disease, but also the impact on nutritional parameters (e.g. growth, lipid profile, vitamin intake, bone mineral density, ..) and on quality of life.
Study Type
OBSERVATIONAL
Enrollment
100
Patients recruited receive a ketogenic diet. The prescription, the type of KD protocol and the route of administration will depend, essentially, on the patient's ability and willingness to feed spontaneously and independently, as well as on compliance with the therapies set and the severity of the clinical conditions.
ASST Fatebenefratelli Sacco (PO "V. Buzzi")
Milan, MI, Italy
RECRUITINGFondazione "Istituto Neurologico Nazionale C. Mondino"
Pavia, PV, Italy
RECRUITINGOspedale Pediatrico Bambino Gesù
Roma, RM, Italy
RECRUITINGNutritional adequacy
evaluate the nutritional adequacy of the diet after 6 months of treatment with ketogenic diet (KD) in terms of adequate growth defined by weight-for-age Z Score (WAZ) and length-for-age Z score (LAZ).
Time frame: 6 months
Nutritional adequacy
Evaluate the nutritional adequacy of the diet after 6 and 12 months of treatment with ketogenic diet (KD) in terms of correct growth defined by: Weight-for-age Z-score (WAZ), length/height-for-age z-score (LAZ), BMI z-score Blood levels of selected parametres (eg cholesterol, HDL, LDL) within normal ranges Body composition (optional)
Time frame: 6 months,(12 months optional)
% of responders to KD
Evaluate the effectiveness of the ketogenic diet (KD) on the clinical outcomes (% of responders): * Epilepsy: at least 50% seizure reduction after 6 months of KD treatment (vs 6 months before trial start( (daily diary records) * ASD: reduction of at least 2-8 in CARS score after 6 months of KD treatment * Chronic migraine: at least 50% episodes reduction after 6 months of KD treatment (vs 6 months before trial start( (daily diary records); reduction \>=50% of PedMiDas score after 6 months of KD treatment (vs baseline) Brain tumors: % survival; % of subjects with progression free survival
Time frame: 6 months,(12 months optional)
Health related quality of life improvement through parent questionnaire
Evaluate the effectiveness of the ketogenic diet (KD) on the patient's quality of life using a non-validated questionnaire administered to parents with qualitative open and closed questions. Improvement will be considered as an improvement in the score of disease burden related questions baseline vs end of observation
Time frame: 6 months,(12 months optional)
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