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Effectiveness and Impact on the Quality of Life of Ketogenic Diet in Pediatric Patients

Recruiting
Conditions
Autism Spectrum Disorder
Chronic Migraine
Brain Tumor, Pediatric
Drug Resistant Epilepsy
Interventions
Other: ketogenic diet (as per clinical practice)
Registration Number
NCT06309251
Lead Sponsor
Danone Nutricia SpA Società Benefit
Brief Summary

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

Detailed Description

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.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
100
Inclusion Criteria
  • Pediatric patients (aged < 18 years) with drug-resistant epilepsy (fail to achieve (and maintain) seizure freedom with adequate trials of two or more antiseizure medications) or genetic, metabolic, neurological (congenital and acquired) diseases treated with ketogenic diet
  • Pediatric patients (aged < 18 years) with metabolic, genetic or neurological (congenital and acquired) diseases (not necessarily associated with drug-resistant epilepsy) treated with ketogenic diet; this includes the new KD indications or the administration of KD in the ICU for status epilepticus.
Exclusion Criteria
  • Patients affected by beta-oxidation cycle disorders, systemic primary carnitine deficiency, primary dyslipidemia, pyruvate carboxylase deficiency, porphyria, mitochondrial disease, defects in ketone body metabolism (ketogenesis or ketolysis), defect in gluconeogenesis.
  • Children with type 1 diabetes
  • Parents (or caregivers) unable to guarantee adherence to the

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Patients requiring KDketogenic diet (as per clinical practice)Pediatric patients with any disease which require a ketogenic diet (KD), i.e. metabolic or genetic disorder or neurological (congenital and acquired) diseases
Primary Outcome Measures
NameTimeMethod
Nutritional adequacy6 months

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).

Secondary Outcome Measures
NameTimeMethod
Nutritional adequacy6 months,(12 months optional)

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)

Health related quality of life improvement through parent questionnaire6 months,(12 months optional)

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

% of responders to KD6 months,(12 months optional)

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

Trial Locations

Locations (3)

ASST Fatebenefratelli Sacco (PO "V. Buzzi")

🇮🇹

Milan, MI, Italy

Ospedale Pediatrico Bambino Gesù

🇮🇹

Roma, RM, Italy

Fondazione "Istituto Neurologico Nazionale C. Mondino"

🇮🇹

Pavia, PV, Italy

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