The project

Monday September 3rd, 2018

The role of medical nutrition therapy is well known in many diseases. In particular, some neurological diseases of children may benefit form a “special diet” which is named “ketogenic diet”.
The aim of this project is to evaluate the impact of ketogenic diet on growth in children that must follow this therapy because they are affected by a rare genetic disease, glucose transporter type I deficiency (GLUT1 deficiency syndrome), or by drug resistant epilepsy.

The ketogenic diet is the first-line and so far the only available treatment for children with GLUT1 deficiency syndrome.
It should be started at birth and, until new therapies will be available, it must be continued all life long. In these children the glucose transporter doesn’t work and the brain cannot receive enough glucose (and so energy) for its correct development and functioning, with consequent onset of intellectual disability and movement disorders.

Ketogenic diet is effective also in drug-resistant epilepsies (in which seizures persist despite antipetileptic medication use), that represent about 20% of all childhood epilepsies.

This treatment is strongly different from healthy and balanced diet recommendations, because it is an high-fat, normo-protein and low-carb diet. It is specifically set in order to increase the production of ketone bodies (which give the name to this special diet), used by the brain as an alternative fuel instead of glucose.

Creating and realizing a special diet for each children requires an important team work, which includes qualified physicians expert in nutrition, neurology and paediatrics, dieticians and nurses (keto-team).
Children and their parent must be educated on which foods can or cannot be consumed and which supplements must be taken daily. It is also essential to evaluate the nutritional status of these children, measuring weight, height and body composition before and after diet initiation.
The correct implementation of this regimen generally does not lead to any adverse effect, but requires regular follow-up visits and examination. The use of this diet in children takes particular attention to avoid interference with growth, an effect of this treatment which has not been studied in depth, yet. A strict auxological monitoring is necessary to identify any eventual growth deflections that could need for further examinations, in order to investigate the presence of a possible reduced secretion of growth hormone, specially in those children who have a genetic disease.

Ensuring an adequate nutritional and growth status consents an improvement in health and quality of life of these children and their families, in particular for those who have to follow KD for many years or life long.

We need your help to realize this project!

Link:

web site: http://spmsf.unipv.it/unadietaspeciale/

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