Angelman
Eating Behavior, Prenatal and Postnatal Growth in Angelman Syndrome
Clinical characteristics of Angelman syndrome include severe intellectual disability, developmental delay and lack of speech. Angelman syndrome is characterised by a loss of expression of certain genes which has a cascading effect on other chromosomes. Eating behaviour has been widely studied within Prader Willi syndrome and it is a behaviour that is very common within individuals with this syndrome. However there is little research into the eating behaviour of individuals with Angelman syndrome and the effect this has on their growth. In a recent study, Line Mertz and colleagues examined eating behaviour and the relationship between the expression of genes and how this effects growth data such as head circumference.
The study consisted of parents filling out a questionnaire about their child’s eating behaviour, including their motivation and the severity to which food interferes with their daily routine. Other measurements such as the child’s birth weight and the circumference of their head were obtained from hospital medical records.
The study showed that children with uniparental disomy (where 2 copies of a chromosome come from the same parent, instead of one copy coming from the mother and one coming from the father) had a significantly larger weight at birth and birth length than children with deletions. This resulted in children developing obesity after 2-3 years old and having significantly more severe eating behaviours compared to children from other genetic syndrome groups. There was no difference in eating behaviour found between children with Angelman syndrome due to deletion or a mutation. This study highlights the relationship between the expression of genes on the postnatal growth within children and suggests that more research needs to be done to look at how genes affect the growth of individuals with Angelman syndrome into adulthood and beyond.