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[Extreme obesity in Prader-Willi Syndrome (PWS)]
J Damiano1, C Ficko, E Garrabé
1Service d'endocrinologie, hôpital Bégin, 69, avenue de Paris, 94160 Saint-Mandé, France.
Introduction:
Prader-Willi Syndrome (PWS) belong to genetic obesities and we report a caricatural observation.
Development:
The early onset of (PWS) is characterised by a severe neonatal hypotonia with poor suck reflex--which may lead to tube feeding--and poor weight gain. Later appears insatiable appetite, morbid obesity associated with short stature, dysmorphic syndrome with small hands and behavioural disorders. Although diagnosis is based on clinical features, it must be confirmed by genetic test looking for the characteristic deletion of the chromosome 15q11-q13 region. PWS is the first example in humans of genetic imprinting.
Conclusion:
Today, the challenge in PWS is it early management which may authorise Growth Hormone administration.