Which ofthe following hormones are raised in Prader- Willi syndrome - (AIIMS Nov 2013)
|A||Growth Hormone (GH)|
|B||Leutinizing Hormone (LH)|
|C||Follicle Stimulating Hormone (FSH)|
o Prader willi Syndrone is associated with elevated circulating levels of Ghrelin.
Prader Willi Syndrome
o Hypotonia (severe hypotonia with poor suck and failure to thrive in early infancy)
o Hyperphagia and Obesity (may be related to elevated Ghrelin tevels)
o Hypogonadotrophic Hypogonadism (CnRH;FSH; LH)
• Genital Hypoplasia (small penis, hypoplastic scrotum and cryptorchidism) in males.
• Genital Hypoplasia (small labia minora and clitoris) in females.
• Pubertal insufficiency.
o Short stature (GH levels)
o Cognitive impairment (usually mild mental retardation) and distinctive behavioral phenotupe such as temper tantrums & obsessive compulsive behavior.
o Characteristic facial appearance includes narrow bifrontal diameter, ‘almond’ shapped palpebral fissure and a down turned mouth with a thin upper lip.