Prader – Willi syndrome is associated with following EXCEPT
**Core Concept**
Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by a deficiency in hypothalamic function, leading to an insatiable appetite and obesity. It is caused by the loss of function of genes on chromosome 15 inherited from the father, typically due to a deletion or uniparental disomy.
**Why the Correct Answer is Right**
PWS is associated with several clinical features, including short stature, intellectual disability, hypogonadism, and a characteristic facial appearance. The hyperphagia and obesity seen in PWS are due to the hypothalamic dysfunction, which affects the regulation of appetite and satiety. This leads to excessive food intake and subsequent weight gain.
**Why Each Wrong Option is Incorrect**
**Option A:** Short stature is a common feature of PWS, so this option is not correct.
**Option B:** Intellectual disability is also a characteristic of PWS, so this option is not correct.
**Option C:** Hypogonadism is a feature of PWS, particularly in males, who may experience undescended testes and infertility, so this option is not correct.
**Option D:** This option is the correct answer because Angelman syndrome is a distinct genetic disorder that is also caused by a deficiency of genes on chromosome 15, but it is inherited from the mother, not the father. It is characterized by developmental delay, intellectual disability, and a distinctive facial appearance, but it does not involve hyperphagia or obesity.
**Clinical Pearl / High-Yield Fact**
A key feature of PWS is the presence of a characteristic "thin upper lip" and "full lower lip" appearance, which can help in diagnosing the condition.
**Correct Answer: D. Angelman syndrome.**