False about Wolman disease is –
**Core Concept**
Wolman disease is a rare genetic disorder caused by a deficiency of the enzyme acid lipase, leading to the accumulation of cholesterol and triglycerides within lysosomes. This accumulation results in cellular dysfunction and ultimately leads to the death of various cells and tissues.
**Why the Correct Answer is Right**
The correct answer, D, is incorrect because Wolman disease is actually characterized by the accumulation of lipids in the adrenal glands and the liver, leading to adrenal insufficiency and liver dysfunction, but it does not typically cause corneal clouding. The clinical features of Wolman disease include hepatosplenomegaly, adrenal insufficiency, and failure to thrive in infants. Corneal clouding is not a typical feature of this disease.
**Why Each Wrong Option is Incorrect**
**Option A:** Wolman disease is indeed a lysosomal storage disorder, characterized by the accumulation of lipids within lysosomes due to the deficiency of acid lipase.
**Option B:** Wolman disease is caused by a deficiency of acid lysosomal lipase, making this statement correct.
**Option C:** Wolman disease is inherited in an autosomal recessive pattern, so this statement is also correct.
**Clinical Pearl / High-Yield Fact**
Wolman disease is a severe and rapidly progressive form of lysosomal storage disorder, and early diagnosis is crucial for the initiation of enzyme replacement therapy or other supportive treatments.
**β Correct Answer:** D. It is characterized by adrenal calcification and corneal clouding