What is A child presents with Hepatosplenomegaly, Abdominal distension, Jaundice, Anemia and Adrenal calcification. Which of the following is the Diagnosis ? and explain in detail?
Wolman disease is a congenital disease characterized by an impaired metabolism of the fats (lipids). It is the most severe type of lysosomal acid lipase deficiency. The lysomal acid lipase deficiency causes a buildup of lipids (fats) in body organs and calcium deposits in the adrenal glands. Reference: GHAI Essential pediatrics, 8th edition