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What is A 2 month old infant is presented with failure to thrive, recurrent emesis, hepatosplenomegaly, and adrenal insufficiency. Adrenal calcification is noted radiologically. What is the most likely diagnosis? and explain in detail?
This child is showing features of Wolman disease. Wolman disease appear in the first few weeks of life and presents with persistent vomiting and diarrhea, hepatosplenomegaly, xanthomatosis and adrenal calcification. Patients have a complete absence of enzyme A of lysosome acid lipase. Investigations: shows liver enzyme abnormalities, decreased adrenal responsiveness to ACTH stimulation, normal or decreased plasma lipids. Cholesterol ester and triglyceride deposition occur in the lysosomes of liver parenchymal and Kuppfer cells, and in macrophages of adrenal gland, lymph node, intestinal mucosa etc.