LET’S LEARN PHYSIOLOGY
Wolman disease
Rare autosomal recessive inborn error of metabolism resulting in deposition of fats in multiple organs
Deficiency of lysosomal acid lipase
Less severe form is called Cholesteryl ester storage disease
Infants present during the first two months of life with failure to thrive, diarrhea and vomiting. Abdominal distention may be present because of hepatomegaly, spleenomegaly and generalized lymphadenopathy
Radiologic features - Bilateral enlargement and calcification of adrenals
Affected infants usually die before 1 year of age due to intestinal malabsorption