Catalog

Wolman disease

No MMS code

Link to WHO Definition

Wolman disease – http://id.who.int/icd/entity/520367511

Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the first weeks of life with abdominal distension and major or even massive hepatosplenomegaly (which can occur in the neonatal period) and sometimes ascites and calcified adrenal glands.