Wolman's Disease

Definition

Wolman disease is a lethal lysosomal storage disease characterized by severe hepatosplenomegaly and adrenal calcification, resulting from acid lipase enzyme deficiency.

Symptoms

  • Hepatosplenomegaly
  • Adrenal calcification
  • Malabsorption
  • Developmental delay

Reasons

LIPA gene mutation.

Diagnosis

Enzyme activity, genetic testing.

Treatment

Palliative care.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of liver failure, emergency services should be consulted.

This page is for general information purposes only.