Mucopolysaccharidosis Type VII (Sly Syndrome)

Definition

Mucopolysaccharidosis type VII is a rare lysosomal storage disease characterized by beta-glucuronidase enzyme deficiency and characterized by hepatosplenomegaly and skeletal anomalies.

Symptoms

  • Hepatosplenomegaly
  • Skeletal anomalies
  • Developmental delay
  • Corneal opacity

Reasons

GUSB gene mutation.

Diagnosis

Urine GAG, enzyme activity.

Treatment

Enzyme replacement therapy.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of respiratory distress, emergency services should be consulted.

This page is for general information purposes only.