Zellweger Syndrome

Definition

Zellweger syndrome is a lethal disease characterized by peroxisome dysfunction, severe neurological findings, and dysmorphic face.

Symptoms

  • Severe hypotonia
  • Characteristic face
  • Liver dysfunction
  • Convulsions

Reasons

PEX gene mutations.

Diagnosis

Plasma very long chain fatty acids, genetic testing.

Treatment

Palliative care.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

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

This page is for general information purposes only.