Properdin deficiency, X-linked

Properdin deficiency, X-linked

Definition

A rare, hereditary, primary immunodeficiency due to a complement cascade protein anomaly characterized by significantly increased susceptibility to Neisseria species infections. It only affects males, typically presenting with severe or fulminant meningococcal disease.

Also known as properdin deficiency, X-linked, properdin deficiency, X-linked, X-linked recessive — per MONDO

Also identified as