/properdin deficiency, X-linked

properdin deficiency, X-linked

Rare Disease

MONDO:0010713

Also Known As

properdin deficiency, X-linkedproperdin deficiency, X-linked, X-linked recessiveCFPDPFDcomplement Factor properdin deficiencyproperdin P Factor deficiencyproperdin deficiency, type 1properdin deficiency, type 2properdin deficiency, type 3

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.