Evolving practice: X-linked agammaglobulinemia and lung transplantation.
X-linked agammaglobulinemia (XLA) is a rare primary humoral immunodeficiency syndrome characterized by agammaglobulinemia, recurrent infections and bronchiectasis. Despite the association with end-stage bronchiectasis, the literature on XLA and lung transplantation is extremely limited. We report a series of 6 XLA patients with bronchiectasis who underwent lung transplantation. Short-term outcomes were excellent however long-term outcomes were disappointing with a high incidence of pulmonary sepsis and chronic lung allograft dysfunction (CLAD).
General Medical Department, Southern Health, Melbourne, Australia.
S
S
Barnes
S
S
Kotecha
J A
JA
Douglass
E
E
Paul
F
F
Hore-Lacy
F
F
Hore-Lacey
R
R
Stirling
G I
GI
Snell
G P
GP
Westall
D000328:Adult; D000361:Agammaglobulinemia / Q000503:physiopathology*; D040181:Genetic Diseases, X-Linked / Q000503:physiopathology*; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged
clinical research; immune modulation; immunosuppression; immunosuppressive regimens; lung transplantation; practice; pulmonology, rejection: chronic