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Evolving practice: X-linked agammaglobulinemia and lung transplantation.

Abstract
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).
AuthorsS Barnes, S Kotecha, J A Douglass, E Paul, F Hore-Lacy, F Hore-Lacey, R Stirling, G I Snell, G P Westall
JournalAmerican journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons (Am J Transplant) Vol. 15 Issue 4 Pg. 1110-3 (Apr 2015) ISSN: 1600-6143 [Electronic] United States
PMID25736826 (Publication Type: Case Reports, Journal Article)
Copyright© Copyright 2015 The American Society of Transplantation and the American Society of Transplant Surgeons.
Topics
  • Adult
  • Agammaglobulinemia (physiopathology)
  • Genetic Diseases, X-Linked (physiopathology)
  • Humans
  • Lung Transplantation
  • Male
  • Middle Aged

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