Evolving Practice: X‐Linked Agammaglobulinemia and Lung Transplantation | Zendy

Barnes S. | Zendy; Kotecha S. | Zendy; Douglass J. A. | Zendy; Paul E. | Zendy; HoreLacey F. | Zendy; Stirling R. | Zendy; Snell G. I. | Zendy; Westall G. P. | Zendy

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Evolving Practice: X‐Linked Agammaglobulinemia and Lung Transplantation

Author(s) -

Barnes S.,

Kotecha S.,

Douglass J. A.,

Paul E.,

HoreLacey F.,

Stirling R.,

Snell G. I.,

Westall G. P.

Publication year - 2015

Publication title -

american journal of transplantation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.89

H-Index - 188

eISSN - 1600-6143

pISSN - 1600-6135

DOI - 10.1111/ajt.13084

Subject(s) - bronchiectasis , medicine , x linked agammaglobulinemia , lung transplantation , immunodeficiency , primary immunodeficiency , lung , common variable immunodeficiency , transplantation , sepsis , incidence (geometry) , immunology , immune system , antibody , physics , receptor , tyrosine kinase , optics , bruton's tyrosine kinase

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).

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