Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: An immunological and behavioural challenge

Abstract

Introduction: Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. Case report: We report a unique case of CF associated with a primary immunodeficiency syndrome - common variable immunodeficiency (CVID). Discussion: Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient. © Royal Academy of Medicine in Ireland 2009.