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.
Keywords Cystic fibrosis – Immunodeficiency – Aspergers – Common variable immunodeficiency