Published online April 16, 2007
PEDIATRICS Vol. 119 No. 5 May 2007, pp. e1203-e1205 (doi:10.1542/peds.2006-2396)
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EXPERIENCE & REASON

Diagnosis of Common Variable Immunodeficiency in a Patient With Primary Ciliary Dyskinesia

Edward W. Skorpinski, MDa, Shiang-Ju Kung, MDb, Ejaz Yousef, MDc,d and Stephen J. McGeady, MDc,d

a Berkshire Allergy & Asthma Center, Wyomissing, Pennsylvania
b Allergy Section, Division of Immunologic and Infectious Diseases, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
c Division of Allergy and Clinical Immunology, Alfred I. duPont Hospital for Children, Nemours Children's Clinic, Wilmington, Delaware
d Division of Allergy and Clinical Immunology, Thomas Jefferson University, Philadelphia, Pennsylvania

ABSTRACT

In this case report we describe the first account in the literature of a patient with primary ciliary dyskinesia and common variable immunodeficiency. A 17-year-old boy with previously diagnosed Kartagener syndrome and stable lung disease developed a deteriorating clinical course that prompted the search for a secondary diagnosis. Although both of these rare conditions can result in similar lung pathology, they require different management strategies, which illustrates the need to consider associated diagnoses in complicated clinical situations.

Key Words: Kartagener syndrome • bronchiectasis • primary ciliary dyskinesia • common variable immunodeficiency

Abbreviations: KS, Kartagener syndrome • PCD, primary ciliary dyskinesia • CVID, common variable immunodeficiency • CT, computed tomography • Ig, immunoglobulin • IVIG, intravenous immunoglobulin • non-CF, non–cystic fibrosis related

Accepted Nov 14, 2006.