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Kim, Park, Ban, Yoo, Shin, Park, and Ye: Kartagener's syndrome with immunoglobulin G subclass deficiency
Case Report

Allergy, Asthma & Respiratory Disease 2013; 1(3): 288-291.

Published online: 30 September 2013

DOI: https://doi.org/10.4168/aard.2013.1.3.288

Kartagener's syndrome with immunoglobulin G subclass deficiency

Ki-Chan Kim, Joo-Han Park, Ga-Young Ban, Hye-Soo Yoo, Yoo-Seob Shin, Hae-Sim Park, Young-Min Ye

Department of Allergy & Clinical Immunology, Ajou University School of Medicine, Suwon, Korea.

Correspondence to: Young-Min Ye. Department of Allergy & Clinical Immunology, Ajou University School of Medicine, 206 World cup-ro, Yeongtong-gu, Suwon 443-721, Korea. Tel: +82-31-219-5150, Fax: +82-31-219-5154, ye9007@ajou.ac.kr

Received 20 June 2013       Revised 18 July 2013       Accepted 26 July 2013

© 2013 The Korean Academy of Pediatric Allergy and Respiratory Disease; The Korean Academy of Asthma, Allergy and Clinical Immunology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

Abstract

Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, the major determinant for diagnosing immunodeficiency, is the most common clinical manifestation of the disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, and rhinorrhea for more than 5 years. Nasal symptoms and dyspnea had not been controlled by intermittent treatment with mucolytics and antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray and computed tomography showed situs inversus, dextrocardia and bronchiectasis. Paranasal sinus series revealed mucosal thickening and haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG was within normal range. Under the diagnosis of Kartagener syndrome with IgG4 deficiency, monthly intravenous IgG (IVIG) treatment was performed for 6 months. Her symptoms were well controlled and the frequency of antibiotics use was markedly decreased. We report a patient having the Kartagener syndrome with IgG4 deficiency that was successfully controlled with a 6-month-treatment of IVIG.

Keywords: Kartagener syndrome, Immunoglobulin G subclass deficiency, Intravenous immunoglobulin G

Figures and Tables

Fig. 1
Radiologic findings at the time of diagnosis. (A) Chest X-ray shows situs inversus with dextrocardia. (B) Chest computerized tomography (CT) shows dextrocardia and bronchiectasis with mucoid impaction in both lower lobes. (C) Paranasal sinus CT shows prominent and diffuse mucoperiosteal thickening on entire paranasal sinus. (D) Abdominal CT shows a mirror image of normal configuration of stomach and liver.
aard-1-288-g001
Fig. 2
Ultrastructural findings of cilia of nasal mucosa show partial defect of outer (→) and inner (-->) dynein arm.
aard-1-288-g002

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ORCID iDs

Hae-Sim Park
https://orcid.org/http://orcid.org/0000-0003-2614-0303

Young-Min Ye
https://orcid.org/http://orcid.org/0000-0002-7517-1715

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