Allergy Asthma Respir Dis.  2013 Sep;1(3):288-291. 10.4168/aard.2013.1.3.288.

Kartagener's syndrome with immunoglobulin G subclass deficiency

• Kim KC
• Park JH
• Ban GY
• Yoo HS
• Shin YS
• Park HS
• Ye YM

• KMID: 2262974
• DOI: http://doi.org/10.4168/aard.2013.1.3.288

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.

Keyword

Kartagener syndrome; Immunoglobulin G subclass deficiency; Intravenous immunoglobulin G

MeSH Terms

Adolescent
Anti-Bacterial Agents
Bronchiectasis
Cough
Dextrocardia
Dyspnea
Estrogens, Conjugated (USP)
Expectorants
Female
Humans
Immunoglobulin G
Immunoglobulins
Immunoglobulins, Intravenous
Kartagener Syndrome
Maxillary Sinus
Reference Values
Sinusitis
Situs Inversus
Sputum
Thorax
Anti-Bacterial Agents
Estrogens, Conjugated (USP)
Expectorants
Immunoglobulin G
Immunoglobulins
Immunoglobulins, Intravenous

Figure

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  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.

• 

  Fig. 2 Ultrastructural findings of cilia of nasal mucosa show partial defect of outer (→) and inner (-->) dynein arm.

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