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Kartagener syndrome
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Kartagener syndrome

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Synopsis

An uncommon autosomal recessive inherited disorder causing abnormal function of lung cilia, which hinders mucosal clearance. A subtype of primary ciliary dyskinesia characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus. Patients typically present with chronic upper and lower respiratory tract infections, rhinitis, tachypnea, cough, chronic ear infections, and situs inversus. Respiratory distress begins in neonatal period. Adults may be diagnosed when infertility becomes an issue, along with respiratory symptoms.

Management of bronchiectasis is geared toward secretion clearance, use of antibiotics, influenza and pneumonia vaccinations, and utilizing many of the treatments of cystic fibrosis. Smoking cessation, treatment of rhinosinusitis, chest physiotherapy, and removal of nasal polyps may be employed. Bilateral lung transplant, if considered, may require modifications or heart-lung transplant due to situs inversus. Prognosis with appropriate therapies is generally good, despite repeated respiratory infections.

For more information, see OMIM.

Codes

ICD10CM:
Q89.3 – Situs inversus

SNOMEDCT:
42402006 – Kartagener Syndrome

Differential Diagnosis & Pitfalls

At first presentation, chronic respiratory infections would suggest a differential including:
Radiologic studies showing situs inversus would narrow the differential considerably.

Best Tests

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References

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Last Updated: 03/29/2017
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Kartagener syndrome
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Kartagener syndrome : Nasal polyps
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