Saturday, 14 May 2011

Triad of Kartagener syndrome

 A 14-year-old boy presents with a history of chronic sinusitis and frequent pneumonias. On physical examination, the patient has normal vital signs and is afebrile. He has mild frontal and maxillary sinus tenderness with palpation. Transillumination of the sinuses is normal. Heart sounds are best heard on the right side of the chest. The boy is coughing copious amounts of yellowish sputum. Which of the following is the most likely diagnosis?
  • a.Cystic fibrosis
  • b.Kartagener syndrome
  • c.Pulmonary dysplasia
  • d.Tuberculosis
  • e.Pulmonary hypertension

 The answer is b."Kartagener syndrome"
Kartagener syndrome is the inheritable disorder of dextrocardia, chronic sinusitis (with the formation of nasal polyps), and bronchiectasis. Patients may also present with situs inversus.The disorder is due to a defect that causes the cilia within the respiratory tract epithelium to become immotile. Cilia of the sperm are also affected.

Frontal and Chest x-ray showing dextrocardia "the typical radiographic sign of Kartagener's syndrom" and bronchiectasis.

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