A child presents with recurrent chest infections and recurrent sinusitis. Chest X-ray reveals dextrocardia and situs inversus. The most likely diagnosis is:
Ans, a. Kartagener's syndrome (Ref: Robbins 9/e p683; Nelson 19/e pi 49?)The most likely diagnosis in a child with history of recurrent chest infections and recurrent sinusitis with dextrocardia and situs inversus on chest X-ray is Kartagener's syndrome.Kartagener's Syndrome (Immotile cilia syndrome/Primary ciliary dyskinesia)* Primary ciliary dyskinesia (PCD) comprises those respiratory disorders having in common the malfunction of airway cilia.* The abnormality results from various inherited primary structural defects in the cilia that lead to repeated and chronic lung and sinus infections.* About 50% of patients with PCD have Kartagener's syndrome: Situs inversus, chronic sinusitis and otitis, and airway disease leading to bronchiectasis.* Recurrent sinopulmonary infections are seenKartagener's Syndrome (Immotile cilia syndrome/Primary ciliary dyskinesia)Ultrastructural changes causing immotility of ciliaImmotility during embryogenesisImmotility in respiratory EpitheliumImmotility of cilia in sperms* Situs inversus* Dextrocardia* Bronchiectasis* Sinusitis* Infertility (male) Major cardiac AnomalyMajor non cardiac anomalyKartagener's syndrome (Immotile cilia syndrome) (Primary ciliary dyskinesia)* Dextrocardia0* Situs inversus* Sinusitis* Bronchiectasis* Infertility
Kartagener's syndrome
Ehler-Danlos syndrome
Marfan's symdrome
William Campbell syndrome
Disorders of the Respiratory Tract