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Clinical Reviews

Siewert-Kartagener syndrome: clinical diagnosis of the most common form of primary ciliary dyskinesia

Authors:

Valladares Garrido MJa, Valladares Garrido Db, Failoc Rojas VEc, Chávez Nd

aFacultad de Medicina. Universidad Norbert Wiener. Lima. Perú.
bFacultad de Medicina. Universidad Nacional de Piura. Servicio de Pediatría. Hospital Santa Rosa. Piura, Perú.
cUniversidad San Ignacio de Loyola. Lima, Perú.
dServicio de Pediatría. Hospital Santa Rosa. Piura, Perú.

Reference of this article.:

Valladares Garrido MJ, Valladares Garrido D, Failoc Rojas VE, Chávez N. Síndrome de Siewert-Kartagener: diagnóstico clínico de la forma más común de discinesia ciliar primaria. Rev Pediatr Aten Primaria. 2021;23:285-8.

Published in Internet: 13/09/2021

Abstract:

Kartagener syndrome (SK) is equivalent to 50% of primary ciliary dyskinesia, with an incidence in the general population of 1: 15 000 cases. The classic triad of SK consists of chronic sinusitis, bronchiectasis and situs inversus. A 23-month-old infant who entered the neonatal intensive care unit with a diagnosis of bronchopneumonia, congestive heart failure, and situs inversus is reported. Dextrocardia was diagnosed by electrocardiogram and imaging studies. The antecedent of the upper and lower infectious respiratory processes from the neonatal stage, observing dextrocardia and accentuation of the vascular bronchovascular tract, the manifest thickening of the mucosa of the maxillary sinuses bilaterally that is observed on the radiography of the sinuses, and the history of having a direct familiar with the same pathology, raised the diagnosis of primary ciliary dyskinesia and specifically Siewert-Kartagener syndrome.

Keywords: Kartagener syndrome. Primary ciliary dyskinesia. Dyneins . Dextrocardia.


 


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ISSN 2174-4106  Publicación Open Acess, incluida en DOAJ, sin cargo por publicación.

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