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Pediatría Atención Primaria
versão impressa ISSN 1139-7632
Resumo
VALLADARES-GARRIDO, Mario J; VALLADARES-GARRIDO, Danai; FAILOC-ROJAS, Virgilio E e CHAVEZ, Nora. Siewert-Kartagener syndrome: clinical diagnosis of the most common form of primary ciliary dyskinesia. Rev Pediatr Aten Primaria [online]. 2021, vol.23, n.91, pp.285-288. Epub 06-Fev-2023. ISSN 1139-7632.
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.
Palavras-chave : Dextrocardia; Dyneins; Primary ciliary dyskinesia; Kartagener syndrome.