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DILATATION DES BRONCHES REVELANT UN SYNDROME DE KARTAGENER : A PROPOS DUN CAS ET REVUE DE LA LITTERATURE

Authors: Abdoulkader A. Anissa-Sahan , Leila Herrak , Asma Jniene , Mustapha Elftouh and Leila Achachi;

DILATATION DES BRONCHES REVELANT UN SYNDROME DE KARTAGENER : A PROPOS DUN CAS ET REVUE DE LA LITTERATURE

Abstract

Primary ciliary dyskinesias (PCD) are rare genetic diseases with autosomal recessive transmission, affecting nearly 1/15000 births, due to a structural/functional defect of cilia. Kartagener syndrome (KS) represents 50% of PCDs and is characterised by the triad of bronchiectasis, chronic sinusitis and situs inversus. It is manifested by clinical signs of upper and lower respiratory infections or infertility. Situs inversus is often discovered incidentally. The diagnosis is confirmed by specialized centers and is based on analysis of ciliary ultrastructure by electron microscopy or ciliary beats by light microscopy/high speed video microscopy , measurement of nasal nitric oxide flow and genetic tests. Treatment is supportive and in line with recommendations for the management of cystic fibrosis or idiopathic adult bronchiectasies. It includes respiratory physiotherapy, antibiotic medication to treat infections, vaccinations and local treatment of the otorhinolaryngeal tract if necessary. We present here the case of a 23-year-old woman with a history of recurrent respiratory infections since her early age, who consulted for cough, chronic bronchorrhea and dyspnea. The evolution to respiratory failure is slowed by the early diagnosis and management of exacerbations.

Keywords

Primary Ciliary Diskynesia Kartageners Syndrome Situs Inversus Diagnosis

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popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
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influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
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impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
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