KARTAGENER SYNDROME AT THE STAGE OF COR PULMONALE: A CASE REPORT

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Tags : kartagenersyndrome, bronchiectasis, chronicsinusitis, situsinversus

Category : Cases reports

Authors : Marie Odette Rasoafaranirina, Harison Michel Tiaray, Odilon Laza, Oninalafenitra Rakotoarisoa, Martin Arnauld Fidy, Anjara Mihaja Nandimbiniaina, Davidson Rakotondrabe, Kiady Ravahatra, Jocelyn Rakotomizao, Joelson Rakotoson, Rondro Nirina Raharimanana, Soloniaina Hélio Razafimahefa

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Kartagener syndrome is highlighted by a triad of symptoms that encompassed bronchiectasis,chronic sinusitis, and situs inversus. It is a rare disease which is characterized by primaryciliary dyskinesia. Also, Kartagener syndrome is defined as genetic disorder along with autosomal recessive inheritance. We report a case of Kartagener syndrome among 30-year-oldman. The diagnostic was make on clinical and paraclinical evidences. A cough associated with
chronic bronchorrhea for about 10 years, an exertional desaturation, and signs of right heart failure marked the clinical picture of the patient. The sinus X ray -(Blondeau incidence)- and the chest-abdominal CT scan confirmed: the situs inversus, the sinusitis, as well as the bronchiectasis. Pulmonary arterial hypertension and right cavities dilatation have been observed on Doppler ultrasound. The patient has benefited of daily respiratory physiotherapy, empiric antibiotherapy based on amoxicillin clavulanic acid for 10 days, oxygen therapy, and bronchodilators. The patient's condition remained stationary with persistence of the exertional desaturation. Kartagener syndrome requires early diagnosis in order to limit the disease progression to chronic respiratory failure.