Kartagener syndrome in a Nigerian African--a case report and literature review. 2009

E K Chuhwak
Department of Medicine and Radiology, Faculty of Medical Sciences, University of Jos. kchuhwak@yahoo.com

BACKGROUND Kartagener syndrome is a type of primary ciliary dyskinesia. It is a rare autonomic recessive disorder with an estimated incidence of about 1 in 32,000 live births. In Nigeria, because of lack of availability of facilities for investigations in order to make a diagnosis, it has hardly been reported. OBJECTIVE This report is intended to remind clinicians of the condition of Kartagener syndrome as the patients might be missed in the presentation as our patient's diagnosis was missed and he was diagnosed to have tuberculosis. METHODS This case is that of a 43-year-old male Nigerian African, who has had an on and off productive cough, since he was 33 years of age. Eight months prior to presentation, the cough worsened and was associated with haemolysis that was followed by swelling of the feet. This swelling of the feet started two months before presentation. He had subfertility. He was found to have partial situs inversus viscerum (dextrocardia which is the hallmark of the diagnosis of Kartagener syndrome). He had lung collapse in the right lower zone, also supporting the diagnosis of Kartagener syndrome. This patient had cor pulmonale from repeated chest infection and died after 30 days of admission.

UI MeSH Term Description Entries
D007619 Kartagener Syndrome An autosomal recessive disorder characterized by a triad of DEXTROCARDIA; INFERTILITY; and SINUSITIS. The syndrome is caused by mutations of DYNEIN genes encoding motility proteins which are components of sperm tails, and CILIA in the respiratory and the reproductive tracts. Kartagener Triad,Ciliary Dyskinesia, Primary, 1,Ciliary Dyskinesia, Primary, 1, With Or Without Situs Inversus,Dextrocardia, Bronchiectasis, and Sinusitis,Kartagener's Syndrome,Kartagener's Triad,Polynesian Bronchiectasis,Siewert Syndrome,Bronchiectasis, Polynesian,Kartageners Syndrome,Kartageners Triad,Polynesian Bronchiectases,Syndrome, Kartagener,Syndrome, Kartagener's,Syndrome, Siewert
D008297 Male Males
D009549 Nigeria A republic in western Africa, south of NIGER between BENIN and CAMEROON. Its capital is Abuja. Federal Republic of Nigeria
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal

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