Chuhwak E K
Department of Medicine and Radiology, Faculty of Medical Sciences, University of Jos.
Niger J Med. 2009 Oct-Dec;18(4):424-7. doi: 10.4314/njm.v18i4.51258.
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.
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.
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.
卡塔格内综合征是原发性纤毛运动障碍的一种类型。它是一种罕见的常染色体隐性疾病，估计活产发病率约为1/32000。在尼日利亚，由于缺乏用于诊断的检查设施，该病鲜有报道。
本报告旨在提醒临床医生注意卡塔格内综合征的情况，因为患者在就诊时可能会被漏诊，就像我们的患者最初被误诊为肺结核一样。
该病例为一名43岁的尼日利亚男性非洲人，自33岁起就时有咳痰性咳嗽。就诊前8个月，咳嗽加重，并伴有溶血，随后出现足部肿胀。足部肿胀在就诊前两个月开始。他存在生育力低下的情况。他被发现有部分内脏反位（右位心，这是卡塔格内综合征诊断的标志）。他右下肺叶出现肺不张，这也支持卡塔格内综合征的诊断。该患者因反复肺部感染导致肺心病，入院30天后死亡。
