Title |
Kartagener syndrome
|
---|---|
Published in |
International Journal of General Medicine, January 2011
|
DOI | 10.2147/ijgm.s16181 |
Pubmed ID | |
Authors |
Nedaa Skeik, Fadi I Jabr |
Abstract |
Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by pseudomonal infection. Situs inversus can be seen in about 50% of cases. Diagnosis can be made by tests to prove impaired cilia function, biopsy, and genetic studies. Treatment is supportive. In severe cases, the prognosis can be fatal if bilateral lung transplantation is delayed. We present a case of a 66-year-old woman with chronic recurrent upper respiratory infections, pseudomonal pneumonia, and chronic bronchiectasis who presented with acute respiratory failure. She was diagnosed with Kartagener syndrome based on her clinical presentation and genetic studies. She expired on ventilator with refractory respiratory and multiorgan failure. |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United Kingdom | 1 | 2% |
Spain | 1 | 2% |
Unknown | 63 | 97% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Bachelor | 13 | 20% |
Researcher | 7 | 11% |
Other | 5 | 8% |
Student > Master | 5 | 8% |
Student > Postgraduate | 4 | 6% |
Other | 12 | 18% |
Unknown | 19 | 29% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 29 | 45% |
Agricultural and Biological Sciences | 11 | 17% |
Biochemistry, Genetics and Molecular Biology | 3 | 5% |
Nursing and Health Professions | 2 | 3% |
Psychology | 1 | 2% |
Other | 1 | 2% |
Unknown | 18 | 28% |