| Kartagener syndrome. | |
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MedLine Citation:
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PMID: 21403791 Owner: NLM Status: PubMed-not-MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Nedaa Skeik; Fadi I Jabr |
Publication Detail:
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Type: Journal Article Date: 2011-01-12 |
Journal Detail:
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Title: International journal of general medicine Volume: 4 ISSN: 1178-7074 ISO Abbreviation: Int J Gen Med Publication Date: 2011 |
Date Detail:
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Created Date: 2011-03-15 Completed Date: 2011-03-18 Revised Date: 2011-07-26 |
Medline Journal Info:
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Nlm Unique ID: 101515487 Medline TA: Int J Gen Med Country: New Zealand |
Other Details:
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Languages: eng Pagination: 41-3 Citation Subset: - |
Affiliation:
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Mayo Clinic, Rochester, MN, USA. skeik.nedaa@mayo.edu |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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