Document Detail


Kartagener's Syndrome.
MedLine Citation:
PMID:  19182755     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.
Authors:
D K Dhar; K C Ganguly; S Alam; A Hossain; U K Sarker; B K Das; M J Haque
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Mymensingh medical journal : MMJ     Volume:  18     ISSN:  1022-4742     ISO Abbreviation:  Mymensingh Med J     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2009-02-02     Completed Date:  2009-08-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9601799     Medline TA:  Mymensingh Med J     Country:  Bangladesh    
Other Details:
Languages:  eng     Pagination:  75-9     Citation Subset:  IM    
Affiliation:
Department of Medicine, Mymensingh Medical College and Hospital, Mymensingh, Bangladesh.
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MeSH Terms
Descriptor/Qualifier:
Anti-Bacterial Agents / therapeutic use
Bronchodilator Agents / therapeutic use
Cilia / pathology*,  ultrastructure
Female
Humans
Kartagener Syndrome / diagnosis*,  physiopathology,  therapy
Middle Aged
Oxygen Inhalation Therapy
Chemical
Reg. No./Substance:
0/Anti-Bacterial Agents; 0/Bronchodilator Agents

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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