Buradasınız

Kartagener Sendromu

Kartagener’s Syndrome

Journal Name:

Publication Year:

Keywords (Original Language):

Abstract (2. Language): 
Primary ciliary dyskinesia is an autosomal recessive, with abnormalities in ciliary structure and function in an outgoing and 20,000 live birth, is a rare disease. Kartagener’s syndrome is characterized by the combination of primary ciliary dyskinesia and situs inversus, and occurs in approximately half of patients with ciliary dyskinesia. It presented here a 12-year-old male patient had a history of recurrent respiratory tract infections and pneumonia.
Abstract (Original Language): 
Primer silier diskinezi otozomal resesif geçişli, silier yapı ve fonksiyonda anormalliklerle giden ve 20000 canlı doğumda bir görülen nadir bir hastalıktır. Kartagener sendromu primer siliyer diskinezi ve komplet situs inversus ile karekterizedir ve siliyer dissgenezilerin yarısını oluşturur. Burada, tekrarlayan solunum yolu enfeksiyonu öyküsü olan ve iyileşmeyen pnömoni nedeni ile başvuran 12 yaşındaki erkek hasta sunulmuştur.
34-36

REFERENCES

References: 

1. Kartagener M: Zur pathogenese der bronchiectasien. I
Mitteilung:bronchiectasien bei situs viscerum inversus. Betr
Klin Tuberk 1933; 83: 498-501.
2. Behrman RE. Nelson Texbook of Pediatrics. 17th ed.
Philadelphia: wb Saunders, 2000: 1391.
3. Eliasson R, Mossberg B, Camner P: The immotile-cilia
syndrome. A congenital ciliary abnormality as an etiologic
factor in chronic airway infections and male sterility. N Engl J
Med 1977; 297 (1): 1-6.
4. De Iongh RU, Rutland J: Ciliary defects in healthy subjects,
bronchiectasis, and primary ciliary dyskinesia. Am J Respir Crit
Care Med 1995; 151 (5): 1559-67.
5. Levison H, Mindorff CM, Chao J, Turner JA, Sturgess JMM,
Stringer DA. Pathophysiology of the ciliary motility syndromes.
Eur J Respir Dis Suppl 1983;127:102-17.
6. Margaret W, Leigh MD. Primary ciliary dyskinesia. Chernick V,
Boat TF (Eds). Kending’s Disorders of the Respiratory Tract in
Children. Philadelphia: WB Saunders Company; 1998:819-25.
7. Phillips GE, Thomas S, Heather S, Bush A. Airway response
of children with primary ciliary dyskinesia to exercise and
(2-agonist challenge. Eur Respir J 1998;11:1389-91.
8. Fraser RS, Müler NL, Colman N, Pare PD. Bronchiectasis and
Other Bronchial Abnormalities In: Fraser and Pare’s diagnosis
of disease of the chest. Philadelphia, WB Saunders, 1999,
2265-97
9. Pedersen M: Specific types of abnormal ciliary motility in
Kartagener’s syndrome and analogous respiratory disorders.
A quantified microphoto- oscillographic investigation of 27
patients. Eur J Respir Dis Suppl 1983; 127: 78-90.
10. Pedersen H. Absence of dynein arms in endometrial cilia:
cause of infertility? Acta Obstet Gynecol Scand 1983; 62 (6):
625-7.
11. Okutan V, Zeren H, Doğan M ve ark. Kartagener Sendromlu Bir
Olgu. Türkiye Klinikleri Tıp Dergisi 1996;16:451-4
12. Rayner CF, Rutman A, Dewar A: Ciliary disorientation alone as
a cause of primary ciliary dyskinesia syndrome. Am J Respir
Crit Care Med 1996; 153 (3): 1123-9.
13. Perraudeau M, Scott J, Walport M, Oakley C, et al. Late
presentation of Kartagener’s syndrome. Consequences of
ciliary dysfunction. BMJ 1994;308:519-21.
14. Swartz MN. Bronchhiectasis. In: Fishman AP ed. Fishman’s
pulmonary disease and Disorders. 3rd ed. McGraw-Hill
Pr;1998:2045-70.

Thank you for copying data from http://www.arastirmax.com