Araştırma Makalesi
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KISTIK FIBROZIS

Yıl 1993, Cilt: 46 Sayı: 4, 657 - 666, 31.12.1994

Öz

Kistik Fibrozis otozomal ressesif kalitumla gecen ve ekzokrin bezlerin fonksiyon bozuklugu ile seyreden; cogul organ tutulumlu, dlimcul
bir hastaliktir. Eskiden yalnizca cocukluk déneminin hastaligi oldugu dustintlirken, giiniimtizde tani ve tedavi imkanlarinin artmasi ile ortalama yasam siiresi 20 yila ¢ikmustir, Hastaligin prognozunu daha cok pulmoner tutulum belirler. Bu nedenle hastalarin takiplerinde pediatrist ve gégiis hastahklar1 uzmanlannuin isbirlizi é6nem tasimaktadir.

Kaynakça

  • 1. Auerbach HS Williams M Kirkpatrick JA et al : Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 2 : 686, 1985,
  • 2. Barnett KE : More news on the cystic fibrosis gene. Gastroenterology 100 : 843, 1991.
  • 3. Bauernfeinde RM Bertele RM Harms K et al : Qualitative and quantitative microbiological analysis of sputa of 102 patients with cystic fibrosis. Infection 15 : 270, 1987.
  • 4. Boland MP Stoski DS MacDonald NE Soucy P et al: Chronic jejunostomy feding with a nelemental formula in undernourished patients with cystic fibrosis. Lancet 2 ; 232, 1988.
  • 5. Colombo C Setchell KDR Pedda M et al : The effects of ursodeoxycholic acid theraphy in liver isease associated with cystic fibrosis. J Pediatr 117 : 482, 1990.
  • 6. Cotting J Lentze M Reichen J: Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and long standing cholestasis, Gut 31 : 918, 1990.
  • 7. Cutting GR Kasch LM Rosenstein BJ et al: A cluster of cystic fibrosis mutations in the first nucleotide binding domain of the cystic fibrosis conductance regülatör protein. Nature 346: 366, 1990.
  • 8. Dagh E Warner JA Besley CR Warner JO : Raised serum solubl interleukin-o receptor concentrations in cystic fibrosis patients with and without evidence of lung disease. Arch Dis Child 67 : 479, 1992.
  • 9. Di Sant’Agnese P and Davis PB: Cystic fibrosis in adults. Am J Med., 66 : 121, 1979.
  • 10. Doring G Albus A Hoiby N : Immunologic aspects of cystic fibrosis. Chest 91 - LOS, 1988.
  • 11. Ferrari M Colombo C Sebastio G et al : Cystic fibrosis patients with liver disease are not genetically distinct. Am J Hum Genet 48 : 815. 1991.
  • 12. Gaskin K Gurwitz D Durie PR et al - Improved respiratory prognosis in patients with cystic fibrosis, with normal fat absorption. J Pediatr 100 : 857, 1982.
  • 13. Gaskin K Waters DLM Howman-Giles R et al : Liver disease and common bile- duct stenosis in cystic fibrosis. N Engl J Med 318 : 340, 1988,
  • 14. Gross K Desanto A Grosfeld JL et al : Intra-abdominal complications of cystic fibrosis. J Pediatr Surg, 20 : 431, 1985
  • 15. Hoffman RD Isenberg JM and Powell (7K : Carbohydrate malabsorption is minimal in school-age cystic fibrosis in children, Dig Dis Sci, 32 : 1071, 1987.
  • 16. Hoiby N Koch : Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax 45 : 881, 1990.
  • 17. Holmes M Murphy V Taylor M Denham B : Intussusception in cystic fibrosis. Arch Dis Child 66 : 726, 1991.
  • 18. Huff DS Huagn NN and Arey JB: Atypical cystic fibrosis of the pancreas with normal] levels of sweat chloride and minimal pancreatic lesions. J Pediatr 94 : 237, 1979.

Cystic Fibrosis

Yıl 1993, Cilt: 46 Sayı: 4, 657 - 666, 31.12.1994

Öz

Cystic fibrosis is an autosomal recessive inherited multisystem disease which manifests with exocrine gland functional deficiency.
Previously it was regarded as an inherited fatal condition of infancy and childhood, recently due to advanced diagnostic methods and treatment modalities mean age of survival increased up to the twenties.
As the prognosis depends mostly on pulmonary disease, the pediatrists and pulmonologists should be in cooperation in the Management and treatment of these patients.

Kaynakça

  • 1. Auerbach HS Williams M Kirkpatrick JA et al : Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 2 : 686, 1985,
  • 2. Barnett KE : More news on the cystic fibrosis gene. Gastroenterology 100 : 843, 1991.
  • 3. Bauernfeinde RM Bertele RM Harms K et al : Qualitative and quantitative microbiological analysis of sputa of 102 patients with cystic fibrosis. Infection 15 : 270, 1987.
  • 4. Boland MP Stoski DS MacDonald NE Soucy P et al: Chronic jejunostomy feding with a nelemental formula in undernourished patients with cystic fibrosis. Lancet 2 ; 232, 1988.
  • 5. Colombo C Setchell KDR Pedda M et al : The effects of ursodeoxycholic acid theraphy in liver isease associated with cystic fibrosis. J Pediatr 117 : 482, 1990.
  • 6. Cotting J Lentze M Reichen J: Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and long standing cholestasis, Gut 31 : 918, 1990.
  • 7. Cutting GR Kasch LM Rosenstein BJ et al: A cluster of cystic fibrosis mutations in the first nucleotide binding domain of the cystic fibrosis conductance regülatör protein. Nature 346: 366, 1990.
  • 8. Dagh E Warner JA Besley CR Warner JO : Raised serum solubl interleukin-o receptor concentrations in cystic fibrosis patients with and without evidence of lung disease. Arch Dis Child 67 : 479, 1992.
  • 9. Di Sant’Agnese P and Davis PB: Cystic fibrosis in adults. Am J Med., 66 : 121, 1979.
  • 10. Doring G Albus A Hoiby N : Immunologic aspects of cystic fibrosis. Chest 91 - LOS, 1988.
  • 11. Ferrari M Colombo C Sebastio G et al : Cystic fibrosis patients with liver disease are not genetically distinct. Am J Hum Genet 48 : 815. 1991.
  • 12. Gaskin K Gurwitz D Durie PR et al - Improved respiratory prognosis in patients with cystic fibrosis, with normal fat absorption. J Pediatr 100 : 857, 1982.
  • 13. Gaskin K Waters DLM Howman-Giles R et al : Liver disease and common bile- duct stenosis in cystic fibrosis. N Engl J Med 318 : 340, 1988,
  • 14. Gross K Desanto A Grosfeld JL et al : Intra-abdominal complications of cystic fibrosis. J Pediatr Surg, 20 : 431, 1985
  • 15. Hoffman RD Isenberg JM and Powell (7K : Carbohydrate malabsorption is minimal in school-age cystic fibrosis in children, Dig Dis Sci, 32 : 1071, 1987.
  • 16. Hoiby N Koch : Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax 45 : 881, 1990.
  • 17. Holmes M Murphy V Taylor M Denham B : Intussusception in cystic fibrosis. Arch Dis Child 66 : 726, 1991.
  • 18. Huff DS Huagn NN and Arey JB: Atypical cystic fibrosis of the pancreas with normal] levels of sweat chloride and minimal pancreatic lesions. J Pediatr 94 : 237, 1979.
Toplam 18 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Yenidoğan, Kardiyoloji
Bölüm Makaleler
Yazarlar

Aydan Kansu

Yayımlanma Tarihi 31 Aralık 1994
Yayımlandığı Sayı Yıl 1993 Cilt: 46 Sayı: 4

Kaynak Göster

APA Kansu, A. (1994). Cystic Fibrosis. Ankara Üniversitesi Tıp Fakültesi Mecmuası, 46(4), 657-666.
AMA Kansu A. Cystic Fibrosis. Ankara Üniversitesi Tıp Fakültesi Mecmuası. Aralık 1994;46(4):657-666.
Chicago Kansu, Aydan. “Cystic Fibrosis”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46, sy. 4 (Aralık 1994): 657-66.
EndNote Kansu A (01 Aralık 1994) Cystic Fibrosis. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46 4 657–666.
IEEE A. Kansu, “Cystic Fibrosis”, Ankara Üniversitesi Tıp Fakültesi Mecmuası, c. 46, sy. 4, ss. 657–666, 1994.
ISNAD Kansu, Aydan. “Cystic Fibrosis”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46/4 (Aralık 1994), 657-666.
JAMA Kansu A. Cystic Fibrosis. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1994;46:657–666.
MLA Kansu, Aydan. “Cystic Fibrosis”. Ankara Üniversitesi Tıp Fakültesi Mecmuası, c. 46, sy. 4, 1994, ss. 657-66.
Vancouver Kansu A. Cystic Fibrosis. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1994;46(4):657-66.