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Twenty years of care for cystic fibrosis adults in Czech Republic


Authors: L. Fila 1;  V. Sedlák 2;  I. Binková 3;  P. Jakubec 4;  R. Bittenglová 5;  J. Musil 1
Authors‘ workplace: Pneumologická klinika 2. lékařské fakulty UK a FN Motol Praha, přednosta doc. MUDr. Jaromír Musil, Ph. D. 1;  Plicní klinika Lékařské fakulty UK a FN Hradec Králové, přednosta doc. MUDr. František Salajka, CSc. 2;  Klinika nemocí plicních a tuberkulózy Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc. 3;  Klinika plicních nemocí a tuberkulózy Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vítězslav Kolek, DrSc. 4;  Klinika tuberkulózy a respiračních nemocí Lékařské fakulty UK a FN Plzeň, přednosta prof. MUDr. Miloš Pešek, CSc. 5
Published in: Vnitř Lék 2009; 55(6): 542-548
Category: Original Contributions

Overview

Introduction:
Most of cystic fibrosis (CF) patients survive now into adulthood and they are transferred to pulmonologist care. Aims: An overview of progress in care for CF adults in Czech Republic and evaluation of relationship of pulmonary function, nutritional status and airway colonization.

Methods:
All adult CF patients followed in pulmonary departments from December 1987 to December 2007 were included into study. Data about survival status, pulmonary function, nutritional status, airway colonization and other pulmonary and extrapulmonary manifestations of CF were collected from patients’ records.

Results:
Total of 206 patients (96 females) were followed. Pancreatic insufficiency was present in 175 (85.0%), liver disease in 61 (29.6%) and insulin treatment in 58 (28.2%) patients. Bone disease was found in 70 (46.7%) from 150 examined patients. Sixty-two patients (23 females) died at mean age 25.4 ± 5.5 years (median 24.3 years). Worse survival was recorded in patients with Burkholderia cepacia complex (BCC) airway colonization (24.4 ± 4.0 vs 28.5 ± 7.0 years, p = 0.012). One hundred forty-four living patients were followed to date of the 31st December 2007 with mean age 27.5 ± 6.5 years (median 26.5 years), FEV1 64.4 ± 28.5% pred. and BMI 20.9 ± 3.1 kg/m2. Worse pulmonary function was present in patients with BCC colonization (FEV1 58.8 ± 21.9 vs 67.8 ± 27.3% pred., p = 0.041) and in malnourished patients (FEV1 49.5 ± 18.5 vs 69.7 ± 25.9% pred., p < 0.0001). BCC colonization was found in 54 (37.5%), Pseudomonas aeruginosa (PA) colonization in 92 (63.9%) and colonization without BCC or PA in 40 (27.8%) patients, respectively. Malnutrition (BMI < 19.0 kg/m2) was recorded in 38 (26.4%) patients.

Conclusion:
This study confirms growing number of CF adults in Czech Republic, close relationship of pulmonary function and nutritional status and also unfavourable influence of BCC colonization.

Key words:
cystic fibrosis – adults


Sources

1. Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease. A clinical and pathological study. Amer J Dis Child 1938; 56: 344–399.

2. Švejcar J, Benešová D, Houštěk J. Cystická fibróza pankreatu. Čas Lék Česk 1948; 87: 1116–1122.

3. Houštěk J, Vávrová V. Cystická fibróza. In: Houštěk J, Syrovátka A (eds). Pokroky v pediatrii 7. Praha: Avicenum 1983: 139–180.

4. Vávrová V, Zemková D, Macek M Jr. Pohled na cystickou fibrózu v roce 2002. Trendy v medicíně 2002; 4: 24–34.

5. Vávrová V, Zemková D, Bartošová J et al. Cystická fibróza – nemoc dospívajících a dospělých? Čas Lék Česk 1999; 138: 654–659.

6. Hellerstein H. Cystic fibrosis of the pancreas in an adult. Ohio Med J 1946; 42: 616–617. Citace in: Widerman E, Millner L, Sexauer W et al. Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after age 18 years. Chest 2000; 118: 427–433.

7. Shwachman H, Kulczycki LL. Long‑term study of one hundred five patients with cystic fibrosis. Studies made over a five- to fourteen-year period. AMA J Dis Child 1958; 96: 6–15.

8. Netoušek M. Mucoviscidosis adultorum hereditaria. Čas Lék Česk 1960; 99: 1525–1527.

9. Ledeč J, Šimonová J. Syndrom mukoviscidózy u dospělých. Vnitř Lék 1966; 12: 525–530.

10. Marel M. Neobvyklý případ mukoviscidózy u mladého muže. Prakt Lék 1986; 66: 794–795.

11. Musil J, Rozehnalová H, Kvapil M. Deset let péče o dospělé nemocné s cystickou fibrózou. Vnitř Lék 1997; 43: 728–732.

12. Kerem E, Conway S, Elborn S et al. Consensus Committee. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005; 4: 7–26.

13. Vávrová V a kol. Cystická fibróza. Praha: Grada Publishing 2006.

14. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 1998; 132: 589–595.

15. Baldwin A, Mahenthiralingam E, Thickett KM et al. Multilocus sequence typing scheme that provides both species and strain differentiation for the Burkholderia cepacia complex. J Clin Microbiol 2005; 43: 4665–4673.

16. Drevinek P, Vosahlikova S, Cinek O et al. Widespread clone of Burkholderia cenocepacia in cystic fibrosis patients in the Czech Republic. J Med Microbiol 2005; 54: 655–659.

17. Houstek J, Hruskovic I, Vyhnálek M et al. Care for cystic fibrosis patients in Czechoslovakia. Acta Univ Carol Med (Praha) 1990; 36: 217–219.

18. Zemková D, Vávrová V, Bartošová J B. Cepacia v ČR – pro Zpravodaj CF. Zpravodaj Klubu rodičů a přátel dětí nemocných cystickou fibrózou 1998: 28–30.

19. Vávrová V, Zemková D, Bartošová J et al. Cystic fibrosis in the Czech Republic from 1960–2000. Alergie 2001; 3 (Suppl 1):S46.

20. Zemková D, Skalická V, Bartošová J et al. Moderní management cystické fibrózy a jeho vliv na zdravotní stav a přežívání českých nemocných. Čes Slov Pediatr 2008; 63: 76–82.

21. Vávrová V, Zemková D, Skalická V et al. Cystic fibrosis in Czech Republic. ECFS Newsletter 2008; 25: 4–9.

22. Stern M, Wiedemann B, Wenzlaff P. German Cystic Fibrosis Quality Assessment Group. From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995–2006. Eur Respir J 2008; 31: 29–35.

23. Bellis G, Cazes MH, Parant A et al. Cystic fibrosis mortality trends in France. J Cyst Fibros 2007; 6: 179–186.

24. Cystic Fibrosis Foundation. Patient Registry 2006 Annual Report, Bethesda, Maryland USA.

25. Cystic Fibrosis Trust. Annual Data Report 2004. Dundee: UK CF Database, University of Dundee Dundee 2006.

26. Dodge JA, Lewis PA, Stanton M et al. Cystic fibrosis mortality and survival in the UK: 1947–2003. Eur Respir J 2007; 29: 522–526.

27. Kulich M, Rosenfeld M, Goss CH et al. Improved survival among young patients with cystic fibrosis. J Pediatr 2003; 142: 631–636.

28. Yankaskas JR, Marshall BC, Sufian B et al. Cystic fibrosis adult care: consensus conference report. Chest 2004; 125 (Suppl 1): 1S–39S.

29. Orenstein DM, Winnie GB, Altman H. Cystic fibrosis: a 2002 update. J Pediatr 2002; 140: 156–164.

30. Stevens DA, Moss RB, Kurup VP et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis. State of the art: Cystic Fibrosis Foundation Consensus Conference. Clin Infect Dis 2003; 37 (Suppl 3): S225–S264.

31. Mastella G, Rainisio M, Harms HK et al.Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Epidemiologic Registry of Cystic Fibrosis. Eur Respir J 2000; 16: 464–471.

32. Flume PA, Strange C, Ye X et al. Pneumothorax in cystic fibrosis. Chest 2005; 128: 720–728.

33. Flume PA, Yankaskas JR, Ebeling M et al. Massive hemoptysis in cystic fibrosis. Chest 2005; 128: 729–738.

34. Moran A, Hardin D, Rodman D et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus. A consensus conference report. Diabetes Res Clin Pract 1999; 45: 61–73.

35. Colombo C, Russo MC, Zazzeron L et al. Liver disease in cystic fibrosis. J Pediatr Gastroenterol Nutr 2006; 43 (Suppl 1): S49–S55.

36. Aris RM, Merkel PA, Bachrach LK et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab 2005; 90: 1888–1896.

37. Edenborough FP, Borgo G, Knoop C et al. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008; 7 (Suppl 1): S2–S32.

38. Flume PA, O’Sullivan BP, Robinson KA et al. Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007; 176: 957–969.

39. Saiman L, Siegel J. Infection control in cystic fibrosis. Clin Microbiol Rev 2004; 17: 57–71.

40. Fila L. Diagnostika cystické fibrózy v dospělosti. Lék Listy 2008; 57: 22–23.

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Diabetology Endocrinology Internal medicine
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