Vnitr Lek 2009, 55(6):542-548

Twenty years of care for cystic fibrosis adults in Czech Republic

L. Fila^1,*, V. Sedlák², I. Binková³, P. Jakubec⁴, R. Bittenglová⁵, J. Musil¹

¹ Pneumologická klinika 2. lékařské fakulty UK a FN Motol Praha, přednosta doc. MUDr. Jaromír Musil, Ph.D.

² Plicní klinika Lékařské fakulty UK a FN Hradec Králové, přednosta doc. MUDr. František Salajka, CSc.

³ Klinika nemocí plicních a tuberkulózy Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc.

⁴ Klinika plicních nemocí a tuberkulózy Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vítězslav Kolek, DrSc.

⁵ Klinika tuberkulózy a respiračních nemocí Lékařské fakulty UK a FN Plzeň, přednosta prof. MUDr. Miloš Pešek, CSc.

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.

Keywords: cystic fibrosis; adults

Received: November 10, 2008; Accepted: January 20, 2009; Published: June 1, 2009  Show citation