Akut pulmoner alevlenme döneminde olan kistik fibrozisli çocuklarda solunum fonksiyonlarının ve fonksiyonel kapasitenin incelenmesi

Öz

Amaç: Kistik fibrozis (KF)’li hastalarda akut pulmoner alevlenme döneminde öksürük ve balgam miktarında artış, solunum fonksiyonlarında azalma görülebilmektedir. Bu çalışmanın amacı, akut pulmoner alevlenme döneminde olan KF’li çocuklarda solunum fonksiyonlarının ve fonksiyonel kapasitenin incelenmesi idi. Yöntem: Akut pulmoner alevlenme döneminde olan 18 KF’li çocuk hasta (yaş=12,94±2,57 yıl, 8 kız (%44,4), 10 erkek (%55,6)) çalışmaya alındı. Olguların fiziksel ve klinik özellikleri kaydedildi. Solunum fonksiyonları spirometre ile fonksiyonel kapasite ise 1-dakika otur kalk testi (OKT) ile değerlendirildi. Bulgular: KF’li çocukların ortalama birinci saniyedeki zorlu ekspiratuar volüm (FEV1) (%) değeri %58,50, zorlu vital kapasite (FVC) (%) değeri %65,22, tepe ekspiratuar akım hızı (PEF) (%) değeri %62,50 ve zorlu ekspirasyonun ortasındaki akım hızı (FEF%25-75) (%) değeri %39,77 olarak bulundu. Çocukların hastalık şiddeti sınıflandırmasına göre %77,7’si hafif obstrüktif akciğer hastalığına sahipti. KF’li çocuklar hastaneye yatış sırasında yapılan 1-dakika OKT’de ortalama 24,88 kez oturup kalktılar ve beklenilen 1-dk OKT sayısının ortalama %41,87’sine ulaştılar. KF’li çocukların 1- dakika OKT sayısı ile FEV1 (%) (r=-0,041; p=0,87), FVC (%) (r=-0,085; p=0,73), PEF (%) (r=-0,216; p=0,38) ve (FEF%25-75) (%) (r=-0,215; p=0,39) değeri arasında anlamlı bir ilişki bulunmadı. Sonuç: Bu çalışmanın sonucunda akut pulmoner alevlenme döneminde olan KF’li çocukların fonksiyonel kapasitelerinin azaldığı belirlendi. Ayrıca çocukların büyük çoğunluğunda hafif şiddette obstrüktif akciğer hastalığının görüldüğü saptandı. Akut pulmoner alevlenme döneminde uygulanacak fizyoterapi ve rehabilitasyon programlarının solunum fonksiyonları ve fonksiyonel kapasiteye etkisi araştırılmalıdır.

Anahtar Kelimeler

• Kistik fibrozis
• Egzersiz testi
• Solunum fonksiyon testi
• Egzersiz kapasitesi

Investigation of pulmonary function and functional capacity in children with cystic fibrosis in acute pulmonary exacerbation period

Öz

Purpose: Patients with cystic fibrosis (CF) may have symptoms such as increased cough and sputum production, decreased pulmonary function in acute pulmonary exacerbation period. The purpose of this study was to assess pulmonary functions and functional capacity in children with CF during acute pulmonary exacerbation period. Methods: Eighteen children with CF who had pulmonary exacerbation (age=12.94±2.57 years, 8 females (44.4%), 10 males (55.6%)) were reviewed. Patients’ physical and clinical characteristics were recorded. Pulmonary functions were assessed by using spirometry. Functional capacity was evaluated by 1-minute sit to stand test (STST). Results: The mean forced expiratory volume in 1 second (FEV1) (%) was 58.50%, forced vital capacity (FVC) (%) was 65.22%, peak expiratory flow (PEF) (%) was 62.50% and forced mid-expiratory flow (FEF25-75%) (%) was 39.77% in children with CF. According to the disease severity classification, 77.7% had mild obstructive lung disease. Children with CF were performed 24.88 times sit to stand repetitions during the test. They reached 41.87% of their predicted STST repetition. No significant correlation was found between the STST repetition and FEV1 (%) (r=-0.041; p=0.87), FVC (%) (r=-0.085; p=0.73), PEF (%) (r=-0.216; p=0.38) and FEF25- 75% (%) (r=-0.215; p=0.39) in children with CF. Conclusion: All children with CF in acute pulmonary exacerbation period had decreased functional capacity. In addition, mild obstructive lung disease was found in the majority of children. The effects of physiotherapy and rehabilitation programs during the acute pulmonary exacerbation period on lung function and functional capacity should be investigated.

Anahtar Kelimeler

• Cystic fibrosis
• Exercise test
• Pulmonary function test
• Exercise capacity

Kaynakça

1. Ong T, Ramsey BW. Cystic Fibrosis: A Review. JAMA. 2023;329:1859-1871
2. Ratjen F, Döring G. Cystic fibrosis. Lancet. 2003;361:681-689.
3. Burtin C, Van Remoortel H, Vrijsen B, et al. Impact of exacerbations of cystic fibrosis on muscle strength. Respir Res. 2013;14:46.
4. Simon H. Cystic fibrosis: Management of pulmonary exacerbations [Internet]. 2024 [Erişim tarihi: 15 Nisan 2024]. Erişim adresi: https://www.uptodate.com.
5. Ulusal Kistik Fibrozis Hasta Kayıt Sistemi. 2021 yıllık rapor [Internet]. [Erişim Tarihi:15 Nisan 2024]. Erişim Adresi: https://www.kistikfibrozisturkiye.org/hasta-kayit-sistemi.
6. Konstan MW, Morgan WJ, Butler SM, et al. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatr. 2007;151:134-139.
7. Emerson J, Rosenfeld M, McNamara S, et al. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol. 2002;34:91-100.
8. Marshall BC, Butler SM, Stoddard M, et al. Epidemiology of cystic fibrosis-related diabetes. J Pediatr. 2005;146:681-687.

9. Dobbin CJ, Bartlett D, Melehan K, et al. The effect of infective exacerbations on sleep and neurobehavioral function in cystic fibrosis. Am J Respir Crit Care Med. 2005;172:99-104.
10. Heltshe SL, Goss CH, Thompson V, et al. Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis. Thorax. 2016;71:223-229.
11. Sanders DB, Bittner RC, Rosenfeld M, et al. Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation. Am J Respir Crit Care Med. 2010;182:627-632.
12. Webb AK, Dodd ME, Moorcroft J. Exercise and cystic fibrosis. J R Soc Med. 1995;88:30-36.
13. Gea J, Sancho-Muñoz A, Chalela R. Nutritional status and muscle dysfunction in chronic respiratory diseases: stable phase versus acute exacerbations. J Thorac Dis. 2018;10:S1332-1354.
14. Bell SC, Bowerman AM, Nixon LE, et al. Metabolic and inflammatory responses to pulmonary exacerbation in adults with cystic fibrosis. Eur J Clin Invest. 2000;30:553-559.
15. Ionescu AA, Nixon LS, Shale DJ. Cellular proteolysis and systemic inflammation during exacerbation in cystic fibrosis. J Cyst Fibros. 2004;3:253-258.
16. Saynor ZL, Gruet M, McNarry MA, et al. Guidance and standard operating procedures for functional exercise testing in cystic fibrosis. European Respiratory Review. 2023;32:230029.
17. Cox NS, Follett J, McKay KO. Modified shuttle test performance in hospitalized children and adolescents with cystic fibrosis. J Cyst Fibros. 2006;5:165-70.
18. Pike SE, Prasad SA, Balfour-Lynn IM. Effect of intravenous antibiotics on exercise tolerance (3-min step test)) in cystic fibrosis. Pediatr Pulmonol. 2001;32:38-43.
19. Miller MR, Hankinson J, Brusasco V, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319-338.
20. Nazer D, Abdulhamid I, Thomas R, et al. Home versus hospital intravenous antibiotic therapy for acute pulmonary exacerbations in children with cystic fibrosis. Pediatr Pulmonol. 2006;41:744-749.
21. Haile SR, Fühner T, Granacher U, et al. Reference values and validation of the 1-minute sit-to-stand test in healthy 5-16-year-old youth: a cross-sectional study. BMJ Open. 2021;11:e049143.
22. Strassmann A, Steurer-Stey C, Dalla Lana K, et al. Population-based reference values for the 1-min sit-to-stand test. Int J Public Health. 2013;58:949-953.
23. Schober P, Boer C, Schwarte LA. Correlation coefficients: appropriate use and interpretation. Anesth Analg. 2018;126:1763-1768.
24. Bouzek DC, Ren CL, Thompson M, et al. Evaluating FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis. Pediatr Pulmonol. 2022;57:1709-1716.
25. Bhatia R, Lesser DJ, Woo MS, et al. Six-minute walk test and health-related quality of life: objective tools to assess improvement in cystic fibrosis patients hospitalized for pulmonary exacerbation. Pediatr Allergy Immunol. 2012;25:86-91.
26. Troosters T, Langer D, Vrijsen B, et al. Skeletal muscle weakness, exercise tolerance and physical activity in adults with cystic fibrosis. Eur Respir J. 2009;33:99-106.
27. Godfrey S, Mearns M. Pulmonary function and response to exercise in cystic fibrosis. Arch Dis Child. 1971;46:144-1451.
28. Nixon PA, Orenstein DM, Kelsey SF, et al. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327:1785-1788.
29. Chetta A, Pisi G, Zanini A, et al. Six-minute walking test in cystic fibrosis adults with mild to moderate lung disease: comparison to healthy subjects. Respir Med. 2001;95:986-991.
30. Aurora P, Prasad SA, Balfour-Lynn IM, et al. Exercise tolerance in children with cystic fibrosis undergoing lung transplantation assessment. Eur Respir J. 2001;18:293-297.
31. Alison JA, Donnelly PM, Lennon M, et al. The effect of a comprehensive, intensive inpatient treatment program on lung function and exercise capacity in patients with cystic fibrosis. Phys Ther. 1994;74:583-591.
32. Paranjape SM, Carson KA, Demissie SM, et al. Use of the modified shuttle walk test during inpatient pediatric cystic fibrosis pulmonary exacerbation treatment. J Acute Care Phys Ther. 2018;9:136-142.
33. Rosenfeld M, Emerson J, Williams-Warren J, et al. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr. 2001;139:359-365.
34. Wieboldt J, Atallah L, Kelly JL, et al. Effect of acute exacerbations on skeletal muscle strength and physical activity in cystic fibrosis. J Cyst Fibros. 2012;11:209-215.
35. Combret Y, Boujibar F, Gennari C, et al. Measurement properties of the one-minute sit-to-stand test in children and adolescents with cystic fibrosis: A multicenter randomized cross-over trial. PloS one. 2021;16:e0246781.