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Primer Siliyer Diskinezili ve Sağlıklı Çocuklarda Aerobik Kapasitenin Karşılaştırılması

Yıl 2014, Cilt: 1 Sayı: 1, 56 - 64, 10.12.2014

Öz

Özet

Amaç: Nadir görülen otozomal resesif bir hastalık olan primer siliyer diskinezinin (PCD) egzersiz kapasitesine etkisi konusundaki bilgiler sınırlıdır. Bu çalışmada, PCD olan çocuklar ve sağlıklı bireylerde aerobik kapasitenin karşılaştırılması amaçlandı.

Gereç ve yöntemler: Çalışmaya PCD tanısı alan 15 çocuk (13.1±3.1 yıl) ve onlarla yaş ve cinsiyet açısından uyumlu 15 sağlıklı çocuk (13.3±3.2 yıl) alındı. Olgulara solunum fonksiyon testi ve modifiye mekik yürüme testi yapıldı. Egzersiz testi öncesinde ve sonrasında kalp hızı, kan basıncı, oksijen satürasyonu (SaO2) ve yorgunluk kaydedildi.

Sonuçlar: İki grubun fiziksel özellikleri birbirine benzerdi (p > 0.05). PCD’li olguların zorlu vital kapasite (FVC), birinci saniyedeki zorlu ekspirasyon volümü (FEV1), zorlu ekspiratuar akımın % 25-75’i , zorlu ekspiratuar akımın % 25’i (FEF%25), zorlu ekspiratuar akımın % 50’si (FEF%50), zorlu ekspiratuar akımın % 75’i (FEF%75), tepe akım hızı (PEF), mekik yürüme testi mesafesi ve egzersiz sırasındaki zirve egzersiz kalp hızı ve SaO2 değerleri sağlıklı olguların değerlerinden anlamlı olarak daha düşüktü (p < 0.05).

Tartışma: PCD’li olgularda erken dönemde solunum fonksiyonları ve maksimal aerobik kapasite olumsuz etkilenmektedir. PCD’li hastaların egzersiz eğitiminin, egzersiz kapasitesi ve havayolu temizlenmesine etkileri araştırılmalıdır

Kaynakça

  • 1. Alpar, R. (2012). Spor Bilimlerinde Uygulamalı İstatistik (2. baskı). Detay Yayıncılık, Ankara.
  • 2. Boon, M., Jorissen, M., Proesmans, M., De Boeck, K. (2013a). Primary Ciliary Dyskinesia, An Orphan Disease. European Journal of Pediatrics, 172 (2), 151-162. doi: 10.1007/s00431- 012-1785-6.
  • 3. Boon, M., Smits, A., Cuppens, H., Jaspers, M., Proesmans, M., Dupont, L. J. ve diğerleri. (2014b). Primary Ciliary Dyskinesia: Critical Evaluation of Clinical Symptoms and Diagnosis in Patients with Normal and Abnormal Ultrastructure. Orphanet Journal of Rare Diseases, 9, 1-10. doi: 10.1186/1750-1172-9-11.
  • 4. Bradley, J., Howard, J., Wallace, E., Elborn, S. (2000). Reliability, Repeatability, and Sensitivity of the Modified Shuttle Test in Adult Cystic Fibrosis. Chest, 117 (6), 1666-1671.
  • 5. Brown, D.E., Pittman, J.E., Leigh, M.W., Fordham, L., Davis, S.D. (2008). Early Lung Disease in Young Children with Primary Ciliary Dyskinesia. Pediatric Pulmonology, 43 (5), 514-516. doi: 10.1002/ppul.20792.
  • 6. Bush, A., Chodhari, R., Collins, N., Copeland, F., Hall, P., Harcourt, J. ve diğerleri. (2007). Primary Ciliary Dyskinesia: Current State of the Art. Archives of Disease in Childhood, 92 (12), 1136-140. doi:10.1136/adc.2006.096958.
  • 7. Coelho, C.C., Aquino Eda, S., de Almeida, D.C., Oliveira, G.C., Pinto Rde, C., Rezende, I.M. ve diğerleri. (2007). Comparative Analysis and Reproducibility of the Modified Shuttle Walk Test in Normal Children and in Children with Cystic Fibrosis. Jornal Brasileiro de Pneumologia, 33 (2), 168-174.
  • 8. Cordier, J-F. (2011). Orphan Diseases. J.S.A. Lucas, W.T. Walker, C.E. Kuehni, R. Lazor, Primary Ciliary Dyskinesia. European Respiratory Monograph 54, (s. 201-217). United Kingdom: European Respiratory Society
  • 9. Cowan, M.J., Gladwin, M.T., Shelhamer, J.H. (2001). Disorders of Ciliary Motility. American Journal of Medical Science, 321 (1), 3-10.
  • 10. Hawkins, M.N., Raven, P.B., Snell, P.G., Stray-Gundersen, J., Levine, B.D. (2007). Maximal Oxygen Uptake as a Parametric Measure of Cardiorespiratory Capacity. Medicine and Science in Sports and Exercise, 39 (1), 103-107.
  • 11. Klijn, P.H., van der Net, J., Kimpen, J.L., Helders, P.J., van der Ent, C.K. (2003). Longitudinal Determinants of Peak Aerobic Performance in Children with Cystic Fibrosis. Chest, 124 (6), 2215-2219.
  • 12. Knowles, M.R., Daniels, L.A., Davis, S.D., Zariwala, M.A., Leigh, M.W. (2013).Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease. American Journal of Respiratory Critical Care Medicine, 188 (8), 913-922. doi: 10.1164/rccm.201301-0059CI.
  • 13. Madsen, A., Green, K., Buchvald, F., Hanel, B., Nielsen, K.G. (2013). Aerobic Fitness in Children and Young Adults with Primary Ciliary Dyskinesia. PLoS One, 8 (8), e71409. doi: 10.1371/journal.pone.0071409.
  • 14. McManus, I.C., Mitchison, H.M., Chung, E.M., Stubbings, G.F., Martin, N. (2003). Primary Ciliary Dyskinesia (Siewert’s/Kartagener’s Syndrome): Respiratory Symptoms and Psycho-Social Impact. BMC Pulmonary Medicine, 27 (3), 4-16. doi: 10.1186/1471-2466-3-4.
  • 15. Miller, M.R., Hankinson, J., Brusasco, V., Burgos, F., Casaburi, R., Coates, A. ve diğerleri. (2005). Standardisation of Spirometry. European Respiratory Journal, 26 (2), 319- 338. doi: 10.1183/09031936.05.00034805.
  • 16. Ozalp, O., Inal-Ince, D., Calik, E., Vardar-Yagli, N., Saglam, Melda., Savci, S. ve diğerleri. (2012). Extrapulmonary Features of Bronchiectasis: Muscle Function, Exercise Capacity, Fatigue, and Health Status. Multidisciplinary Respiratory Medicine, 7 (1), 1-6. doi: 10.1186/2049-6958-7-3.
  • 17. Ozturk, A., Mazicioglu, M.M., Hatipoglu, N., Budak, N., Keskin, G., Yazlak, Z. ve diğerleri. (2011). Reference Body Mass Index Curves for Turkish Children 6 to 18 Years of Age. Journal of Pediatric Endocrinology and Metabolism, 21 (9), 827-836. doi: 10.1515/JPEM.2008.21.9.827.
  • 18. Phillips, G.E., Thomas, S., Heather, S., Bush, A. (1998). Airway Response of Children with Primary Ciliary Dyskinesia to Exercise and Beta2-Agonist Challenge. European Respiratory Journal, 11 (6), 1389-1391. doi: 10.1183/09031936.98.11061389.
  • 19. Pianosi, P., Leblanc, J., Almudevar, A. (2005). Peak Oxygen Uptake and Mortality in Children with Cystic Fibrosis. Thorax, 60 (1), 50-54. doi: 10.1136/thx.2003.008102.
  • 20. Rogers, D., Prasad, S. A., Doull, I. (2003). Exercise Testing in Children with Cystic Fibrosis. Journal of the Royal Society of Medicine, 96 (43), 23-29.
  • 21. Schidlow, D.V. (1994). Primary Ciliary Dyskinesia (the Immotile Cilia Syndrome). Annals of Allergy, 73 (6), 457-468.
  • 22. Valerio, G., Giallauria, F., Montella, S., Vaino, N., Vigorito, C., Mirra, V. ve diğerleri. (2012). Cardiopulmonary Assessment in Primary Ciliary Dyskinesia. European Journal of Clinical Investigation, 42 (6), 617-622. doi: 10.1111/j.1365-2362.2011.02626.x.
  • 23. Wilkes, D.L., Schneiderman, J.E., Nguyen, T., Heale, L., Moola, F., Ratjen, F. ve diğerleri. (2009). Exercise and Physical Activity in Children with Cystic Fibrosis. Paediatric Respiratory Reviews, 10 (3), 105-109. doi: 10.1016/j.prrv.2009.04.001.
  • 24. Williams, C.A., Benden, C., Stevens, D., Radtke, T. (2010). Exercise Training in Children and Adolescents with Cystic Fibrosis: Theory into Practice. International Journal of Pediatrics, 2010, 1-7. doi: 10.1155/2010/670640.

A Comparison of Aerobic Capacity Between Primary Ciliary Dyskinesia and Healthy Children

Yıl 2014, Cilt: 1 Sayı: 1, 56 - 64, 10.12.2014

Öz

Abstract:

Purpose: Information regarding the effects of primary ciliary dyskinesia (PCD), a rare usually autosomal recessive disease, on exercise capacity is limited. In this study, we aimed to compare aerobic capacity of children with PCD those with age-and sex matched healthy counterparts. 

Material and Methods: Fifteen patients with PCD (13.1±3.1 years) were participated, and 15 age- and sex-matched healthy children (13.3±3.2 years) were enrolled as controls. Patients underwent pulmonary function testing, a modified shuttle walk test was performed. Heart rate, blood pressure, oxygen saturation (Sa02), and fatigue were recorded before and after the exercise test.

Results: Physical characteristics of the two groups were similar (p>0.05). The PCD subjects’ forced vital capacity (FVC), forced expiratory volume in one second (FEV1), forced expiratory flow between 25% and 75% (FEF25-75%), forced expiratory flow at 25% (FEF25%), forced expiratory flow at 50% (FEF50%), forced expiratory flow at 75% (FEF75%), peak expiratory flow (PEF),  shuttle walk test distance, and peak exercise heart rate, Sa02 values during exercise were significantly lower than those of healthy subjects (p<0.05).

Discussion: Pulmonary function and maximal aerobic capacity in patients with PCD are negatively affected at an early stage. The effects of exercise training, exercise capacity and airway clearance in PCD patients should be investigated.

Key Words: Primary ciliary dyskinesia; Pulmonary function test; Exercise test; Physical activity; Children.  

Kaynakça

  • 1. Alpar, R. (2012). Spor Bilimlerinde Uygulamalı İstatistik (2. baskı). Detay Yayıncılık, Ankara.
  • 2. Boon, M., Jorissen, M., Proesmans, M., De Boeck, K. (2013a). Primary Ciliary Dyskinesia, An Orphan Disease. European Journal of Pediatrics, 172 (2), 151-162. doi: 10.1007/s00431- 012-1785-6.
  • 3. Boon, M., Smits, A., Cuppens, H., Jaspers, M., Proesmans, M., Dupont, L. J. ve diğerleri. (2014b). Primary Ciliary Dyskinesia: Critical Evaluation of Clinical Symptoms and Diagnosis in Patients with Normal and Abnormal Ultrastructure. Orphanet Journal of Rare Diseases, 9, 1-10. doi: 10.1186/1750-1172-9-11.
  • 4. Bradley, J., Howard, J., Wallace, E., Elborn, S. (2000). Reliability, Repeatability, and Sensitivity of the Modified Shuttle Test in Adult Cystic Fibrosis. Chest, 117 (6), 1666-1671.
  • 5. Brown, D.E., Pittman, J.E., Leigh, M.W., Fordham, L., Davis, S.D. (2008). Early Lung Disease in Young Children with Primary Ciliary Dyskinesia. Pediatric Pulmonology, 43 (5), 514-516. doi: 10.1002/ppul.20792.
  • 6. Bush, A., Chodhari, R., Collins, N., Copeland, F., Hall, P., Harcourt, J. ve diğerleri. (2007). Primary Ciliary Dyskinesia: Current State of the Art. Archives of Disease in Childhood, 92 (12), 1136-140. doi:10.1136/adc.2006.096958.
  • 7. Coelho, C.C., Aquino Eda, S., de Almeida, D.C., Oliveira, G.C., Pinto Rde, C., Rezende, I.M. ve diğerleri. (2007). Comparative Analysis and Reproducibility of the Modified Shuttle Walk Test in Normal Children and in Children with Cystic Fibrosis. Jornal Brasileiro de Pneumologia, 33 (2), 168-174.
  • 8. Cordier, J-F. (2011). Orphan Diseases. J.S.A. Lucas, W.T. Walker, C.E. Kuehni, R. Lazor, Primary Ciliary Dyskinesia. European Respiratory Monograph 54, (s. 201-217). United Kingdom: European Respiratory Society
  • 9. Cowan, M.J., Gladwin, M.T., Shelhamer, J.H. (2001). Disorders of Ciliary Motility. American Journal of Medical Science, 321 (1), 3-10.
  • 10. Hawkins, M.N., Raven, P.B., Snell, P.G., Stray-Gundersen, J., Levine, B.D. (2007). Maximal Oxygen Uptake as a Parametric Measure of Cardiorespiratory Capacity. Medicine and Science in Sports and Exercise, 39 (1), 103-107.
  • 11. Klijn, P.H., van der Net, J., Kimpen, J.L., Helders, P.J., van der Ent, C.K. (2003). Longitudinal Determinants of Peak Aerobic Performance in Children with Cystic Fibrosis. Chest, 124 (6), 2215-2219.
  • 12. Knowles, M.R., Daniels, L.A., Davis, S.D., Zariwala, M.A., Leigh, M.W. (2013).Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease. American Journal of Respiratory Critical Care Medicine, 188 (8), 913-922. doi: 10.1164/rccm.201301-0059CI.
  • 13. Madsen, A., Green, K., Buchvald, F., Hanel, B., Nielsen, K.G. (2013). Aerobic Fitness in Children and Young Adults with Primary Ciliary Dyskinesia. PLoS One, 8 (8), e71409. doi: 10.1371/journal.pone.0071409.
  • 14. McManus, I.C., Mitchison, H.M., Chung, E.M., Stubbings, G.F., Martin, N. (2003). Primary Ciliary Dyskinesia (Siewert’s/Kartagener’s Syndrome): Respiratory Symptoms and Psycho-Social Impact. BMC Pulmonary Medicine, 27 (3), 4-16. doi: 10.1186/1471-2466-3-4.
  • 15. Miller, M.R., Hankinson, J., Brusasco, V., Burgos, F., Casaburi, R., Coates, A. ve diğerleri. (2005). Standardisation of Spirometry. European Respiratory Journal, 26 (2), 319- 338. doi: 10.1183/09031936.05.00034805.
  • 16. Ozalp, O., Inal-Ince, D., Calik, E., Vardar-Yagli, N., Saglam, Melda., Savci, S. ve diğerleri. (2012). Extrapulmonary Features of Bronchiectasis: Muscle Function, Exercise Capacity, Fatigue, and Health Status. Multidisciplinary Respiratory Medicine, 7 (1), 1-6. doi: 10.1186/2049-6958-7-3.
  • 17. Ozturk, A., Mazicioglu, M.M., Hatipoglu, N., Budak, N., Keskin, G., Yazlak, Z. ve diğerleri. (2011). Reference Body Mass Index Curves for Turkish Children 6 to 18 Years of Age. Journal of Pediatric Endocrinology and Metabolism, 21 (9), 827-836. doi: 10.1515/JPEM.2008.21.9.827.
  • 18. Phillips, G.E., Thomas, S., Heather, S., Bush, A. (1998). Airway Response of Children with Primary Ciliary Dyskinesia to Exercise and Beta2-Agonist Challenge. European Respiratory Journal, 11 (6), 1389-1391. doi: 10.1183/09031936.98.11061389.
  • 19. Pianosi, P., Leblanc, J., Almudevar, A. (2005). Peak Oxygen Uptake and Mortality in Children with Cystic Fibrosis. Thorax, 60 (1), 50-54. doi: 10.1136/thx.2003.008102.
  • 20. Rogers, D., Prasad, S. A., Doull, I. (2003). Exercise Testing in Children with Cystic Fibrosis. Journal of the Royal Society of Medicine, 96 (43), 23-29.
  • 21. Schidlow, D.V. (1994). Primary Ciliary Dyskinesia (the Immotile Cilia Syndrome). Annals of Allergy, 73 (6), 457-468.
  • 22. Valerio, G., Giallauria, F., Montella, S., Vaino, N., Vigorito, C., Mirra, V. ve diğerleri. (2012). Cardiopulmonary Assessment in Primary Ciliary Dyskinesia. European Journal of Clinical Investigation, 42 (6), 617-622. doi: 10.1111/j.1365-2362.2011.02626.x.
  • 23. Wilkes, D.L., Schneiderman, J.E., Nguyen, T., Heale, L., Moola, F., Ratjen, F. ve diğerleri. (2009). Exercise and Physical Activity in Children with Cystic Fibrosis. Paediatric Respiratory Reviews, 10 (3), 105-109. doi: 10.1016/j.prrv.2009.04.001.
  • 24. Williams, C.A., Benden, C., Stevens, D., Radtke, T. (2010). Exercise Training in Children and Adolescents with Cystic Fibrosis: Theory into Practice. International Journal of Pediatrics, 2010, 1-7. doi: 10.1155/2010/670640.
Toplam 24 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Sağlık Kurumları Yönetimi
Bölüm Makaleler
Yazarlar

Senem Şimşek

Deniz İnce Bu kişi benim

Aslıhan Çakmak Bu kişi benim

Ebru Kütükcü Bu kişi benim

Melda Sağlam Bu kişi benim

Naciye Yağlı Bu kişi benim

Nagehan Emiralioğlu Bu kişi benim

Uğur Özçelik Bu kişi benim

Hazal Sonbahar Bu kişi benim

Cemile Özdemir Bu kişi benim

Hülya Arıkan Bu kişi benim

Nural Kiper Bu kişi benim

Yayımlanma Tarihi 10 Aralık 2014
Gönderilme Tarihi 12 Nisan 2015
Yayımlandığı Sayı Yıl 2014 Cilt: 1 Sayı: 1

Kaynak Göster

APA Şimşek, S., İnce, D., Çakmak, A., Kütükcü, E., vd. (2014). Primer Siliyer Diskinezili ve Sağlıklı Çocuklarda Aerobik Kapasitenin Karşılaştırılması. Hacettepe University Faculty of Health Sciences Journal, 1(1), 56-64.