Possibilities of computed bronchophonography in the diagnosis of external respiratory dysfunction in patients with cystic fibrosis

The degree of respiratory organ injury in cystic fibrosis determines the prognosis of the disease. Objective: to evaluate external respiratory function in children with cystic fibrosis. The study enrolled 48 children followed up at the Omsk Cystic Fibrosis Center. A control group consisted of 42 non-addicted smoking children with no evidence for respiratory diseases in the history. External respiratory function was evaluated using computed bronchophonography; spirography was additionally carried out in children over 6 years of age. Computed bronchophonography revealed obstructive respiratory failure in all children with severe cystic fibrosis. Chronic respiratory tract infection with Pseudomonas aeruginosa and bronchiectasis were associated with the higher values of the acoustic work of breathing at frequencies over 5000 Hz. It was established that there was a moderate negative correlation between the value of the acoustic work of breathing in the high frequency range and the forced expiratory volume in 1 second in %. Conclusion. Computed bronchophonography could reveal obstructive external respiratory dysfunction in children less than 6 years of age. 

1. Регистр больных муковисцидозом в Российской Федерации. 2014 г. М: МЕДПРАКТИКА-М, 2015; 64. (Register of patients with cystic fibrosis in the Russian Federation. Year 2014. Moscow: MEDPRAKTIKA-M, 2015; 64. (in Russ))

2. Ашерова И.К. Снижение тяжести течения заболевания, повышение выживаемости и качества жизни больных муковисцидозом на основе совершенствования междисциплинарной специализированной помощи. Автореф. дисс. … д.м.н. М: Федер. науч.-клин. центр дет. гемато- логии, онкологии и иммунологии им. Дмитрия Рогачева, 2012; 48. (Asherova I.K. Reducing the severity of the disease, increasing the survival and quality of life in patients with cystic fibrosis by improving interdisciplinary specialized care. Avtoref. diss. … d.m.n. Moscow: Federal Research and Clinical Center Pediatric Hematology, Oncology and Immunology named Dmitry Rogachev, 2012; 48. (in Russ))

3. Castellani C., Cuppens H., Macek M.Jr. et al. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice. J Cyst Fibros 2008; 6: 8: 179–196.

4. Капранов Н.И. Муковисцидоз: медико-социальное значение, диагностика, возможности терапии. Врач 2006; 4: 12–16. (Kapranov N.I. Cystic fibrosis: medical and social importance, diagnosis, treatment possibilities. Vrach 2006; 4: 12–16. (in Russ))

5. Павлинова Е.Б., Мингаирова А.Г., Сафонова Т.И. и др. Оценка эффективности современной муколитической терапии у детей, больных муковисцидозом (двухлетний опыт применения дорназы альфа). Вопр соврем педиатр 2007; 6: 2: 102–106. (Pavlinova Ye.B., Mingairova A.G., Safonova T.I. et al. Efficiency estimate of the modern mucolytic therapy among children, suffering from cystic fibrosis (two year experience of dornase alfa application). Vopr sovremen pediatr 2007; 6: 2: 102–106. (in Russ))

6. Гембицкая Т.А., Черменский А.Г., Бойцова Е.В. Муковис- цидоз сегодня: достижения и проблемы, перспективы этиопатогенетической терапии. Врач 2012; 2: 5–8. (Gembitskaya T.A., Chermensky A.G., Boytsova E.V. Cystic fibrosis today: achievements and challenges, perspectives etiopathogenic therapy. Vrach 2012; 2: 5–8. (in Russ))

7. Каширская Н.Ю., Капранов Н.И. Место ингаляционных форм антибактериальных препаратов в терапии бактериальной инфекции легких у больных муковисцидозом. Атмосфера. Аллергология и пульмонология 2012; 3: 5–12. (Kashirskaya N.Yu., Kapranov N.I. Place of inhaled forms of antibacterial drugs in the treatment of bacterial lung infection in cystic fibrosis patients. Аtmosfera. Аllergologiya i pul'monologiya 2012; 3: 5–12. (in Russ))

8. Verhaeghe C., Delbecque K., de Leval L. et al. Early inflammation in the airways of a cystic fibrosis foetus. J Cyst Fibros 2007; 6: 4: 304–308.

9. Цыпленкова С.Э., Мизерницкий Ю.Л. Современные возможности функциональной диагностики внешнего дыхания у детей. Рос вестн перинатол и педиатр 2015; 5: 14–20. (Tsyplenkova S.E., Mizernitskiy Yu.L. Current possibilities of functional diagnosis of external respiration in children. Ros Vestn Perinatol i Pediat 2015; 5: 14–20. (in Russ))

10. Гусейнов А.А. Акустический анализ дыхательных звуков в диагностике заболеваний легких. Пульмонология 2009; 2: 51–55 (Guseinov A.A. The acoustic analysis of respiratory sounds in the diagnosis of lung diseases. Pul'monologiya 2009; 2: 51–55. (in Russ))