The safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis

Introduction Airway clearance techniques, which include positive expiratory pressure (PEP) devices, are essential in the pulmonary rehabilitation of cystic fibrosis (CF). Bottle-PEP is a low-cost but an effective alternative. Objective The aim of this study is to document the sustainability and safety of Bottle-PEP therapy as a home rehabilitation aid. Methods The study has been designed as a prospective case series. Patients with CF at the age of 6-18 years in acute exacerbation period were included in the study. Bottle-PEP training was given by a competent physiotherapist to those patients who did not use any method, and those who currently use another device were followed up with their existing devices. Thus, patients divided into two groups were followed up for 1 year. The patients were evaluated by phone every 2 weeks for exacerbation, regular and proper use of the device, and satisfaction during their follow-up. The patients were evaluated every 3 months with pulmonary function tests, 6-minute walking test (6MWT) and quality of life. Results Thirty-four patients were included in the study. The acute exacerbation score of the patients was 4.5 in the Bottle-PEP group and 6 in the other group, showing no significant difference (p = .1). Treatment compliance scores were compared, the median value of the Bottle-PEP group was 24 the other group was 27 and there was no significant difference (p = .6). During follow-up of, there were no significant differences in FEV1, 6MWT and quality of life data (p > .05). Conclusion Bottle-PEP treatment is not different from other devices in terms of long-term usability and safety in patients diagnosed with cystic fibrosis

Primer siliyer diskinezili hastalarda konvansiyonel pulmoner rehabilitasyon ile yüksek frekansli göğüs duvari ossilasyonu yönteminin karşılaştırılması

ÖZET:Giriş: Primer Siliyer Diskinezili hastalarda erken tanı ve pulmoner rehabilitasyon (PR) ile mukosilyer klirensin arttırılması bronşektazi gelişiminin önlenmesi açısından önemlidir.PSD’de PR yöntemleri arasında en sık ve en etkin kullanılan yöntem konvansiyonel pulmoner rehabilitasyon (KPR) olarak adlandırılan postüral drenaj ve perküsyondur. Yeni bir teknik olan yüksek frekanslı göğüs duvarı ossilasyon (YFGDO) yöntemi kistik fibrozisli hastalarda sık kullanılan bir PR yöntemi olmakla birlikte PSD’de YFGDO kullanımı ile ilgili bir çalışma yoktur. Amaç: PSD’de KPR ve YFGDO yöntemlerinin etkinlik ve güvenilirliğini karşılaştırmaktır.Gereç ve yöntem: Marmara Üniversitesi Hastanesi Çocuk Göğüs Hastalıkları BD’de PSD tanısı ile izlenmekte olan 6 yaş üzerinde 24 hasta araştırmaya dahil edildi. Hastalara hem KPR hem de YFGDO yöntemi randomize olarak uygulandı. KPR grubuna solunum fonksiyon testi (SFT) yapıldıktan sonra çocuk servisine 5 gün süre ile yatırılarak solunum fizyoterapisti tarafından günde 2 kez 30’ar dakika süreyle PR uygulandı. Ardından aynı gruba 5 gün süre ile evde sabah ve akşam 30 dakika süreyle YFGDO yöntemi uygulandı. Diğer gruba ise önce YFGDO ardından KPR uygulandı. Her iki gruba da işlem sırasında satürasyon takibi yapıldı ve 5. günün sonunda SFT tekrarı yapıldı. İki tedavi yöntemi arasında hastaya 2 gün evde bakım vericisi tarafından PR uyguladı.Hastalara her iki yöntemle PR uygulandıktan sonra bu yöntemleri değerlendirmeleri istendi (Konfor 1-5, Etkinlik 1-5 arasında puanlandırıldı).Bulgular: Her iki PR yöntemi sonrasında hastaların SFT değerlerinde anlamlı artış saptandı. Her iki PR yöntemindeki % beklenen FVC, FEV1, PEFve FEF25-75 artış değerleri karşılaştırıldığında anlamlı fark saptanmadı. Cinsiyet, yaş dağılımı, tartı ve boy Z-skor, balgam mikrobiyolojisi ile her iki PR yöntemi sonrası % beklenen FVC, FEV1, PEF ve FEF25-75 değerlerindeki artış arasında anlamlı ilişki saptanmadı. YFGDO yöntemi hastalar tarafından daha konforlu bulundu. Her iki PR yöntemi hastalar tarafından etkin bulundu, PR sırasında desatürasyon gelişmedi. PSD hastalarının 3 ay sonraki takibinde PR’ye uyumu sorgulandığında 8 (%33) hastanın düzenli olarak sabah akşam KPR yaptığı, 13 (%54,2) hastanın ise KPR’yi düzensiz olarak yaptığı, 3 (%12,5) hastanın KPR’yi hiç yapmadığı saptandı. Hastaların çalışma sonrası % beklenen FVC, FEV1, PEF, FEF 25-75 değerlerinde anlamlı düşme oldu ve çalışma öncesi bazal değere döndüğü saptandı. Çalışmadan 3 ay sonra SFT değerleri ile çalışma sonrası SFT değerleri arasında anlamlı fark saptandı. Sonuç: Her iki PR yöntemi sonrasında SFT’de anlamlı artış saptandı. Solunum fonksiyon testleri açısından KPR ile YFGDO yöntemi arasında fark yoktu. YFGDO uygulaması hastalar tarafından daha konforlu bulundu ve iyi tolere edildi. Çalışmadan 3 ay sonra hastalar değerlendirildiğinde hastaların SFT parametrelerinde bazal değere düştüğü görüldü ve hastaların sadece %33’ünün düzenli sabah akşam KPR yaptığı saptandı. PSD’de PR yapılması ile hastalığın klinik gidişinin daha iyi olduğu bilinmekte olup hastaların yaş ve klinik durum özelliklerine göre PR yönteminde değişiklikler yapılması ve kişiye en uygun tedavi yönteminin bulunması PR uyumunu arttıracaktır. Anahtar kelimeler: Primer siliyer diskinezi, konvansiyonel pulmoner rehabilitasyon, yüksek frekanslı göğüs duvarı ossilasyonu, solunum fonksiyon testi, çocukABSTRACT:Background:Early diagnosis and enhancement of mucociliary clearance by pulmonary rehabilitation (PR) is important to prevent the development of bronchiectasis in primary ciliary dyskinesia (PCD). Percussion and postural drainage are the most commonly used PR methods. High-frequency chest wall oscillation (HFCWO) is a new PR method which creates oscillation on the chest wall and moves the secretions from small airways to the large airways. HFCWO is as effective as conventional pulmonary rehabilitation (CPR) in cystic fibrosis patients. There is no study evaluating the efficacy of HFCWO in PCD patients. Aim: The aim of this study was to evaluate the efficacy and safety of CPR and HFCWO in PCD patients.Methods: Study included 24 patients with PCD older than 6 years old; all followed in our pediatric pulmonology clinic. Both CPR and HFCWO were applied randomly. CPR group patients were hospitalized for 5 days and PR was performed 2 times a day for a period of 30 minutes by respiratory physiotherapist. Then HFCWO was applied to the same group for 5 days at home. HFCWO was applied first to the other group and then hospitalized for CPR. Oxygen saturation was monitored during PR sessions. Pulmonary function tests (PFT) were performed on the 1st and 5th day of both therapies. Patients were asked about the comfort and efficiency level after both methods. Comfort: (1: very uncomfortable, 5: very comfortable) Efficacy (1: minimally efficient, 5: maximally efficient)Results: PFT values of patients increased significantly after both PR methods. There was no significant difference in % predicted FVC, FEV1, PEF and FEF25-75 increased values with CPR and HFCWO. There was no correlation between increased values with CPR and HFCWO. There was no correlation between gender, age, distribution, weight and height Z-score, sputum microbiology and % predicted FVC, FEV1, PEF and FEF25-75 values of both PR methods. HFCWO was found more comfortable. Two PR methods were found efficient and no desaturation was occured during PR.The compliance with CPR was low 3 months after the PR sessions. Eight (33%) patients did CPR regularly morning and evening, 13 (54.2%) patients irregularly, 3 (12.5%) patients had never done CPR after the study. Therefore PFT values was significantly decreased and was returned to baseline after 3 months of CPT sessions (Decline in FVC: p=0.013, FEV1 : p=0.003, PEF: p= 0.007). Conclusion:PFTs were significantly increased after both PR methods. There were no differences in PFTs and SpO2 between the CPR and HFCWO groups. HFCWO was found more comfortable and tolerated well by the patients. Both PR methods were found efficient. When the patients were evaluated 3months afte rthe study, we determined that only 33% of patients did CPR regularly 2 times a day and the PFT values were declined to the baseline. The prognosis of PCD is known to have been better with performing regular PR. Personalized modifications should be done to the method of PR according to age and clinical status of patients. So finding the most appropriate treatment method will improve compliance with CPR.Key words: Primary ciliary dyskinesia, conventional pulmonary rehabilitation, high frequency chest wall ossilation, pulmonary function test, chil

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Background: Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF). Methods: We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection. Results: Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/10 0 0 pwCF. Incidence was higher in lung-transplanted patients (n = 23) versus non transplanted patients (n = 107) (8.43 versus 2.36 cases/10 0 0). Incidence was higher in pwCF versus the age-matched general population in the age groups < 15, 15-24, and 25-49 years (p < 0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p = 0.133). Conclusions: SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination. (c) 2021 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. This is an open access article under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/

Primer siliyer diskinezili hastalarda konvansiyonel pulmoner rehabilitasyon ile yüksek frekansli göğüs duvari ossilasyonu yönteminin karşılaştırılması

: Giriş: Primer Siliyer Diskinezili hastalarda erken tanı ve pulmoner rehabilitasyon (PR) ile mukosilyer klirensin arttırılması bronşektazi gelişiminin önlenmesi açısından önemlidir. PSD’de PR yöntemleri arasında en sık ve en etkin kullanılan yöntem konvansiyonel pulmoner rehabilitasyon (KPR) olarak adlandırılan postüral drenaj ve perküsyondur. Yeni bir teknik olan yüksek frekanslı göğüs duvarı ossilasyon (YFGDO) yöntemi kistik fibrozisli hastalarda sık kullanılan bir PR yöntemi olmakla birlikte PSD’de YFGDO kullanımı ile ilgili bir çalışma yoktur. Amaç: PSD’de KPR ve YFGDO yöntemlerinin etkinlik ve güvenilirliğini karşılaştırmaktır. Gereç ve yöntem: Marmara Üniversitesi Hastanesi Çocuk Göğüs Hastalıkları BD’de PSD tanısı ile izlenmekte olan 6 yaş üzerinde 24 hasta araştırmaya dahil edildi. Hastalara hem KPR hem de YFGDO yöntemi randomize olarak uygulandı. KPR grubuna solunum fonksiyon testi (SFT) yapıldıktan sonra çocuk servisine 5 gün süre ile yatırılarak solunum fizyoterapisti tarafından günde 2 kez 30’ar dakika süreyle PR uygulandı. Ardından aynı gruba 5 gün süre ile evde sabah ve akşam 30 dakika süreyle YFGDO yöntemi uygulandı. Diğer gruba ise önce YFGDO ardından KPR uygulandı. Her iki gruba da işlem sırasında satürasyon takibi yapıldı ve 5. günün sonunda SFT tekrarı yapıldı. İki tedavi yöntemi arasında hastaya 2 gün evde bakım vericisi tarafından PR uyguladı.Hastalara her iki yöntemle PR uygulandıktan sonra bu yöntemleri değerlendirmeleri istendi (Konfor 1-5, Etkinlik 1-5 arasında puanlandırıldı). Bulgular: Her iki PR yöntemi sonrasında hastaların SFT değerlerinde anlamlı artış saptandı. Her iki PR yöntemindeki % beklenen FVC, FEV1, PEFve FEF25-75 artış değerleri karşılaştırıldığında anlamlı fark saptanmadı. Cinsiyet, yaş dağılımı, tartı ve boy Z-skor, balgam mikrobiyolojisi ile her iki PR yöntemi sonrası % beklenen FVC, FEV1, PEF ve FEF25-75 değerlerindeki artış arasında anlamlı ilişki saptanmadı. YFGDO yöntemi hastalar tarafından daha konforlu bulundu. Her iki PR yöntemi hastalar tarafından etkin bulundu, PR sırasında desatürasyon gelişmedi. PSD hastalarının 3 ay sonraki takibinde PR’ye uyumu sorgulandığında 8 (%33) hastanın düzenli olarak sabah akşam KPR yaptığı, 13 (%54,2) hastanın ise KPR’yi düzensiz olarak yaptığı, 3 (%12,5) hastanın KPR’yi hiç yapmadığı saptandı. Hastaların çalışma sonrası % beklenen FVC, FEV1, PEF, FEF 25-75 değerlerinde anlamlı düşme oldu ve çalışma öncesi bazal değere döndüğü saptandı. Çalışmadan 3 ay sonra SFT değerleri ile çalışma sonrası SFT değerleri arasında anlamlı fark saptandı. Sonuç: Her iki PR yöntemi sonrasında SFT’de anlamlı artış saptandı. Solunum fonksiyon testleri açısından KPR ile YFGDO yöntemi arasında fark yoktu. YFGDO uygulaması hastalar tarafından daha konforlu bulundu ve iyi tolere edildi. Çalışmadan 3 ay sonra hastalar değerlendirildiğinde hastaların SFT parametrelerinde bazal değere düştüğü görüldü ve hastaların sadece %33’ünün düzenli sabah akşam KPR yaptığı saptandı. PSD’de PR yapılması ile hastalığın klinik gidişinin daha iyi olduğu bilinmekte olup hastaların yaş ve klinik durum özelliklerine göre PR yönteminde değişiklikler yapılması ve kişiye en uygun tedavi yönteminin bulunması PR uyumunu arttıracaktır. Anahtar kelimeler: Primer siliyer diskinezi, konvansiyonel pulmoner rehabilitasyon, yüksek frekanslı göğüs duvarı ossilasyonu, solunum fonksiyon testi, çocuk ABSTRACT: Background:Early diagnosis and enhancement of mucociliary clearance by pulmonary rehabilitation (PR) is important to prevent the development of bronchiectasis in primary ciliary dyskinesia (PCD). Percussion and postural drainage are the most commonly used PR methods. High-frequency chest wall oscillation (HFCWO) is a new PR method which creates oscillation on the chest wall and moves the secretions from small airways to the large airways. HFCWO is as effective as conventional pulmonary rehabilitation (CPR) in cystic fibrosis patients. There is no study evaluating the efficacy of HFCWO in PCD patients. Aim: The aim of this study was to evaluate the efficacy and safety of CPR and HFCWO in PCD patients. Methods: Study included 24 patients with PCD older than 6 years old; all followed in our pediatric pulmonology clinic. Both CPR and HFCWO were applied randomly. CPR group patients were hospitalized for 5 days and PR was performed 2 times a day for a period of 30 minutes by respiratory physiotherapist. Then HFCWO was applied to the same group for 5 days at home. HFCWO was applied first to the other group and then hospitalized for CPR. Oxygen saturation was monitored during PR sessions. Pulmonary function tests (PFT) were performed on the 1st and 5th day of both therapies. Patients were asked about the comfort and efficiency level after both methods. Comfort: (1: very uncomfortable, 5: very comfortable) Efficacy (1: minimally efficient, 5: maximally efficient) Results: PFT values of patients increased significantly after both PR methods. There was no significant difference in % predicted FVC, FEV1, PEF and FEF25-75 increased values with CPR and HFCWO. There was no correlation between increased values with CPR and HFCWO. There was no correlation between gender, age, distribution, weight and height Z-score, sputum microbiology and % predicted FVC, FEV1, PEF and FEF25-75 values of both PR methods. HFCWO was found more comfortable. Two PR methods were found efficient and no desaturation was occured during PR. The compliance with CPR was low 3 months after the PR sessions. Eight (33%) patients did CPR regularly morning and evening, 13 (54.2%) patients irregularly, 3 (12.5%) patients had never done CPR after the study. Therefore PFT values was significantly decreased and was returned to baseline after 3 months of CPT sessions (Decline in FVC: p=0.013, FEV1 : p=0.003, PEF: p= 0.007). Conclusion:PFTs were significantly increased after both PR methods. There were no differences in PFTs and SpO2 between the CPR and HFCWO groups. HFCWO was found more comfortable and tolerated well by the patients. Both PR methods were found efficient. When the patients were evaluated 3months afte rthe study, we determined that only 33% of patients did CPR regularly 2 times a day and the PFT values were declined to the baseline. The prognosis of PCD is known to have been better with performing regular PR. Personalized modifications should be done to the method of PR according to age and clinical status of patients. So finding the most appropriate treatment method will improve compliance with CPR. Key words: Primary ciliary dyskinesia, conventional pulmonary rehabilitation, high frequency chest wall ossilation, pulmonary function test, chil

Sleep disordered breathing in childhood

Sleep disordered breathing (SDB) in childhood is linked with significant end-organ dysfunction across various systems, particularly with cardiovascular, neurocognitive and metabolic consequences. If we understand the pathophysiology of SDB, diagnose it promptly and treat appropriately, we may be able to prevent morbidity associated with SDB and also save health resources around the world. In this article, we highlight articles on this topic published in medical journals in the past year

Cathelicidin (LL-37) and human 2-defensin levels of children with post-infectious bronchiolitis obliterans

IntroductionThe antimicrobial peptides (AMPs) human -defensins and cathelicidin (LL-37) are key factors in innate and adaptive immune responses of the respiratory tract and play an important role in many respiratory diseases. No data are available in the literature about the levels of these AMPs in paediatric patients with post-infectious bronchiolitis obliterans (BO). This study aimed to determine human -defensin 2 (hBD2) and LL-37 levels and compare between post-infectious BO patients and the control group. MethodThe patients diagnosed with post-infectious BO between September 2012 and 2013 and age- and-gender matched healthy controls were enrolled in this multi-center study. Serum hBD2 and LL37 levels were determined with the enzyme-linked immunosorbent assay method. ResultsSixty-three post-infectious BO patients and 65 healthy children (median age 7355 and 78.74 +/- 36.32 months, respectively) were enrolled in the study. The mean of hBD2 levels in patients and the control group were 1.06 +/- 0.24 and 0.67 +/- 0.72 ng/mL, respectively (P<0.001). The mean of LL-37 levels in patients and the control group were 72.13 +/- 29.06 and 50.10 +/- 21.98 ng/mL, respectively (P<0.001). No correlation was found between these AMPs levels and chronological age, age at the time of diagnosis, gender, Z-scores of weight and length, hospitalization numbers, the disease history before diagnosis and 25-OH D vitamin levels. ConclusionThis is the first study to demonstrate the higher levels of serum hBD2 and LL-37 levels in paediatric post-infectious BO patients. These AMPs may have important roles in the immune systems and pathogenesis of these patients

Coping and psychopathology in children with malignancy and bronchiectasis

Aim: We aimed to evaluate the coping styles and social support perceived by the children with two different chronic diseases (cancer and bronchiectasis), their mothers' coping styles and compare them with a control group without any chronic physical or psychiatric disorder. Methods: Our sample consisted of 114 children and adolescents, with an age range from 9 to 15 years. The data were collected by using schedule for affective disorders and schizophrenia for school-age children-present and lifetime version, kid-coping orientation to problems experienced (Kid-COPE), social support appraisals scale (SSAS), and COPE. Results: All three groups were similar with respect to age and sex distribution. Around 50% to 60% of the children in both patient groups had a psychiatric diagnosis. Remarkably, 30% of the children had an internalizing disorder. The most commonly used coping style by the mothers was religious coping in all groups. Kid-COPE scores did not significantly differ between groups. The scores on Family and Friend subscales of SSAS in the bronchiectasis group were significantly lower when compared with those of participants in hematology-oncology and control groups. Conclusion: Chronic medical illnesses may have a similar psychological impact on children regardless of disease-specific clinical presentations and outcomes. Future studies need to focus on identifying protective and risk factors that potentially mediate psychosocial well-being

Prevalence of sleep-disordered breathing and associated risk factors in primary school children in urban and rural environments

Introduction Sleep-disordered breathing (SDB) in primary school children is a significant problem, yet its prevalence is not well known outside large urban settings. Information on the burden and risk factors of SDB in children could be used to improve resource allocation when providing care across a large country. The objectives of this study were to assess the prevalence of SDB among school-aged children comparing rural and urban settings, and to investigate associated risk factors. Methods In this cross-sectional study, a random sample of primary school students in Turkey were selected from urban and rural areas and data were collected using the Pediatric Sleep Questionnaire, asthma, and allergic rhinitis questionnaires completed by the parents. Results Questionnaires were collected from a total of 139 schools from 58 provinces. A total of 11,013 students were contacted, and 9045 (73%) completed the study. There was no difference in the prevalence of SDB between rural and urban settings (16% vs. 15%,p = 0.612). Multivariate logistic regression analysis revealed that current wheezing, current rhinoconjunctivitis, being overweight, parental snoring, and current mold at home were significant risk factors for SDB in both rural and urban children. Current tobacco smoke exposure (OR = 1.48, 95%CI = 1.19-1.85), near roadway air pollution exposure (OR = 1.40, 95%CI = 1.108-1.791), and mold at home in the first year of life (OR = 1.68, 95%CI = 1.26-2.23) were associated with SDB in urban children. History of maternal/paternal adenotonsillectomy was a significant predictor of SDB in the rural setting (OR = 1.63, 95%CI = 1.12-2.39). Conclusion The prevalence of SDB is high in children living in both settings but associated risk factors may vary. Children residing in rural areas should also be screened for sleep-disordered breathing during routine health visits