Bulent Karadag

Non-Cystic-Fibrosis Bronchiectasis in Children: A Persisting Problem in Developing Countries

Background: Non-cystic-fibrosis (non-CF) bronchiectasis in childhood is still one of the most common causes of childhood morbidity in developing countries. The management of these patients remains problematic, and there are few studies of long-term outcome. Objective: The aim of this retrospective study was to define the general characteristics, underlying causative factors and long-term follow-up results of non-CF bronchiectasis patients. Methods: One hundred and eleven consecutive children, diagnosed with non-CF bronchiectasis were included in the study. General characteristics and underlying causes were recorded from the medical records. Clinical outcomes were evaluated in terms of lung function tests, annual exacerbation rates and patient/parent perception of health status. Results: Mean age of the patients was 7.4 ± 3.7 years at presentation, and patients had been followed 4.7 ± 2.7 years on average. In 62.2% of the patients, an underlying etiology was identified, whereas postinfectious bronchiectasis was the most common (29.7%). In spite of intensive medical treatment, 23.4% of the patients required surgery. The annual lower respiratory infection rate has decreased from a mean of 6.6 ± 4.0 to 2.9 ± 2.9 during follow-up (p < 0.0001). Lung function tests were also found to be improved (mean FEV1% 63.3 ± 21.0 vs. 73.9 ± 27.9; p = 0.01; mean FVC% 68.1 ± 22.2 vs. 74.0 ± 24.8; p = 0.04). There was clinical improvement in both the surgical (73%) and medical (70.1%) groups (p > 0.05). Conclusion: In conclusion, bronchiectasis remains a disease of concern to pediatricians, particularly in developing countries. Infections are still important causes of bronchiectasis, and clinical improvement can be achieved by appropriate treatment. Although medical treatment is the mainstay of management, surgery should be considered in selected patients.

Management of primary ciliary dyskinesia in European children: recommendations and clinical practice

The European Respiratory Society Task Force on primary ciliary dyskinesia (PCD) in children recently published recommendations for diagnosis and management. This paper compares these recommendations with current clinical practice in Europe. Questionnaires were returned by 194 paediatric respiratory centres caring for PCD patients in 26 countries. In most countries, PCD care was not centralised, with a median (interquartile range) of 4 (2–9) patients treated per centre. Overall, 90% of centres had access to nasal or bronchial mucosal biopsy. Samples were analysed by electron microscopy (77%) and ciliary function tests (57%). Nasal nitric oxide was used for screening in 46% of centres and saccharine tests in 36%. Treatment approaches varied widely, both within and between countries. European region, size of centre and the country’s general government expenditure on health partly defined availability of advanced diagnostic tests and choice of treatments. In conclusion, we found substantial heterogeneity in management of PCD within and between countries, and poor concordance with current recommendations. This demonstrates how essential it is to standardise management and decrease inequality between countries. Our results also demonstrate the urgent need for research: to simplify PCD diagnosis, to understand the natural history and to test the effectiveness of interventions.

Flexible bronchoscopy for diagnosis and follow up of childhood endobronchial tuberculosis.

Background: In this study, our aim was to determine the clinical and bronchoscopic outcome of the endobronchial tuberculosis (ETB). Methods: Patients with suspected tuberculosis (TB) or TB patients with an inadequate response to 8 weeks of antituberculosis treatment were enrolled in the study. Results: Seventy patients were included to the study and 118 flexible bronchoscopies were performed. ETB was present in 33 (47%) patients. There was isolated compression in 14 cases, caseous lesions in 13, granuloma formation in 6, polypoid lesions in 2, adenopathy protrusion in 1, and mucosal erosion in 1 case. The mean duration of bronchoscopic resolution of endobronchial lesions was 5.50 ± 2.74 months. Mycobacterium tuberculosis was isolated from gastric lavage in 10% and from bronchoalveolar lavage in 12.8% of 70 cases. When both of the procedures were performed concurrently, the isolation rate increased to 20%. Transient hypoxia resolving with nasal O2 was observed in 3 patients as a complication of bronchoscopy. Conclusions: Bronchoscopy offered a safe and rapid means of confirming the diagnosis of ETB.

Home Ventilation for Children with Chronic Respiratory Failure in Istanbul

Background: The number of children on home mechanical ventilation (HMV) has increased markedly in Europe and North America but little is known about the HMV use and outcomes in children in Turkey. Objective: To review clinical conditions and outcome of children who were discharged from the hospital on respiratory support. Methods: Thirty-four patients assessed at the Marmara University Hospital in Istanbul who had been receiving ventilatory support at home for more than 3 months were included in the study. Results: Thirty-four patients with a median age of 5.1 years were discharged home with ventilatory support. HMV was started in 2001 at our institution and the number of children treated has increased substantially since then (2001: n = 1, 2002: n = 3, 2003: n = 3, 2004: n = 2, 2005: n = 14, 2006: n = 11).Ventilatory support was started at a median age of 1.8 years and continued for 13 months. Eleven (32.4%) patients received invasive mechanical ventilation via tracheostomy and 23 (67.6%) patients received noninvasive mechanical ventilation. Sixteen children (47.1%) were on noninvasive mechanical ventilation via nasal mask while 7 (20.6%) used a face mask. Seven (20.6%) patients received ventilatory support for 24 h and 27 (79.4%) patients were supported only during sleep. Twenty-four (70.6%) children received supplemental oxygen in addition to ventilatory support. Three patients successfully came off ventilatory support; 11 patients died during follow-up. None of the patients had home nursing and there were no life-threatening complications. Conclusions: A rapidly rising trend of HMV use in chronic respiratory failure (CRF) has been observed in this study. HMV can be safely applied in selected children with CRF with close monitoring and proper follow-up in developing countries despite the lack of home nursing.

Differences and similarities in non-cystic fibrosis bronchiectasis between developing and affluent countries

Non-CF bronchiectasis remains a major cause of morbidity not only in developing countries but in some indigenous groups of affluent countries. Although there is a decline in the prevalence and incidence in developed countries, recent studies in indigenous populations report higher prevalence. Due to the lack of such data, epidemiological studies are required to find the incidence and prevalence in developing countries. Although the main characteristics of bronchiectasis are similar in developing and affluent countries, underlying aetiology, nutritional status, frequency of exacerbations and severity of the disease are different. Delay of diagnosis is surprisingly similar in the affluent and developing countries possibly due to different reasons. Long-term studies are needed for evidence based management of the disease. Successful management and prevention of bronchiectasis require a multidisciplinary approach, while the lack of resources is still a major problem in the developing countries.

The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results

Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of the European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort). We identified eligible datasets by performing a systematic review of published studies containing clinical information on PCD, and by contacting members of past and current European Respiratory Society Task Forces on PCD. We compared the contents of the datasets, clarified definitions and pooled them in a standardised format. As of April 2016 the iPCD Cohort includes data on 3013 patients from 18 countries. It includes data on diagnostic evaluations, symptoms, lung function, growth and treatments. Longitudinal data are currently available for 542 patients. The extent of clinical details per patient varies between centres. More than 50% of patients have a definite PCD diagnosis based on recent guidelines. Children aged 10–19 years are the largest age group, followed by younger children (≤9 years) and young adults (20–29 years). This is the largest observational PCD dataset available to date. It will allow us to answer pertinent questions on clinical phenotype, disease severity, prognosis and effect of treatments, and to investigate genotype–phenotype correlations. The iPCD Cohort offers a unique opportunity to study PCD in an international retrospective cohort of >3000 patients http://ow.ly/rn0m304Jgsu

Withdrawal of inhaled steroids in children with non‐cystic fibrosis bronchiectasis

Background:  To study the effects of inhaled steroid withdrawal on bronchial hyperreactivity, sputum inflammatory markers and neutrophilic apoptosis in children with non‐cystic fibrosis (non‐CF) bronchiectasis.

Effect of night symptoms and disease severity on subjective sleep quality in children with non-cystic-fibrosis bronchiectasis.

Night‐time symptoms and hypoxemia during sleep may affect sleep quality in children with chronic lung disease such as bronchiectasis. Poor sleep quality may impair growth, learning, and emotional development of children. Our aim was to assess the sleep quality and associated factors in children with non‐cystic fibrosis bronchiectasis.

Flexible bronchoscopy as a valuable tool in the evaluation of persistent wheezing in children

BACKGROUND Persistent wheezing is a common problem in early childhood and leads to a diagnostic dilemma, excessive investigations, drug administration and additional cost. OBJECTIVE To determine the efficacy and the safety of FOB in children with persistent wheezing despite bronchodilator and inhaled steroid therapy. METHODS Patients with persistent wheezing that lasted at least 6 weeks and did not respond to bronchodilator and inhaled steroid therapy and to whom flexible bronchoscopy was performed were included to the study. RESULTS Between 1997 and 2009; 113 patients were enrolled to the study. Sixty-three percent of the children were male. Median age was 14 months at presentation and median duration of symptoms was 5 months. Bronchoscopy revealed pathological findings in 48% of the patients. Thirty-eight patients had malacia disorders, 14 had foreign body aspiration and two had external compression of airways which were later diagnosed as vascular ring. Major and minor complications were not seen in 92% of the patients while transient hypoxia was seen in 6%, stridor in 1% and tachycardia in 1% of the patients. CONCLUSION Flexible bronchoscopy provided rapid and definitive diagnosis for our patients with persistent wheezing without any major complications. This study is one of the largest studies concerning persistent wheezing. Early bronchoscopic evaluation can reduce cost by providing rapid and accurate diagnosis and preventing unnecessary investigations and drug administration. Flexible bronchoscopy is a safe procedure and should be considered in the evaluation of children with persistent wheezing.

Comparison of conventional pulmonary rehabilitation and high‐frequency chest wall oscillation in primary ciliary dyskinesia

Enhancement of mucociliary clearance by pulmonary rehabilitation (PR) is advocated in primary ciliary dyskinesia (PCD). Our primary aim was to compare the efficacy and safety of postural drainage, percussion and vibration [conventional PR (CPR)], and high frequency chest wall oscillation (HFCWO) by studying change in pulmonary function. Our secondary aim was to evaluate patient preferences regarding the two methods.