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Advances in technology in recent years increased the long-term survival rate of patients with chronic respiratory failure. As a result, the number of patients receiving long-term home mechanical ventilation (LTHV) support is increasing worldwide. The most important step in the management of patients with home mechanical ventilation (HMV) is to ensure adequate oxygenation and ventilation. Successful care at home requires the presence of trained caregivers and accessibility to medical devices and a care team. The aims of this study were, to describe the characteristics of children on LTHV in Istanbul, to compare the patients receiving non-invasive ventilation (NIV) and invasive ventilation, and to evaluate the frequency and risk factors of hospital admission in this population.

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.

Introduction Tracheostomy care in children may be challenging, due to lack of knowledge of healthcare providers (HCPs). The aim of this study was to determine the level of knowledge of HCP who follow patients with tracheostomy and to increase this level with theoretical training and training in a simulation laboratory. Materials and Methods ISPAT (IStanbul PAediatric Tracheostomy), a multidisciplinary team for tracheostomy care was established and a training program was prepared. Participants were subjected to theoretical and practical pretests which evaluated their knowledge levels and skills for care, follow-up, and treatment of a patient with tracheostomy. After the theoretical and practical training given to the participants with a simulation model, theoretical and practical posttests were applied. Results Fifty-one HCP from nine tertiary pediatric clinics in Istanbul were enrolled in the training program. Only six (11.8%) of them had received standardized training programs previously. Regarding the theoretical tests, seven of the 33 questions were indicated as essential. The knowledge level of the participants based on the essential questions significantly increased after the training (p < 0.05 for all of the essential questions). The total number of correct answers and correct answers of three subheadings also significantly increased after the practical training (p < 0.001 for all). Ninety-five percent of the participants assessed the course as good or excellent in general. Conclusion Training in a simulation laboratory in combination with theoretical education can improve the knowledge and skills of the HCP enabling improved care of children with a tracheostomy. Simulation Center of Koc University

Background Due to advances in technology, home ventilation in children has increased in recent years. The provision of proper care for a home-ventilated (HV) child can have a strong impact on the lifestyle of caregivers. The aim of this study was to evaluate the depression and anxiety levels of the mothers of home-ventilated children during the current COVID-19 pandemic and compare them to those of mothers of healthy peers. Methods This cross-sectional study was conducted on HV children (n=21) and a control group of healthy peers (n=32) by means of a questionnaire completed by the mothers of the children of both groups. Psychometric scales, such as the Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), and the State-Trait Anxiety Inventory-Trait (STAI-T), were used to assess participants. Results During the pandemic signs of depression were present in eight (38.1 %) of the case group and eight (25%) of the healthy control (HC) group. Comparison of the BDI scores from before and during the pandemic showed no difference between mothers of the HV children (p=0.09). Scores for BDI and STAI-T were higher in the case group than in the control group, whereas there was no significant difference in STAI-S scores. Conclusion Depression and anxiety levels of mothers of HV children were found to be higher during the COVID-19 pandemic. Economic, medical and social support resources are needed in order to reduce levels of depression and anxiety and help mothers of those children dependent on technology. This article is protected by copyright. All rights reserved.

Background Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC). Objective Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC. Materials and Methods Sixty-five caregivers of children with tracheostomy were included. First, participants were evaluated with written test about TC and participated in the practical tests. Then, they were asked to participate in a standardized training session, including theoretical and practical parts. Baseline and postintervention assessments were compared through written and practical tests conducted on the same day. Results A significant improvement was observed in the written test score after the training. The median number of correct answers of the written test including 23 questions increased 26%, from 12 to 18 (p < .001). The median number of correct steps in tracheostomy tube change (from 9 to 16 correct steps out of 16 steps, 44% increase) and suctioning the tracheostomy tube (from 9 to 17 correct steps out of 18 steps, 44% increase) also improved significantly after the training (p < .001, for both). Conclusion Theoretical courses and practical hands-on-training (HOT) courses are highly effective in improving the practices in TC. A standardized training program including HOT should be implemented before discharge from the hospital. Still there is a need to assess the impact of the program on tracheostomy-related complications, morbidity, and mortality in the long term.

Introduction: The pediatric tracheostomy related mortality mostly occurs due to accidental decannulation and cannula obstruction. The caregivers may be discharged with inadequate knowledge. Aims and objectives: Simulation training can highlight skills regarding tracheostomy care. Our aim is to investigate if a standardized hands-on-training improves knowledge and skills among caregivers. Methods: Before the traning, we applied a pretest to caregivers in order to assess their knowledge and current practises. Skills were evaluated by two simulation scenarios in a patient model: cannula change and aspiration. After one hour theoretical and practical hands-on training, the same post-test was applied, skills were reevaluated regarding cannula change and aspiration with practical tests. Results: The multicenter study included 65 caregivers of patients with tracheostomy. The median score of the theoretical test including 23 questions improved from 12 to 18 (p

Introduction: Tracheostomy care in children may be challenging, due to lack of knowledge of health care providers. The aim of this study was to determine the level of knowledge of health care providers who follow patients with tracheostomy and to increase this level with theoretical education and training in a simulation laboratory. Materials and Methods: Volunteer participants were subjected to a theoretical pretest which evaluated their knowledge level for the care, follow-up and treatment of patients with tracheostomy. Then, practical testing with three subheadings evaluating the change of the tracheostomy cannula was applied. After theoretical training and one-on-one practical training given to the participants with a simulation model, theoretical and practical posttests were applied. Results: Fifty-one health care providers from six tertiary pediatric clinics in Istanbul were enrolled in the educational course. Only 6 (11.8%) of them received standardised training programme previously. Regarding the theoretical tests, seven of the 33 questions were indicated as crucial. The knowledge level of the participants based on the crucial questions significantly increased after the training (p value

Background: Chest physiotherapy (CP) is a recommended treatment modality in primary ciliary dyskinesia (PCD). Objective: Primary aim was to compare the efficacy and safety of the conventional chest physiotherapy (CCP) and oscillatory positive expiratory pressure therapy (OPEPT). Secondary aims were to compare the exacerbation rate, time until the first exacerbation, patient compliance and comfort between the two CP methods. Methods: This is a 6 month randomized, controlled crossover trial. Patients >6 years of age with PCD were randomized into two groups, first group was assigned to OPEPT (Acapella®) for 3 months while second group was assigned to CCP. Groups were crossed over to the other modality after a 15-day washout period. Pulmonary function tests (PFTs) and compliance were monitored by monthly clinic visits. Results: There was a significant increase in FEV1 , FEF25-75 , and PEF values (p = .018, p = .020, and p = .016, respectively) in the OPEPT group and in FVC values (p = .007) in CCP group compared to baseline. However PFT increase at 3rd month was not superior to each other with both physiotherapy methods. Median acute pulmonary exacerbation rate and time period until the first exacerbation were similar in both groups (p = .821, p = .092, respectively). Comfort and effectiveness of OPEPT was higher than CCP according to patients (p = .029 and p = .042, respectively). There were no adverse effects with either therapy. Conclusions: OPEPT was as effective as CCP in PCD patients. OPEPT was more comfortable and effective than CCP according to patients. OPEPT might be an efficient alternative method for airway cleareance in PCD patients.

Background The prevalence of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and developing countries in recent years. Although the main features remain similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of our recent non-CF bronchiectasis patients and to compare these with our historical cohort in 2001. Methods One hundred four children with non-CF bronchiectasis followed between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. Results Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 ± 17.1 vs. 63.3 ± 22.1 and 76.6 ± 15.1 vs. 67.3 ± 23.1, respectively; p p p = 0.001), decrease in idiopathic cases (19.2% vs. 37.8%; p = 0.03) with no change in postinfectious and immunodeficiencies as underlying etiology. Sputum cultures were positive in 77.9% of patients which was 46.9% in the historical cohort (p = 0.001). Conclusion Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD in the recent cohort.

Aim Pycnodysostosis is a rare autosomal, recessive, skeletal dysplasia caused by a mutation in the cathepsin k gene. Pycnodysostosis is characterized by short stature, characteristic facial appearance (delayed closure of fontanelles and cranial sutures, mandibular hypoplasia and angle disorder, blue sclera), and acroosteolysis of the distal phalanges. Our aim was to describe the otorhinolaryngologic findings, differential diagnoses, various treatment options, and followup in eight cases of pycnodysostosis. Method This retrospective clinical study used data from eight patients diagnosed with pycnodysostosis by a single pediatric endocrinologist primarily based on clinical and radiographic findings. All patients were referred to the otorhinolaryngology outpatient clinic by the pediatric endocrinology unit of the Marmara University between February 2013 and March 2015. Detailed medical histories were obtained in all cases and otorhinolaryngologic physical examination, blood assays, electrocardiogram, lateral skull X-rays, chest radiograph, cephalometric investigations, tympanograms, and audiograms were also carried out. Sleep videos of patients were recorded and those with upper airway problems were evaluated for sleep apnea by polysomnography. Informed consent form was obtained from the parents of all patients. Results Eight patients (7 females and 1 male) displaying proportionate dwarfism were included in the study. They had a mean age of 14.7 years (range: 13–16 y), the mean height of 141.3 cm (range 132–155 cm), and mean weight of 44.4 kg (range: 39.6–49.3 kg). All patients had facial dysmorphism with frontal bossing and the hands and feet had short digits with overlying cutaneous wrinkles that tapered off with large overriding nails. Midfacial hypoplasia and malocclusion were observed in seven of the eight patients (87.5%), four (50%) had micrognathia, and five (62.5%) had proptosis. Tympanograms and audiograms of all patients were type A and normal, and the mean of the pure tone audiogram was 13.3 dB (range: 10–16 dB). All patients had a narrow and grooved palate with disturbed dentition; two of them (25%) had mild markedness of the tongue base, five (62.5%) had grade 3 and three (37.5%) had grade 2 tonsillar hypertrophy, and five (62.5%) had adenoid hypertrophy. One patient (12.5%) had grade 3 Mallampati, four (50%) showed grade 2 Mallampati while three (37.5%) patients displayed grade 1 Mallampati score. Further, while six (75%) patients had no uvular pathology, one (12.5%) patient presented with uvular elongation and another patient had a bifid uvula. Cephalometric measurements such as PAS-UP (mean 5.67 mm; range: 5.0–7.6 mm) and PAS-TP (mean 9.61 mm; range: 8.5–12.2 mm) were lower than that of normal subjects. Video recordings showed that six of the eight patients (75%) had respiratory distress and four (50%) had sleep apnea. Polysomnography in these patients with sleep apnea showed that two had mild OSA (AHI: 18.2 and 20.1 events/hour) and two had severe OSA (AHI: 53.4 and 62.8 events/hour). For upper airway problems, an adenotonsillectomy was performed in two (25%) patients while two others required an adenoidectomy. Positive pressure ventilation was recommended in two patients with persistent sleep apnea after adeno/adenotonsillectomy. However, because of the parental objections, the follow-up polysomnographs could not be obtained. Conclusion Pycnodysostosis is a very rare form of bone dysplasia. Otorhinolaryngologically, proper follow-up of these patients and appropriate treatment of upper airway problems are important to achieve an acceptable quality of life. Adeno/adenotonsillectomy and positive pressure ventilation, used as conservative approaches in treating upper airway problems, are effective and could be used instead of an aggressive surgery such as tracheotomy or maxillomandibular advancement. This study, to the best of our knowledge, is the largest ENT case series on pycnodysostosis.

Introduction Bronchiolitis obliterans (BO) is mainly caused by infections and hematopoietic stem cell transplantation (HSCT). This study aimed to investigate the health-related quality of life (HRQOL) of children with BO compared to the healthy children and also to assess the HRQOL according to the etiology. Methods Postinfectious (group 1) and post-HSCT BO (group 2) patients and healthy children were included in the study. HRQOL was assessed by the Short Form-36 (SF-36) and St George's Respiratory Questionnaire (SGRQ). Correlations between demographic and clinical characteristics, pulmonary function tests, high-resolution chest tomography scores, and HRQOL were assessed. Results Thirty-seven postinfectious and post-HSCT BO patients and 34 healthy children were included in the study. Mean age was 13.8 +/- 0.7 years. Mean forced vital capacity and forced expiratory volume(1)were 60.7 +/- 2.7% predicted, and 49.8 +/- 3.1% predicted, respectively. The SF-36 scores were lower in BO patients compared to healthy children (P < .01). Patients with better lung functions had higher SF-36 scores, but lower SGRQ. The number of inhaled therapies, acute exacerbations, hospitalizations were inversely correlated with SF-36. A positive correlation was found between these parameters and total SGRQ scores (r = .507,P = .02;r = .409,P = .12;r = .326,P = .049, respectively). SF-36 scores were better in group 1 for subscales of physical role functioning and social role functioning compared to group 2. (P = .01,P = .01, respectively). Conclusion The HRQOL of patients with BO measured by SF-36 was low compared to healthy children. SF-36 scores were more affected in post-HSCT BO patients. HRQOL of children with chronic lung disease should be taken into consideration in the management of these patients.

Background The diagnosis of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and nondeveloped countries in recent years. Although the main features are similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of non-CF bronchiectasis patients and to compare these data with our previous cohort in 2001. Methods 104 children with non-CF bronchiectasis being followed-up between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and distribution of microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months of the follow-up. Results Mean FEV1 and FVC % of predicted at presentation were improved compared with previous study (76.6±17.1 vs. 63.3±22.1 and 76.6±15.1 vs. 67.3±23.1, respectively; p

Introduction and Aim: Since January 1, 2015, newborn screening program has been implemented in our country. The aim of this study is to report the clinical and genotype characteristics of patients who were diagnosed as CF after false negative CFNBS results since January 2015. Material and Methods: 1. The IRT value is greater than 90 ng/ml and 2. IRT value greater than 70 were considered suspicious and referred to a sweat test. The sweat test value above 60 mmol/L was considered positive. Results: 63 patients were diagnosed as CF in our clinic between 2015-2018. While 84.2% (n = 53) of the patients were diagnosed by positive CFNBS result, 15.8% of the patients (n = 10) had false negative results of IRT values. Of the 10 patients included in the study, 50% were girls, with a mean age of 30.7 ± 8.8 months (12-39 months); The mean age of diagnosis was 152 ± 147 days (28-480 days). Genetic mutation was detected in all patients. The first IRT level was above 90 µg/l in 40% (n = 4) of the patients. 40% of the patients had pancreatic insufficiency and Pseudobartter was detected in 70% of patients. Pseudomonas aeroginosa growth in sputum culture was detected in 60% of patients whereas MRSA growth was 10%. 30% of the patients had anemia. Conclusion: Regardless of the results of the newborn screening tests, patients with symptoms supporting CF should be further evaluated. For the patients with intermediate sweat test results, genetic analysis should be performed for CF. As seen in our study, it should be kept in mind that IRT negativity may also occur in mutations that cause severe CF.

Primary ciliary dyskinesia (PCD) is a genetic disease characterized by impaired mucociliary function. Immunofluorescence (IF) staining of ciliary proteins is gaining importance in the diagnosis of PCD in recent years. In this study the results of IF of 36 Turkish patients with suspected PCD were reported. Methods: Thirty six patients were included in the study. Nasal brush samples were evaluated by IF staining with antibodies against DNAH5, GAS8, DNAH11, RSPH9 proteins. In some cases, further IF analyses were performed tackling the proteins DNALI1 and CCDC39. Results: Mean age of patients was 12,9±0,7 years. In 64% of patients, the symptoms started in the neonatal period. Twenty two patients had consanguinity between parents. Fifty-two percent of the patients had dextrocardia. Out of 36 patients, 12 had an outer dynein arm defect, 7 had an inner dynein arm defect, 7 had an N-DRC defect and 4 had a radial spoke defect. In 6 patients(16.6%), no defect was detected by IF examination. Comparison of ciliary defects with clinical features did not reveal significant difference in lung function tests, the number of antibiotic usage and hospitalization with IF defect type(p>0.05). Conclusion: Examination of ciliary proteins by IF analysis is a faster and a cheaper diagnostic tool for PCD. In this study, 30 patients out of 36 could be confirmed to have the disease, emphasizing the power of IF in the diagnosis of PCD. The remaining 6 individuals should be subject to further IF analyses and complementary diagnostic tests.

Introduction: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by impaired mucociliary function. Conventional chest physiotheraphy(CCPT) and vibratory positive expiratory pressure therapy system(Acapella®) are commonly used treatment options for chest physiotherapy. The purpose of this study is to investigate the efficacy and safety of Acapella® in patients with PCD. Materials and Methods: Patients older than 6 years of diagnosed with PCD were randomized into two groups according to physiotherapy method. First group used Acapella® for the first three months while second group used conventional chest physiotheraphy (CCPT). At the end of the 3 months; study groups switched their physiotherapy methods. Pulmonary function test (PFT) were performed. The exacerbation rate and the time period until the first exacerbation were recorded. Reults: Thirty patients were included in the study. There was no significant difference between the numbers of exacerbations of patients using Acapella® and CCPT (p: 0.821). The mean time from the start of the study to the first exacerbation in Acapella® group was 33.75 ± 17.55 days; in CCPT group was 31.00 ± 24.69 days (p: 0.09). There was a significant increase in PEF, FEV1 and FEF25-75 values during the use of Acapella®(p:0.047, 0.015, 0.061). There was no statistically significant PFT change between CCPT and Acapella® at baseline and 3 months of airway clearance with one of the methods (p> 0.05). At the end of study, patients reported that all of them preffered Acapella®. Conclusion: Acapella® was found to be equally effective with CCPT in PCD patients.

Introduction: Children with bronchopulmonary dysplasia (BPD) have an increased risk for persistent structural and functional lung abnormalities. The multiple breath washout (MBW) test assesses efficiency of ventilation distribution and the lung clearance index (LCI) is considered a sensitive marker of ventilation inhomogeneity. Aim: To determine LCI and functional residual capacity (FRC) in infants with BPD and assess the correlations of LCI and FRC with lung structure and clinical parameters. We hypothesized that LCI would be elevated in infants with BPD and would correlate with structural changes. Methods: Preterm infants born ≤32 weeks gestational age (GA) with BPD were included. Demographics and clinical parameters were obtained from medical records. LCI and FRC were measured by MBW. Lung structure was assessed on chest CT with the PRAGMA-BPD scoring system. Volumes and percentages of regions with normal appearance, low attenuation, high attenuation and bronchial thickening were determined. Results: 15 infants with a median corrected age [IQR] of 7.0 [2.5] months were included. Median GA was 26.9 [1.3] weeks and birth weight was 907.8 [240] g. 14 infants had severe and one had mild BPD. Mean LCI was 7.2±0.7 and FRC was 162±52 ml. Only in 3 patients MBW was abnormal. Architectural distortion was present in all CTs. Median total lung volume was 238,7 [92.9] ml and healthy lung volume was 226,7 [108] ml. LCI and FRC did not correlate with lung volumes on CT and architectural distortion. FRC % predicted strongly correlated with GA (r=0.726, p Conclusion: LCI and FRC are not useful in the follow-up of patients with BPD.

Introduction and aim Obstructive sleep apnea syndrome (OSAS) is frequent in children with Down syndrome (DS) and polysomnography (PSG) is recommended for all children with DS. However PSG is not always available and alternative diagnostic methods are needed. The aim of the study was to evaluate the feasibility and validity of home polygraphy (HPG) in children with DS. Methods A national DS association was contacted and children aged 6 to 18 years who accepted to participate were recruited. Otorhinolaryngologic evaluation, in-lab PSG and HPG were performed. OSAS was diagnosed by PSG with an apnea-hypopnea index (AHI) more than or equal to 1. OSAS severity was classified as moderate-to-severe if AHI was more than or equal to 5. Receiver operating characteristic curves were calculated for HPG using PSG as the gold standard. Results Nineteen children (12 girls) completed the study. Median age was 11.3 years. Demographic and clinical characteristics were similar in children with and without OSAS. Eighty-nine percent of HPG studies were technically acceptable at the initial night and the success rate was 100% when two failed studies were repeated. PSG revealed OSAS in six (32%) children, two had mild and four had moderate-to-severe OSAS. All four patients with moderate-to-severe OSAS diagnosed with PSG have been diagnosed with the same severity on HPG. HPG had 100% sensitivity and 83% specificity when AHI ≥ 3 was set as diagnostic criteria. Conclusion HPG is a feasible and reliable test of OSAS in children with DS and may be useful in diagnosis and treatment of patients with moderate-to-severe OSAS in this patient group.

BACKGROUND Cystic fibrosis (CF) genotyping has garnered increased attention since the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 led to the identification of over 1700 mutations on chromosome 7. Yet, little is known about the genetic profile of CF patients in Turkey. This study sought to determine the mutation distribution among CF patients seeking care at Marmara University. METHODS Two hundred fifty previously diagnosed CF patients were included in the study. CFTR gene exons 1 to 27 were amplified by a polymerase chain reaction and whole DNA sequencing was performed. Duplications and deletions were investigated by the multiplex ligation-dependent probe amplification (MLPA) technique in patients with one or two unidentified mutations in sequence analysis. RESULTS CFTR mutation analysis revealed 80 mutations and five large deletions were present in our study population. The five most common mutations were (delta) F508 (c.1521-1523delCTT) (28.4%), 1677delTA (c.1545-1546delTA) (6.4%), 2789 + 5G- > A (c.2657 + 5G > A) (5.8%), N1303K (c.3909C > G) (2.4%), and c.2183AA- > G (c.2051-2052delAAinsG) (4.0%). Large deletions were found in 16 patients. Four novel mutations and two novel deletions were detected in this study. CONCLUSIONS We have identified four novel mutations and two novel deletions using next-generation DNA sequencing and the MLPA technique and obtained an overall mutation detection rate of 91.4%. Detection of novel variants in CF patients will assist in genetic counseling and in determining appropriate patients for new therapies.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss. On physical examination, auscultation revealed diminished breath sounds on the upper lobes. Chest radiograph showed apical pleural thickening and volume loss. Computerized tomographic scan (CT) of chest revealed ground glass densities and tubular bronchiectasis predominantly in upper lobes bilaterally, with interlobular septal thickening of the pleura and enlarged mediastinal lymph nodes. Thoracoscopic lung biopsy was performed and histological evaluation showed subpleural fibrosis and elastic staining demonstrated fragmented elastic fiber deposition in the subpleural area and adjacent pulmonary parenchyma suggesting IPPFE. To our knowledge this is the first case in childhood. Therefore, pediatricians should be aware of this disease for the diagnosis and appropriate management.La fibroelastosis pleuroparenquimatosa idiopàtica (FEPPI) es un trastorno raro incluido recientemente en las neumonías intersticiales idiopáticas según la actualización de la clasificación de la Sociedad Torácica Estadounidense (American Thoracic Society) y la Sociedad Respiratoria Europea (European Respiratory Society). La FEPPI se caracteriza por fibrosis parenquimatosa pleural y subpleural que produce reducción del volumen, sobre todo en los lóbulos pulmonares superiores. La edad al momento de la aparición varía, aunque la FEPPI ocurre principalmente entre los 30 y los 50 años de edad. En este artículo, presentamos el caso de un paciente de 16 años con antecedentes de disnea de esfuerzo, tos seca y pérdida de peso en los últimos dos años. Durante el examen físico, con la auscultación se detectó disminución de los ruidos respiratorios en los lóbulos superiores. En la radiografía de tórax se observó engrosamiento pleural apical y volumen reducido. En la tomografia computarizada (TC) del tórax se observaron densidades en vidrio esmerilado y bronquiectasia tubular predominantemente en ambos lóbulos superiores, con engrosamiento septal interlobulillar de la pleura y adenopatía mediastínica. Se realizó una biopsia pulmonar por toracoscopia y en el examen histológico se observaron fibrosis subpleural y tinción de las fibras elásticas que demostraba depósito de fibras elásticas en el área subpleural y el parénquima pulmonar adyacente, lo que sugería FEPPI. Hasta donde sabemos, este es el primer caso durante la niñez. Por lo tanto, los pediatras deben estar atentos a esta enfermedad para realizar un diagnóstico y tratamiento adecuados.

Introduction: Bronchiolitis obliterans (BO) is mainly caused by infections and in certain cases after hematopoietic stem cell transplantation (HSCT). Aim of this study is to evaluate the quality of life (QoL) in children with BO. Methods: 23 BO (post infectious and HSCT) patients were included to the study. QoL was assessed by Short Form-36 (SF-36) and St George9s Respiratory Questionnaire (SGRQ). Correlation between demographic and clinical data of patients, pulmonary function tests (PFT) and QoL were assessed. Results: Mean age was 14,5±4,4 years, and follow up time was 7,6±4,9 years. Five of the patients (%21,7) had HSCT as the underlying etiology versus 18 (%78,3) had post infectious BO. QoL assessment, according to the SF-36, revealed a moderate correlation between FEV1 and social role functioning (r:0,43, p:0,04). FEV1 was found to be inversely correlated with SGRQ impact and total scores. Number of exacerbations were moderately correlated with SGRQ symptom scores and total scores (r:0,47, p:0,02). Number of hospital admissions were significantly correlated with SF-36 physical functioning, emotional role functioning, social role functioning and SGRQ symptom and total score. In HSCT group, hospital admissions within last year was significantly higher than the post infectious BO group (p:0,01).SF-36 physical role functioning and social role functioning scores were significantly lower in the HSCT group (p:0,01). Discussion: QoL measures were significantly reduced in BO patients. QoL was found to be decreased in patients with lower PFTs and increased number of hospital admissions. Early initiation of respiratory treatments and regular follow-up may improve the QoL in BO patients.

Aim Sleep disordered breathing is a common problem in childhood that encompasses a spectrum of disorders extending from primary snoring to obstructive sleep apnea. This study aims to investigate the results of children undergoing evaluation with polysomnography in the sleep laboratory of a tertiary care hospital. Material and methods Demographic and clinical features as well as sleep associated symptoms, scores of pediatric sleep questionnaire and Pittsburgh sleep quality index and polysomnography results are retrospectively evaluated. Results Totally 131 patients were evaluated, of which 47.3% (n=62) were females and 52.7% (n=69) were males. Mean age was 101.85±59.15 months at the time of the study. Fifty percent (n=59) of patients complained of snoring and 43.7% (n=52) of patients complained of apnea during sleep. Mean obstructive hypopnea-apnea index was 5.12±11.72. Mean obstructive hypopnea-apnea index of snorers (6.93±13.53) was significantly higher than the mean obstructive hypopnea-apnea index of nonsnorers (2.32±5.43) (p=0.011). Mean obstructive hypopnea-apnea index of patients experiencing apnea during sleep (7.52±14.25) was significantly higher than the mean obstructive hypopnea-apnea index of the children who do not experience apnea (2.61±5.84) (p=0.008). No significant correlation was observed between obstructive hypopnea-apnea index and scores of pediatric sleep questionnaire and Pittsburgh sleep quality index. The prevalence of obstructive sleep apnea was 33.6% (n=44). Forty nine patients (39.8%) were treated after polysomnography. Frequently suggested treatment options were noninvasive mechanical ventilation (n=23, 46.9%), intranasal steroid (n=15, 30.6%), montelukast (n=11, 22.4%) and adenotonsillectomy (n=9, 18.4%). Conclusions Polysomnography is the gold standard in the diagnosis of sleep disordered breathing in children. Pediatricians should be able to recognize early signs and symptoms of sleep disordered breathing and refer the patients in risk to centers where evaluation with polysomnography is available.

INTRODUCTION: Infantile hemangiomas are the most common cause of tumors in children. They usually located on head and neck. Airway hemangiomas usually cause respiratory symptoms, especially subglottic hemangiomas that early diagnosis and treatments are very important. AIM AND METHODS: To examine of the features of airway hemangiomas and to determine of the approach of diagnosis and treatment. The data of 8 patients obtain from several center from retrospectively. RESULTS: Three of patients were girl (% 37.5), and 5 of the patients were boy (% 63.5). Average age of diagnosis was 5.2 ± 5.4 months (1-17 months). Average duration of follow up was 18,6±16 (3-48 months). The primary symptom was stridor in 7 of patients (% 87,5) and hemoptysis in 1 of patients. All of patients were performed with flexible bronchoscopy. 4 of patients required screening method like magnetic resonans (MR) for neck to confirm diagnosis. Medical treatment was applied to 7 patients, 1 of the patients were treated with surgical. The choices of treatment were propronalol+prednisolon, propronalol+metilprednisolon, metilprednisolon, propranolol; 3, 2, 1,1, respectively. All of patients were still on treatment. During follow up 5 of the patients were asymptomatic, 3 of the patients were symptomatic as stridor when there was an upper respiratory tract infection. CONCLUSION: Airway hemangiomas are an important differential diagnosis of stridor in infants. Early diagnosis and treatment of airway hemangiomas are very important. The first choice of treatment is usually medical but surgical treatment may perform firstly in proper cases as an alternative treatment choice.

Background: Oral hygiene significantly affects children9s well-being. Effective oral health care is important for ventilated patients. Oropharyngeal colonization is associated with several systemic diseases, including cardiovascular diseases and lung infections. The aim of the study was to observe and compare the dental health by using decayed, missing, filled teeth (DMF-T) index, gingival and plaque index in home ventilated children with healthy controls. Materials&Methods: Eleven home ventilated children who were followed at Marmara University Pediatric Pulmonology Department and 14 healthy controls were recruited to the study. Clinical examination was performed in each child and the dental status was determined by DMF-T index. Level of oral hygiene was determined by gingival and plaque index according to criteria described by Silness and Loe. Statistical analysis was performed using SPSS version 21.0. Results: Home ventilated children had higher DMF-T index compared to healthy controls although the difference was not statistically significant ( p=0.275). Gingival index and plaque index was significantly higher in home ventilated children compared to healthy controls (p = 0.024 and p = 0.001 respectively). Conclusion: Many systemic diseases increase the risk for oral diseases. Home ventilated children have high prevalence of dental caries and poor oral hygiene compared to healthy controls. The inability to maintain adequate oral hygiene by themselves and effect from medications may explain the high incidence of the oral diseases in this population. Strategies to promote physician-dentist collaborations and family education are needed to improve continuity of oral health for ventilated children.

Introduction: Interstitial lung disease (ILD) in childhood is a group of diseases characterized by interstitial and alveolar inflammation and fibrosis. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare ILD which have not been reported in children. It is characterized by predominantly upper lobe pleural thickening and subpleural elastosis and intraalveolar collagenous fibrosis. Case: 15-year-old male presented with dry cough, exertional dyspnea and weight loss since two years. Physical examination revealed failure to thrive, and tachypnea with severe restriction (FVC 30%, FEV1 32%, FEV1 / FVC 109%) findings on pulmonary function test. Total lung capacity and CO diffusion capacity were low. Thorax tomography revealed pleural thickening and subpleural fibrosis mainly in the upper lobes and bronchiectasis. Interstitial lung disease was considered due to clinical and laboratory findings. Lung biopsy was performed. Histopathologic diagnosis was consistent with pleuroparenchymal fibroelastosis. Oral prednisolone was started and patient was referred to lung transplantation clinic. He is currently followed with supportive treatment. Conclusion: Although interstitial lung disease is rare in childhood it must be considered in children with chronic cough, exertional dyspnea, persistent tachypnea, failure to thrive and diffuse radiologic findings. This case showed that idiopathic pleuroparenchymal fibrosis can present during childhood.

Although voriconazole, a triazole antifungal, is a safe drug, treatment with this agent is associated with certain adverse events such as hepatic, neurologic, and visual disturbances. The current report presents two cases, one a 9-year-old boy and the other a 17-year-old girl, who experienced neurologic side effects associated with voriconazole therapy. Our aim is to remind readers of the side effects of voriconazole therapy in order to prevent unnecessary investigations especially for psychological and ophthalmologic problems. The first case was a 9-year-old boy with cystic fibrosis and invasive aspergillosis that developed photophobia, altered color sensation, and fearful visual hallucination. The second case was a 17-year-old girl with cystic fibrosis and allergic bronchopulmonary aspergillosis, and she experienced photophobia, fatigue, impaired concentration, and insomnia, when the dose of voriconazole therapy was increased from 12 mg/kg/day to 16 mg/kg/day. The complaints of the two patients disappeared after discontinuation of voriconazole therapy. Our experience in these patients reminded us of the importance of being aware of the neurologic adverse events associated with voriconazole therapy in establishing early diagnosis and initiating prompt treatment. In addition, although serum voriconazole concentration was not measured in the present cases, therapeutic drug monitoring for voriconazole seems to be critically important in preventing neurologic side effects in pediatric patients.

Introduction: Pulmonary complications (PC) are significant causes of morbidity and mortality after hematopoietic stem cell transplantation (HSCT) in children. PC can occur due to infectious and noninfectious causes. In our study we aimed to evaluate pulmonary functions and complications in children after HSCT. Method: 23 children who underwent HSCT at Bahcesehir University, Pediatric Hematology Department between 2011-2014 were enrolled in the study. Demographic characteristics, spirometry tests, lung volumes, CO diffusion capacity, 6-minute walk test results and pulmonary complications were evaluated. Results: 17 patients (74%) were male, mean age was 10.4 ± 4.6 years. Age at HSCT was 8.0 ± 4.7 years, average time after HSCT was 2.4 ± 1.1 years. Indication for HSCT was thalassemia major in 11 patients, acute lymphoblastic leukemia in 5 patients, acute myeloid leukemia in 3 patients, aplastic anemia in 2 patients, severe combined immunodeficiency in one patient and dyskeratosis congenita in one patient. Infectious or noninfectious PC was observed in 12 (52.2%) children (pneumonia: 9 pts, bronchiolitis obliterans: 2 pts, restrictive lung disease: 1 pt). PC developed at mean 6 months (range 1-34 months) after transplantation. No significant diffference was observed in pulmonary function tests and 6-minute walk test results of patients with or without pulmonary complications. Conclusion: Our study showed that pulmonary complications are frequent after HSCT. Failure to demonstrate differences between groups may be due to the low number of cases. Close monitoring and identifying patients at risk is important since early diagnosis and treatment of PC reduces morbidity and mortality.