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Öğe Health-related quality of life in patients with bronchiolitis obliterans(Wiley, 2020) Atağ, Emine; Baş İkizoğlu, Nilay; Ergenekon, Pınar; Kalın, Sevinç; Ünal, Füsun; Gökdemir, Yasemin; Erdem Eralp, Ela; Yalçın, Koray; Öktem, Sedat; Ersu, Refika; Karakoç, Fazilet; Karadağ, BülentIntroduction 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.Öğe Home mechanical ventilation in children: The experience of pediatric pulmonology divisions in Istanbul(European Respiratory Society Journals Ltd, 2022) Yanaz, Mürüvvet; Ünal, Füsun; Hepkaya, Evrim; Yazan, H.; Oksay, S. Can; Köstereli, Ebru; Yeğit, C. Yılmaz; Başkan, A. Kılıç; Onay, Z. R.; Gülieva, Aynur; Soyyiğit, Aslınur; Kalyoncu, M.; Küçük, Hanife Büşra; Ergenekon, Almala Pınar; Atağ, Emine; Uzuner, Selçuk; Baş İkizoğlu, Nilay; Kılınç, Ayşe Ayzıt; Ay, P.; Eralp, E. Erdem; Gökdemir, Yasemin; Öktem, Sedat; Çakır, Erkan; Girit, Saniye; Uyan, Zeynep Seda; Cokuğraş, Haluk; Ersu, Refika; Karadağ, Bülent; Karakoç, Feyza[Abstract Not Available]Öğe Non-invasive ventilation for children with chronic lung disease(Frontiers Media S.A., 2020) Atag, Emine; Krivec, Uros; Ersu, RefikaAdvances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.Öğe Pediatric flexible bronchoscopy in the intensive care unit: A multicenter study(Wiley, 2021) Atağ, Emine; Ünal, Füsun; Yazan, Hakan; Girit, Saniye; Uyan, Zeynep Seda; Ergenekon, Almala Pınar; Yayla, Esra; Merttürk, Edanur; Telhan, Leyla; Meral, Özge; Küçük, Hanife Büşra; Gündüz, Mehmet; Gökdemir, Yasemin; Eralp, Ela Erdem; Kıyan, Gürsu; Çakır, Erkan; Ersu, Refika; Karakoç, Fazilet; Öktem, SedatIntroduction Flexible bronchoscopy (FB) is frequently used for assessment and treatment of patients with respiratory diseases. Our aim was to investigate the contribution of FB to diagnosis and therapy in children admitted to the intensive care units (ICU) and to evaluate the safety of FB in this vulnerable population. Methods Children less than 18 years of age who underwent FB in the five neonatal and pediatric ICUs in Istanbul between July 1st, 2015 and July 1st, 2020 were included to the study. Demographic and clinical data including bronchoscopy indications, findings, complications, and the contribution of bronchoscopy to the management were retrospectively reviewed. Results One hundred and ninety-six patients were included to the study. The median age was 5 months (range 0.3-205 months). The most common indication of FB was extubation failure (38.3%), followed by suspected airway disease. Bronchoscopic assessments revealed at least one abnormality in 90.8% patients. The most common findings were airway malacia and the presence of excessive airway secretions (47.4% and 35.7%, respectively). Positive contribution of FB was identified in 87.2% of the patients. FB had greater than 1 positive contribution in 138 patients and 80.6% of the patients received a new diagnosis. Medical therapy was modified after the procedure in 39.8% and surgical interventions were pursued in 40% of the patients. Therapeutic lavage was achieved in 18.9%. There were no major complications. Conclusion Flexible bronchoscopy is a valuable diagnostic and therapeutic tool in neonatal and pediatric ICUs and is not associated with major complications.Öğe Sleep disordered breathing in patients with primary ciliary dyskinesia(Wiley-Blackwell, 2013) Öktem, Sedat; Karadağ, Bülent; Erdem, Ela; Gökdemir, Yasemin; Karakoç, Fazilet; Dağlı, Elif; Ersu, RefikaBackground Upper airway manifestations of primary ciliary dyskinesia (PCD) can cause obstructive sleep apnea syndrome (OSAS). Also abnormalities of lung mechanics and gas exchange may lead to sleep abnormalities in these patients. Objectives To determine the rate of OSAS and sleep quality in PCD patients, and whether these are related to upper respiratory system manifestations and severity of lung disease in these patients. Methods Twenty-nine PCD patients and healthy controls were included to the study. Respiratory symptoms within the previous month were separately scored with the severity of the symptoms. Physical examination, pulmonary function tests, and ear-nose-throat assessments were obtained. All patients completed the Turkish version of Pittsburgh Sleep Quality Index (PSQI), sleep questionnaire, and underwent overnight polysomnography. Categorical variables were compared with chi-square and Fisher's exact test while continuous variables were compared with Student's t-test. Results Eleven PCD patients reported themselves to be poor sleepers, compared to only one subject in the control group (P=0.002). Sixty-five percent of PCD patients had habitual snoring (HS). Fifty-two percent of the PCD patients had OSAS in polysomnography. OSAS rate was higher in PCD patients who snored (P=0.008). HS and OSAS were more common in PCD patients who had cigarette smoke exposure in their homes (P<0.001 and P=0.02, respectively). Conclusions Patients with PCD have decreased sleep quality and higher rate of sleep disordered breathing compared to controls and higher rate of OSAS compared to population rates. Cigarette smoke exposure is an important risk factor for OSAS in PCD patients. Assessment and treatment of sleep disorders in PCD should be a part of disease management. Pediatr Pulmonol. 2013; 48:897-903. (c) 2012 Wiley Periodicals, Inc.
