Publications by authors named "Ugur Ozcelik"

128 Publications

Spondyloepimetaphyseal dysplasia EXTL3-deficient type: Long-term follow-up and review of the literature.

Am J Med Genet A 2021 Jun 4. Epub 2021 Jun 4.

Department of Pediatric Genetics, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Spondyloepimetaphyseal dysplasia (SEMD) is a group of genetic skeletal disorders characterized by disproportionate short stature, and varying degrees of vertebral, epiphyseal, and metaphyseal involvement of the skeleton. According to the Nosology and classification of genetic skeletal disorders 2019 revision, more than 20 types of SEMD have been identified, and SEMD with immune deficiency, EXTL3 type is one of the newcomers. Affected individuals display variable skeletal abnormalities and neurodevelopmental findings. Liver and kidney cysts have also been reported frequently. Patients may exhibit varying degrees of immune deficiency as well. To date, only 14 patients from 9 unrelated families with SEMD with immune deficiency, EXTL3 type have been reported in the literature. We report a new patient who is currently 15 years old in whom cystic liver lesions were detected in the prenatal period. Disproportionate short stature, mild developmental delay and a T NK B immunological profile were detected in the postnatal follow-up. Exome sequence analysis revealed a previously reported homozygous missense variant in exon 3 c.953C > T; p.(Pro318Leu) in EXTL3.
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http://dx.doi.org/10.1002/ajmg.a.62378DOI Listing
June 2021

Evaluation of sleep-disordered breathing and its relationship with respiratory parameters in children with mucopolysaccharidosis Type IVA and VI.

Am J Med Genet A 2021 May 7. Epub 2021 May 7.

Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

The aims of the study were to evaluate the prevalence of sleep-disordered breathing (SDB) by using polysomnography (PSG) in children with MPS IVA and MPS VI who underwent enzyme replacement therapy (ERT) and to analyze the effect on SDB of having upper airway surgery, pulmonary functions, and exercise capacity. A retrospective cross-sectional study was conducted on patients with MPS IVA (n:17) and MPS VI (n:11) aged under 19 years who underwent polysomnography. Descriptive and nonparametric analyses were performed for demographic, PSG, pulmonary function and exercise capacity variables. The frequency of sleep apnea in the study sample was 85.7% (24/28). Four patients (14.3%) had no sleep apnea, 15 (53.6%) had mild, and nine (32.1%) had moderate-to-severe sleep apnea. Two patients (7.1%) had central sleep apnea and 22 had obstructive sleep apnea (OSA) (78.6%). Forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were negatively correlated to apnea-hypopnea index (AHI) (r = -0.594, p = .009; r = -0.636, p = .005, respectively). Despite ERT and previous upper airway surgery, the prevalence of OSA was high in patients with MPS IVA-MPS IV, emphasizing the importance of PSG screening for sleep disorders. Pulmonary function tests may be useful for predicting sleep apnea in patients with MPS IVA and MPS VI.
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http://dx.doi.org/10.1002/ajmg.a.62229DOI Listing
May 2021

Clinical characteristics of children with cystic fibrosis infected with unusual bacteria.

Minerva Pediatr (Torino) 2021 Apr 15. Epub 2021 Apr 15.

Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Objectives-aim: Pulmonary infections are usually caused by bacterial microorganisms such as Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae, and Burkholderia cepacia complex in cystic fibrosis (CF) patients. Unusual bacteria (UB) have been described by new isolation techniques recently in the respiratory samples of CF patients. The aim is to investigate the effects of the presence of UB in the respiratory cultures of CF patients on clinical outcomes, necessity of treatment and prognosis.

Methods: The UB were identified by MALDI-TOF (matrix-assisted laser desorption/ionization time-of-flight) mass spectrometry technology.

Results: Rhizobium radiobacter were detected in 2, Chyrseobacterium species (gleum and indolgenes) in 5, Aeromonas hydrophila in 1, Orchobacterium anthropy in 1,Wautersiella falsenii in 1, Leclercia adecarboxylata in 1, Delftia acidovorans in 1, Cupriavidus Gilardi in 1, R.radiobacter twith Elizabethkingia miricola in 1 and R.radiobacter with C.gleum in 1 patient. Median age of the first UB growth was 3 years. After the first UB growth, the median follow-up time was 15 months. Before the UB growth, 60.0% of the patients had respiratory colonization with methicillin-susceptible S.aureus (MSSA). UB growth were accompanied with MSSA in 66.6% of the patients. Median percentage of FEV1 before and during the UB growth for patients who could perform spirometry, were 80 and 102, respectively. Median body mass index before and during the UB growth were 16 and 16.2, respectively. These UB were not detected during the follow-ups except in one patient.

Conclusions: The UB growth did not cause any additional symptoms and decrease in BMI and FEV1 in patients with CF. MSSA may be a facilitating factor for UB growth as majority of the patients had MSSA colonization before and during the UB growth.
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http://dx.doi.org/10.23736/S2724-5276.21.06189-2DOI Listing
April 2021

Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula.

Respir Med 2021 May 28;181:106376. Epub 2021 Mar 28.

Hacettepe University, Ihsan Dogramacı Children's Hospital, Pediatric Pulmonology, Ankara, Turkey. Electronic address:

Introduction: Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center.

Methods: Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy.

Results: A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025). CONCLUSİON: Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.
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http://dx.doi.org/10.1016/j.rmed.2021.106376DOI Listing
May 2021

Does cystic fibrosis make susceptible to celiac disease?

Eur J Pediatr 2021 Mar 25. Epub 2021 Mar 25.

Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Patients with cystic fibrosis (CF) have a higher incidence of celiac disease (CD) than the healthy population; however, the actual incidence of coexisting CF and CD is unclear. In this report, we aimed to evaluate the frequency of CD and CF coexistence and to assess the clinical findings of affected patients during follow-up. We conducted a retrospective review of patients with CF to reveal the frequency of CD and also investigated the clinical characteristics and clinical response to gluten-free diet in patients with CD. The incidence of CD in 515 patients with CF was 1.4%. The median age at the time of CF diagnosis was 2 months (1-6 months). CD was diagnosed in six patients with poor weight gain, fatty stools, and low z score for BMI and one patient with poor weight gain despite a high protein and calorie diet and pancreatic enzyme replacement. The median age of CD diagnosis was 8 years (2-12 years). Except for one patient who was recently diagnosed, the other six patients gained weight and their accompanying symptoms resolved after starting a gluten-free diet.Conclusion: CD should be investigated in patients with CF in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment. What is Known: • CFTR dysfunction may be a risk factor for CD, due to increased intestinal permeability and intestinal inflammation, pancreatic exocrine insufficiency that results in higher antigen load and increased antibodies against to nutritional antigens such as anti-gliadin IgA antibodies. • Although coexistence of CF and CD are rare in the same patient; there is still no consensus on when children with CF should be screened for CD. What is New: • Physicians should consider the investigation of CD in patients with CF, in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment. • CFTR dysfunction has been emphasized to develop susceptibility to CD, and patients with CF who have persistent gastrointestinal symptoms despite appropriate and adequate nutritional and enzyme replacement treatment should be screened for CD.
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http://dx.doi.org/10.1007/s00431-021-04011-4DOI Listing
March 2021

Complicated pneumonia due to exogenous toxic substances in children.

Turk J Pediatr 2021 ;63(1):149-154

Departments of Radiology, Hacettepe University Faculty of Medicine Ankara, Turkey.

Background: Toxic substances may be inhaled or aspirated not always by patients themselves but sometimes by healthcare providers unintentionally by nasogastric administrations or medications. Aspirated toxic particles may cause injury in the pulmonary epithelium at various levels of the respiratory tract, leading to a spectrum varying from simple symptoms to severe chronic disease. Imaging features of substances are different depending on their structure and are not well known. Moreover, on follow-up, radiologic findings are not well correlated with clinical findings which progress or recover more rapidly.

Case: In this case series, we report three different chemical pneumonitis due to aspiration of exogenous substances of paint thinner, activated charcoal and liquid paraffin. We present three cases with chest radiographs, distinctive computed tomography findings and follow-up images.

Conclusions: Aspiration of exogenous materials reveals typical imaging findings such as the metallic density of charcoals, fat attenuation of liquid paraffin and necrotic areas within consolidation in hydrocarbon poisoning. Recognition of imaging findings is very important for the diagnosis and characterization of toxic substances.
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http://dx.doi.org/10.24953/turkjped.2021.01.018DOI Listing
January 2021

The Frequency and Related Factors of Non-Tuberculosis Mycobacteria Infections among Patients with Cystic Fibrosis.

Pediatr Int 2021 Mar 7. Epub 2021 Mar 7.

Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Background: Non-tuberculous mycobacteria (NTM) can cause chronic lung infection particularly in patients who have structural lung disease such as cystic fibrosis (CF). We evaluated the incidence and management of NTM infections in patients with CF in our center.

Methods: A retrospective cohort study was carried out on CF patients having at least one positive NTM isolate between 2012-2020.

Results: Ten patients (2.1 %) had at least one positive NTM culture from respiratory samples. All of them were vaccinated with Bacille Calmette-Guérin (BCG) vaccine which it is in the national vaccination program in our country. Eight patients had Mycobacterium abscessus complex (MABSC), one had Mycobacterium avium and one had Mycobacterium szulgai growth in their respiratory samples. Three patients had transient, 2 had persistent and 5 had active NTM infection (NTM pulmonary disease). Patients with NTM pulmonary disease received antibiogram directed antimycobacterial therapy. In patients with NTM pulmonary disease, the median ppFEV1 and BMI decreased by 17% and 1%, respectively, at the time of the first NTM isolation when compared with the values one year before first NTM isolation. Culture conversion was not seen in any patient infected with MABSC.

Conclusions: The NTM infection incidence is lower in our country than those countries where the BCG vaccine is not routinely applied. The BCG vaccine may be a protective factor for NTM infection. Further studies are needed about the prevalence of NTM infections, facilitating and protective factors and appropriate management of NTM infections in patients with CF.
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http://dx.doi.org/10.1111/ped.14688DOI Listing
March 2021

Differentially expressed genes associated with disease severity in siblings with cystic fibrosis.

Pediatr Pulmonol 2021 May 31;56(5):910-920. Epub 2020 Dec 31.

Department of Medical Biology, Hacettepe University Faculty of Medicine, Sıhhiye, Ankara, Turkey.

Cystic fibrosis (CF) is an autosomal recessive disease caused by CFTR gene mutations. Despite having the same mutation, CF patients may demonstrate clinical variability in severity and prognosis of the disease. In this study, we aimed to determine differentially expressed genes between mild and severe siblings with same genotype. We performed targeted real-time polymerase chain reaction based transcriptomic analysis of nasal epithelial cells obtained from two families with two siblings with Class II mutations (F508del/F508del) and (F508del/G85E), one family with three siblings with Class IV mutation (I1234V/I1234V). In severe siblings with Class II mutations, TNFRSF11A, KCNE1, STX1A, SLC9A3R2 were found to be up regulated. CXCL1, CFTR, CXCL2 were found to be down regulated. In the severe sibling with Class IV mutation; mainly genes responsible from complement and coagulation system were identified. Comparison of CF patients to non-CF control; showed that ICAM1 was up regulated whereas EZR, TNFRSF1A, HSPA1A were down regulated in patients. As a result of this study, differentially expressed genes responsible for clinical severity among affected siblings carrying the same mutation were identified. The results will provide an opportunity for the development of novel target molecules for treatment of disease.
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http://dx.doi.org/10.1002/ppul.25237DOI Listing
May 2021

The Effect of Self-Efficacy, Social Support and Quality of Life on Readiness for Transition to Adult Care Among Adolescents with Cystic Fibrosis in Turkey.

J Pediatr Nurs 2021 Mar-Apr;57:e79-e84. Epub 2020 Dec 3.

Pediatric Pulmonology Department, Faculty of Medicine, Hacettepe University, Turkey.

Purpose: The aim of this study is to examine the effects of self-efficacy, social support and quality of life on readiness for transition to adult care in adolescents with cystic fibrosis.

Design And Methods: A descriptive and cross-sectional study design was used. Data were collected from 50 adolescent between 14 and 17 years old with cystic fibrosis, by using The Transition Readiness Assessment Questionnaire, Social Support Appraisals Scale for Children, Self-Efficacy Questionnaire for Children and health-related quality-of-life instrument, the KIDSCREEN-10.

Results: A positive correlation was found between the readiness levels of adolescents for transition to adult care and self-efficacy levels. In path analysis, self-efficacy was found to have a significant effect on the level of readiness for transition to adult care. There was not statistically significant relationship between the level of readiness for transition to adult care and health-related quality of life and perceived social support. Path analysis revealed that health-related quality of life and perceived social support had significant effects on the self-efficacy levels of adolescents.

Conclusions: Self-efficacy were associated with readiness for the transition to adult care. Although perceived social support and quality of life were not related with transition readiness these variables had significant effects on perceived self-efficacy, which was determined as a factor affecting the readiness for the transition to adult care.

Practice Implications: In adolescents with cystic fibrosis, self-efficacy, social support and quality of life levels should be taken into account when planning preparation programs for transition to adult care.
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http://dx.doi.org/10.1016/j.pedn.2020.11.013DOI Listing
April 2021

Risk factors for recurrent pulmonary exacerbation in idiopathic pulmonary hemosiderosis.

Pediatr Pulmonol 2021 May 17;56(5):1060-1068. Epub 2021 Mar 17.

Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objectives: To evaluate the risk factors of recurrent pulmonary exacerbation and poor prognosis in children with idiopathic pulmonary hemosiderosis (IPH).

Methods: In this multicenter study, 54 patients with a diagnosis of IPH were included. Medical records were retrospectively reviewed from three tertiary care hospitals between 1979 and 2019. Also, current information and the long-term progress of patients was determined by contacting the families by telephone.

Results: A total of 54 children were included. The median age of onset of symptoms was 4.5 years (3 months to 15.8 years). The median time from onset to diagnosis was 0.9 years (0.25 months to 12 years). The mean number of recurrent episodes per child in the recurrence-positive group was 3.55 (1-15). Univariate analysis demonstrated that patients presenting with hypoxia or requiring transfusion at the time of presentation had significantly more recurrence episodes (p = .002). Multivariate analysis showed that the presence of hypoxia at the time of initial presentation was a significant independent predictor of recurrent episodes (p = .027). The median follow-up was 3.3 years (0.75 months to 27 years). There was a significant relationship between the presence of hypoxia, transfusion history, antinuclear antibody positivity, and elevated transaminases at the time of initial evaluation and treatment response.

Conclusions: The present study provides substantial information regarding factors that may affect recurrent exacerbations and prognosis in children with IPH. Demonstrating hypoxia as an independent risk factor in recurrence episodes could guide physicians in the planning of treatment strategies.
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http://dx.doi.org/10.1002/ppul.25189DOI Listing
May 2021

Nonmyeloablative hematopoietic stem cell transplantation in a patient with hereditary pulmonary alveolar proteinosis.

Pediatr Pulmonol 2021 02 24;56(2):341-343. Epub 2020 Nov 24.

Department of Pediatric Hematology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

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http://dx.doi.org/10.1002/ppul.25174DOI Listing
February 2021

Current Approach in the Diagnosis and Management of Allergic Bronchopulmonary Aspergillosis in Children With Cystic Fibrosis.

Front Pediatr 2020 20;8:582964. Epub 2020 Oct 20.

Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Allergic bronchopulmonary aspergillosis (ABPA) is a complex pulmonary disorder characterized by a hypersensitivity reaction to , and almost always seen in patients with cystic fibrosis (CF) and asthma. Fungal hyphae leads to an ongoing inflammation in the airways that may result in bronchiectasis, fibrosis, and eventually loss of lung function. Despite the fact that ABPA is thought to be more prevalent in CF than in asthma, the literature on ABPA in CF is more limited. The diagnosis is challenging and may be delayed because it is made based on a combination of clinical features, and radiologic and immunologic findings. With clinical deterioration of a patient with CF, ABPA is important to be kept in mind because clinical manifestations mimic pulmonary exacerbations of CF. Early diagnosis and appropriate treatment are important in preventing complications related to ABPA. Treatment modalities involve the use of anti-inflammatory agents to suppress the immune hyperreactivity and the use of antifungal agents to reduce fungal burden. Recently, in an effort to treat refractory patients or to reduce adverse effects of steroids, other treatment options such as monoclonal antibodies have started to be used. Intensive research of these new agents in the treatment of children is being conducted to address insufficient data.
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http://dx.doi.org/10.3389/fped.2020.582964DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7606581PMC
October 2020

Telephone surveillance during 2019 novel coronavirus disease: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?

J Telemed Telecare 2020 Nov 12:1357633X20972008. Epub 2020 Nov 12.

Department of Pediatric Pulmonology, Hacettepe University, Turkey.

Introduction: The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease.

Methods: A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients.

Results: Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised.

Discussion: Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.
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http://dx.doi.org/10.1177/1357633X20972008DOI Listing
November 2020

Obstructive sleep apnea in children with hypothalamic obesity: Evaluation of possible related factors.

Pediatr Pulmonol 2020 12 6;55(12):3532-3540. Epub 2020 Oct 6.

Department of Pediatrics, Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Introduction: Hypothalamic obesity (HO) is a type of obesity that is caused by hypothalamic damage. HO can be complicated by obstructive sleep apnea syndrome (OSAS) due to anatomical narrowing of the upper airway and hypothalamic damage-induced dysfunction of the sleep control mechanisms. We aimed to explore the presence and severity of OSAS in children with HO and hypothesized that OSAS is more severe and frequent in HO than exogenous obesity (EO).

Methods: This cross-sectional study was conducted among children aged 6.6-17.9 years. Subjects with HO (n = 14) and controls with EO (n = 19) were consecutively recruited through an endocrinology clinic. All patients underwent full-night polysomnography. The primary outcomes were obstructive apnea-hypopnea index (OAHI) and the severity of OSAS. We analyzed the polysomnography findings, biochemical parameters, Brodsky and modified Mallampati scores, and blood pressure compared with the controls. We explored the different obesity types and these variables in association with OAHI using multiple linear regression (MLR).

Results: Age and body mass index z scores (BMI-z) were similar between the EO and HO groups. The OAHI of HO (5.8) was higher than that of EO (2.2). In MLR, the predicted OAHI was formulated as an equation using regression coefficients of obesity type (HO), age, and BMI-z (R  = .41). In the logistic regression analysis, the odds ratio of moderate/severe OSA was 5.6 for HO.

Conclusions: Children with HO have a higher risk of moderate/severe OSAS than children with EO. Polysomnography should be considered in all patients with HO.
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http://dx.doi.org/10.1002/ppul.25097DOI Listing
December 2020

Psychiatric and general health effects of COVID-19 pandemic on children with chronic lung disease and parents' coping styles.

Pediatr Pulmonol 2020 12 28;55(12):3579-3586. Epub 2020 Sep 28.

Department of Pediatric Pulmonology, School of Medicine, Ihsan Dogramaci Children's Hospital, Hacettepe University, Ankara, Turkey.

Background: We aim to assess the anxiety and depressive symptoms related to the COVID-19 pandemic in children with chronic lung disease and their parents and also to evaluate parents' coping strategies.

Methods: Parents of children aged 4-18 years, with chronic lung disease (study group n = 113) and healthy control (n = 108) were enrolled in the study. General Health Questionnaire-12, specific COVID-19 related anxiety questions, The Coping Orientation to Problems Experienced inventory, coronavirus-related psychiatric symptom scale in children-parental form were used to analyze the psychiatric effects of COVID-19. Parents were also asked about how online education affected their family life and children. All data were compared between children/parents in the study and control groups. Risk factors related with anxiety scores of children were also analyzed.

Results: Talking about the pandemic, concern about coronavirus transmission, taking precaution to prevent coronavirus transmission, making pressure to protect from COVID-19 were significantly higher in parents within the study group (p < .05). Parents in the study group used more problem-focused coping than parents in the control group (p = .003). Anxiety symptoms score was higher in children of the study group (p = .007). Parents in the study group found online education more useful than parents in the control group.

Conclusion: Children with chronic lung diseases and their parents have more anxiety due to COVID-19 pandemic and these parents use more mature coping strategies to manage the stress of the pandemic. Longitudinal and larger studies should be done in all aspects of online education in children with chronic lung diseases.
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http://dx.doi.org/10.1002/ppul.25082DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7537125PMC
December 2020

Late Diagnosis of Infants with PCD and Neonatal Respiratory Distress.

J Clin Med 2020 Sep 4;9(9). Epub 2020 Sep 4.

Institute of Social and Preventive Medicine, University of Bern, 3012 Bern, Switzerland.

Neonatal respiratory distress (NRD) is common among infants with primary ciliary dyskinesia (PCD), but we do not know whether affected neonates receive a timely diagnosis. We used data from the international PCD cohort and assessed the proportion of patients with PCD who had a history of NRD and their age at diagnosis, stratifying by presence of laterality defects. First we analyzed data from all participants diagnosed after 2000, followed by individuals from a subgroup diagnosed using stricter criteria. Among the 1375 patients in the study, 45% had a history of NRD and 42% had laterality defects. Out of the 476 children with definite PCD diagnosis, 55% had a history of NRD and 50% had laterality defects. Overall, 30% of children with PCD were diagnosed during the first 12 months of life. This varied from 13% in those with situs solitus and no NRD, to 21% in those with situs solitus and NRD, 33% in those with situs anomalies but no NRD, and 52% in those with both situs anomalies and NRD. Our results suggest that we need to improve our knowledge of the neonatal presentation of infants with PCD and apply it so that these patients will receive appropriate care sooner.
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http://dx.doi.org/10.3390/jcm9092871DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7563976PMC
September 2020

Diverse clinical characteristics of Aspergillus growth in patients with cystic fibrosis.

Turk J Pediatr 2020 ;62(4):560-568

Departments of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background And Objectives: Patients with cystic fibrosis (CF) have a varying spectrum of clinically significant Aspergillus disease in addition to allergic bronchopulmonary aspergillosis (ABPA). Here we aimed to review the different clinical phenotypes related with Aspergillus growth on the airway culture of patients with CF, we also aimed to investigate the effect of Aspergillus growth on lung function tests.

Method: The medical records of 100 patients with CF who had Aspergillus growth on airway culture within the period of April 2001 and June 2016 were retrospectively analyzed. Age, gender, symptoms, physical examination findings, pulmonary function tests, the diagnosis of ABPA, and airway culture results were recorded for every visit. Patients with Aspergillus growth on airway cultures were classified into different groups as ABPA, Aspergillus sensitization, Aspergillus colonization and Aspergillus bronchitis.

Results: Medical records of 83 patients and 147 sputum cultures were attained from 100 patients. The mean age of the patients was 17.6±7.6 years and the mean age of the first Aspergillus growth in sputum culture was 12.5±6.7 years. At first isolation, Aspergillus fumigatus SC was the most common Aspergillus SC in sputum (76.3%) and 14.5% of these patients required hospitalization. Aspergillus sensitization was diagnosed in 3.6% (n= 3) of the patients. Aspergillus colonization was diagnosed in 18.1% (n= 15) of all patients and led to a decline in FEV1%, FVC% and FEF25-75% which was not statistically significant, furthermore. ABPA was detected in 9.6% (n= 8) of all patients and led to a statistically significant decline in FEV1% (p= 0.02); nonsignificant decline in FVC% and FEF25-75%. Aspergillus bronchitis was detected in 43.4% (n= 36) of all patients and led to nonsignificant decline in FEV1%, FVC% and FEF25-75%.

Conclusion: ABPA is recognized as the most common Aspergillus associated disorder in CF patients and is related to deteriorated pulmonary function tests; however Aspergillus colonization and bronchitis may also be associated with worsening lung function.
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http://dx.doi.org/10.24953/turkjped.2020.04.005DOI Listing
January 2020

Motor repertoire is age-inadequate in infants with cystic fibrosis.

Pediatr Res 2021 Apr 27;89(5):1291-1296. Epub 2020 Jul 27.

Department of Pediatric Chest Diseases, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: General movements (GMs) in infants occur as fidgety movements (FMs) between postterm 9 and 20 weeks. We aimed to evaluate FMs and motor repertoire in infants with cystic fibrosis (CF) and their relation with clinical findings.

Methods: Demographic and clinical characteristics were recorded. FMs and motor repertoire were analyzed from a 5-min video recording of each infant. Videos were rated based on the Prechtl General Movement Assessment and motor optimality score (MOS) was calculated.

Results: The analysis included 18 infants with CF and 20 healthy infants at postterm age of 3-5 months. MOS was significantly lower in the infants with CF compared to controls (p < 0.05). Fifty percent of the infants with CF had abnormal or absent/sporadic FMs. MOS was negatively associated with hospitalization duration (r = -0.378, p = 0.036); and positively associated with vitamin A level in CF infants (r = 0.665, p = 0.026).

Conclusions: Infants with genetically anticipated severe CF phenotype tended to have lower MOS. MOS may be used in addition to genetic testing to predict disease severity in infants with CF. Infants with CF, absent/sporadic FMs, and lower MOS could be considered for planning specific age-adequate early intervention programs.

Impact: Motor repertoire was age-inadequate in infants with cystic fibrosis (CF). 50% of infants with CF had abnormal or absent/sporadic fidgety movements (FMs). Motor optimality score (MOS) was positively associated with vitamin A level and negatively correlated with hospitalization duration in infants with CF. MOS tended to decrease as genetically anticipated disease severity increased; thus, MOS might enable us to predict disease severity in CF. The relationship between motor repertoire and phenotype and genotype is unclear and warrants further study. CF infants with absent/sporadic FMs, and lower MOS could be considered for planning early intervention.
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http://dx.doi.org/10.1038/s41390-020-1082-4DOI Listing
April 2021

Mutations of the CFTR gene and novel variants in Turkish patients with cystic fibrosis: 24-years experience.

Clin Chim Acta 2020 Nov 18;510:252-259. Epub 2020 Jul 18.

Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey. Electronic address:

Background: Cystic fibrosis, the most prevalent autosomal recessive genetic disease, is caused by mutations in the CFTR gene. The spectrum and frequency of CFTR mutations in Turkish patients show heterogeneity.

Methods: We investigated CFTR gene mutations in samples from 604 cystic fibrosis patients diagnosed at Hacettepe University, the largest referral CF center in Turkey, by different techniques such as strip assay and direct sequencing. We also analyzed the effects of novel variants and predicted pathogenicity by integrating information from different insilico tools.

Results: We showed that mutation detection rate increased to 76.7% with direct sequencing of the coding region and exon/intron boundaries. Ten variants were described for the first time. All variants except T788R were reported as pathogenic.

Conclusion: Characterization of patients with CFTR mutations that occur at very low frequencies is necessary for mutation-based treatments. Population specific genetic screening panels should be designed since none of them are suitable for Turkish patients due to heterogeneous mutation distribution. The preliminary data obtained from in silico results of novel variants will pave the way for functional analysis by using samples obtained from patients. These observations will facilitate the discovery and development of new targeted and personalized therapies.
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http://dx.doi.org/10.1016/j.cca.2020.07.033DOI Listing
November 2020

Variation in the bombesin staining of pulmonary neuroendocrine cells in pediatric pulmonary disorders-A useful marker for airway maturity.

Pediatr Pulmonol 2020 09 29;55(9):2383-2388. Epub 2020 Jun 29.

Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objectives: Pulmonary neuroendocrine cells (NEC) increase with age due to pulmonary maturity. The aim of this study was to determine whether open lung biopsies from patients with interstitial lung diseases have increased pulmonary NEC compared with neuroendocrine cell hyperplasia of infancy (NEHI). Our second aim was to assess pulmonary NECs in the lung autopsy of children without lung disease who died from different causes.

Methods: Lung tissue of 5 infants with NEHI; 21 patients with pediatric interstitial lung disease (chILD); 17 lung autopsies of infants at varying age without lung disease were included. The percentage of the airways containing neuroendocrine cells, the average percentage of neuroendocrine cells (NECs) per airway, and the number of neuroendocrine bodies (NEBs) in each case were analyzed.

Results: The mean percentage of the airways containing neuroendocrine cells were 95% in the NEHI group, 30% in the chILD group, 89% under Intrauterine 37 weeks, 70% between intrauterine 37 to 40 weeks, 52% at postnatal 4 days to 6 months of autopsy ages. In the NEHI group, diffuse NE cell distribution and large NEBs were noticed in the lung biopsy. In the chILD group, neuroendocrine cells were dispersed, did not form clusters and NE cells showed solitary distribution. In the lung autopsy group, linear NE cells were detected at younger aged fetuses and solitary distribution of NE cells was detected with the older increasing age.

Conclusions: Our findings confirm that NECs are seen in many other childhood interstitial lung diseases; NE cell hyperplasia may be a marker of decreased pulmonary development and NE cells decrease with the increasing age of the fetus during Intrauterine life.
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http://dx.doi.org/10.1002/ppul.24910DOI Listing
September 2020

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

Pediatr Pulmonol 2020 09 26;55(9):2302-2306. Epub 2020 May 26.

Division of Pediatric Allergy and Immunology, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey.

Background: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs.

Methods: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy.

Results: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups).

Conclusions: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.
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http://dx.doi.org/10.1002/ppul.24854DOI Listing
September 2020

Factors associated with severe lung disease in an adult population with cystic fibrosis: a single-center experience

Turk J Med Sci 2020 06 23;50(4):945-952. Epub 2020 Jun 23.

Department of Chest Diseases, Medicana Kadıköy, İstanbul, Turkey

Background/aim: The patients with cystic fibrosis (CF) are living longer compared to the past, but respiratory failure is still the most common cause of mortality. The aim of this study is to investigate factors associated with severe lung disease in a cohort of adult patients with CF.

Materials And Methods: Demographic data, clinical and laboratory findings of the patients aged 18 years and more were collected and the patients were grouped according to forced expiratory volume in 1 s (FEV1) as severe group: <40% and nonsevere ≥40%. Associations were investigated between groups and clinical outcomes.

Results: A total of 76 patients were enrolled in the study. The mean age was 24.5 ± 5.25 years and 36 (47.4%) patients were female. In the severe group; the mean age was higher (27.1 ± 6.0 vs 23.6 ± 4.7, P = 0.013), the median Chrispin-Norman score of severe lung disease group was higher (14 (6–22) vs 5.5 (0–20), P < 0.001), hospitalization at least once in a year for intravenous antibiotic was more common (12/18 (66%) vs 19/58 (32%), P = 0.014). There was a positive correlation between body mass index (BMI) and lung function, indicating that lower nutritional status was related to lower FEV1, r2 = 0.21, P < 0.001. The median FEV1% was lower in patients with CF-related diabetes (38 (14–95) vs 66 (13–121), P = 0.042). Dornase alpha use and physiotherapy rate were higher in severe lung disease group (P = 0.008 and P < 0.001, respectively).

Conclusion: Lower BMI, older age, presence of CF-related diabetes, higher radiologic scores, use of dornase alpha and physiotherapy and higher hospitalization rate for intravenous antibiotic therapy are significantly associated with severe lung disease.
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http://dx.doi.org/10.3906/sag-1912-101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379455PMC
June 2020

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

Pediatr Pulmonol 2020 08 4;55(8):2011-2016. Epub 2020 May 4.

Department of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey.

Background: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population.

Methods: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS.

Results: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS.

Conclusion: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.
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http://dx.doi.org/10.1002/ppul.24805DOI Listing
August 2020

A rare cause of acute abdominal pain in a patient with Primary ciliary dyskinesia with situs inversus totalis.

Turk J Pediatr 2020 ;62(1):156-159

Departments of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Primary ciliary dyskinesia (PCD) is a rare, genetic disease characterized by ciliary dysfunction. Patients may present with respiratory distress during neonatal period; chronic sinopulmonary disease, bronchiectasis, recurrent otitis media, sinusitis and infertility in later periods. About 50% of PCD patients have situs inversus totalis and 6-12% have situs ambiguous known as heterotaxy syndromes. Herein, we present a case of PCD and accompanying situs inversus who had acute abdominal pain and was diagnosed with torsion of one of the multiple spleens. Evaluation of acute abdominal pain in these patients has great importance since the internal organs are not at their typical locations.
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http://dx.doi.org/10.24953/turkjped.2020.01.024DOI Listing
January 2020

Cystic fibrosis in Turkey.

Lancet Respir Med 2020 04;8(4):e17

Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey.

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http://dx.doi.org/10.1016/S2213-2600(20)30055-2DOI Listing
April 2020

Respiratory viruses: What is their role in acute exacerbations in children with cystic fibrosis?

Pediatr Pulmonol 2020 07 30;55(7):1646-1652. Epub 2020 Mar 30.

Department of Pediatrics, Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Respiratory viruses (RVs) are frequently present in the airways of patients with cystic fibrosis (CF) during pulmonary exacerbations (PEx).

Method And Objectives: This prospective, longitudinal study was performed to examine the role of RVs in acute exacerbations in children with CF. Sputum samples or additional midturbinate swabs were tested from all children using a polymerase chain reaction panel. The primary aims of the study were to determine the prevalence and etiologic role of RVs in exacerbations of CF and to compare changes with RV-positive and RV-negative infections. The secondary aims were to determine the predictive factors for RV-related exacerbations.

Results: From 50 patients with PEx, 23 (48.9%) sputum samples were virus-positive. With a combination of sputum and swab, viral positivity increased to 56%. The virus-positive group presented more frequently with hypoxia (oxygen saturation <93%) than the virus-negative group (P = .048). Virus-positive exacerbations were not associated with an increase in colonization rates or greater lung function decline over 12 months.

Conclusions: RVs frequently present during PEx of CF. However, predicting viral infections is difficult in this group. Only the presence of hypoxia may raise the suspicion of an accompanying viral agent. The combination of sputum and nasal swab samples increases the diagnostic yield in viral infections of CF. Despite their high frequency, the presence of RVs had no impact on clinical outcomes, such as a decline in lung function and increased colonization rates.
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http://dx.doi.org/10.1002/ppul.24750DOI Listing
July 2020

Plasma Ceramides and Sphingomyelins of Pediatric Patients Increase in Primary Ciliary Dyskinesia but Decrease in Cystic Fibrosis.

Lipids 2020 05 2;55(3):213-223. Epub 2020 Mar 2.

Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, Ankara, Sıhhıye, 06100, Turkey.

We investigated plasma sphingomyelin (CerPCho) and ceramide (Cer) levels in pediatric patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Plasma samples were obtained from CF (n = 19) and PCD (n = 7) patients at exacerbation, discharge, and stable periods. Healthy children (n = 17) of similar age served as control. Levels of 16-24 CerPCho and 16-24 Cer were measured by LC-MS/MS. Concentrations of all CerPCho and Cer species measured at exacerbation were significantly lower in patients with CF than PCD. 16, 18, 24 CerPCho, and 22, 24 Cer in exacerbation; 18, 24 CerPCho, and 18, 20, 22, 24 Cer at discharge; 18, 24 CerPCho and 24 Cer at stable period were significantly lower in CF patients than healthy children (p < 0.001 and p < 0.05). All CerPCho and Cer levels of PCD patients were significantly higher except 24 CerPCho and 24 Cer during exacerbation, 24 CerPCho at discharge, and 18, 22 CerPCho levels at stable period (p < 0.001 and p < 0.05) compared with healthy children. There was no significant difference among exacerbation, discharge, and stable periods in each group for Cer and CerPCho levels. This is the first study measuring plasma Cer and CerPCho levels in PCD and third study in CF patients. The dramatic difference in plasma levels of most CerPCho and Cer species found between two diseases suggest that cilia pathology in PCD and CFTR mutation in CF seem to alter sphingolipid metabolism possibly in opposite directions.
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http://dx.doi.org/10.1002/lipd.12230DOI Listing
May 2020

Impact of mannose-binding lectin 2 gene polymorphisms on disease severity in noncystic fibrosis bronchiectasis in children.

Pediatr Pulmonol 2020 05 2;55(5):1190-1198. Epub 2020 Mar 2.

Department of Pediatrics, Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Mannose-binding lectin (MBL) is a complement protein involved in the innate immune system, and is associated with some chronic respiratory diseases including noncystic fibrosis (non-CF) bronchiectasis in adults. The aim of this study was to investigate the frequency of MBL2 gene polymorphisms in children with non-CF bronchiectasis, and the effect of MBL deficiency on disease severity.

Methods: Fifty children with non-CF bronchiectasis (bronchiectasis group) and 50 healthy controls (control group) were included. The demographic findings, number of acute pulmonary exacerbations in the previous year, airway cultures, pulmonary function tests, and radiologic scores of the bronchiectasis group were recorded. DNA extraction was performed in both groups and MBL2 gene polymorphisms in codons 52, 54, 57 in exon 1 and H/L, Y/X in the promoter region were studied using real-time polymerase chain reaction. Haplotypes were made by genotypes, and MBL serum expression was classified according to the genotypes in the literature.

Results: The bronchiectasis group consisted of 23 (46%) patients with primary ciliary dyskinesia, 5 (10%) with primary immunodeficiency diseases, and 22 (44%) with idiopathic bronchiectasis. There were no statistically significant differences between the bronchiectasis and control groups in terms of allele and genotype frequencies of polymorphisms in codons 52, 54, 57 in exon 1 and promoter H/L. However, the YX heterozygote genotype was more frequent in the control group (82%) compared with the bronchiectasis group (50%) (P = .002). The frequency of patients with intermediate serum MBL expression genotype was higher in the bronchiectasis group (20%) than in the control group (0%) (P = .001). In the bronchiectasis group, there were no significant differences in growth, annual pulmonary exacerbation rates in the last year, pulmonary function tests, radiologic scores, and microbiologic findings between low, intermediate, and high-expressing genotypes.

Conclusions: In children with non-CF bronchiectasis, MBL genotype was different from healthy controls. MBL deficiency associated only with MBL genotype was not related to disease severity in this group of patients.
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http://dx.doi.org/10.1002/ppul.24711DOI Listing
May 2020

Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non-cystic fibrosis bronchiectasis.

Pediatr Pulmonol 2020 05 28;55(5):1207-1216. Epub 2020 Feb 28.

Department of Child Health and Diseases, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: To the best of our knowledge, there is no study in the literature investigating the extrapulmonary outcomes of children with non-cystic fibrosis (CF) bronchiectasis and CF under the framework of the International Classification of Functioning, Disability, and Health (ICF) model. The purpose of the present study is to evaluate the children with CF and non-CF bronchiectasis using the ICF model.

Materials And Methods: Children with CF, non-CF bronchiectasis, and healthy counterparts were evaluated (20 participants in each group) according to the ICF items in domain b (body functions), domain s (body structures), and domain d (activities and participation). The pulmonary functions, respiratory and peripheral muscle strength tests, and posture analysis were carried out for domain b. For domain d, however, the Glittre-activities of daily living test and Pediatric Outcome Data Collection were used.

Results: Muscle strength of shoulder abductors and hip extensors in children with CF was significantly lower than healthy children and adolescents (P < .05). The severity of lateral and posterior postural abnormalities in children with CF and non-CF bronchiectasis was higher than those of healthy children (P < .05). Among the patient groups, global function, sports/physical function, expectations, transfers/basic mobility, and pain/comfort were the most affected participation dimensions (P < .05).

Conclusions: This study highlights the need for comprehensive up-to-date evaluation methods according to the ICF model for understanding rehabilitation requirements in CF and non-CF bronchiectasis in different age groups.
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http://dx.doi.org/10.1002/ppul.24708DOI Listing
May 2020

From Diagnosis to Treatment of Pediatric Tuberculosis: Ten Years Experience in a Single Institution.

Clin Pediatr (Phila) 2020 05 22;59(4-5):476-482. Epub 2020 Feb 22.

Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

. To describe the patient characteristics, clinical-epidemiologic-microbiologic profile, and treatment outcomes, and to draw attention to tuberculosis (TB) in Turkey. . A retrospective, descriptive study was undertaken of 93 children aged 0 to 18 years who were admitted to Hacettepe University Pediatric Pulmonology Department for treatment from January 2005 to December 2015. Review of hospital records was performed for all children diagnosed as having TB. . Pulmonary TB was detected in 51.6% of the patients, extrapulmonary involvement in 33.3%, and pulmonary TB with extrapulmonary involvement in 15.1%. The history of contact with an adult with TB was found in 29% of cases. The most common extrapulmonary TB was TB lymphadenitis. The most common symptom was cough followed by fever and night sweats. One fifth of the patients had normal physical examinations at the time of diagnosis. One fifth (20.4%) of the patients had culture, 17.2% had polymerase chain reaction, and 15% had acid-resistant bacillus positivity. In 21.4% of patients with culture growth, at least one anti-TB drug resistance was found. The mean duration of treatment of patients treated according to the national guideline was 8.6 months (range = 6-36 months). Recovery was seen in 91.4% of patients. . The most important way to prevent childhood TB is to fight adult TB through early diagnosis and effective treatment. The presence of contact history must direct us to search for TB. While evaluating children with persisted nonspecific symptoms, TB must be kept in mind even if physical examinations are normal.
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http://dx.doi.org/10.1177/0009922820906488DOI Listing
May 2020