Publications by authors named "Dilber Ademhan Tural"

10 Publications

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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

Evaluation of Allergic Disease Prevalence and Related Risk Factors in Children with Autoimmune Thyroiditis.

Int Arch Allergy Immunol 2021 Apr 19:1-9. Epub 2021 Apr 19.

Department of Pediatric Allergy and Immunology, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, University of Health Sciences, Ankara, Turkey.

Background: Allergic and autoimmune diseases are caused by an impaired immunological response resulting from different types of T-helper (Th) cells. Since the Th cell production is in a certain balance, an inverse relationship between the 2 disease groups may exist. In this study, we aimed to investigate the frequency of allergic diseases in children with autoimmune thyroiditis (AT).

Methods: Symptoms of allergic diseases were investigated by the (International Study of Asthma and Allergies in Childhood) ISAAC questionnaire. The questionnaire was administered to a group of 300 children with AT and a control group of 300 children with no known autoimmune disease. The risk factors for allergic diseases and sociodemographic characteristics were investigated. A multivariable logistic regression analysis was performed with the risk factors of allergic diseases.

Results: Asthma, wheezing during the last year, wheezing with exercise, disease-free night cough, and night cough were significantly less common in patients with AT. Allergic rhinitis symptoms, except physician-diagnosed allergic rhinitis, are found significantly less frequently in patients with AT. It was found that the presence of AT and an increase in the number of children in the family reduce the risk of allergic diseases; cat-dog contact before 1 year of age and the presence of asthma, eczema, or allergic rhinitis in the mother increase the risk of allergic diseases.

Conclusions: Asthma and allergic rhinitis symptoms were significantly less common in children with AT. AT reduces the risk of allergic diseases.
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http://dx.doi.org/10.1159/000515499DOI Listing
April 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

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

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

Clinical implications of fungal isolation from sputum in adult patients with cystic fibrosis.

Turk J Med Sci 2021 Jan 12. Epub 2021 Jan 12.

Background/aim: Cystic fibrosis is an autosomal recessive disease with a defect in mucociliary activity that is characterized by recurrent pulmonary infections. Bacterial agents most frequently implicated in airway colonization and infection are Haemophilus influenzae, Staphylococcus spp. and Pseudomonas spp. Fungal isolation from sputum species is more common in adults. However, growth of fungal agent only in sputum culture in patients with cystic fibrosis is insufficient for the diagnosis of fungal diseases. There is limited and contraversial data about the clinical significance of fungal isolation in sputum cultures. The aim of the present study was to investigate the clinical outcomes including characteristics, lung function, therapy modalities and frequency of exacerbations and clinical significance of fungal isolation from sputum samples in adult patients with CF.

Materials And Methods: This retrospective cohort study included patients who have been admitted between October 2017 and January 2019 in an Adult Cystic Fibrosis Unit. Patients were grouped according to fungal pathogenicity as; fungal disease group, colonization group, and non-isolated group. The data of the last one year, including patients demographics, clinical data, laboratory results, treatment modalities, results of cultured bacteria and fungus from sputum samples, respiratory function parameters, frequency of exacerbation and hospitalization were compared between groups.

Results: A total of 330 sputum samples from 88 adult patients with CF were collected during the preceeding one year. Patients were divided into three groups, fungal disease group (n = 10, 11.4%), colonization group (n = 49, 55.7%), and non-isolated group (n = 29, 32.9%). Presence of pulmonary exacerbation and number of admission to emergency department and the number of positive cultures for bacteria from sputum were significantly higher in the fungal disease group (p = 0.03, p = 0.01 and p ?0.001). Patients in the fungal disease group had higher rate of administered antibiotics by parenteral route than other groups (p = 0.001) whereas lung function parameters were similar. Use of nutritional supplementation and parenteral antibiotherapy in the preceeding one year were the factors associated with elevated risk of fungal isolation.

Conclusion: Frequent use of parenteral antibiotics and use of nutritional supplementation were found to be independent risk factors for fungal isolation from sputum in adult CF patients.
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http://dx.doi.org/10.3906/sag-2006-94DOI Listing
January 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

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

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