Publications by authors named "Ayse Tana Aslan"

43 Publications

Evaluation of age at diagnosis and clinical findings of children with primary ciliary dyskinesia.

Pediatr Pulmonol 2021 Jun 16. Epub 2021 Jun 16.

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

Objective: Patients with primary ciliary dyskinesia (PCD) may present with different clinical findings at different ages, and age at diagnosis may differ. We aimed to review clinical factors that affected age at diagnosis of patients with PCD.

Study Design: All 70 patients with PCD who were followed in our pediatric pulmonology department were included. Demographic features, clinical findings, PrImary CiliAry DyskinesiA Rule (PICADAR) scores and pulmonary function tests of patients were recorded and clinical factors that affected age at diagnosis were evaluated.

Results: The mean age at diagnosis was 8.3 ± 4.6 years. Most of patients (95.7%) had a persistent wet cough. The mean PICADAR score was 6.5 ± 3.2, and there was a negative correlation between PICADAR and age at diagnosis (r = -0.271, p = .023). The mean ages at diagnosis of patients with situs abnormality and recurrent wheezing were earlier than in patients without situs abnormality and recurrent wheezing (6.7 ± 4.3 and 6.8 ± 4.3, p = .002 vs. 9.8 ± 4.3 and 9.0 ± 4.6 years, p = .040, respectively). The mean age at diagnosis of patients with bronchiectasis was later than in patients without bronchiectasis (10.8 ± 3.9 and 6.9 ± 4.4 years, p = .001). Other clinical features were not statistically significant according to age at diagnosis (p > .05). There was no statistically significant relation between age at diagnosis and sex, sibling or relative with PCD and parental consanguinity (p > .05).

Conclusion: Although most patients diagnosed with PCD had symptoms, the diagnosis may be delayed. High PICADAR score is a useful guide to evaluate PCD. Situs abnormality and recurrent wheezing could be clues for early diagnosis of PCD. Early diagnosis of PCD may prevent bronchiectasis.
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http://dx.doi.org/10.1002/ppul.25533DOI Listing
June 2021

Pulmonary Mycobacterium abscessus Infection in an 11-Year-Old Child, Successfully Treated with Inhaled/Parenteral Amikacin: A Case Report and Review of Literature.

J Trop Pediatr 2021 05;67(2)

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

Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case of pulmonary M. abscessus infection in a pediatric patient with primary ciliary dyskinesia and he was successfully treated with parenteral amikacin, linezolid and oral clarithromycin combined with inhaled amikacin. Clinical improvement was observed after adding inhaled amikacin to the treatment.
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http://dx.doi.org/10.1093/tropej/fmab031DOI Listing
May 2021

Sleep disturbances in children with cystic fibrosis, primary ciliary dyskinesia and typically developing children during COVID-19 pandemic.

J Paediatr Child Health 2021 May 18. Epub 2021 May 18.

Department of Public Health, Gazi University Faculty of Medicine, Ankara, Turkey.

Aim: We aimed to investigate sleep disturbances in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) and typically developing (TD) children during the COVID-19 pandemic.

Methods: Primary care givers of children with CF and PCD aged 3-16 years were asked to enrol in the study. Primary care givers of TD children were included as control group. The Sleep Disturbance Scale for Children (SDSC) was used, and questions related to sleep habits during the pandemic were asked. Results of the three groups were compared.

Results: Primary care givers of 33 children with CF, 16 children with PCD and 66 TD children were included in the study. There were no differences in terms of age and gender between the three groups. Changes in sleep patterns during the pandemic were more common among TD children and their families, with 75% of the children and 80% of their families sleeping later than before. The sleep initiation and maintenance disorder scores were higher in TD children (P = 0.001), whereas the sleep breathing disorder scores were higher in children with PCD (P = 0.001), and the sleep hyperhidrosis scores were higher in children with CF and PCD (P = 0.011). No relationships were found between sleep parameters and clinical findings of children with lung disease.

Conclusions: Children's sleep habits have changed during the pandemic. Children with chronic lung diseases and even TD children may experience sleep disturbances during this period.
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http://dx.doi.org/10.1111/jpc.15553DOI Listing
May 2021

Hepatopulmonary Syndrome and Multiple Arteriovenous Fistulas in a Child with Niemann-Pick Disease.

Pediatr Allergy Immunol Pulmonol 2021 03;34(1):30-32

Department of Interventional Radiology, Faculty of Medicine, Gazi University, Ankara, Turkey.

Niemann-Pick disease (NPD) is caused by abnormal storage of sphingomyelin. NPD may affect the pulmonary system and cause hypoxia. In the present case, both hepatopulmonary syndrome (HPS) and pulmonary arteriovenous fistulas (PAVFs) developed in a child with NPD and were successfully treated with repeated embolization. We have reported the case of a 16-year-old-girl with NPD who suffered severe hypoxia, dyspnea, fatigue, had multiple PAVFs, and was diagnosed with type 2 HPS. To improve oxygenation, 10 PAVFs were embolized. She needed re-embolization after 9 months because of hypoxia redevelopment. Pulmonary involvement, HPS, and/or PAVFs could be responsible for hypoxemia in patients with NPD, who should, therefore, be investigated for HPS and PAVFs. Embolization could be beneficial. Some patients may need repeated embolization.
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http://dx.doi.org/10.1089/ped.2020.1244DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8082032PMC
March 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

Pigeon breeder`s disease as a cause of hypersensitivity pneumonia in children.

Turk J Pediatr 2020 ;62(4):623-633

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

Background And Objectives: Hypersensitivity pneumonia is a complex condition due to exposure time, intensity, different clinical presentation, and treatment practices. We aimed to evaluate the patients that were diagnosed with hypersensitivity pneumonia (HSP) due to exposure to pigeons and a review of the literature for diagnosis and treatment of Pigeon Breeder`s Disease (PBD) in children.

Method: Between the years of 2009-2018, patients who were diagnosed with HSP due to PBD were included in the study in a pediatric pulmonology department. Findings of our patients, treatments, and prognoses were compared with 17 articles in the literature about PBD in children.

Results: In a 9 year-period, 6 patients were diagnosed as HSP due to PBD. The mean age of the patients was 8.8 ± 5.4 years and the average duration of pigeon exposure was 60.1 ± 6.5 days. Precipitating antibodies were positive in 3 patients. In four cases, symptoms were resolved with only prevention of pigeon exposure. Two patients who had close contact with pigeons needed oxygen supplementation and steroid therapy.

Conclusion: Hypersensitivity pneumonia should be considered for the differential diagnosis of patients that present with respiratory distress, cough, fever, and weight loss. Prolonged exposure and close contact may worsen the clinical symptoms. In most cases, only exposure prevention is enough, while steroid therapy, oxygen support, and intensive care monitoring may be required in severe cases.
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http://dx.doi.org/10.24953/turkjped.2020.04.012DOI Listing
January 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

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

What triggered massive hemoptysis in Goodpasture syndrome with isolated pulmonary involvement in a 14-year-old boy?

Turk J Pediatr 2019 ;61(4):611-614

Department of Pediatric Intensive Care Unit, Gazi University Faculty of Medicine, Ankara, Turkey.

Şişmanlar-Eyüboğlu T, Aslan AT, Gezgin-Yıldırım D, Buyan N, Emeksiz S, Kalkan G. What triggered massive hemoptysis in Goodpasture syndrome with isolated pulmonary involvement in a 14-year-old boy? Turk J Pediatr 2019; 61: 611-614. Goodpasture syndrome is a rare, autoimmune disease associated with the development of antibodies against a specific antigen both in glomerular basement membrane and alveolar basement membrane. It is very rare in childhood and its incidence is not known. Although the mechanism of the disease is the same in lung as in the kidney, sometimes it presents with involvement of only one organ. Pulmonary involvement may be lifethreatening in patients with massive hemoptysis. Herein we report a 14-yearold boy with isolated lung involvement. He had massive hemoptysis following bronchoscopy and recovered succesfully with treatment.
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http://dx.doi.org/10.24953/turkjped.2019.04.022DOI Listing
May 2020

An adolescent case of extensive Behçet`s disease successfully treated with Infliximab.

Turk J Pediatr 2019 ;61(4):585-588

Divisions of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey.

Isıyel E, Bakkaloğlu S, Oğuz D, Yenicesu İ, Boyunağa Ö, Özdemir Y, Damar Ç, Kandur Y, Akçaboy M, Aslan AT, Şişmanlar T, Hasanoğlan E, Buyan N. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behçet`s Disease. We present a 14-year-old boy, admitted to our hospital for recurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombus and pulmonary arterial aneurysm were identified. He was diagnosed with Behçet`s Disease, and then he received prednisone and cyclophosphamide. However, his cardiac thrombus enlargened. After his treatment was replaced with infliximab, the pulmonary aneurysms regressed, and the cardiac thrombus disappeared. In conclusion, infliximab should be considered as a reliable option for vascular Behçet`s Disease resistant to conventional treatment.
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http://dx.doi.org/10.24953/turkjped.2019.04.016DOI Listing
May 2020

Passive Smoking and Disease Severity in Childhood Pneumonia Under 5 Years of Age.

J Trop Pediatr 2020 08;66(4):412-418

Department of Medical Education, Faculty of Medicine, Gazi University, Ankara, Turkey 06560.

Objective: To objectively investigate the effect of passive smoking on pneumonia and disease severity in children aged less than 5 years by using cotinine as an indicator of passive smoking.

Methods: Between December 2015 and April 2016, children aged less than 5 years with pneumonia and age-matched healthy controls were included in this study, which was conducted at three tertiary pediatric pulmonology centers. A questionnaire was given to the parents regarding demographic data and smoking status at home. Urinary cotinine/creatinine ratio (CCR) was measured. The data from the pneumonia and control groups, as well as children with mild and severe pneumonia within the pneumonia group, were compared.

Results: A total of 227 subjects were included in the study; there were 74 children in the pneumonia group and 153 in the control group. The mean age of all the children was 33.4 ± 1.28 months. Of all subjects, 140 were male and 102 were exposed to passive smoking by their parents at home. There were statistically significant differences in age, number of people in the home, and mother's and father's age between the control and pneumonia groups (p < 0.05). No difference was found in the CCR in the control and pneumonia group (p > 0.05). Age and urinary CCR were significantly different between children with mild and severe pneumonia (p < 0.05).

Conclusion: We showed that passive smoking exposure was associated with the development of severe pneumonia in children. Further studies are needed to examine the underlying cause in detail.
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http://dx.doi.org/10.1093/tropej/fmz081DOI Listing
August 2020

Ten-year follow-up of children with hydatid cysts.

Turk Pediatri Ars 2019 25;54(3):173-178. Epub 2019 Sep 25.

Department of Medical Education, Gazi University Faculty of Medicine, Ankara, Turkey.

Aim: Hydatid cystic disease is an endemic parasitic disease that is common in the world. We aimed to review the demographic, clinical and laboratory findings, and treatments and outcomes of children with hydatid cyst disease, and to determine the factors affecting treatment response in two pediatric pulmonology centers in the central region of Turkey.

Material And Methods: The clinical records of patients aged below 18 years who were followed up between January 2006 and December 2016 because of hydatid cyst disease were reviewed retrospectively. The patients' ages at the time of diagnosis, sexes, living areas (rural /urban), dog contact history, presence of hydatid cyst in other family members, symptoms, organs involved, dimensions of cysts, laboratory results, treatments and post treatment responses, follow-up, and outcomes were noted.

Results: In a period of 10 years, 50 pediatric patients were followed up with a diagnosis of hydatid cyst. The mean age was 9.3±0.5 years and 33 (66%) of the patients were male. Fifteen patients were living in a rural area and 35 were living in an urban area. Fifteen patients had a history of contact with a dog and 10% had a positive family history. Thirty-six patients had lung involvement, 25 had liver involvement, 14 (28%) had both lung and liver involvement, and six patients had organ involvement other than lung and liver. The indirect hemagglutination test for hydatid cyst was positive in 24 of 40 patients and Echinococcus granulosus-specific IgE positivity was detected in 8 of 17 patients. Surgery was performed in 31 patients with lung involvement and PAIR was performed in 13 patients who had liver involvement. Cyst excision was performed in two patients who had isolated spinal involvement. All patients were treated with albendazole, and additional praziquantel treatment was given to seven patients. Relapse occurred in seven patients in this period. The relapse frequency was higher in patients who had organ involvement other than in the lung and liver (p<0.05), and these patients' treatment durations were longer compared with the others (p<0.05).

Conclusion: Hydatid cysts can involve different organs in children. Patients with organ involvement other than the lung and liver should be followed up carefully in terms of recurrence.
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http://dx.doi.org/10.14744/TurkPediatriArs.2019.24119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6776445PMC
September 2019

Pleural Thickening after Pleural Effusion: How can we Follow-Up in Children?

J Trop Pediatr 2020 02;66(1):85-94

Department of Medical Education, Gazi University Medicine Faculty, Ankara, Turkey.

Introduction: No clear information exists about the factors affecting pleural thickening following parapneumonic effusion in children. We aimed to investigate factors that affect the resolving time of pleural thickening after parapneumonic effusion.

Methods: Between the years of 2007-18, 91 patients, which were followed due to diagnosis of pleural thickening after parapneumonic effusion, were assessed. Ages, complaints, physical examination findings, laboratory results, chest x-ray and ultrasonography findings, treatments, duration of treatment and recovery time of the patients were examined terms in of pleural thickening resolving time.

Results: The mean age of patients was 7.5 ± 5.0 years. Pleural thickening resolving time was 151 ± 6.8 days. The resolving time for pleural thickening was delayed with older ages, longer duration of complaints, fever before hospital admission and treatment, lower oxygen saturation at the time of admission, crackles in the physical examination, higher white blood cell count and pleural fluid density (p = 0.018, p = 0.001, p = 0.021, p = 0.020, p = 0.024, p = 0.025, p = 0.021, p = 0.019). In addition, the amount of effusion measured by thorax ultrasonography, fibrinolytic usage, and complications had a role in the delayed resolving time (p = 0.034, p = 0.001, p = 0.034). Pleural thickening resolved in 80% of the patients.

Conclusion: In this report, 80% of pleural thickening, following parapneumonic effusion resolved within 5 months. Patients who do not have a complication during follow-up are not required to monitor with frequent chest x-ray. Patients with a higher amount of pleural effusion, complications and need for fibrinolytic treatment should be followed more carefully.
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http://dx.doi.org/10.1093/tropej/fmz036DOI Listing
February 2020

Neurocognitive disorders and sleep in children with primary ciliary dyskinesia.

Pediatr Pulmonol 2018 10 10;53(10):1436-1441. Epub 2018 Jul 10.

Department of Chest Diseases, Gazi University Faculty of Medicine, Ankara, Turkey.

Background: Primary ciliary dyskinesia (PCD) patients have higher incidence of sleep disordered breathing which lead neurocognitive impairments such as attention-deficit/hyperactivity disorder (ADHD). It may effect academic performance of children and may cause impairment in emotional relationships. This study aim to evaluate hyperactivity and attention deficiency in PCD patients and investigate the relationship between sleep and hyperactivity and attention deficiency in PCD patients.

Method: Fifteen PCD patients aged 8-18 years and 31 age-matched healthy controls were compared. Ear, nose, and throat examination and home sleep testing were performed in PCD patients. Pediatric sleep questionnaire, Conners' Parents and Teacher scale and Stroop test were applied in both groups in order to investigate the relation between sleep disordered breathing and ADHD in PCD children.

Results: PCD patients had chronic rhinosinusitis (100%), tonsillar hypertrophy (80%) and adenoidal hypertrophy (60%). FEF25-75 was low in pulmonary function test. Sixty percent of the PCD patients had mild obstructive sleep apnea syndrome in home sleep testing. Mean AHI was 1.54 ± 0.27. Compared with the controls PCD patients had higher PSQ scores. Hyperactivity scores on Conners' Parents scale and inattention findings in Stroop test were higher in PCD patients than the healthy controls (P < 0.05).

Conclusion: Most of PCD children had mild obstructive sleep apnea syndrome. Hyperactivity and inattention findings were higher in PCD patients. Sleep disordered breathing assessment should be a routine part of PCD patients management and these patients should be carefully monitored in terms of hyperactivity and inattention.
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http://dx.doi.org/10.1002/ppul.24133DOI Listing
October 2018

Isolated acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus in an 8-year-old girl.

Auto Immun Highlights 2018 Mar 27;9(1). Epub 2018 Mar 27.

Department of Radiology, Faculty of Medicine, Gazi University, Ankara, Turkey.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which has broad pleuropulmonary manifestations. One of the rare and mortal complications is acute lupus pneumonitis, which is reported very rarely, especially in childhood. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation that required a lung biopsy for diagnosis. Although she had improvement with the administration of steroids, steroid treatment was reduced due to the drug's side effects resulting in the addition of azathioprine and mycophenolate mofetil to the treatment regimen. After the new regimen failed to result in clinical improvement, hydroxychloroquine treatment was started and a significant improvement was observed. Acute lupus pneumonitis is an uncommon manifestation of SLE and diagnosis may be difficult in patients without other organ involvement.
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http://dx.doi.org/10.1007/s13317-018-0104-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5869323PMC
March 2018

Left upper lobe atelectasis due to plastic bronchitis.

Turk J Pediatr 2017 ;59(2):207-209

Department of Radiology, Gazi University Faculty of Medicine, Ankara, Turkey.

Şişmanlar T, Aslan AT, Öztunalı Ç, Boyunağa Ö. Left upper lobe atelectasis due to plastic bronchitis. Turk J Pediatr 2017; 59: 207-209. Plastic bronchitis is a rare condition in children, characterized by expectoration of branching bronchial casts. It can cause atelectasis in the lung. Herein we reported a 4.5-year-old boy with left upper lobe atelectasis due to plastic bronchitis. Although his chest X-ray is specific for left upper left atelectasis, thoracic computerized tomography had been performed and was compatible with obliterated left upper lobe bronchus. Typical radiological appearance of the left upper lobe atelectasis is not well known by clinicians which results unnecessary further examinations such as computerized tomography which exposes high dose radiation. We want to emphasize the long-term side effects of radiation and avoid unnecessary examinations in children.
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http://dx.doi.org/10.24953/turkjped.2017.02.016DOI Listing
November 2018

Unknown vascular compression of the airway in patients with congenitalheart disease and persistent lower respiratory symptoms

Turk J Med Sci 2017 11 13;47(5):1384-1392. Epub 2017 Nov 13.

Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up.Results: During the 8-year study period, 62 of 253 CHD patients had persistent respiratory symptoms, of which 11 cases were diagnosed as AC via bronchoscopy and/or thoracic computed tomography angiography. The most frequently affected structures were the left main bronchus and trachea, and the most common compressing structure was the right pulmonary artery. The ADR was near total compression in 3 patients and >0.50 in 3 patients. During follow-up, 5 of the 11 patients with AC underwent surgery, 2 died, and 4 were followed clinically. Patients with ADR of >0.50 did not require surgery and were followed clinically. Conclusion: CHD patients with persistent respiratory symptoms associated with lower respiratory airway obstruction should be evaluated via invasive examination. An AC diagnostic algorithm for pediatric CHD patients was developed.
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http://dx.doi.org/10.3906/sag-1612-30DOI Listing
November 2017

Early severe anemia as the first sign of cystic fibrosis.

Eur J Pediatr 2016 Sep 5;175(9):1157-1163. Epub 2016 Aug 5.

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

Unlabelled: Severe anemia is reported to occur rarely in patients with cystic fibrosis (CF). This study aimed to determine the factors associated with early severe anemia in infants with CF. This study included 231 infants with CF from 3 pediatric CF centers ten year period that were retrospectively reviewed in terms of severe anemia as the first sign of CF. Factors that could affect anemia, such as age, pancreatic insufficiency, mutations, vitamin A and E, and albumin level were evaluated. Clinical and laboratory findings in CF patients that presented with severe anemia and no respiratory symptoms were compared to those in CF patients that did not present with severe anemia. Severe anemia as the first sign of CF was noted in 17 of 231 patients. Patient age, prolonged PT/INR and the albumin level differed significantly between the 2 groups of patients (P < 0.001). Feeding pattern, pancreatic insufficiency, vitamin E and A levels, and the types of genetic mutations did not differ between the 2 groups. The mean hemoglobin level was 5.59 ± 0.21 g/dL and respiratory symptoms began a mean 6.3 months after diagnosis of CF in the anemia group.

Conclusion: In early infancy severe anemia in the absence of respiratory symptoms can be the first sign of CF. CF should be considered in the differential diagnosis of severe anemia in infants. Anemia can occur several months before respiratory symptoms in patients with CF and may be caused due to several reasons.

What Is Known: • Severe anemia as a first sign is reported to occur rarely in patients with cystic fibrosis. • Although anemia is well known in cystic fibrosis, factors that cause severe anemia are not known clearly. What is New: • This study shows the importance of severe anemia as the first sign of cystic fibrosis. • Anemia can occur several months before respiratory symptoms in patients with CF.
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http://dx.doi.org/10.1007/s00431-016-2752-4DOI Listing
September 2016

The effect of vitamin D on lower respiratory tract infections in children.

Turk Pediatri Ars 2016 Jun 1;51(2):94-9. Epub 2016 Jun 1.

Department of Public Health, Gazi University School of Medicine, Ankara, Turkey.

Aim: Lower respiratory tract infections including mainly pneumonia represent an important public health problem leading to high mortality and mobidity rates in children aged below five years in developing countries including our country. Vitamin D deficiency has been associated with increased risk of rickets/osteomalacia, various cancers, autoimmune diseases, hyperproliferative skin diseases, cardiovascular system diseases and infectious diseases. Vitamin D has an important role in cellular and humoral immunity and pulmonary functions. Vitamin D deficiency and lower respiratory tract infection are common health problems in children in our country and no clinical study investigating the relationship between these problems has been conducted so far. In this case-control study, we aimed to assess the association between vitamin D level and lower respiratory tract infection in children.

Material And Methods: Sixty-three children aged between six months and five years with lower respiratory infections and 59 age-matched children who had no history of respiratory symptoms in the last month and no accompanying chronic disease were compared in terms of vitamin D levels. The children in the patient group were also evaluated by the clinical picture.

Results: No significant correlation was found between vitamin D levels and lower respiratory tract infection in terms of disease and its severity. However, it was found that vitamin D deficiency/ insufficiency was observed with a high rate in all children included in the study.

Conclusions: Although no correlation was found between vitamin D level and lower respiratory tract infection, it is recommended that vitamin D level should be measured in children with lower respiratory tract infection and vitamin D supplementation should be given to all children especially in winter months based on the fact that the level of vitamin D was lower than normal in approximately half of the children included in the study and considering the effects of vitamin D on infections, pulmonary functions and immunity.
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http://dx.doi.org/10.5152/TurkPediatriArs.2016.3383DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4959747PMC
June 2016

Respiratory Bronchiolitis-Associated Interstitial Lung Disease in Childhood: New Sequela of Smoking.

Pediatrics 2015 Oct 7;136(4):e1026-9. Epub 2015 Sep 7.

Pediatric Pneumology and Allergy, Children's Hospital, Ludwig Maximilians University, Munich, Germany.

Childhood interstitial lung diseases are rare disorders of largely unknown etiology characterized by variable types and degrees of parenchymal inflammation. Disease spectrum and prognosis considerably from those in adults. Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a well-described entity occurring almost exclusively in adults who are current heavy cigarette smokers. We describe an 11-year-old boy with failure to thrive, dry cough, and exertional dyspnea for 1 year who was diagnosed with RB-ILD due to heavy passive smoking exposure. Although RB-ILD is well defined in smoking adults, there are no reports in the English literature in nonactive smokers, especially in childhood.
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http://dx.doi.org/10.1542/peds.2015-1303DOI Listing
October 2015

Life-threatening, giant pneumatoceles in the course of surfactant protein C deficiency.

Pediatr Pulmonol 2015 Jul 9;50(7):E25-8. Epub 2015 Feb 9.

Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Munich, Germany.

Chronic interstitial lung diseases are rare in childhood and can present with a wide spectrum of histological abnormalities and radiological-clinical phenotypes. A 17-month-old female infant with malnutrition, recurrent lower respiratory tract infections, and failure to thrive since 3 months of age was diagnosed as surfactant protein C deficiency. Diffuse, giant, and life-threatening pneumatoceles developed during the course. They were treated with empiric drug treatment and oxygen support, and resolved rapidly. Substantial clinical and radiological improvement was observed 1 year after treatment initiation. Large-giant pneumatoceles can develop in the course of surfactant protein C deficiency and may be associated with biopsy. They can resolve with medical treatment. If available, genetic testing should be attempted as a first step for diagnosis.
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http://dx.doi.org/10.1002/ppul.23162DOI Listing
July 2015

Genotype alone does not predict the clinical course of SFTPC deficiency in paediatric patients.

Eur Respir J 2015 Jul 5;46(1):197-206. Epub 2015 Feb 5.

Department of Pediatric Pneumology, Hauner Children's Hospital, Ludwig-Maximilians University, Comprehensive Pneumology Center Munich (CPC-M), German Center for Lung Research (DZL), Munich, Germany

Patients with interstitial lung disease due to surfactant protein C (SFTPC) mutations are rare and not well characterised. We report on all subjects collected over a 15-year period in the kids-lung register with interstitial lung disease and a proven SFTPC mutation. We analysed clinical courses, interventions and outcomes, as well as histopathological and radiological interrelations. 17 patients (seven male) were followed over a median of 3 years (range 0.3-19). All patients were heterozygous carriers of autosomal dominant SFTPC mutations. Three mutations (p.L101P, p.E191 K and p.E191*) have not been described before in the context of surfactant protein C deficiency. Patients with alterations in the BRICHOS domain of the protein (amino acids 94-197) presented earlier. At follow-up, one patient was healthy (2 years), six patients were "sick-better" (2.8 years, range 0.8-19), seven patients were "sick-same" (6.5 years, 1.3-15.8) and three patients were "sick-worse" (0.3 years, 0.3-16.9). Radiological findings changed from ground-glass to increasing signs of fibrosis and cyst formation with increasing age. Empiric treatments had variable effects, also in patients with the same genotype. Prospective studies with randomised interventions are urgently needed and can best be performed in the framework of international registers.
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http://dx.doi.org/10.1183/09031936.00129414DOI Listing
July 2015

Unfinished battle with childhood tuberculosis: is it curable with less drugs and shorter duration?

Tuberk Toraks 2013 ;61(4):320-6

Division of Pediatric Chest Diseases, Department of Children's Health and Diseases, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Introduction: Tuberculosis is still a global health problem all over the world despite its mortality has been decreased with effective treatment regimens. Poor treatment adherence, acquired drug resistance, treatment failure and relapse are the major problems during the course of the tuberculosis treatment. Intermittent regimens have the advantages of reducing the side effects and the cost of the therapy and increasing the adherence, especially in resource-limited areas; and have been documented to be as effective as daily regimen in the paediatric population. In this study, we compared the results of 6-month and 9-month intermittent-therapy regimens with two drugs, given to the children with pulmonary and extrapulmonary tuberculosis at our hospital.

Materials And Methods: One hundred and fifteen patients with pulmonary and extrapulmonary tuberculosis other than meningitis, who had been given intermittent anti-tuberculosis therapy between 1986 and 2001, were evaluated retrospectively. Fifty one patients were given isoniazid and rifampin daily for 15 days, followed by the same drugs and doses twice weekly for a total of 9-months. Also, 64 patients were treated with the same regimen for a total of 6-months.

Results: Clinical recovery was observed in 75% and 79% of pulmonary tuberculosis patients at the first month of therapy in group 1 (9-month group) and group 2 (6-month group), respectively. Radiological recovery was noted between 0-6 months in 81% of the patients in group 1 and 86% of the patients in group 2. According to the clinical and radiological recovery times, no significant difference was detected between the two groups (p> 0.05). Similar results had been observed in extrapulmonary tuberculosis (p> 0.05). Follow-up periods ranged from 7 months to 15 years. There was no case of early relapse. Late relapse was noted in 4 patients, who had been received 9-month therapy (group 1).

Conclusion: Six-month intermittent therapy with two drugs is as efficacious as 9-month intermittent-therapy in childhood pulmonary and extrapulmonary tuberculosis, other than meningitis.
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http://dx.doi.org/10.5578/tt.3890DOI Listing
March 2015

Multicentric analysis of childhood tuberculosis in Turkey.

Turk J Pediatr 2013 Mar-Apr;55(2):121-9

Department of Pediatrics, Faculty of Medicine, Necmettin Erbakan University, Konya-Turkey.

Only a few series of pediatric tuberculosis (TB) have been reported in the last 20 years. The purpose of this study was to evaluate the clinical, radiological, microbiological, and treatment characteristics of childhood TB. A total of 539 children with childhood TB diagnosed over a 12-year period (1994-2005) in 16 different centers in Turkey participated in the study. The medical records of all childhood TB patients were investigated. A total of 539 children (274 males, 265 females) with childhood TB aged 10 days-17 years participated in the study. Age distribution was nearly equal among all age groups. We detected the index case in 39.8% of the patients. More than one index case was detected in 17.3% of the patients. A minimum 15-mm induration is accepted on tuberculin skin test (TST) following Bacillus Calmette-Guérin (BCG) vaccination. The TST was positive in 55.3% of the patients. Acid-fast bacillus smear was positive in 133, and polymerase chain reaction for Mycobacterium tuberculosis was positive in 45 patients. In 75 patients (13.9%), cultures yielded M. tuberculosis. One hundred fifty-one patients (28%) did not present for followup, and families of 5 patients (0.9%) discontinued the treatment. Pulmonary TB (n=285) and meningeal TB (n=85) were the most frequent diseases. In 29% of the patients, there was poor adherence to treatment or patients were lost to follow-up. We have demonstrated that household contact screening procedures play a major and important role, especially considering the high ratio of cases with contact index cases. We also recommend that the positive TST values should be reviewed according to the local cut-off data and should be specified in as many countries as possible. In view of the considerably high percentages of patients lost to follow-up and treatment discontinuation observed in our study, we suggest that application of directly observed treatment short-course (DOTS) is preferable.
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December 2013

Home sleep study characteristics in patients with mucopolysaccharidosis.

Sleep Breath 2014 Mar 22;18(1):143-9. Epub 2013 May 22.

Pediatric Metabolic Unit, Gazi University Hospital, Ankara, Turkey,

Background: Mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders caused by the deficiency of hydrolases involved in the degradative pathway of glycosaminoglycans. In MPS, upper airway obstruction may result from multiple causative factors which may impact severely upon morbidity and mortality.

Methods: We evaluated upper airway obstructive disease and related clinical findings through home sleep study in 19 patients (11 with MPS VI, 4 with MPS I, 4 with MPS II) with MPS followed at Gazi University Pediatric Metabolic Unit. Patients underwent home-based sleep measurements, and sleep respiratory problems were asked in a detailed clinical history. Measurements of apnea, apnea-hypopnea index (AHI), hypopnea index, oxygen desaturation index, and minimal oxygen saturation were obtained through home sleep study.

Results: For 19 children, the disorder was normal in 1, mild (AHI=1.5-5/h) in 5, moderate (AHI=5-10/h) in 2, and severe (AHI>10/h) in 11. The prevalence of OSA was 94.7 % (18/19) in patients with MPS. Snoring, witnessed apnea, pectus carinatum, and macroglossia were the main clinical findings. Echocardiograms showed evidence of pulmonary hypertension in 13 patients.

Conclusion: Home sleep study is a quick and accessible screening test to determine the abnormalities of breathing during sleep and enables clinicians to take necessary action for patients with severe manifestations.
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http://dx.doi.org/10.1007/s11325-013-0862-zDOI Listing
March 2014

Pediatric pulmonology in a developing country: our focus.

Turk J Pediatr 2011 Jan-Feb;53(1):11-8

Department of Pediatrics, Selçuk University Faculty of Medicine, Konya, Turkey.

This study was designed to determine the profile of our pediatric pulmonology unit in Turkey, a developing country, by investigating the patients admitted to our unit for the first time. Our objectives were: to determine the profile of patients admitted for the first time, to compare their initial diagnoses before referral to our hospital with the diagnoses determined in our unit, to determine the definitive diagnoses for patients requiring advanced intervention with invasive diagnostic methods, and to follow the treatments, operations and invasive-noninvasive mechanical ventilation practices. With these objectives in mind, the records of 412 patients who visited the pediatric pulmonology clinic within a six-month period were reviewed. The referral diagnoses, consisting mostly of primary ciliary dyskinesia, recurrent lung infections caused by immune deficiency and bronchiectasis, as well as definitive diagnoses were recorded. Tuberculosis (14%), cystic fibrosis (7.8%), bronchiectasis (4.6%), immune deficiency (1.6%), hydatid cyst (2%), and primary ciliary dyskinesia (1%) were the most commonly diagnosed diseases. Final diagnosis in 145 of the 412 patients (35.2%) differed from the referral diagnosis. Consanguineous marriages are encountered more commonly in developing countries like Turkey, leading to an increased incidence of genetic diseases such as primary ciliary dysgenesis, cystic fibrosis and immune deficiencies. Infectious diseases such as hydatid cyst and tuberculosis are also common. In any country in which there is a unique distribution of diseases, in other words, a characteristic and unique disease spectrum, courses and instructional fellowship programs should be arranged accordingly.
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June 2011

Home mechanical ventilation: outcomes according to remoteness from health center and different family education levels.

Turk J Pediatr 2010 May-Jun;52(3):267-73

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

Throughout the world, home mechanical ventilation (HMV) is being increasingly employed to treat patients suffering from chronic respiratory failure. This present study aimed to examine the characteristics and outcomes of 27 children seen in our department over a four-year period who were treated with HMV. The causes of chronic respiratory failure were as follows: 16 (59.3%) neuromuscular disease, 6 (22.2%) primary respiratory diseases, 3 (11.1%) congenital heart disease, and 2 (7.4%) storage disease. The mean age was 59.4 months (1 day-15 years); mean follow-up for invasive ventilation was 356 (0-1200) days and for non-invasive HMV was 517 (30-1440) days. With respect to maternal educational level, 13 had graduated from elementary school and 14 from high school or university. Nine of our patients resided in Ankara, while 18 lived in rural areas of Turkey. Eleven of the 27 patients died during the HMV period (1-36 months) at home. Five patients were weaned from HMV between 1-19 months. Our experience showed that HMV can be applied successfully in chronic respiratory failure patients in Turkey. Length of the follow-up period and mortality rate were not affected by the patient's place of residence (city center or rural) or maternal level of education.
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September 2010

Long-term clarithromycin in cystic fibrosis: effects on inflammatory markers in BAL and clinical status.

Turk J Pediatr 2009 Sep-Oct;51(5):416-23

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

Macrolides have antiinflammatory effects that are potentially useful in cystic fibrosis (CF). In this placebo-controlled, randomized, double-blind crossover study, 18 CF patients were randomized to receive either clarithromycin (CM) (Group 1) or placebo (Group 2) for three months. After 15 days, the treatments were crossed over. Bronchoalveolar lavage (BAL) was obtained in the beginning and at the end of each treatment period. There was no significant difference in median cell counts and median cytokine levels at baseline, after CM use and after placebo use between the two groups. In Group 2, the median neutrophil elastase (NE) level decreased with CM. Patients had less acute pulmonary exacerbations and median clinical score decreased with CM in both groups. Median z-scores for weight increased with CM in Group 2. We could not demonstrate a fall in proinflammatory cytokines in BAL; however, some improvement in clinical status could be shown with three-month CM.
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February 2010

The role of human leucocyte antigens in children with hydatid disease: their association with clinical condition and prognosis.

Parasitol Res 2010 Mar 29;106(4):795-800. Epub 2010 Jan 29.

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

Hydatid disease (HD) is a parasitosis caused by Echinococcus granulosus, which is still an important health problem worldwide, and our country is an endemic region for HD. There is little information regarding the role of human leucocyte antigen (HLA) in genetic susceptibility or resistance to HD. In this study, we aimed to investigate the HLA profile of Turkish children with HD and to compare them with healthy individuals. We also planned to investigate whether HLAs have a potential role in the predisposition to or prevention of the occurrence of HD and to study the relationship between the clinical features of HD and the HLA profile of the patients. The study included 81 children (25 boys, 56 girls) with HD aged between 3 and 18 years. All the patients' and control subjects' HLA class I and II antigens were examined, antigen allele frequencies were calculated, and clinical characteristics were also evaluated. The frequency of HLA-B18, -DR1, and -DR15 alleles were significantly different between the patients and healthy groups; HLA-DR15 antigen might be associated with HD occurrence, and the presence of HLA-B18 and HLA-DR1 antigens might be associated with HD resistance. Compared with the healthy group, patients with lung HD had a significant increase in HLA-B44 frequency, and liver HD patients had a significant increase in HLA-DR15 antigen frequency. Furthermore, presence of HLA-DR11 was found to be a significant factor associated with cure of the disease. We concluded that HLA types have significant impact on the development of HD and clinical course of disease.
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http://dx.doi.org/10.1007/s00436-009-1719-zDOI Listing
March 2010

Successful unilateral partial lung lavage in a child with pulmonary alveolar proteinosis.

J Clin Anesth 2009 Mar;21(2):127-30

Pulmonary Medicine Unit, Department of Pediatrics, Hacettepe University, Sihhiye, Ankara, Turkey.

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within the alveoli. A 4-year-old child with autoimmune PAP, who was successfully treated with a series of unilateral partial bronchoalveolar lavages by selectively ventilating the other lung with a cuffed endotracheal tube, is presented.
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http://dx.doi.org/10.1016/j.jclinane.2008.06.035DOI Listing
March 2009