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

Introduction Flexible bronchoscopy (FB) is frequently used for assessment and treatment of patients with respiratory diseases. Our aim was to investigate the contribution of FB to diagnosis and therapy in children admitted to the intensive care units (ICU) and to evaluate the safety of FB in this vulnerable population. Methods Children less than 18 years of age who underwent FB in the five neonatal and pediatric ICUs in Istanbul between July 1st, 2015 and July 1st, 2020 were included to the study. Demographic and clinical data including bronchoscopy indications, findings, complications, and the contribution of bronchoscopy to the management were retrospectively reviewed. Results One hundred and ninety-six patients were included to the study. The median age was 5 months (range 0.3-205 months). The most common indication of FB was extubation failure (38.3%), followed by suspected airway disease. Bronchoscopic assessments revealed at least one abnormality in 90.8% patients. The most common findings were airway malacia and the presence of excessive airway secretions (47.4% and 35.7%, respectively). Positive contribution of FB was identified in 87.2% of the patients. FB had greater than 1 positive contribution in 138 patients and 80.6% of the patients received a new diagnosis. Medical therapy was modified after the procedure in 39.8% and surgical interventions were pursued in 40% of the patients. Therapeutic lavage was achieved in 18.9%. There were no major complications. Conclusion Flexible bronchoscopy is a valuable diagnostic and therapeutic tool in neonatal and pediatric ICUs and is not associated with major complications.

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

Background: COVID-19 outbreak lead to nationwide lockdown on the March 16th, 2020 in Turkey. We aimed to quantitively determine the change in frequency of upper and lower respiratory tract infections and asthma in pediatric population associated with COVID-19. Methods: The electronic medical record data of pediatric population admitted to the emergency department (ED), outpatient and inpatient clinics and pediatric intensive care unit (PICU) were analyzed with the diagnosis of Influenza, upper and lower respiratory tract infections (URTI, LRTI) acute bronchiolitis and asthma. The data of the first year of the pandemic was compared with the previous year. Results: In total 112496 admissions were made between April 1, 2019 and March 31, 2021 in our hospital. A decline was observed in ED admissions (-73%) and outpatient clinic (-70%) visits, hospitalizations (-41.5%) and PICU admissions (-42%). The admissions with the diagnosis of Influenza and URTI had a decline from 4.26% to 0.37% (p=0.0001), and from 81.54% to 75.62% (p=0.0001), respectively. An increase was observed in the LRTI, acute bronchiolitis and asthma (from 8.22% to 10.01% (p=0.0001), from 2.76% to 3.07% (p=0.027) and from 5.96% to 14% (p=0.0001), respectively). Conclusions: A dramatic decrease was observed in the number of admissions to ED and inpatient clinics and outpatient clinic visits and PICU admissions, and, when the rates of admissions were compared, the general rate of admissions to ED showed a decrease while inpatient, outpatient clinics and PICU admissions demonstrated an increase during the pandemic.

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

Objective To investigate whether partial arterial carbon dioxide pressure (PaCO 2 ) level in arterial blood gas analysis that was used to predict nocturnal hypoventilation (NH) is concordant with nocturnal end-tidal CO 2 (PetCO 2 ) measurement obtained by a noninvasive method of oxycapnography in children with neuromuscular disease (NMD). Methods Twenty-one patients aged 6-18 years with a confirmed diagnosis of NMD were enrolled. Each patient underwent a nocturnal oxycapnography study using an orinasal probe and a pulse oximetry finger probe to record PetCO 2 , oxygen saturation (SpO 2 ), pulse rate, and respiratory rate. Arterial blood gas analysis was performed to record PaCO 2 levels on three occasions at night (23:00 pm, 03:00 am, 07:00 am). Results The mean overnight PaCO 2 level of the three blood gas analyses (mean PaCO 2 noct) was 41.78±4.69 mmHg. A significant change was observed between mean PaCO 2 23:00 and PaCO 2 07:00 levels (p=0.032). There was no significant difference between PaCO 2 , PetCO 2 , and SpO 2 levels in the NMD group. The interclass correlation coefficient between PaCO 2 07:00 and PetCO 2 levels was 0.791 (95% CI: 0.533-0.923); the interclass correlation coefficient between overnight mean PaCO 2 and PetCO 2 levels was 0.811 (95% CI:0.533-0.923). Conclusion Our study indicates that nocturnal PetCO 2 and PaCO 2 levels were statistically comparable but the use of PaCO 2 alone is not adequate to make an early diagnosis of NH in NMD. There is a need for making more restrictive definitions for NH, and conducting studies with larger study populations to reach an agreement on the best definition of hypoventilation, and updating consensus guidelines.

Objective: We aimed to evaluate the demographic, epidemiological, and clinical characteristics of children with coronavirus disease‐2019 (COVID-19), and to determine variations of the clinical presentation of the disease by age. Method: A total of 104 confirmed COVID-19 patients aged between 0-18 years were retrospectively analyzed. The study period was between March 15, 2020 and July 1, 2020. A confirmed COVID-19 was defined as a child who has positive real-time reverse transcription-polymerase chain reaction (rRT-PCR). The patients were analyzed in terms of demographic, epidemiological, clinical, laboratory and imaging features, and clinical outcomes. Results: The median age (inter-quartile range) of the patients was 11.8 (8.4) years, and 53 (51%) were male. Ninety-eight (94%) of the patients had an epidemiological risk factor. The most frequent symptoms were cough (62%), fever (42%), and fatigue (33%). Seven patients (7%) were asymptomatic, 84 (81%) were mild, 9 (9%) were moderate, and 4 (4%) were in severe or critical condition. Ninety-eight (94%) of the patients underwent a chest x-ray, and 12% had pneumonia. The patients younger than 5 years were more likely to have fever (p = 0.019), rhinorrhea (p = 0.041), and diarrhea (p = 0.035). Muscle/join pain (26%), loss of smell/taste (20%), and headache (19%) were striking extra-pulmonary symptoms in older patients. Only 8% of the patients were hospitalized, and 4% were admitted to the intensive care unit. No death occurred. Conclusion: The most of the pediatric COVID-19 cases have mild symptoms without pneumonia. They may not present with fever or cough which are considered characteristic symptoms of the disease and the frequency of signs and symptoms may vary by age. Children younger than 5 years old are more likely to have fever, rhinorrhea, and diarrhea. School-age children and adolescents may present with extra-pulmonary symptoms such as muscle/join pain, smell/taste loss, and headache.

Pulmonary arteriovenous malformations (PAVM) are generally congenital lesions caused by abnormal capillary development. Lesions can be in the form of isolated anomaly or as part of autosomal dominantly inherited hereditary hemorrhagic telengiectasia (HHT). HHT is the most common hereditary vascular disease characterized by mocucutaneuos telengiectasia and visceral arteriovenous malformations. PAVMs can be asymptomatic or can present with effort dyspnea, palpitations and fatigue especially in cases with HHT. Herein, we present a 13 year-old girl diagnosed with PAVM with polycythemia, clubbing, cyanosis and radiological features; and had accompanying history of epistaxis in family and telengiectasia in oral mucosa as parts of HHT. She was treated by endovascular embolization.

Introduction: Although liver support systems play a major role as a bridge to transplantation, they may sometimes serve as a lifesaving treatment eliminating this need. Case presentation: We report on a 4-year-old boy who developed acute liver failure due to an influenza A (H3N2) infection as confirmed by clinical and laboratory data (molecular typing, stage 3 encephalopathy, brain edema, increased levels of ammonia, bilirubin, and international normalized ratio 5.2). Testing for any possible underlying liver disease showed no congenital or acquired liver pathology. Oral oseltamivir treatment was initiated and liver support therapy with plasma exchange was performed as a bridge to transplantation. A total of three plasma exchange sessions every other day, with fresh frozen plasma 1.5 times the total blood volume for the first course and 1 times for the subsequent courses, were performed. After the first plasma exchange, encephalopathy improved to stage 2, accompanied by substantial decreases in the elevated liver function tests. At the end of three plasma exchange sessions, the patient’s clinical condition improved significantly. At 14 days after admission, deep tendon reflexes of the patient were normal and levels of alanine transaminase, aspartate transaminase, bilirubin, ammonia, and international normalized ratio returned to normal. Liver support treatment with plasma exchange resulted in complete recovery and the patient was discharged on the 17th day of admission. Conclusion: Acute supportive treatment with plasma exchange proved to be life-saving in our case.

Introduction: The aim of this study was to asses the surveillance of influenza A/other respiratory viruses and risk factors in hospitalized children with the symptoms of influenza-like illness during two consecutive influenza seasons. Methodology: All children hospitalized with adiagnosis of influenza-like illness had been investigated for Influenza A and other respiratory antigens in pharengeal/nasopharyngeal secretions. Results: A total of 132 hospitalized children between December 2013-May 2014 and December 2014-May 2015 were enrolled in this study. At least one respiratory virus was found to be positive by RT-PCR in 78 (59%) patients, influenza A (H3N2) was detected in only 8 (6%) patients. In 54 (41%) patients samples no respiratory viral pathogen was detected and in 70 (53%) patients, one non- influenza A virus was detected. The respiratory viral pathogens detected in decreasing rates were:RSV (n = 46, 35%), HCoV (n = 10, 7.5%), adenovirüs (n = 7, 5%), rhinovirüs (n = 6, 4.5%), HMPV (n = 5, 4%), Influenza B (n = 4, 3%) ve human Bocavirus (n = 2, 1.5%). In 10 patients, coinfection was detected, however none was with H3N2. In the H3N2 (+) group, the following risk factors were identified: age older than three years (p < 0.05), asthma history (p < 0.05) and chronic lung diseases (p < 0.05). Conclusion: Influenza A virus was detected in 6% of hospitalized patients with influenza-like illness. Viruses other then Influenza, especially RSV, can cause similar symptoms compatible with Influenza-like-illness.

Objectives: This study aims to analyze the coherence between T-SPOT tuberculosis test (T-SPOT.TB) and tuberculin skin test (TST) with different cut-off values in screening latent tuberculosis infection (LTBI) both prior to and at the sixth month of anti-tumor necrosis factor (anti-TNF) treatment.Patients and methods: This prospective multicentric study included 57 children (34 girls, 23 boys, mean age 12.4 +/- 3.9 years; range, 6 to 18 years) diagnosed with immune-mediated inflammatory diseases (IMIDs) evaluated with TST and T-SPOT.TB for screening LTBI both prior to and at the sixth month of treatment with anti-TNF agents. Coherence between two tests was analyzed for TST cut-off values suggested by the local guidelines and also for different possible cut-off values of TST.Results: Tuberculin skin test was positive (>= 5 mm) in 28.1% (n=16) of patients in the screening prior to treatment and in 33.3% (n=19) at the sixth month of treatment. T-SPOT.TB test was positive in 8.8% (n=5) of patients both prior to and at the sixth month of treatment. Coherence between two tests was poor or fair when compared with all possible TST cut-off values both prior to and at the sixth month of anti-TNF therapy.Conclusion: Our results show poor coherence between T-SPOT.TB and TST for all possible cut- off values of TST. Thus, using both tests would be beneficial in screening LTBI until further studies bring new evidence on the subject.

Tuberculosis and hydatid cyst are still major health problems in developing countries. Co-existence of these two infections is also seen commonly. In this article, we present a 15-year-old-girl patient who applied with cardiac tamponade accompanied by pleural and pericardial effusion and was diagnosed as pericardial hydatid cyst and pericardial tuberculosis. To the best of our knowledge, this is the first case with such feature reported in the literature.

Amac: Bronsiyolitis obliterans (BO) nadir gorulen ve alt solunum yollarinda agir hasar sonrasi olusan obstruktif ve inflamatuvar reaksiyon ile karakterize kronik akciger hastaligidir. Pnomoniden alti hafta sonra devam eden hisilti, oksuruk, krepitan rallerin duyulmasi ve yuksek cozunurluklu bilgisayarli tomografide mozaik perfuzyon, vaskuler atenuasyon ve santral bronsektazi bulgulari varliginda dusunulur. Gastroosefageal reflusu (GOR) olan hastalarda tekrarlayan aspirasyonlar akcigerlerde kimyasal ve enfeksiyoz hasara yol acabilmektedir. Calismamizda tekrarlayan hisilti nedeniyle izlenen, klinik ve radyolojik bulgulari ile bronsiyolitis obliterans tanisi alan hastalarda GOR birlikteliginin arastirilmasi ve GOR varliginin hastaligin prognozuna etkisi olup olmadiginin belirlenmesi amaclanmistir. Gerec ve Yontemler: Persistan hisiltili hastalar arasindan onceden BO tanisi alan 84 hasta calisma grubunu ve ayni yas ve cinste olan 25 GOR(+) ve 25 GOR(-) hasta secilerek kontrol grubu olusturuldu. Alti aylik izlem sonrasinda klinik ve radyolojik duzelme degerlendirildi. Bulgular: Hastalarin ortalama tani yasinin 2.9 yas oldugu ve ilk hisilti atagi ile basvurdugu yasin ise ortalama 9.6 ay oldugu belirlendi. Bronsiyolitis obliteransli hastalarin % 63’u erkek ve GOR sikligi % 41 bulundu. Seksen dort hastanin 68’ine kontrol HRCT cekilebildi ve 37 hastada (%54) klinik ve radyolojik duzelme goruldu, 16 hastada (%23) sadece klinik duzelme, 5 hastada (%7) sadece radyolojik duzelme gozlendi. Geriye kalan 10 hastada klinik ve radyolojik duzelme saptanmadi. Reflu varliginin klinik ve radyolojik prognoz uzerinde istatistiksel olarak anlamli fark olusturmadigi saptandi (p=0.428). Sonuc: Sonuc olarak BO’lu hastalarda etiyolojide GOR olasiligi veya kronik obstruktif akciger hastaligina sekonder GOR gelisme ihtimali oldugundan GOR birlikteligi arastirilmalidir. Calismamizda %41 oraninda GOR birlikteligi saptanmistir. Ancak GOR varliginin hastaligin klinik ve radyolojik prognozunda etkisi olmadigi gozlenmistir. Bu hastalarda GOR’u daha duyarli yontemlerle arastiran ve daha fazla hastayi degerlendiren kontrollu calismalara ihtiyac oldugu dusunulmustur.

Purine Nucleoside Phosphorylase (PNP) deficiency is a rare autosomal recessive metabolic disorder. In PNP-deficiency disorder, the deficient enzyme leads to accumulation of toxic metabolites, especially in lymphocytes and the metabolites exert toxic effect on T-cell generation. Purine nucleoside phosphorylase deficiency causes decreased numbers of T cells and lymphopenia. The patients suffering from PNP-deficiency may be admitted with recurrent infections, as well as neurological and autoimmune findings. We hereby presented a case admitted with the symptom of hematuria in which we established the diagnosis of PNP-deficiency early on the basis of detection of lymphopenia and low level of uric acid.

Yazıcı, Selçuk (Balikesir Author), Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behcet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8.2%, lung disease secondary to gastro-esophageal reflux 3.7%, foreign body aspiration 2.7%, tuberculosis % 2.7, congenital malformation 1.8% and asthma 1.8%, respectively. In 15.4% of cases, etiology was not identified clearly. 91% of the patients were medically treated. In ten years, the frequency of asthma and tuberculosis in etiology had decreased but primary ciliary dyskinesia and primary immune deficiency had increased. Non-cystic fibrosis bronchiectasis can be followed up for a long time with medical treatment.

Amaç: İnek sütü alerjisi çocukluk çağında en sık görülen gıda alerjisidir. Tanıda gecikme olduğunda sistemik reaksiyonlar ortaya çıkarken, yanlış tanı konulduğunda annenin ve çocuğun yetersiz beslenmesine neden olmaktadır. Diğer alerjik hastalıklar gibi besin aler- jileri de son yıllarda giderek büyüyen bir sorundur. Amacımız, inek sütü alerjisi vakala- rının özelliklerini saptamak ve sıklığının artıp artmadığını belirlemektir. Gereç ve Yöntemler: Çalışmamız retrospektif ve kesitsel olarak planlandı. Hasta verileri Pediatrik Allerji Bölümü hasta dosyalarından toplandı. Gıda alerjisi tanısı konulan has- talar arasından inek sütü alerjisi saptanan hastalar çalışmaya alındı. Bulgular: 2003-2011 yılları arasında gıda alerjisi tanısı ile takip edilen 264 olgu arasından inek sütü alerjisi tanısı alan 105 olgu çalışmaya alındı. Hastaların çoğu 2 yaşından küçük (%78,1), erkek cinsiyette (%62,9) idi ve anamnezinde süt alımını takiben kesin öykü vardı (%79,1). Olguların %75,2’sini IgE aracılı inek sütü alerjileri oluşturmaktaydı. Hastaların yarısından fazlası 2010 ve 2011 yıllarında başvuran hastalardı. Bu artışdan daha çok 1 yaş altındaki hastaların fazla olması sorumluydu (p=0.015). En sık tutulan sistem, deri (%67,5) ve multisistem (%15,7) olarak bulundu. Sonuç: Detaylı bir öykü inek sütü alerjisi tanısında önemli bir rol oynar. Yaşamın ilk yılı içinde çok sayıda hasta çeşitli semptom ve bulgularla karşımıza çıkar. Genellikle hayatı tehdit eden bir durum olmamakla birlikte beslenme, çocuğun gelişimi ve büyümesinde rol oynadığı için doğru tanı çok önemlidir., Objective: Cow’s milk allergy is the most frequent food allergy in childhood. Delayed diagnosis may cause a number of systemic dysfunctions, while incorrect diagnosis may result in deficient nutrition of the mother and the child. As in other types of allergies, food allergy is also a growing problem in recent years. Our aim was to determine the characte- ristics of our patients allergic to cow’s milk, and see if there was an increasing trend. Material and Methods: The study was of retrospective and cross-sectional design. Patient data were collected from Pediatric Allergy Department patient files. Cases of food allergy were reviewed and patients fulfilling the criteria of cow’s milk allergy were included in the study. Results: A total of 105 cases with cow’s milk allergy out of 264 food allergies who were followed up between 2003-2011 were investigated. Most of the patients were under 2 years of age (78.1%), were males (62.9%) and presented with a history of allergy (79.1%). IgE mediated cases constituted the majority (75.2%). More than half of the patients admitted in 2010 and 2011. This was mostly due to the increased number of patients less than twel- ve months of age (p=0.015). Most frequently involved system was skin (67.5%) and mul- tisystem involvement was 15.7% Conclusion: A detailed history is the key element of diagnosing cow’s milk allergy. There is a considerable number of cases within the first year of life which may present with dif- ferent signs and symptoms. Although not a life threatening situation, correct diagnosis is vital as nutrition is a key component in the development and growth of child.

Objective To evaluate tubal patency by transvaginal sonosalpingography. Design Comparative study of transvaginal sonosalpingography with chromolaparoscopy in subjects with unknown tubal function. Setting Outpatient infertility clinic at Zeynep Kamil Maternity Hospital in Istanbul, Turkey. Patients Forty-two cases of unknown tubal function with infertility complaints were included. Interventions Isotonic saline was injected into the uterine cavity through a catheter. Main Outcome Measures Transvaginal sonosalpingography is a safe, easy, and cost-effective procedure for screening tubal status. Results The results obtained from transvaginal sonosalpingography and laparoscopy were completely consistent for 29 cases (76.32%) and partially consistent for 8 cases (21.05%). Transvaginal sonosalpingography accurately showed patency in 26 patients and bilateral nonpatency in 3 patients. Conclusions Transvaginal sonosalpingography, with its accuracy and safety, is a promising screening and diagnostic technique in the evaluation of tubal patency on ambulatory basis.