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Aim: Coronavirus disease 2019 (COVID-19) is a severe acute respiratory syndrome with a high mortality rate and has been labeled a global pandemic in March 2020. Gaucher Disease (GD) is one of the rare inherited lysosomal storage diseases (LSDs). We aimed to call attention to the frequency, susceptibility of COVID-19 infection, and the factors that prevent this infection in patients with GD as compared to other LSDs. Material and Methods: The study was conducted retrospectively between September and December 2020. Participants were divided into two groups: GD group (19 patients) and the control group (19 patients, those with other LSDs). All patients were contacted by phone to collect data about their health status, and any possible contact with Covid-19 patients. Results: Six of the GD patients (36.8%) had contacted a confirmed COVID-19 infected person but only three (15.8%) had developed a mild COVID-19 with fever and fatigue that did not require hospital admission. Four of the control group patients (21.1%) had experienced contact with a person with a confirmed COVID-19 infection. Three of the control group patients, that comprised of patients with various LSDs other than GD (15.8%) were positive on COVID-19 PCR tests and two of them had developed a mild COVID-19 infection. One of these (with Mucopolysaccharidosis type 1) had severe symptoms and required hospitalization. Conclusion: There is no consensus on the management of rare diseases such as lysosomal storage diseases during the COVID-19 pandemic. Developing plans regarding the management of COVID-19 infections in LSDs will be useful when drawing up consensus guidelines.

There is limited data on the pattern and severity of myocardial injury in patients with COVID-19 vaccination associated myocarditis.We aimed to define the myocardial damage occurring after BNT162b2 vaccination, raise awareness about adverse reactions developing after vaccination, and determine the patterns and scope of Cardiac magnetic resonance imaging (MRI) findings.A total of 9 patients diagnosed with vaccine-associated myopericarditis were followed up.The mean age of the patient at diagnosis was 15.3 ± 1.0 (range: 14-17) years, and all patients were male. Seven patients presented with myocarditis symptoms after their second vaccine dose, one patient presented with pericarditis symptoms after his first dose, and the other patient presented with myocarditis symptoms after his booster dose. The median time at presenting to the hospital was 3 (range: 2-22) days. Seven (77.7%) patients had abnormal electrocardiography (ECG) findings, and the most prevalent finding was diffuse ST-segment elevation. Initial cardiac MRI results were abnormal in all patients, where 8 (88.8%) patients had late gadolinium enhancement, and 5 (55.5%) had myocardial edoema. Three patients showed local left ventricular wall-motion abnormalities. In their follow-up MRIs 3-6 months later, myocardial edoema was present in 2 (28.5%) patients, while late gadolinium enhancement was present in all patients (7/7, 100%, 2 patients did not have control MRI time). Hypokinetic segments were still present in one of the 3 patients. No negative cardiac events were observed in the short-term follow-up of any patient.Further follow-up evaluation and larger multicenter studies are needed to determine the clinical significance of persistent cardiac MRI abnormalities.

Objective The aim of this study is to determine parents' knowledge levels and attitudes about childhood human papillomavirus (HPV) vaccination and reveal the differences between parents who are health care workers and nonhealth care workers. The HPV vaccine can potentially prevent initial HPV infection and HPV-related genital and anal precancerous disorders and cancers. Methods Questions and attitudes toward childhood HPV vaccination were asked to 564 parents who agreed to participate in the study. Results In our study, the rate of health care worker parents considering having their children vaccinated against HPV (63.1%) was higher than that of nonhealth care worker parents (11.4%). Only 5% of parents had their children previously vaccinated against HPV; however, there was no difference between parents who were health care workers and those who were not. In addition, this rate was relatively low compared to other paid vaccines (49.8, 35.3, and 18.8%, respectively). Parents who had not previously vaccinated their children against HPV were considering getting their children vaccinated, especially if a pediatrician recommended it. Conclusion The parents' high level of health literacy may not be effective in deciding to vaccinate their children against HPV. Therefore, adequate information and awareness should be provided to all parents about HPV vaccination as early as possible for all children of appropriate age. Pediatricians should be given important roles in raising the awareness of parents about childhood HPV vaccination by health authorities.

Objective Encouraged by reports of favorable outcomes following the use of corticosteroids in patients with moderate-to-severe coronavirus 2019 (COVID-19) pneumonia, we aimed to present our experience with early short-term corticosteroid use at our center in pediatric patients with COVID-19 pneumonia. Methods One hundred and twenty-nine pediatric patients were included in the study. Patients were divided into four groups according to the type and dose of corticosteroids given: Group 1 (those receiving dexamethasone 0.15 mg/kg/d); Group 2 (those receiving methylprednisolone 1 mg/kg/d); Group 3 (those receiving methylprednisolone 2 mg/kg/d); and Group 4 (those receiving pulse methylprednisolone 10–30 mg/kg/d). Results Of 129 patients, 19 (14.7%) patients were assigned to Group 1, 30 (23.3%) patients to Group 2, 30 (23.3%) patients to Group 3, and 50 (38.8%) patients to Group 4. Thirty-two (24.8%) patients were followed in the pediatric intensive care unit (PICU), of whom 13 (10%) required mechanical ventilation, and 7 (%5.4) died. In Group 4, the hospitalization length was significantly longer than in other groups (p Conclusion Although children usually have a milder course of COVID-19 than adults, underlying diseases and obesity increase the severity of disease manifestations also in children. Further studies are needed to define the exact role of corticosteroids in COVID-19 patients.

İmmün trombositopenik purpura (İTP), izole trombositopeni ile kendini gösteren hematolojik bir hastalıktır. Etiyolojisinde son bir aydaki viral enfeksiyonlar sıklıkla bulunur. COVİD-19 enfeksiyonu sonrası çeşitli hematolojik komplikasyonların yanı sıra ITP gelişimi de görülebilmektedir. Bu yazıda COVİD-19 enfeksiyonu sırasında teşhis edilen ve kanama bulgusu olmayan bir çocuk akut ITP olgusu sunulmaktadır.

In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up.MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively.A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p 0.001, p 0.001, p 0.001, p 0.001, p = 0.005, p 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization.MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points • In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

Amaç: COVID-19 pandemisinin başlangıcından itibaren viral bulaşı ve hastalık yayılmasını azaltmak ve engellemek amacıyla dünya genelinde olduğu gibi ülkemizde de medikal olmayan birincil önlemler alındı. Çalışmamızda bu önlemlerin çocuk hastalarda önceki dört yıla göre bronşiolit ve suçiçeği oranlarına etkisini belirlemeyi ve değerlendirmeyi, ayrıca pandemi döneminde viral solunum yolu enfeksiyonlarına hangi etkenlerin sebep olduğunu değerlendirmeyi amaçladık.Gereç ve Yöntemler: Bir ay ile 18 yaş arasındaki hastaların tanı ve laboratuvar verileri geriye dönük olarak hastane bilgi sisteminden alındı. Bronşiolit ve suçiçeği tanılı hasta sayılarının Ocak 2016’ dan Aralık 2020’ ye kadar olan aylara göre dağılımı gösterildi. Pandemi döneminde, hastaların ilk başvurusunda alınan nazofaringeal aspirat örneklerinde polimeraz zincir reaksiyonu (polymerase chain reaction, PCR) yöntemiyle saptanan viral etkenler araştırıldı.Bulgular: Ocak 2016-Aralık 2020 tarihleri arasında hastanemize başvuran 2.254.877 çocuk hastanın verileri incelendi. Toplamda 38.458 bronşiolit ve 954 suçiçeği tanılı hasta vardı. Pandemi döneminde bronşiolit vakalarında önceki yıllara göre %85.6 oranında azalma görüldü ve önceki yıllarda görülen suçiçeği piklerinin hiçbiri görülmedi. Pandemi döneminde bronşiolit hastalarında en sık etkenin Rhinovirüs olduğu bulundu. Respiratuvar Sinsitiyal Virüs (RSV) ikinci sırada yer aldı. İnfluenza sıklığında da önemli bir azalma gözlendi.Sonuç: Çalışmamız, sosyal hayatı kısıtlayan ve sosyal mesafeyi ön planda tutan önlemlerin viral enfeksiyonların yayılmasını engellediğini ortaya koymuştur. Ayrıca pandemi döneminde Rhinovirüs enfeksiyonu sıklığında artış olduğunu göstermiştir.Anahtar Sözcükler: Bronşiolit, Suçiçeği, COVID-19, Rhinovirus, Objective: Primary preventive nonpharmaceutical interventions were introduced to reduce viral transmission and disease spread at the beginning of the COVID-19 pandemic. Therefore, herein, we aimed to determine and assess the impact of the nonpharmaceutical interventions on bronchiolitis and varicella infection rates in the pediatric population during the pandemic compared to the previous four years. We also aimed to evaluate which viruses cause viral respiratory tract infections during the pandemic period.Material and Methods: Diagnosis and laboratory data of the patients who were one month to 18 years of age were retrospectively retrieved from hospital records. The distribution of the number of patients with bronchiolitis and chickenpox diagnoses was shown monthly between January 2016 and December 2020. Viral agents detected by polymerase chain reaction (PCR) in the nasopharyngeal aspirate samples obtained at the first application of the patients during the pandemic period were investigated.Results: The data of 2.254.877 pediatric patients admitted to our hospital from January 2016 to December 2020 were examined. There were 38.458 bronchiolitis and 954 chickenpox cases reported both as inpatients and outpatients. There was a 85.6% decrease in the rate of bronchiolitis compared to previous years, and chickenpox peak was not observed in the pandemic period. Rhinovirus was found to be the most common etiologic agent of bronchiolitis during the pandemic period and Respiratory Syncytial Virus (RSV) came second. A significant decrease in the frequency of influenza was also observed.Conclusion: Our study reveals that the measures which curtail social life and prioritize social distancing prevent the spread of viral infections. It has also shown that there is an increase in the frequency of Rhinovirus infection during the pandemic period.

Bruselloz pastörize edilmemiş süt ve süt ürünlerinin ağız yoluyla alınmasıyla veya enfekte hayvan salgılarıyla yakın temas yoluyla bulaşan, gram negatif bir kokobasil olan Brucella türlerinin neden olduğu ülkemizde de yaygın olarak görülen zoonotik bir hastalıktır. Bruselloz özgül olmayan semptom ve bulgulara sahip olması nedeniyle diğer birçok hastalığı taklit edebilir. Kırım Kongo Kanamalı Ateşi (KKKA) brusellozun ayırıcı tanısında yer alan zoonotik bir hastalıktır. Bu yazıda KKKA ön tanısı ile hastanemize sevk edilen ve bruselloz tanısı konulan iki pediatrik olgu sunulmuştur., Brucellosis is a common zoonotic disease in our country caused by Brucella species, a gram negative coccobacillus that is transmitted by oral ingestion of unpasteurized milk and dairy products or by close contact with infected animal secretions. Because brucellosis has nonspecific symptoms and findings, it can mimic many other diseases. Crimean-Congo Hemorrhagic Fever (CCHF) is a zoonotic disease included in the differential diagnosis of brucellosis. In this article, two pediatric cases referred with a pre-diagnosis of CCHF and diagnosed with brucellosis were presented.

Introduction The aim of this study was to make the differential diagnosis between acute appendicitis and multisystem inflammatory syndrome in children (MIS-C) for patients presenting with the complaint of acute abdominal pain (AAP) and to identify the determining factors for the diagnosis of MIS-C. Materials and Methods Eighty-one children presenting with AAP/suspected AAP were evaluated. Of these, 24 (29.6%) were included in the MIS-C group (MIS-C/g) and 57 were included in the suspected appendicitis group (S-A/g), which consisted of two subgroups: appendicitis group (A/g) and control observation group (CO/g). Results Comparing MIS-C/g, A/g, and CO/g, duration of abdominal pain (2.4, 1.5, 1.8 days), high-grade fever (38.8, 36.7, 37°C), severe vomiting, and severe diarrhea were higher in MIS-C/g. Lymphocytes count (LC) was lower, while values of C-reactive protein (CRP), ferritin, and coagulopathy were higher in MIS-C/g (p Conclusion The duration of abdominal pain, presence of high-grade and prolonged fever, and evaluation of hemogram in terms of high neutrophil count and low LC exhibit high sensitivity and negative predictive value for MIS-C presenting with AAP. In case of doubt, inflammatory markers such as CRP, ferritin, D-dimer, and serology for SARS-CoV-2 should be studied to confirm the diagnosis.

Community-acquired pneumonia (CAP) in children continues to be one of the prominent causes of pediatric morbidity and mortality worldwide. By determining the risk factors associated with the development of complicated CAP (CCAP), new approaches for early diagnosis and effective treatment can be identified.This retrospective cohort study enrolled patients with CAP and CCAP who visited the pediatric ward of the study hospital between January 1, 2017 and December 31, 2017. For patients with CCAP, data regarding medical procedures performed, surgical intervention, and hospitalization duration were collected.A total of 111 patients, 93 (83.7%) with CAP and 18 (16.3%) with CCAP, aged between 3 months and 18 years were hospitalized because of severe pneumonia. The mean age of the patients was 3.6 ± 1.2 years and 60 (54%) of them were female. The mean age of patients with CCAP was higher than that of patients with CAP (4.2 ± 3.3 vs. 2.8 ± 2.1 years respectively); however, the difference was not significant (p = 0.012). Patients with CCAP exhibited a significantly higher C-reactive protein level than those with CAP (10.06 ± 7.55 vs. 4.43 ± 3.37 g/L respectively; p = 0.007). Hypoxia upon admission was noted more commonly in the CCAP group than in the CAP group (p 0.001).Findings related to hypoxia, respiratory distress, and pleural effusion on imaging are important distinguishing factors associated with the development of complications in patients hospitalized with CAP. Therefore, CCAP etiology, diagnosis, and treatment approaches should be established and protective measures adopted.

Tetanus is an acute-onset disease that results from exposure to the exotoxins produced by Clostridium tetani. It is characterized by tonic muscle spasms and has high morbidity and mortality. Respiratory failure is the most severe problem related to generalized tetanus. Mechanical ventilation is almost always mandatory for the therapy of respiratory insufficiency in patients with tetanus-developed airway obstruction. The patient presented herein was 15 years old and unvaccinated, and had generalized tetanus and respiratory failure. He was transported to pediatric intensive care unit following intubation. In this report, we aimed to present the follow-up and treatment of a patient with severe tetanus in pediatric intensive care unit.

Coronavirus disease of 2019 (COVID-19) is a cause of significant morbidity and mortality worldwide. Although COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported. The mechanism of cardiac injury and arrhythmias is unclear. Also, drugs currently used to treat the COVID-19 may prolong the QT interval and may have a proarrhythmic propensity. The study aims to investigate the effects of COVID-19 infection with asymptomatic and mild symptoms on trans-myocardial repolarization parameters in children without treatment. A total of 105 COVID-19 patients were compared with 40 healthy children. The patient and control group data were compared by calculating the QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e, Tp-e dispersion, Tp-e/QT ratio, and Tp-e/QTc ratio on the 12-lead surface electrocardiogram. The mean age was determined as 11.2 ± 0.3 years in the patient group, and 10.8 ± 2.1 years in the control group. In the COVID-19 group, QTd, QTcd, Tp-e, Tp-e dispersion, Tp-e/QT ratio and Tp-e/QTc ratio were statistically higher than the control group. The ventricular repolarization was impaired even in asymptomatic children with COVID-19 infection. These results suggest the need to further assess the long terms risks of prolonged QT dispersion in the setting of COVID-19 infection.

Currently, there is not any specific effective treatment for COVID-19. There are many studies published and ongoing especially on adult patients. Treatment options in pediatric patients are determined according to the agents used in adult patients. Although coronavirus disease 2019 (COVID-19) is mild in nearly all children, a small proportion of pediatric patients develop severe or critical illness. Supportive therapy forms the basis of the treatment as the symptoms and disease course in children are mild. There are currently no randomized controlled trials of drugs that can be used to treat COVID-19 in children. However, in severe clinical cases, the drugs used in adults are evaluated and used on a case-by-case basis. There is a growing need for well-designed controlled clinical trials to better define the safety and efficacy of potential treatments for COVID-19 in children.

Background: Infectious complications are one of the most life-threatening complications and result in substantial mortality and morbidity in children who have been burned. The goal of the study is to assess the risk factors for sepsis in pediatric burn patients in a referral hospital. Methods: This study was performed at the Pediatric Burn Unit of Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital during the period between January 2014 and June 2017. The patients were evaluated for age, sex, burn etiology, burned body surface area (BSA), the presence of inhalation injury, sepsis, positive cultures, the micro-organisms cultured samples, and septic focus. Results: A total of 181 patients were included in the study. The most common cause of burns was scalds in 120 patients (66.3%). Forty-one patients (22.7%) developed health-care-associated infection and sepsis. Gram-negative micro-organisms were isolated in 40 (97.6%) patients (Acinetobacter spp., Pseudomonas aeruginosa, Klebsiella pneumonia) with sepsis. Carbapenem resistance was detected in 31 (93.8%) of 40 patients. Mortality was observed in 11 patients (6.1%) in the group with sepsis. Burn surface area, burn depth, C-reactive protein (CRP) values, mortality, Garces index, and Baux index were higher in the group with sepsis (p < 0.05). Multiple regression analysis revealed that mechanism of injury (flame), burned BSA >= 25%, C-reactive protein >= 6 mg/dL (area under the curve [AUC]: 0.76 p < 0.001 and AUC: 0.90, p < 0.001, respectively) at admission were independent parameters for development of sepsis in pediatric burn patients. Conclusion: Multi-drug-resistant Pseudomonas aeruginosa and Acinetobacter baumannii were important agents of blood stream infection in burned children. Burned BSA >= 25% and CRP >= 6 mg/dL were risk factors for developing sepsis in pediatric burn patients.

Background Burkholderia cepacia complex is an aerobic, non-spore-forming, catalase-positive, nonfermentative, Gram-negative bacterium common in environment. It is a serious pathogen especially for patients with cystic fibrosis (CF). But pathogenicity of Burkholderia is not limited to patients with CF. Herein, we aimed to reveal clinical patterns and outcomes of Burkholderia infections in pediatric patients in our hospital and also antimicrobial susceptibility of the isolated strain. Methods This retrospective study was conducted in Ankara Hematology Oncology Children's Training and Research Hospital. Patients with isolates of Burkholderia spp. between January 6, 2013, and January 12, 2018, were included in the study. Results Burkholderia spp. was isolated from 55 patients. 94.6% of these patients had underlying diseases and had prior hospitalization within a year. Burkholderia gladioli grew in 15 patients' samples (27.3%); 38 patients grew B. cepacia (69.1%). None of the patients that B. gladioli was isolated was diagnosed as CF;. all had nosocomial infections. B. gladioli seemed to be more susceptible to aminoglycosides, piperacillin-tazobactam, carbapenems and ciprofloxacin than B. cepacia (P = 0.00), whereas B. cepacia seemed to be more susceptible to ceftazidime than B. gladioli (P = 0.032). In addition, B. cepacia was more susceptible to trimethoprim-sulfamethoxazole and levofloxacin than B. gladioli, but this difference was not statistically significant (P = 0.76). Conclusions The incidence of nosocomial infections caused by Burkholderia spp. is rare especially in pediatric literature. In our study, nosocomial Burkholderia infections occurred mostly in intensive care unit patients. The surveillance of Burkholderia infections is still very important, and the clinicians should be aware of changing epidemiology and increasing resistance of the microorganism. Besides, there are no internationally agreed minimal inhibitory concentration breakpoints and disk-diffusion test thresholds for susceptibility testing for Burkholderia. Thus, the methods which were used for antibiotic susceptibility testing in our center might cause uncertainty about the results and internationally agreed minimal inhibitory concentration breakpoints and disk-diffusion test thresholds for susceptibility testing for Burkholderia is still a gap to fill for the current literature.