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Introduction: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs., Methods: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators., Results: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1., Conclusion: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes., (© 2024 Wiley Periodicals LLC.)

Background: Antibiotic-associated diarrhea is one of the most frequent side effects of antimicrobial therapy. We assessed the epidemiological data of antibiotic-associated diarrhea in pediatric patients in our region., Methods: The prospective multi-center study included pediatric patients who were initiated an oral antibiotic course in outpatient clinics and followed in a well-established surveillance system. This follow-up system constituded inclusion of patient by the primary physician, supply of family follow-up charts to the family, passing the demographics and clinical information of patient to the Primary Investigator Centre, and a close telephone follow-up of patients for a period of eight weeks by the Primary Investigator Centre., Results: A result of 758 cases were recruited in the analysis which had a frequency of 10.4% antibiotic-associated diarrhea. Among the cases treated with amoxicillin-clavulanate 10.4%, and cephalosporins 14.4% presented with antibiotic-associated diarrhea. In the analysis of antibiotic-associated diarrhea occurrence according to different geographical regions of Turkey, antibiotic-associated diarrhea episodes differed significantly (p = 0.014), particularly higher in The Eastern Anatolia and Southeastern Anatolia. Though most commonly encountered with cephalosporin use, antibiotic-associated diarrhea is not a frequent side effect., Conclusion: This study on pediatric antibiotic-associated diarrhea displayed epidemiological data and the differences geographically in our region., (© 2023. The Author(s).)

Objectives: multi-system ınflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease., Methods: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C., Results: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5‒15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity., Study Limitations: The number of patients in the this study was small., Conclusions: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C., (Copyright © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)

Background: The prevalence of community-acquired methicillin-resistant S. aureus (CA-MRSA) has been increasing worldwide. We aimed to investigate the prevalence of MRSA in community-acquired S. aureus infections, the risk factors for CA-MRSA infection and the clinical features of CA-MRSA., Methods: A multi-center study with prospective and retrospective sections was conducted. Patients ≥ 3 months old and ≤18 years of age who were diagnosed with community-acquired S. aureus infections were included in this study and the patients` information were reviewed from the medical and microbiological database of the hospital. A standard question form about living conditions and exposure risk factors was administered to the parents of patients. The CA-MRSA infections were compared with the methicillin-susceptible S. aureus (CAMSSA) infections in terms of the queried risk factors and clinical variables., Results: We identified 334 pediatric patients with S. aureus infection, 58 (17.4%) had an infection with CAMRSA. The refugee rate was higher in the CA-MRSA group. There was no significant difference regarding the exposure risk. The treatment modalities and outcomes were similar., Conclusions: The study was not able to show reliable clinical variables or epidemiological risk factors except for being a refugee for CA-MRSA infections. Empirical antibiotic treatment should therefore be determined according to the local CA-MRSA prevalence in patients presenting with a possible staphylococcus infection.

Introduction: Health care workers (HCWs) are disproportionately exposed to infectious diseases and play a role in nosocomial transmission, making them a key demographic for vaccination. HCW vaccination rates are not optimal in many countries; hence, compulsory vaccination policies have been implemented in some countries. Although these policies are effective and necessary under certain conditions, resolving HCWs' hesitancies and misconceptions about vaccines is crucial. HCWs have the advantage of direct contact with patients; hence, they can respond to safety concerns, explain the benefits of vaccination, and counter antivaccine campaigns that escalate during pandemics, as has been observed with COVID-19., Method: A short survey was carried out in May-June 2020 on the vaccination status of HCWs working with pediatric patients with COVID-19. The survey inquired about their vaccination status (mumps/measles/rubella [MMR], varicella, influenza, and diphtheria/tetanus [dT]) and willingness to receive hypothetical future COVID-19 vaccines. The respondents were grouped according to gender, age, occupation, and region., Results: In total, 4927 HCWs responded to the survey. Most were young, healthy adults. The overall vaccination rates were 57.8% for dT in the past 10 years, 44.5% for MMR, 33.2% for varicella, and 13.5% for influenza. Vaccination rates were the highest among physicians. The majority of HCWs (81%) stated that they would be willing to receive COVID-19 vaccines., Conclusion: Although vaccination rates for well-established vaccines were low, a majority of HCWs were willing to receive COVID-19 vaccines when available. Education and administrative trust should be enhanced to increase vaccination rates among HCWs.

Background: Invasive Trichosporon infections are rarely seen opportunistic fungal infections in children and mainly affect immunocompromised patients. This multicenter retrospective study has rewieved the characteristics, risk factors, treatment modalities and outcomes of bloodstream infections caused by Trichosporon species in children diagnosed over the past ten years in Turkey., Methods: The study was performed with the participation of 12 of 55 hospitals invited from Turkey. In each center, the patients with bloodstream infections caused by Trichosporon spp. between January 2010 and December 2020 were retrospectively ascertained and the results were reported to the study coordinator by means of a simple case report. Data were collected on patient demographics, underlying condition(s), treatment of.infections caused by Trichosporon spp, and 7 and 30- day mortality rates., Results: A total of 28 cases with fungemia caused by Trichosporon spp. were included in the study. The most common underlying disease was paediatric cancers (39.3%). T. asahii infections were detected in 78.5 % (n=22) of patients. A various spectrum of antifungal treatment regimens were used including intravenous amphotericin B monotherapy in 35.7%, intravenous amphotericin B and voriconazole combination in 32.1% and intravenous voriconazole monotherapy in 28.6% of the patients. The overall mortality rate was 28.5 %. The mortality rates were 12.5% in the voricanozole, 30% in the amphotericin B and 33.3% in combined voriconazole -amphotericin B arms CONCLUSIONS: Invasive Trichosporon infections with an important impact of patients quality of life are almost related to underlying diseases with an overall mortality rate of 28.5%. Voriconazole was found to be associated with lower mortality rates when compared with other treatment regimens., Competing Interests: Declaration of Conflict of Interest No potential conflict of interest has been declared by the author(s). All authors have reviewed and approved the finalized version of the manuscript, and contributed significantly to the survey. No financial support was provided relevant to this article. The manuscript has not been previously published nor is it being considered for publication elsewhere, (Copyright © 2021 SFMM. Published by Elsevier Masson SAS. All rights reserved.)

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 1 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., (© 2020 Wiley Periodicals, Inc.)

Background: Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017., Methods: The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively., Results: There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients., Conclusions: Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future., (© 2019 Wiley Periodicals, Inc.)

Background: Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis of unknown etiology. In the present study, we investigated whether there is a relationship between KD and dynamic thiol/disulphide homeostasis., Methods: This case-control study involved KD patients and healthy controls. Plasma total, native and disulphide thiol and the disulphide/native, disulphide/total and native thiol/total thiol ratios of all patients and the control group were analyzed simultaneously., Results: A total of 20 patients with KD (male/female, 12/8) and 25 age- and gender-matched healthy controls (male/female, 12/13) were evaluated. Native, total thiol and native thiol/total thiol ratio were significantly lower in KD patients than in the control group (P < 0.001). In contrast, disulphide thiol, disulphide/native thiol and disulphide/total thiol ratios were significantly higher in KD patients than control subjects (P < 0.001). In KD patients with coronary artery lesion (CAL), the native thiol and total thiol were significantly lower than in KD patients without CAL. In KD patients with CAL, the ratios of disulphide/total thiol and disulphide/native thiol were significantly higher than in those without CAL (P = 0.02 and P = 0.02, respectively), whereas the ratio of native/total thiol was significantly lower (P = 0.02)., Conclusion: The KD patients had lower plasma thiol (native and total) and higher disulphide thiol than controls, indicating that dynamic thiol/disulphide homeostasis might be an important indicator of inflammation in KD. Alteration and shifting of thiol/disulphide homeostasis to the oxidized side are correlated with the pathogenesis of KD and CAL., (© 2019 Japan Pediatric Society.)

Kaman A, Tanır G, Çakmakçı E, Demir P, Öz FN, Aydın Teke T, Metin Ö, Gayretli Aydın ZG, Karaman A. Characteristics, diagnosis, and treatment modality of pediatric patients with cystic echinococcosis: a single centre experience. Turk J Pediatr 2019; 61: 704-713. Cystic echinococcosis (CE) is among the most common zoonotic infections worldwide. Studies about CE are limited in childhood. The aim of this study was to evaluate clinical, radiological and laboratory characteristics of childhood CE at a tertiary care pediatric hospital. Medical records of children with CE were analyzed between January 2005 and January 2015. A total of 130 patients with a median age of 10.4 years (IQR= 7.2-years-13.2 years) were evaluated. The anatomic locations of cysts were as follows; liver (76.9%), lung (36.9%), spleen (6.2%), pelvic region (3.8%) and kidney (2.3%). The most common symptoms were abdominal pain and cough in the patients with liver cysts and lung cysts, respectively. The indirect hemagglutination (IHA) test positivity was 58%. Elevated serum total immunoglobulin E levels were detected in 59% of the patients. Fourty-four patients with liver CE, 33 patients with lung CE were treated surgically and 23 patients with liver CE were treated with percutaneous aspiration, injection and re-aspiration (PAIR) along with medical treatment. The recurrence was observed in five patients with liver CE. It was demonstrated that CE mainly involves liver but lung cysts are more frequently symptomatic and prone to be complicated than liver cysts in children. IHA test positivity together with abdominal ultrasonography are useful to diagnose liver CE but thorax CT is usually needed to diagnose lung CE. Liver cysts that are sized greater than 5 cm are more frequently treated with PAIR or surgery but smaller liver cysts can be treated medically.

Malaria is a potentially life-threatening disease caused by infection with the Plasmodium protozoa transmitted by an infective female Anopheles mosquito. Despite successful control programs in many countries, malaria remains to be a major disease burden worldwide, with approximately 584,000 deaths annually. The incidence of the disease and responsible species may differ due to increased human movements between countries. Plasmodium falciparum infection carries a poor prognosis with a high mortality if untreated, but it has an excellent prognosis if diagnosed early and treated appropriately. In the present study, we described a patient diagnosed with falciparum malaria and treated with atovaquone-proguanil who had a history of traveling to Uganda.

Neurocysticercosis is a parasitic infection of the central nervous system caused by the larval stage of Taenia solium. Although this zoonotic infection is one of the major public health problems in some developing countries, it is extremely rare in Turkey. In this article, we present the case of a pediatric patient with neurocysticercosis who was misdiagnosed with brain abscess because of focal seizures in another hospital.

Objective: Leishmaniasis is an infectious disease that is caused by a protozoan parasite of the Leishmania genus and that occurs worldwide. Leishmaniasis is endemic in southeastern Turkey and the neighboring Middle Eastern countries. The purpose of this study was to describe the clinical characteristics of patients admitted to our hospital with a diagnosis of cutaneous leishmaniasis (CL)., Methods: A total of 16 CL patients [11 (69%) boys and five (31%) girls] were admitted between January 2014 and December 2015. The data of the patients were retrospectively recorded from their medical records., Results: Their mean age was 74.3±32.3 months (range: 1-10.5 years). Double lesions were most commonly seen in eight (50%) patients. The face and neck was the most commonly involved site (87.5% of the patients). Skin smears for a parasitological examination were positive in nine (56%) patients. Two patients (12.5%) with limb lesions were treated with intralesional meglumine antimoniate. Fourteen patients were treated with systemic agents., Conclusion: We felt that the increase in human movement that include travels and forced migration due to the war might make it possible for CL to appear in non-endemic provinces such as Ankara. In particular, in patients with painless cutaneous lesion(s) who came from endemic areas such as Syria, CL should be kept in mind by the clinicians that residing in even non-endemic areas .

Kawasaki disease is an acute, systemic vasculitis of unknown etiology. Although gastrointestinal involvement does not belong to the classic diagnostic criteria; diarrhea, abdominal pain, hepatic dysfunction, hydrops of gallbladder, and acute febrile cholestatic jaundice are reported in patients with Kawasaki disease. We describe here two cases presented with fever, and acute jaundice as initial features of Kawasaki disease.

Parapneumonic effusion (PPE) and empyema are most often seen as a complication of bacterial pneumonia and occasionally associated with atypical bacteria or viruses. The aims of this study were to describe and compare demographic characteristics, clinical, laboratory, microbiological findings and treatment modalities of patients with PPE and empyema. We retrospectively reviewed 116 pediatric patients with PPE and empyema. Seventy (60.3%) had pleural empyema and 46 patients (39.6%) had PPE. The median age of patients with empyema [72.0 months (IQR 68.0 months)] was lower than the patients with PPE [92.5 (IQR 80.0 months)] (p=0.003). Children in the empyema group had significantly more dyspnea symptoms than the children with PPE (p=0.022). Mean fever duration before hospitalization was similar in both groups. Streptococcus pneumoniae and group A streptococcus were the most common causes of empyema. All of the patients were treated with intravenous antibiotics. In addition to medical treatment, tube thoracostomy was performed in 59 of 70 (84.3%) patients in empyema group; 27 (45.8%) of them required intrapleural fibrinolysis also. In the presence of antibiotic treatment failure or in cases with moderate or large pleural effusion with loculations and clinical deterioration; it is necessary to perform drainage of the purulent fluid by tube thoracostomy, to add intrapleural fibrinolytics or to perform video-assisted thoracoscopic surgery (VATS), in order to enhance prompt recovery.

Langerhans cell histiocytosis is a rare non-malignant disease with clinical heterogeneity. The disease may present with various clinical findings and may imitate many other conditions. In this report we describe a 34-month-old girl who presented with chronic otitis and otorrhea, skull fracture, rash, vulvar edema, erythema and erosion in labia majors which initially suggested child abuse but the patient was diagnosed with Langerhans cell histiocytosis.

The purpose of this study was to evaluate the clinical, radiological, microbiological characteristics, side effects of anti-TB drugs and treatment outcome of childhood definite or probable TB. The medical records of all childhood TB patients were investigated in the department of pediatric infectious disease of a tertiary care hospital between January 2005 and December 2012. Patients who followed-up until the anti-TB treatment was completed were included in the study. One hundred forty four pediatric cases with active TB were included in the study (27 definite, 117 probable). Twenty-four (16.7 %) patients were asymptomatic at admission. Pulmonary TB was the most common type. The most common localizations for extrapulmonary TB were superficial lymphadenitis. An index case was detected in 39.6% of the patients. In conclusion, the absence of constitutional symptoms does not exclude TB. Household contact screening plays an important role in the diagnosis of childhood TB especially in asymptomatic patients.

A 20-month-old boy presented with 1-year history of persistent fever, cough, and progressive abdominal distention. Abdominal ultrasonography showed hepatomegaly and multiple calcifications in the liver and spleen. Thoracic computed tomography showed multiple mediastinal lymph nodes and consolidation in both lungs. Additionally, there was a 2-cm thick retroperitoneal soft tissue mass destroying the T7-8 and L1-L2 vertebral bodies. The patient was preliminarily diagnosed with miliary tuberculosis (TB) and Pott's disease, and began administering anti-TB treatment consisting of isoniazid, rifampin, ethambutol, and pyrazinamide. Acid-resistant bacilli analysis and mycobacterial culture of the biopsy specimen of Pott's abscess were positive. Mycobacterial culture and PCR of gastric aspirate were also positive. The patient's condition progressively improved with anti-TB treatment and he received 12 months of antiTB therapy. At the end of the treatment all of the patient's symptoms were relieved and he was well except for kyphosis. Miliary TB complicated by Pott's abscess is a very rare presentation of childhood TB. The presented case shows that when Pott's abscess is diagnosed and surgically corrected without delay, patients can recover without squeal.

Streptococcus pneumoniae is a leading cause of bacterial meningitis in children. It is also responsible for bacteremia, sepsis, pneumonia, sinusitis and acute otitis media in young children worldwide. The serotypes included in the 7-valent conjugated pneumococcal vaccine (PCV7)-1, 5, 6A, 6B, 14, 19F, 23F-are those most commonly responsible for invasive pneumococcal disease (IPD) globally. Unvaccinated children are at greater risk for meningitis. The rate of non-vaccine serotypes as causes of invasive disease has increased. Although the incidence rate of IPD is highest in children aged <2 years, the rare, non-vaccine serotypes of S. pneumoniae may be responsible for acute meningitis in older, unvaccinated children. In this report, we present a pediatric case of meningitis due to S. pneumoniae serotype 33D, which has not been previously identified as a cause of IPD in those countries where PCV7 is routinely administered, including Turkey.

The aim of this study was to elucidate the clinical presentations, diagnostic clues and management of deep neck infections (DNI) in pediatric patients. Demographic characteristics, clinical manifestations, predisposing factors, duration of symptoms before presentation, history of previous antibiotic use, duration of hospitalization, laboratory and radiographic evaluations, management, complications, and outcomes of 25 patients (19 male/6 female; mean age: 47.9±39.0 months) diagnosed with DNI were analyzed retrospectively. Retropharyngeal abscesses occurred especially in preschool children, whereas peritonsillar abscesses occurred especially in school children. Nine of 25 (36%) patients underwent surgical intervention in addition to medical therapy. Contrast-enhanced computed tomography provided additional information in half of the patients. DNIs should be considered in the differential diagnosis of children who present with fever and neck mass even in the absence of more specific findings. Medical treatment can be considered an option to surgical treatment by utilizing the advantages of imaging techniques and empirical antibiotics.