BackgroundAllergic bronchopulmonary aspergillosis (ABPA), is a hypersensitivity reaction in the lung against the antigens of Aspergillus fumigatus. The aim of this study is to determine the incidence of ABPA, and to determine the risk factors leading to the development of ABPA.MethodsThe study was designed as a retrospective cohort study. Using the CF Registry System of Turkey data, the incidence of ABPA was determined and the clinical and demographic characteristics of 44 patients newly diagnosed with ABPA in 2022 and 132 patients with similar mutation weight and age range without ABPA diagnosis in that year were examined and compared. Also risk factors affecting the development of ABPA were determined.ResultsLow pulmonary function test values, having had at least one pulmonary exacerbation in that year, receiving intravenous antibiotic treatment, using inhaled antibiotics, having high number of pulmonary exacerbations, having Pseudomonas aeruginosa colonization, having low body mass index (BMI), having cystic fibrosis related diabetes mellitus were found to be among the risk factors for the development of ABPA.ConclusionsEarly recognition and treatment of ABPA is essential to prevent further damage to the lungs. Patients with frequent pulmonary exacerbations, low BMI and low pulmonary function test values, chronic colonization should definitely be considered for ABPA.

AbstractProtein aggregate myopathies can result from pathogenic variants in genes encoding protein chaperones. DNAJB4 is a cochaperone belonging to the heat shock protein-40 (HSP40) family and plays a vital role in cellular proteostasis. Recessive loss-of-function variants in DNAJB4 cause myopathy with early respiratory failure and spinal rigidity, presenting from infancy to adulthood. This study investigated the broader clinical and genetic spectrum of DNAJB4 myopathy. In this study, we performed whole-exome sequencing on seven patients with early respiratory failure of unknown genetic etiology. We identified five distinct pathogenic variants in DNAJB4 in five unrelated families of diverse ethnic backgrounds: three loss-of-function variants (c.547 C > T, p.R183*; c.775 C > T, p.R259*; an exon 2 deletion) and two missense variants (c.105G > C, p.K35N; c.181 A > G, p.R61G). All patients were homozygous. Most affected individuals exhibited early respiratory failure, and patients from three families had rigid spine syndrome with axial weakness in proportion to appendicular weakness. Additional symptoms included dysphagia, ankle contractures, scoliosis, neck stiffness, and cardiac dysfunction. Notably, J-domain missense variants were associated with a more severe phenotype, including an earlier age of onset and a higher mortality rate, suggesting a strong genotype‒phenotype correlation. Consistent with a loss of function, the nonsense variants presented decreased stability. In contrast, the missense variants exhibited normal or increased stability but behaved as loss-of-function variants in yeast complementation and TDP-43 disaggregation assays. Our findings suggest that DNAJB4 is an emerging cause of myopathy with rigid spine syndrome of variable age of onset and severity. This diagnosis should be considered in individuals presenting with suggestive symptoms, particularly if they exhibit neck stiffness during infancy or experience respiratory failure in adults without significant limb muscle weakness. Missense variants in the J domain may predict a more severe phenotype.

Ewing sarkomu, dünya çapında en sık görülen ikinci kemik sarkomudur. Tanı anında uzak metastazlara sık rastlanılır, en sık metastaz yeri akciğerlerdir ve en önemli prognostik faktör de tanı anındaki metastatik hastalığın varlığıdır. Ewing sarkomunun prognozu, sistemik kemoterapi ve multimodalite yaklaşımı ile son zamanlarda iyileşmiştir. Biz bu makalede sağ 12. kostadan köken almış ve plevra metastazı sonrası tanı almış olan, kemoterapi tedavisi sonrası yapılan görüntülemelerde kitle tamamen kaybolduğu için cerrahi rezeksiyona gerek duyulmayan bir Ewing Sarkomu olgusunu literatürde ender olarak görülmesi nedeniyle sunmak istedik.

Objective: Recent evidence suggests that alexithymic deficits in emotional processing may also affect physical health, and alexithymia may also be associated with organic disorders. The emotional well-being of patients with primary ciliary dyskinesia (PCD) is often negatively affected by uncertainty about the prognosis, lack of ongoing medical care, and lack of symptom control. This study aims to evaluate the frequency of alexithymia and its possible impact on the management of childhood PCD. Materials and Methods: Subjects were recruited from patients with PCD and healthy volunteers aged 8-18 years. The questionnaire included sociodemographic characteristics and self-report scales. Data were compared between patient and control groups. Results: In the >14 years of age group, the total Toronto Alexithymia Scale (TAS-20) score was significantly higher in the patients (56.60 +/- 13.01) compared to the control group (46.47 +/- 7.50) (p = .007). There were 6 (30) patients with a TAS-20 score >= 61. There was a significant correlation between TAS-20 and Pediatric Quality of Life (PedsQL) score (child), but no correlation between TAS-20 and Kovacs Children Depression Inventory (CDI) and PedsQL score (parents). The TAS-20 score was significantly higher in patients with bronchiectasis (p = .035), nasal polyps (p = .045), and siblings with PCD (p = .001). Furthermore, the TAS-20 score had a significant negative correlation with pulmonary function tests. Conclusion: Although this study is based on limited data from a single center and cannot be generalized to all PCD patient communities, our results show that PCD patients are more likely to have alexithymia compared to healthy controls and highlight the need to evaluate for alexithymia in patients with PCD. It is important for pediatric pulmonologists to have a thorough knowledge of the alexithymic features associated with PCD and to refer patients to pediatric psychiatry when necessary, especially in patients who are noncompliant with treatment protocols.

This study aims to investigate and analyze the clinical features of coexisting human bocavirus (HBoV) positivity and plastic bronchitis (PB). We present three cases with no known history of cardiac surgery who presented with cough, progressive dyspnea, and atelectasis. They tested positive for HBoV in a real-time polymerase chain reaction of both nasal lavage fluids. They were diagnosed with PB as a result of bronchoscopy and pathology. PB is a rare disease characterized by forming thick rigid casts in the tracheobronchial tree, which can progress to respiratory failure. While asthma or atopy-related type 1 PB was observed in one patient, two patients were diagnosed with PB, which was thought to have developed secondary to viral infections of unknown etiology. As far as we know, HBoV-associated PB cases are rare in the literature. Besides the commonly known infectious agents, we identified a relationship with HBoV in all the presented cases.

IntroductionBronchiolitis obliterans is characterized by partial or total occlusion of the bronchioles due to inflammation and fibrosis, and the most common form is postinfectious bronchiolitis obliterans (PIBO). This study aimed to retrospectively present our intravenous immunoglobulin (IVIG) treatment experience in PIBO patients with a clinically severe course despite receiving commonly used treatment protocols. Materials and MethodsThe study included patients aged 0-18 with subtle immunological abnormalities who were followed up in our center for PIBO between 2010 and 2021. Clinical evaluation, body mass index (BMI), computerized tomography (CT) image scoring, and immunological parameters were recorded before and after IVIG treatment. ResultsOf the 11 patients included in the study, 90% were male, the mean age at diagnosis was 27.1 months (range: 5-68 months) and the mean current age was 81.4 months (range: 15-188 months). The number of hospital visits due to infection and the frequency of hospitalizations decreased markedly in the patients who underwent IVIG therapy. Oxygen therapy was discontinued in all patients, and improvements in radiological severity scores were observed. BMI z-scores improved over the baseline values after IVIG therapy. ConclusionCorticosteroids are considered the best first-line treatment to control inflammation in PIBO. In our study group, PIBO patients showed favorable clinical and radiological responses to regular IVIG treatment, possibly due to minor immune deficiency secondary to steroids or as a result of undetected adaptive and innate immune defects involved in the etiology of severe PIBO.

This retrospective study aimed to describe the clinical and radiological features, diagnostic methods, laboratory findings, organ involvement, and treatment strategies of 22 patients who followed up with late‐onset childhood sarcoidosis and compare them with the literature.

Introduction: Alexithymia, which is defined as the difficulty in recognizing, recognizing, distinguishing and expressing emotions, is a term that has emerged in the clinical field. Although it is controversial whether alexithymia is a personality trait or a symptom associated with mental problems, it is known that alexithymia is associated with the symptoms of physical and mental health problems. In this study, we planned to measure the alexithymia level of cystic fibrosis patients followed in our clinic. Material-Method: Patients aged 5-18 years who were followed up with the diagnosis of Cystic Fibrosis (CF) in the Pediatric Chest Diseases Outpatient Clinic of the Meram Medical Faculty Hospital were included in the study. Patients younger than 5 years old and patients older than 18 years were excluded from the study. Patients filled out a questionnaire including sociodemographic characteristics and Toronto Alexithymia Scale. Results: This study included 22 girls (46.8%) and 25 (53.2%) boys. According to the Toronto Alexithymia scale, the total score of the children with CF was 57.45±9.8, the difficulty of recognizing emotion sub-score of the scale was 16.61±6.19, the difficulty of expressing emotions was 13.73±3.45, and the extroverted thinking sub-score was 27.12±3.24. The alexithymia total score of 12 children (25.5%) was above 60 points. A total of 23 children (48.9%) had a total scale score above 50. Discussion: It is known that children with chronic diseases may have difficulties in recognizing and expressing their emotions. In this study, it was observed that children with CF had difficulties in recognizing and expressing emotions, and their expressive thoughts increased.

OBJECTIVE:Only a few studies have investigated the frequency and severity of respiratory syncytial virus (RSV) infections after the end of the pandemic regulations. This study aims to investigate the frequency and severity of RSV infections before, during, and after the pandemic in Turkey. MATERIALS AND METHODS:Patients under 18 years of age and those who tested positive for RSV between April 2018 and March 2023 were retrospectively reviewed. All patients were divided into three groups (pre-COVID-19, COVID-19, and post-COVID-19) according to admission date. Among inpatients, data were compared between the three groups to determine the impact of the pandemic on RSV epidemiology and clinical outcomes. RESULTS:A total of 9567 patients were tested for RSV, of which 1073 (11.2%) were positive and included in the study. Hospitalization occurred in 447 (41.7%) patients. Inpatients were younger than outpatients (p < .000). Among the three inpatient pandemic groups, clinical outcomes were statistically significantly worse in the post-COVID-19 group than in the other two groups. SpO2 was lower (p < .000), inhaled salbutamol requirement was higher (p < .000), length of stay was longer (p = .031), and ICU admission was higher (p = .023). CONCLUSION:Although the RSV trend changed within 2 years after the COVID-19 outbreak, it returned to its usual seasonality last year. After the repeal of all COVID-19 measures and the normal life began, the number of RSV-positive patients and RSV-related hospitalizations increased, and the clinical outcomes of RSV worsened. This may be a result of decreased herd immunity due to a change in society's attitude toward epidemic diseases.

Cystic fibrosis (CF) is an autosomal recessive disease caused by cystic fibrosis transmembrane regulator protein (CFTR) gene mutations. The incidence of CF varies considerably among different ethnic groups worldwide, although it is most common in the Caucasian race. This study aims to examine the clinical course and treatment of CF among refugee patients in our country.

Chronic cough is one of the most common complaints of childhood to consult a doctor. There are many causes such as respiratory tract infections, gastroesophageal reflux, persistent bacterial bronchitis, asthma, cystic fibrosis, congenital malformations, and foreign body aspiration in children under the age of five. However, neither transient hypogammaglobulinemia of infancy (THI) among the causes of chronic cough, nor chronic cough among the application complaints of THI are not questioned. In this study, we aimed to draw attention to the role of THI in the etiology of chronic cough under the age of five. Our study included 55 pediatric patients under the age of five who applied to the pediatric pulmonary diseases outpatient clinic in between January 2015 and December 2020 with the complaint of chronic cough, who were excluded from other causes of chronic cough in etiology, and who met the criteria for THI according to the European Society for Immunodeficiencies (ESID). Demographic, clinical and laboratory characteristics and follow-ups of these patients were reviewed retrospectively. In our study, the mean age of 55 patients at admission was 21.73 ± 11.50 months (median age: 18 months), and the mean age of IgG recovery was 38.65 ± 16.81 months. The mean recovery time was 16.93 ± 12.85 months. Of the patients, 22.4% had a history of consanguinity, 23.4% had prematurity, and 18.2% had a frequent sickness in siblings. The most common complaint accompanying chronic cough in patients was frequent respiratory tract infection, 16.3%. Along with IgG, 26.4% of the patients had low IgA and 31.5% had low IgM in laboratory testing. In antibody responses, isohemagglutinin, anti-tetanus, anti-pneumococcal, anti-HBs vaccine responses were found to be positive in 90.6%, 63.9%, 66.7% and 97.7% of the cases, respectively. 72.7% of the patients received inhaler treatment, 45.5% received antibiotic prophylaxis, and 2.2% received intravenous immunoglobulin (IVIg) treatment. After the IgG value of the patients returned to normal, it was observed that 86.3% of the patients’ cough complaint have disappeared. Transient hypogammaglobulinemia of infancy mostly presents with recurrent lower and upper respiratory tract infections. The most common complaint is cough. It is not questioned whether the cough is chronic or not. In this study, we aimed to investigate the follow-up and prognosis of patients under the age of five who had a chronic cough complaint, when other causes of cough were ruled out and THI was detected. In the study, when the IgG levels of the patients return to normal, the cough complaints disappear to a large extent, showing that THI may also be among the causes of chronic cough.

BackgroundCystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. MethodsThis retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. ResultsIn 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. ConclusionsPatients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

Objectives-aim: Pulmonary exacerbations (PE) results in a progressive decline in pulmonary function in patients with cystic fibrosis (CF). We aimed to evaluate the effects of acute PE attacks in infancy on pulmonary functions at 6 years in CF patients. Methods: This is a retrospective cohort study. Eligible patients were included in theTurkish Cystic Fibrosis Registry of Turkey (CFRT)-2019 data. Patients who had 2 or less PE in the first 2 years of age were defined as Group1, and those more than 2 were defined as Group 2. Results: 110 patients were included in the study, 57 female, 22 patients' PE attack information is unknown, 59 of the patients were in Group 1. The mean number of PE in the first 2 years of age was 2.2 (±2.38). There were no significant differences between the groups according to IV antibiotic needs, positive bacterial culture results, and CFTR mutations' distribution during the first PE. The mean of percent predictive FEV1 and FVC were significantly lower in Group2 than in Group1 (p:0.029; 0.032 res.). There were no significant differences in BMI between the groups. There were no correlations between the first PE's age and spirometry indices and the number of hospitalizations. The current BMI of the patients who have chronic colonization is significantly lower than their first PE's BMI value (p: 0.028). Conclusion: Patients with more than 2 PE had worse pulmonary function than 2 or less. Frequent PE at an early age and bacterial colonization should be prevented to preserve the lung functions of CF patients.

Objective Mycobacterium tuberculosis is an immobile aerobic bacillus that causes tuberculosis (TB) disease. We aimed to evaluate the association between coronavirus disease 2019 (COVID-19), COVID-19-related drugs, TB reactivation, and TB incidence during the pandemic.Methods Eight patients who were diagnosed as having TB in Meram Medical Faculty, Necmettin Erbakan University between March 1, 2020, and December 31, 2021, at the beginning of the pandemic, were enrolled in this study. The presence of COVID-19 infection was confirmed using COVID-19 antibody tests and the patients' COVID-19 history. We evaluated the demographic data, laboratory findings, imaging tests, and pathology results of all patients. Results We checked all our patients with TB using COVID-19 antibodies (immunoglobulin [Ig]G thorn IgM) or polymerase chain reaction. Seven of the eight patients were female (87.5%). The median age was 16 years. Family screening of all patients was negative, and they had bacillus Calmette-Guerin vaccine scars. Two patients had chronic diseases. One was diagnosed as having primary ciliary dyskinesia in our department (patient no. 8) and the second was under follow-up by the rheumatology department with a diagnosis of juvenile idiopathic rheumatoid arthritis.Conclusion There has been an increase in the incidence of TB in children, especially in adolescents, during the pandemic period. This may be due to the pathogenic structure of the COVID-19 virus with an unknown mechanism. In addition, lifestyle changes and changes in health care policies during the pandemic may have caused this. Further research should be performed on this topic.

ÖZET Trakeal darlık çocuklarda nadir görülen, asemptomatik olabileceği gibi hayatı tehtid eden ağır solunum yetmezliği tablolarıyla da karşımıza çıkabilen bir hastalıktır. Etyolojide edinsel ve konjenital nedenler yer alır. Tanıda iyi bir öykü almak, fizik muayene yapmak ve fleksible bronkoskopi önemlidir. Özellikle hışıltılı solunum ile başvuran çocuklarda trakeal stenoz ayırıcı tanıda yer almalıdır. Bu yazıda hışıltılı solunum şikayeti ile başvuran trakeal stenoz vakası sunulmuştur. Anahtar Kelimeler: Trakeal stenoz, hışıltı, çocuk, solunum yetmezliği

OBJECTIVE:The clinical course of new COVID-19 variants in adolescents is still unknown. The aim of this study is to evaluate the clinical characteristics of COVID-19 in adolescents and compare the differences between the original version and the delta variant. MATERIALS AND METHODS:The medical records of patients aged 10-18 years treated for COVID-19 between April 1, 2020 and March 31, 2022 were retrospectively reviewed. Patients were divided into four groups (asymptomatic, mild, moderate, and severe) for COVID-19 severity and into two groups according to the diagnosis date (first-second year). The primary endpoint of the study was hospital admission. RESULTS:The mean age of patients was 171.81 ± 29.5 months, and most of them were males (n: 435, 53.3%). While the patient number was 296 (43.52%) in the first year of pandemic, it raised to 520 (54.11%) in the second year (p < 0.01). The severity of COVID-19 was mild in 667 (81.7%) patients. In the comparison of patients according to the diagnosis date (first-second years); the parameters of anosmia, ageusia, weakness, muscle pain, vomiting, hospital admission, and length of stay in hospital were statistically different (p < 0.05). In the comparison of hospitalized patients between years, the necessity of oxygen support (p < 0.001), endotracheal intubation rates (p < 0.05), length of stay in the hospital (p < 0.001), and the severity of COVID-19 (p < 0.05) was significantly higher in the second year. CONCLUSION:The clinical course for adolescents diagnosed with COVID-19 has linearly changed with the delta variant. Our results confirmed that the delta variant is more transmissible, requires more oxygen support, increases endotracheal intubation, and prolongs the length of stay in the hospital.

Introduction: Pediatric poisoning is a common cause of emergency department admissions. The epidemiology of pediatric poisonings can vary in different countries, even different regions of the same country. It is important to determine the regional factors to decrease morbidity and mortality. This study was aimed to evaluate the epidemiological features, clinical signs, and risk factors of pediatric poisonings. Methods: This retrospective descriptive study was carried out in the pediatric emergency department of a tertiary hospital and medical records between January 2016 and December 2020 were retrospectively reviewed. Demographic characteristics, laboratory results and treatments were recorded. Results: 835 patients aged 4 months-18 years including 450 females (53.9%) were included in the study. Poisonings were most common in summer (n = 280, 33.5%) and in the daytime (n = 490, 58.7%). The mean age of suicidal poisoning was significantly higher then accidental poisonings (p < 0.001). Pharmaceutical agents were statistically significantly higher in patients poisoned due to suicidal attempts (p < 0.001). On the other hand, there was not a statistically significant difference between accidental and suicidal poisoning in terms of clinical presentation, length of stay in hospital, and, ICU requirement (p > 0.05). Conclusions: Childhood poisoning is one of the important emergencies that need attention. Pediatric emergency physicians should always keep in mind the possibility of poisoning, even with the lack of medical history. It could be estimated the possible ingested agents according to the age group, and this allows physicians to avoid delay in treatment of these patients.

Introduction: Pediatric poisoning is a common cause of emergency department admissions. The epidemiology of pediatric poisonings can vary in different countries, even different regions of the same country. It is important to determine the regional factors to decrease morbidity and mortality. This study was aimed to evaluate the epidemiological features, clinical signs, and risk factors of pediatric poisonings. Methods: This retrospective descriptive study was carried out in the pediatric emergency department of a tertiary hospital and medical records between January 2016 and December 2020 were retrospectively reviewed. Demographic characteristics, laboratory results and treatments were recorded. Results: 835 patients aged 4 months-18 years including 450 females (53.9%) were included in the study. Poisonings were most common in summer (n = 280, 33.5%) and in the daytime (n = 490, 58.7%). The mean age of suicidal poisoning was significantly higher then accidental poisonings (p < 0.001). Pharmaceutical agents were statistically significantly higher in patients poisoned due to suicidal attempts (p < 0.001). On the other hand, there was not a statistically significant difference between accidental and suicidal poisoning in terms of clinical presentation, length of stay in hospital, and, ICU requirement (p > 0.05). Conclusions: Childhood poisoning is one of the important emergencies that need attention. Pediatric emergency physicians should always keep in mind the possibility of poisoning, even with the lack of medical history. It could be estimated the possible ingested agents according to the age group, and this allows physicians to avoid delay in treatment of these patients.

Introduction: Vitamin D intoxication (VDI) is a well-known cause of hypercalcemia in children and leads to serious kidney, heart, and neurological problems. In the treatment of VDI, the goal is to correct hypercalcemia. Our aim was to evaluate the clinical features of patients with VDI, identify the causes of VDI in our region, and help guide precautions and treatment of VDI. Materials and methods: The medical records of patients with VDI presenting between January 2015 and December 2019 were retrospectively analyzed. Results: In total, 38 patients aged 0.3-4 years including 20 males (52.6%) were included in the study. Vomiting (65.8%), loss of appetite (47.4%), and constipation (31.6%) were the most common symptoms. The cause of intoxication was prescribed D3 vials in 23 patients, non-prescribed D3 vials in nine patients, and incorrectly produced fish oil supplement in six patients. Admission serum calcium and 25 (OH) D levels were 3.75 +/- 0.5mmol/L and 396 +/- 110ng/mL, respectively. A statistically significant correlation was found between the serum calcium levels at the time of diagnosis and the dose of vitamin D received, serum 25 (OH) D, phosphorus, and parathyroid (PTH) levels. Nephrocalcinosis was present in 15 (39.5%) patients. The mean time to achieve normocalcemia was 6.18 +/- 2 days. The mean time to achieve normocalcemia in patients treated with pamidronate was 5.94 +/- 0.7 days. Conclusion: Stoss therapy should not be administered for children of families with problems of adherence to treatment. It should be noted that VDI may develop as a result of improperly produced nutritional supplements. General practitioners and pediatricians must be aware of VDI risks and explain them to parents. Pamidronate is effective for treating VDI in children. (C) 2021 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.