THE ASSOCIATION BETWEEN AMINOGLYCOSIDE EXPOSURE AND OTOTOXICITY IN CHILDREN WITH CYSTIC FIBROSIS.

INTRODUCTION: Pulmonary exacerbations increase the requirement of aminoglycoside (AG) antibiotics in people with cystic fibrosis (pwCF). Several studies have shown that AGs have a cumulative effect on ototoxicity. We aimed to investigate the relationship between AG exposure and ototoxicity by using three different methods in patients with CF. MATERIALS-METHODS: The multicenter study included 121 pwCF aged between 5-18 years with a history of parenteral AG exposure. Standard pure tone audiometry (sPTA), extended high frequency audiometry (EHF-PTA), and distortion-product otoacoustic emissions (DPOAE) tests were performed. Mitochondrial mutation analysis for m1555G>A was performed in 61 patients. RESULTS: Median age was 12.85 years and 52.1% (n=63) were male. 18.2% (n=22) of the patients had received parenteral AGs more than 5 courses/lifetime. Ototoxicity was detected in at least one of the tests in 56.2% (n= 68) of the patients. Only 10.7 % (n=13) of the patients had reported a symptom indicating ototoxicity. 30.3% (n=30) of the patients had ototoxicity in the low-exposure group while it was 45.5% (n=10) in the high-exposure group according to EHF-PTA (p>0.05). Median number of parenteral amikacin courses was significantly higher in the ototoxic group [2(1.25-5.75) vs 2(1-3); p=0.045]. No m1555A>G mutation was detected in 61 patients who screened for mitochondrial mutation analysis. CONCLUSION: As AG ototoxicity occurs primarily at high frequencies, EHF-PTA is important in early detecting otoxicity. EHF-PTA and DPOAE detected ototoxicity in some patients with normal PTA results. All pwCF with a history of AG exposure should be evaluated for hearing loss since symptoms may only be noticed in the late period.

Diagnostic approach to the etiology of recurrent pneumonia in children.

BACKGROUND: Recurrent pneumonia (RP) accounts for 7.7%-9% of childhood pneumonia. Identifying the cause of RP is crucial for infection control and reducing mortality and morbidity. This study aimed to investigate the etiology, risk factors, and diagnostic procedures of RP based on the literature using a diagnostic algorithm. METHODS: Our study included RP patients aged 0-18 years who were followed up as outpatients or inpatients between 2018 and 2021. Patients were analyzed retrospectively using the national health database. Patients with RP were identified by ICD diagnosis codes. Etiology and risk factors were determined based on the occurrence of RP in the same or different areas. RESULTS: The rate of RP was found to be 14.4% among the cases of recurrent lower respiratory tract infection. Of these, 27.6% occurred in the same area and 72.4% in different areas. The underlying etiology was identified in 224 of 250 RP patients (89.6%). RP in different areas was mainly due to neuromuscular diseases, whereas asthma, right middle lobe syndrome, and congenital lung or airway structural disease were common causes of RP in the same area. Malnutrition, passive smoke exposure, and prematurity were common risk factors in both groups. CONCLUSIONS: Systematic etiological investigations should take into account the characteristics of the patient population and geographical region. The use of diagnostic algorithms based on recurrence in the same or different areas is particularly beneficial. Whether asthma is causally related to RP episodes or is a coincidental association due to inadequate differential diagnosis remains unclear.

Impact of Long COVID on Lung Function in Children.

Objective: While the coronavirus disease-2019 (COVID-19) pandemic has generally resulted in milder illness among children than adults, persistent respiratory symptoms have been increasingly reported in this population. Methods: We conducted a prospective, single-center cohort study focusing on children experiencing prolonged respiratory symptoms after contracting COVID-19. Spirometry, 6- minute walk tests (6MWTs), and tests of lung volume, the diffusing capacity of the lungs for carbon monoxide (DLCO), and fractional exhaled nitric oxide (FeNO) were performed on COVID-19 survivors at least 4 weeks after infection and a group of healthy control subjects. Results: Fifty-five children with long-term COVID and 55 healthy control subjects were recruited. The weight, height, and body mass index Z-scores were similar in the groups. Within a median duration of 85 days (minimummaximum: 35-194) following COVID-19 infection, a restrictive pattern was observed to be more common in the study group (p=0.021). In children with long COVID, 6MWT distances, DLCO Z-scores, and the predicted values of spirometry and lung volume tests were found to be significantly lower but in the normal range. The average predicted values for DLCO, FeNO, and 6MWT were similar in the two groups. Conclusions: Prolonged respiratory symptoms often persist long after COVID-19 infection, necessitating comprehensive evaluation of affected children. Close monitoring, including spirometry and lung volume assessments, is crucial for children with abnormalities in lung imaging. However, FeNO measurements were found to be ineffective in monitoring long COVID.

Implementation of a high fidelity simulation based training program for physicians of children requiring long term invasive home ventilation: a study by ISPAT team.

Introduction: The number of children requiring long-term invasive home ventilation (LTIHV) has increased worldwide in recent decades. The training of physicians caring for these children is crucial since they are at high risk for complications and adverse events. This study aimed to assess the efficacy of a comprehensive high-fidelity simulation-based training program for physicians caring for children on LTIHV. Methods: A multimodal training program for tracheostomy and ventilator management was prepared by ISPAT (IStanbul PAediatric Tracheostomy) team. Participants were subjected to theoretical and practical pre-tests which evaluated their knowledge levels and skills for care, follow-up, and treatment of children on LTIHV. Following the theoretical education and hands-on training session with a simulation model, theoretical and practical post-tests were performed. Results: Forty-three physicians from 7 tertiary pediatric clinics in Istanbul were enrolled in the training program. Seventy percent of them had never received standardized training programs about patients on home ventilation previously. The total number of correct answers from the participants significantly improved after the theoretical training (p < 0.001). The number of participants who performed the steps correctly also significantly increased following the hands-on training session (p < 0.001). All of the 43 participants who responded rated the course overall as good or excellent. Conclusion: The knowledge and skills of clinicians caring for children on LTIHV can be enhanced through a comprehensive training program consisting of theoretical training combined with hands-on training in a simulation laboratory.

Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war.

Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1-258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1-12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3-258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization.  Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: • Children who have chronic diseases are the group that is most affected by wars. • The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: • Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. • Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees.

Childhood interstitial lung disease in Turkey: first data from the national registry.

The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data-entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3-11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (- 2.0 [- 3.36 to - 0.81]) than in group 2 (- 0.80 [- 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040).   Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.

Long-term outcomes of standardized training for caregivers of children with tracheostomies: The IStanbul PAediatric Tracheostomy (ISPAT) project.

BACKGROUND AND OBJECTIVES: Children with tracheostomies are at increased risk of tracheostomy-related complications and require extra care. Standardized training programs for caregivers can improve tracheostomy care and reduce complications. In this study, we compared caregiver knowledge and skill scores after a standardized theoretical and practical training program on tracheostomy care (IStanbul PAediatric Tracheostomy (ISPAT) project) immediately and 1 year post-training and evaluated how this training affected the children's clinical outcomes. MATERIALS AND METHODS: We included 32 caregivers (31 children) who had received standardized training a year ago and administered the same theoretical and practical tests 1 year after training completion. We recorded tracheostomy-related complications and the number and reasons for admission to the healthcare centers. All data just before the training and 1 year after training completion were compared. RESULTS: After 1 year of training completion, the median number of correct answers on the theoretical test increased to 16.5 from 12 at pretest (p < 0.001). Compared with pretest, at 1-year post-training practical skills assessment scores, including cannula exchange and aspiration, were significantly higher (both p < 0.001) and mucus plug, bleeding, and stoma infection reduced significantly (p = 0.002, 0.022, and 0.004, respectively). Hands-on-training scores were better than pretest but declined slightly at 1 year compared to testing immediately after training. Emergency admission decreased from 64.5% to 32.3% (p = 0.013). Hospitalization decreased from 61.3% to 35.5% (p = 0.039). CONCLUSION: Our findings indicate that caregiver training can lead to a persistent increase in knowledge and skill for as long as 1 year, as well as improvements in several measurable outcomes, although a slight decrease in scores warrants annual repetitions of the training program.

Clinical and Radiological Evaluation and Follow-Up of Patients with Noncardiac Plastic Bronchitis.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease characterized by obstruction of the airway by fibrinous mucus plugs. The etiology can be idiopathic or secondary to systematic diseases such as congenital heart diseases. Definitive diagnosis is made by pathological examination of the sputum or bronchial sample taken by bronchoscopy. In this study, the clinical status and treatment status of patients with PB were evaluated. MATERIALS AND METHODS: Medical records of the patients diagnosed as PB were reviewed ret- rospectively. Age, gender, clinical symptoms, radiology, bronchoscopic findings, and pathology results were documented. RESULTS: Six patients with PB were included in this study (female:male, 2:4). The median age of the diagnosis was 45 months. The most common symptoms are persistent wet cough and short- ness of breath. The duration of symptoms ranged from 30 to 90 days. Atelectasis was the most common radiological finding. Diagnosis was made with pathological examination of the mucus in all patients. All of the patients were treated with bronchoscopic removal of the mucus, and 4 patients required oral prednisolone therapy. Symptoms and radiological findings resolved completely in all patients. CONCLUSION: Although PB is a rare disease, it should be kept in mind in relation to patients with persistent radiological and clinical respiratory symptoms.

Investigation of Childhood Pneumonia With Thoracic Ultrasound: A Comparison Between X-ray and Ultrasound.

ABSTRACT: Childhood pneumonia is a common entity, and chest x-rays are widely used as an initial diagnostic step. To avoid radiation exposure in the pediatric age group, we assessed whether the diagnostic accuracy of ultrasound (US) imaging is sufficient in the diagnosis. One hundred thirty-three children with pneumonia (72 girls/61 boys) were participated to study between 2019 and 2021. All participants had a chest x-ray. Radiologists who perform the US scans and interpret the x-rays were blinded to each other. A comparative analysis was also done to assess US findings on pneumonia for different age groups. We compare the diagnostic accuracy of US and x-rays by McNemar test and receiver operating characteristic curves. Intraclass correlation coefficient values were calculated for the assessment of interobserver agreement of x-ray evaluation. The participants' ages ranged from 1 month to 17 years and 8 months with a median age of 24 months (Q 1 : 8 and Q 3 : 66 months). Hospital stay lengths were longer, consolidation depths were greater, and presence of air bronchogram or pleural effusion was more frequent in school-age children. The proportion of consolidation seen on chest x-ray and transthoracic US scan was significantly different ( P < 0.001). The area under the curve was greater in the US than in the chest x-ray (area under the curve, 0.94 and 0.76 respectively). There was a good agreement between the 2 interpreters on chest x-ray assessment ( κ = 0.661). The thoracic US can be used as a safe and efficient imaging tool in the diagnosis of pediatric pneumonia.

The First Definition of Pulmonary Component of Hypereosinophilic Syndrome: Bronchial Casts.

Hypereosinophilic syndrome is a heterogeneous disease characterized by eosinophilic tissue inflammation and eosinophilia. Pulmonary involvement could be seen in up to 55% among children with hypereosinophilic syndrome. A 3-year-old boy with chronic hypereosinophilia and respiratory complaints was diagnosed with idiopathic hypereosinophilic syndrome. Atelectasis was detected in the radiological evaluation, and bronchial casts with eosinophilic structures were removed by bronchoscopy. Steroid, inhaled hypertonic saline, inhaled bronchodilator, inhaled corticosteroid, and leukotriene receptor antagonist were used for 1 year in the management of hypereosinophilic syndrome, and related eosinophilic casts and repetitive bronchoscopies were administered for removal of the casts. The patient was successfully managed with an inhaled N-acetyl cysteine treatment. In children, the long-term prognosis of hypereosinophilic syndrome is uncertain. Comprehensive diagnostic tests are required for the early diagnosis and management of pediatric hypereosinophilic syndrome. In the presented case, the rare occurrence of pulmonary involvement of hypereosinophilic syndrome in a 3 year-old-boy with recurrent hypereosinophilic casts and its management were discussed.

Did Hospital Admissions Caused by Respiratory Infections and Asthma Decrease During the COVID-19 Pandemic?

Objective: Despite maximum isolation measures taken during the coronavirus disease-2019 (COVID-19) pandemic, the workload of health institutions has increased. Consequently, changes in the number of admissions in clinics and emergency departments by disease groups have been observed. We aimed to quantitatively investigate the changes in the frequency of respiratory infections and asthma in the pediatric population during the COVID-19 pandemic. Methods: We analyzed electronic medical record data of patients who were admitted to the emergency department (ED), outpatient and inpatient clinics, and pediatric intensive care unit (PICU) because of influenza, upper and lower respiratory tract infections (URTI, LRTI), acute bronchiolitis, and asthma during the two-year period (one year pre-COVID period and first year of the pandemic). Results: There were 96,648 admissions in the pre-COVID period and 15,848 during the pandemic. We observed a decline in ED admissions (-73%), outpatient clinic visits (-70%), hospitalizations (-41.5%), and PICU admissions (-42%). While the admission rates of patients with influenza [from 4.26% to 0.37% (p=0.0001)] and URTI [from 81.54% to 75.62% (p=0.0001)] decreased, the ratio of the number of admissions to the total number of admissions due to LRTI [from 8.22% to 10.01% (p=0.0001)], acute bronchiolitis [from 2.76% to 3.07% (p=0.027)], and asthma [from 5.96% to 14% (p=0.0001)] increased. Conclusions: The decrease in viruses that cause acute bronchiolitis and asthma attacks lead to a reduction in admissions to ED of this patient group. The fact that parents preferred not to admit their child in ED during this period may have paved the way for the development of more severe LRTIs.

Efficacy of standardized tracheostomy training with a simulation model for healthcare providers: A study by ISPAT team.

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.

The ISPAT project: Implementation of a standardized training program for caregivers of children with tracheostomy.

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.

Effects of tracheostomy on mothers of children with cerebral palsy.

OBJECTIVE: We aimed to investigate psychosocial changes such as burnout, caregiver burden, depression, and coping in mothers of children with cerebral palsy (CP) after tracheostomy placement. DESIGN: A total of 48 children with CP and chronic respiratory insufficiency and their mothers participated in the study. The children with tracheostomy were classified as T (+) (n = 26) and those who did not have tracheostomy were classified as T (-) (n = 22). The patients with tracheostomy through invasive mechanical ventilation were classified as TIV (+). The data about the clinical conditions of the patients and the sociodemographic characteristics of the mothers were recorded. Zarit caregiver burden, Maslach burnout, Beck depression, and Coping Orientation to Problems Experienced (COPE) questionnaires were applied to all mothers. FINDINGS: Beck depression score was 14 ± 8.24 in T (+) group and 16.09 ± 9.65 in T (-) group (p = .576). There was no significant difference between the T (+) and T (-) groups in the Maslach burnout inventory, Zarit caregiver burden scale, and their subgroups. The first two methods from the COPE scales were identical. In the T (+) group, the third coping method was found as "planning" and in the T (-) group as "active coping". Maslach burnout inventory, Zarit 1, and Zarit 2 scores were higher in patients with only tracheostomy compared to those with TIV (p = .002, p = .018, p = .021). CONCLUSION: In the study presented, the placement of tracheostomy in children with CP does not increase mother's depression tendencies and mood changes.

Assessment of Nocturnal Hypoventilation by Different Methods and Definitions in Children with Neuromuscular Disease: Oxycapnography and Blood Gas Analysis.

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.

Geographical barriers to timely diagnosis of cystic fibrosis and anxiety level of parents during newborn screening in Turkey.

BACKGROUND: Despite the availability of cystic fibrosis (CF) screening countrywide, diagnostic delay is still a crucial issue. The objectives of this study were to explore the stages of the NBS process, determine the risk factors associated with diagnostic delay and evaluate parent anxiety and experience throughout the process. METHODS: This is a multicenter cross-sectional study. A questionnaire was completed by parents of newborns diagnosed with CF via NBS in 17 centers. Socio-demographic characteristics, parent knowledge and experiences related to NBS, sweat test availability in the region of residence, and time to the definitive CF diagnosis were assessed through this questionnaire. Parents' anxiety levels were evaluated through the State-Trait Anxiety Inventory scales 1 and 2. Delayed diagnosis (DD) was defined as a definite CF diagnosis beyond the 8th week of life. Predictors of delayed CF diagnosis were evaluated by univariate and multivariate analysis. RESULTS: A total of 220 CF patients diagnosed via NBS were enrolled; 82 (37.3%) babies had DD. Multivariable analysis indicated that residence in the Southeast Anatolia region of Turkey (OR = 10.79, 95% CI = 2.37-49.2) was associated with a higher incidence of DD compared with other regions in Turkey. Of the total, 216 (98.1%) of the caregivers regarded the NBS program as useful and 180 (82%) reported high anxiety levels. CONCLUSION: The organization of newborn screening should take into account regional and socio-cultural characteristics to improve the early diagnosis of CF and also reduce the anxiety level of parents.

Pediatric flexible bronchoscopy in the intensive care unit: A multicenter study.

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.

Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations.

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.

Screening for Latent Tuberculosis in Children With Immune-mediated Inflammatory Diseases Treated With Anti-tumor Necrosis Factor Therapy: Comparison of Tuberculin Skin and T-SPOT Tuberculosis Tests.

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.