Pulmonology Centers Research Articles

Newborn Screening Program for Cystic Fibrosis in Türkiye: Experiences from False-Negative Tests and Requirement for Optimization.

Since January 2015, the Cystic Fibrosis National Newborn Bloodspot Screening (CF-NBS) program has been implemented in Turkey with two samples of immune reactive trypsinogen (IRT-1/IRT-2) testing. This study aimed to evaluate the Turkish national CF screening program, which included patients referred to a tertiary pediatric pulmonology center, to ascertain the optimal cut-off values for IRT-1/IRT-2 and to identify alternative strategies for mitigating the number of late-diagnosed false-negative patients (FNPs) who initially exhibited screen negative results but were diagnosed subsequently based on clinical suspicion. The study also compared NBS-positive patients to FNPs to determine the influence of delayed diagnosis. A retrospective cohort study. Screening for CF was conducted in accordance with the national CF-NBS program within 48-72 hours of birth by collecting a few drops of heel blood on Guthrie paper. A cut-off value of 90 μg/l was accepted for the first IRT, while 70 μg/l was accepted for the second sample. Infants with elevated IRT values in both samples were referred to the CF centers for a sweat test (ST). Based on the diagnosis, the NBS-positive infants referred to our CF center for ST analysis were divided into three groups: CF; cystic fibrosis-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID); and false-positive NBS. In addition, the study included NBS-negative patients who initially received negative screen results but were subsequently diagnosed with CF based on clinical suspicion. Of the 227 NBS-positive infants referred within the study period, 53 (23.34%) were diagnosed with CF (true-positive NBS), 11 were classified as CRMS/CFSPID (4.84%), and 163 were classified as falsepositive NBS (71.8%). CF was diagnosed in 66 infants, 53 (80.3%) of whom were confirmed using the NBS test, while the 13 (19.7%) patients who were missed on the NBS test were diagnosed based on clinical suspicion (FNP). The study findings indicate that the IRT/IRT approach exhibited a sensitivity of 80.3% and a positive predictive value (PPV) of 23.3%. The current study is the first to analyze the NBS program for CF using data from the Western Anatolian Region of Türkiye. Due to the low sensitivity and PPV of the IRT/IRT protocol and the high proportion of false-positive infants and FNPs, the current national program is not practicable for Türkiye. False-negative results significantly delay the diagnosis and invalidate the screening objectives. It is essential to establish optimal cut-off values for IRT-1/IRT-2 or revise existing strategies to reduce the number of FNPs missed by the screening program.

Assessing the Psychological Well-being of Healthcare Workers During the COVID-19 Pandemic

During the COVID-19 pandemic, working in any medical organization is associated with the risk of coronavirus infection. This applies first and foremost to specialized medical organizations dealing with the treatment of patients infected with coronavirus. Medical workers experiencing increased psychological stress are at an increased risk of infection with the SARS-CoV-2 virus while providing medical care. Therefore, studying the psychological states of medical workers – doctors, nurses, orderlies - is of particular importance and relevance. Our research goal is to investigate the psychological responses of medical professionals amid the COVID-19 pandemic. The research focused on the Aktobe Medical Center (AMC), which hosts the regional pulmonology center for COVID-19 patients and the Regional Clinical Infectious Diseases Hospital (RCIDH). The study is a selective, single-stage cross-sectional analysis. To assess the psychological reactions of medical workers, standardized questionnaires are used, including the PSM-25, GAD-7, and MFI-20. Statistical analysis of the data is conducted using the STATISTICA 10.0 software package from StatSoft, Inc., USA. An analysis of the research results indicates that medical personnel, nurses, and administrative staff in medical organizations associated with the risk of coronavirus infection are susceptible to adverse psychophysiological disorders. The study revealed that stress levels, anxiety, and asthenia in doctors and nursing staff are significantly higher compared to those in nurses and administrative personnel. Medical workers at risk of COVID- 19 clearly require support measures, as the potential for occupational infection has been definitively established. Additionally, the psychological risk is largely influenced by the nature of their work and their direct contact with the population and patients infected with the coronavirus. Senior and mid-level medical workers at the forefront of combating coronavirus infection is at a high risk of experiencing psycho-emotional and psychophysiological health issues. Doctors and nurses have been found to exhibit high levels of physical and mental asthenia, anxiety, and moderate-to-high levels of stress. Evaluating potential risk factors for psychophysiological disorders among healthcare workers not only provides an objective assessment of their health status, but also aids in the effective and rational organization of their work.

Integrating novel interventional techniques into pediatric pulmonology training.

To review the evolution of pediatric pulmonology interventions and propose strategies for advancing training in the field. I examined the historical development of pediatric pulmonology interventions and current training practices, including hands-on courses and fellowship programs. I reviewed a survey of US pediatric pulmonology centers to assess variability in procedural expertise. Historically, foreign body removal dominated pediatric pulmonology interventions. Advancements in technology have expanded the field to include techniques such as endobronchial and transbronchial biopsies, airway lumen restoration, and cryotherapy, enabling more accurate tissue sampling with larger specimens while maintaining safety. Hands-on courses, offered globally and at major conferences, provide opportunities for skill development, self-assessment, and networking. However, limited availability leads to high demand and long waiting lists. A survey of US pediatric pulmonology centers revealed significant variability in procedural expertise, highlighting the need for uniform training across institutions. To better integrate interventional techniques, I propose that pediatric pulmonology training could benefit from a structured, tiered approach: (1) expanding hands-on workshops and incorporating them into fellowship programs, (2) facilitating collaborations between centers of excellence to allow trainees to rotate through institutions with advanced expertise, and (3) developing an additional year of training for an "Advanced Pediatric Pulmonologist" certification. This approach aims to ensure proficiency in the latest interventional techniques, standardize care, and foster advancements across the field.

Type and morphology affect the success rate of bronchoscopy for postintubation tracheal stenosis.

With advancements in respiratory interventional techniques, bronchoscopic intervention technology has emerged as a viable approach for managing postintubation tracheal stenosis (PITS). However, there was a paucity of research investigating the potential impact of stenosis characteristics and morphology (such as stenosis degree, length, type, and morphology) on bronchoscopic intervention treatment prognosis for PITS patients. This study was to assess the impact of various preoperative stenosis characteristic factors on the bronchoscopic cure rate among patients. This is a retrospective study analyzing the medical records of patients with PITS who received bronchoscopic intervention at the tertiary interventional pulmonology center. Among the cases, 115 individuals achieved a in a success rate of 79.86% for bronchoscopic intervention therapy and were assigned to Group S. On the other hand, 29 cases required surgical intervention, accounting for a surgical treatment rate of 20.14% and were assigned to Group F. The stenosis in the Group F predominantly exhibited irregular shapes with scar granulation accompanied by tracheal chondromalacia collapse. Patients in group S experienced fewer total procedures, rigid bronchoscopy treatment, intraoperative hypoxemia, needed emergency re-bronchoscopy in 24 h and transferred to ICU postoperatively. Patients with pure scar and granuloma, the rate of bronchoscopic success cure was higher than patients with scar granulation accompanied by tracheal chondromalacia (odds ratio: 8.208; 95% confidence interval: 2.755-24.459), and regular stenosis morphology was associated with a higher bronchoscopic success cure rate (odds ratio: 9.463; 95% confidence interval: 3.128-28.623). Irregular airway stenosis, chondromalacia or airway collapse are key factors affecting the success rate of bronchoscopic treatment for post-intubation tracheal stenosis. Level of evidence: 4 (historically controlled studies).

Airway collapse hinders recovery in bronchoscopy therapy for postintubation tracheal stenosis patients.

Expiratory central airway collapse (ECAC) following postintubation airway stenosis (PITS) is a rare phenomenon. The impact of airway malacia and collapse on the prognosis and the success rate of bronchoscopic interventional therapy in patients with PITS had been inadequately investigated. The aim of this research was to assess the influence of airway malacia and collapse on the efficacy of bronchoscopic interventional therapy in patients with PITS. This retrospective analysis examined the medical documentation of individuals diagnosed with PITS who underwent bronchoscopic intervention at the tertiary interventional pulmonology center of Emergency General Hospital from 2014 to 2021. Data pertaining to preoperative, perioperative, and postoperative stages were documented and subjected to analysis. The patients in malacia and collapse group (MC group) exhibited a higher frequency of perioperative complications, including intraoperative hypoxemia, need for reoperation within 24h, and postoperative intensive care unit admission rate (P < 0.05, respectively). Meanwhile, patients in group MC demonstrated significantly worse postoperative scores (higher mMRC score and lower KPS score) compared to those in pure stenosis group (P < 0.05, respectively), along with higher degrees of stenosis after treatment and a lower success rate of bronchoscopic intervention therapy cured (P < 0.05, respectively). Pearson analysis results showed that these terms were all significantly correlated with the occurrence of airway malacia and collapse in the airway (P < 0.05, respectively). The presence of malacia or collapse in patients with PITS was associated with increased perioperative complications following bronchoscopic interventional therapy, and significantly reduced the long-term cure rate compared to patients with pure tracheal stenosis. Trial registration Chinese Clinical Trial Registry on 06/12/2021. ChiCTR2100053991.

Type one chiari malformation as a cause of central sleep apnea and hypoventilation in children

ObjectivesChiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. MethodsThis is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008–2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging. ResultsWe included 104 children with CM1 with a median age of 7 (interquartile range (IQR) 5–13) years. The median extent of tonsillar descent (TD) was 13 (IQR 10–18) mm. Syringomyelia was present in 19 children (18%). Of all children, 53 (51%) had normal PSG, 35 (34%) showed periodic breathing or central apnea and hypopnea index ≥5 h−1, and 16 (15%) displayed features of compensated central hypoventilation and end-tidal or transcutaneous carbon dioxide 99th percentile level above 50 mmHg. TD had the best predictive value for central breathing disorders. In a linear model, both age (61%) and TD (39%) predicted median breathing frequency (R = 0.33, p < 0.001). ConclusionsAlthough severe CSA is a rare complication of brainstem compression in pediatric patients with CM1, short arousal-triggered episodes of periodic breathing and mild compensated central hypoventilation are common. TD shows the best but still poor prediction of the presence of a central breathing disorder. This highlights the use of PSG in patient evaluation. Posterior fossa decompression surgery effectively treats central breathing disorders.

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: When to suspect and what to do

Childhood Interstitial lung Disease (chILD) is a heterogeneous group of more than 200 respiratory disorders characterized by the impairement of alveolar-capillary gas exchange and lung function. These disorders usually have a chronic course. Morbidity and mortality greatly vary depending on the disorder considered. Interstitial lung disease are associated with respiratory distress. During infancy the most common symptom to pay attention to is tachypnea, whilst in older children clinical presentation may be more insidious. When chILD is suspected, it is advisable to send the patient to a pediatric pulmonology center in order to set up a diagnostic work-up and an adequate treatment. A European protocol called the “Delphi method” has been standardizing empirical treatment since 2015.

Features of the course of bronchial asthma in patients who have suffered a new coronavirus infection

Objective: to evaluate the clinical status of patients with bronchial asthma in the post-Covid period depending on the severity of the acute period of COVID-19. Materials and methods: a case-control study was conducted of patients diagnosed with asthma who had suffered a coronavirus infection, who turned to the Nizhny Novgorod Regional Pulmonology Center for consultation about asthma. The analysis included 56 patients. The survey methods included two stages: retrospective and prospective. The following scales were assessed: the severity of asthma, the level of asthma therapy, asthma control according to the Global Initiative for Asthma (GINA), and the severity of shortness of breath (mMRC test). Results: the analysis revealed statistically significant differences on all scales, regardless of the severity of the acute course of COVID-19, p &lt;0.005. In the process of data analysis, depending on the severity of the acute period of infection, statistically significant differences in the clinical status before and after COVID-19 were found in patients with mild and moderate coronavirus infection. In patients who had mild COVID-19, clinical status indicators worsened on all scales: asthma severity before COVID-19 ─ 2.00 [2.00–2.00], after COVID-19 ─ 2.00 [2.00–3.00], р&lt;0.005; control according to GINA before COVID-19 ─ 2.00 [2.00–2.00] after COVID-19 ─ 3.00 [2.00–3.00]; step therapy before COVID-19 ─ 3.00 [2.25–3.00], after ─ 4.00 [4.00–5.00], р&lt;0.005; severity of shortness of breath before COVID-19 ─ 1.00 [0.25–1.00], after COVID-19 ─ 2.00 [1.00–2.00], р&lt;0.005. Patients who had moderate COVID-19 also worsened all indicators of clinical status on all scales: severity of asthma before COVID-19 ─ 2.00 [2.00–2.00], after COVID-19 ─ 2.00 [2,00–3.00], p&lt;0.005; control according to GINA before COVID-19 ─ 2.00 [2.00–2.00], after COVID-19 ─ 3.00 [2.00–3.00]; level of therapy before COVID-19 ─ 3.00 [3.00–3.00], after COVID-19 ─ 4.00 [4.00–5.00], p&lt;0.005; severity of shortness of breath before COVID-19 ─ 1,00 [1.00–1.00], after COVID-19 ─ 2.00 [1.00–2.00], p&lt;0.005. Conclusion. еhe results of our study demonstrate the negative impact of SARS-CoV-2 on the clinical status of patients with asthma who have suffered a new coronavirus infection.

Lung isolation—a personalized and clinically adapted approach to control bronchoscopy-associated acute massive airway hemorrhage

BackgroundThe current concept of bronchoscopy-associated massive airway hemorrhage is not accurate enough, and the amount of bleeding as the only evaluation criterion cannot comprehensively evaluate magnitude of the effects and the severity.ObjectiveTo propose the concept of bronchoscopy-associated acute massive airway hemorrhage, analyze its impact on patients and highlight the treatment approach of acute massive airway hemorrhage without ECMO support.DesignsA retrospective cohort study.SettingInclude all patients who received bronchoscopy intervention therapy at Interventional Pulmonology Center of Emergency General Hospital from 2004 to December 2021.Patients223 patients met the inclusion criteria.InterventionPatients were divided into two groups: acute massive airway hemorrhage group (n = 29) and non-acute massive airway hemorrhage group (n = 194).Main outcome measuresPerioperative adverse events between two groups were the main outcome. Secondary outcome was the impact of lung isolation on patient in group Acute.ResultsThe incidence of acute massive airway hemorrhage was 0.11%, and the incidence of non-acute massive airway hemorrhage was 0.76% in this study. There were significant differences in the incidence of intraoperative hypoxemia, lowest SpO2, hemorrhagic shock, cardiopulmonary resuscitation, intraoperative mortality, and transfer to ICU between acute group and non-acute group (P<0.05, respectively). Lung isolation was used in 12 patients with acute massive airway hemorrhage, and only 2 patients died during the operation.ConclusionBronchoscopy-associated acute massive airway hemorrhage had more serious impact on patients due to rapid bleeding, blurred vision of bronchoscopy, inability to stop bleeding quickly, blood filling alveoli, and serious impact on oxygenation of the lung lobes. Polyvinyl chloride single-lumen endotracheal intubation for lung isolation, with its characteristics of low difficulty, wide applicability and available in most hospitals, may reduce the intraoperative mortality of patients with bronchoscopy-associated acute massive airway hemorrhage.Trial registrationChinese Clinical Trial Registry on 13/03/2022. Registration number: ChiCTR2200057470.

The Profile of the Oncological Refugee Patient from Ukraine ― Single Institution Experience

The aim of the study was to assess the patient's profile according to the ICD-10 code and the cancer pathway, with particular emphasis on the use of radiotherapy (RT). Due to the outbreak of war in Ukraine on February 24, 2022 and the influx of refugees to Poland, including patients (pts) with suspected or diagnosed cancer or during oncological treatment, actions were taken to coordinate care for this group of pts. At the request of the National Health Fund and the Association of Public Oncology Centers, an ONCOHOTLINE was launched and further activities were coordinated by the National Health Fund and the National Institute of Public Health NIH - National Research Institute. A refugee coordinator was appointed in each voivodeship. All oncology centers were obliged to send once a week to the Refugee Coordinator in a given voivodeship schedules regarding free dates in their clinics according to diagnoses, patient admission hours, location of a given clinic. Pts were registered in accordance with the queue for Polish citizens, with particular emphasis on the urgent mode for pts during treatment, whose treatment was interrupted due to the ongoing war in Ukraine. The records of pts are kept based on the following categories: date of border crossing, date of admission to the treatment center, ICD-10 code, reason for hospitalization, additional information. The official list of pts - refugees from Ukraine admitted to Lower Silesian Oncology, Hematology and Pulmonology Center in Wroclaw (DCOPiH) - was analyzed. From March 14, 2022 to February 10, 2023 149 refugees from Ukraine were admitted to DCOPiH - 105 women, 44 men; the average age was 56 (SD 16). Among the refugees 107 cancer pts were identified - 74 women, 33 men; average age 54 (SD 14). The profile of diagnoses according to the clinical classification code was: C50 - 22, C34 - 10, C53 - 10, C18 - 8, C16 - 7, C92 - 6, C20 - 5, C90 - 4, C56 - 4, C54 - 4, C61- 3, C91- 3, C85 - 3, C43 - 2, C49 - 2; 13 with single diagnoses: C38, C19, C62, C95, C11, C09, C08, C81, C12, C67, C71, C51. 28 of 107 pts required RT - 19 women, 9 men; mean age 52 (SD 15). 21 pts - 75% of the total received definitive RT and respectively 7 (25%) palliative. The collected data was collated with the total number of pts treated with RT in DCOPiH in this period. 7 pts diagnosed with C50 underwent adjuvant definitive RT after breast conserving surgery (another 10 are during RT planning), which was 1,8 % of pts with C50 and RT; respectively for C53 - 10 pts which was 13,7 % and for C61 - 3 pts, which was 0,8 %. Despite a small group of refugees from Ukraine treated with RT, % of pts diagnosed with cervical cancer is significant, the most common cancer among women is breast cancer and it is consistent with the worldwide profile of cancers and so the intention of treatment. The centrally adopted coordination solutions made it possible to distribute pts to many oncology centers. Granting full reimbursement rights and Personal ID Number implement to obtain a full range of services for each Ukrainian patient.

Использование природно-климатических факторов северной Осетии в лечении и реабилитации хронической респираторной патологии.

The effectiveness of treatment of patients with chronic respiratory pathology largely depends on an integrated approach that includes successive stages, including climate therapy in combination with natural factors. T h e p u r p o s e of the study: to analyze the activities of the republican center for pulmonological care and to show the importance of using the natural and climatic features of North Ossetia in the treatment and rehabilitation of patients with chronic respiratory pathology. R e s e a r c h m e t h o d s : statistical, expert assessments, direct observation. The statistical reporting materials of the Republican Center for Pulmonological care for 2018–2022 were analyzed. R e s u l t s . The expediency of organizing local specialized medical rehabilitation organizations and their relevance in the republic is shown. The activity of the pulmonological center became especially relevant in the post-ovarian period, when the need for rehabilitation measures for people who have had a coronavirus infection COVID‑19 has significantly increased. At the end of 2021, the flow of patients to the pulmonology center increased significantly compared to 2020. The growing trend in the number of patients treated with chronic respiratory pathology was noticeably manifested in 2022. Beds for the treatment of chronic obstructive pulmonary disease have been the most in demand for all years. In relation to 2020, the number of people treated in 2022 increased by 44.8%. Despite the fact that bronchial asthma occupies the second place in the structure of hospitalization for pulmonological beds, a more noticeable increase in patients is registered with chronic bronchitis (by 30%) and bronchiectatic disease (by 23.8%). The number of people treated with asthma and asthmatic status increased by 18.3% in 2022. More than 70% of the treated patients were persons over the working age. Taking into account the specifics of respiratory pathology, the expediency of organizing local specialized medical and rehabilitation medical organizations located in climatic conditions familiar to patients that do not require acclimatization is important. The organization of local pulmonological centers in the region is of particular importance for carrying out medical and rehabilitation measures for people with impaired respiratory system function after suffering from COVID‑19 coronavirus infection. The identified trends should be taken into account when planning the volume of specialized medical care in the republic.

Association of Upon-Diagnosis Blood Eosinophilic Count with Frequency and Severity of Annual Exacerbation in Chronic Obstructive Pulmonary Disease: A Prospective Longitudinal Analysis.

There is a controversy regarding the relationship between blood eosinophil count and COPD exacerbation. We aimed to determine whether peripheral eosinophils upon COPD diagnosis could affect the frequency and severity of annual acute exacerbation of COPD (AECOPD). This prospective study was conducted on 973 newly diagnosed COPD patients who were under 1-year follow-up in a pulmonology center in Iran. The Cox proportional model, polynomial regression, and receiver operator characteristic curves were conducted to evaluate the impact of the eosinophil levels on AECOPD. A linear regression model was conducted to evaluate the continuous association of eosinophilic count with AECOPDs. Patients with eosinophil >200 cells/microliter were higher pack-year smokers with more pulmonary hypertension prevalence compared to COPD patients with <200 cells/microliter. There was a positive correlation between the eosinophilic count and the frequency of AECOPDs. Eosinophil >900 cells/microliter and eosinophil >600 cells/microliter had a sensitivity of 71.1% and 64.3%, respectively, in predicting the occurrence of more than one AECOPD. Eosinophilic count cutoff of 800 cells/microliter had the highest Youden index with sensitivity and specificity of 80.2% and 76.6%, respectively, for incident AECOPD in newly diagnosed patients. Using a linear model, increasing 180 cells/microliter in serum eosinophils was associated with further exacerbation. Evaluating gender, BMI, smoking pack-year, FEV1/FVC, CAT score, GOLD score, pulmonary hypertension, annual influenza, pneumococcal vaccinations, leukocytosis, and blood eosinophils, only blood eosinophils (hazard ratio (HR) = 1.44; 95% confidence interval = 1.33-2.15; p value = 0.03) and GOLD score (HR = 1.19; 95% CI = 1.30-1.52; p value = 0.03) were found as independent risk factors of AECOPD >3 episodes/year. Requirement for ICU admission, invasive ventilation, and mortality rate due to AECOPDs was similar between eosinophilic and noneosinophilic groups. Eosinophilia upon COPD diagnosis is a factor of recurrent AECOPDs. To reduce the risk of AECOPDs and the burden of disease, clinicians may consider inhaler corticosteroids and domiciliary oxygen with a lower threshold for eosinophilic-COPD patients regardless of their clinical status.

Evaluation of specificity and sensitivity of IRT/IRT protocol in the cystic fibrosis newborn screening program: 6-year experience of three tertiary centers.

We aimed to evaluate cutoff values of immunoreactive trypsinogen (IRT)/IRT and determine relationship between IRT values and clinical characteristics of children with cystic fibrosis (CF). This study is cross-sectional study. Data of children with positive newborn screening (NBS) between 2015 and 2021 were evaluated in three pediatric pulmonology centers. Age at admission, sex, gestational age, presence of history of meconium ileus, parental consanguinity, sibling with CF, and doll-like face appearance, first and second IRT values, sweat chloride test, fecal elastase, fecal fat, biochemistry results, and age at CF diagnosis were recorded. Sensitivity and specificity of IRT cutoff values were evaluated. Of 815 children with positive NBS, 58 (7.1%) children were diagnosed with CF. Median values of first and second IRT were 157.2 (103.7-247.6) and 113.0 (84.0-201.5) μg/L. IRT values used in current protocol, sensitivity was determined as 96.6%, specificity as 17.2% for first IRT, and 96.6% sensitivity, 20.5% specificity for second IRT. Positive predictive value (PPV) was determined as 7.1%. When cutoff value for first IRT was estimated as 116.7μg/L, sensitivity was 69.0% and specificity was 69.6%, and when cutoff value was set to 88.7μg/L for second IRT, sensitivity was 69.0% and specificity was 69.0%. Area under curve was 0.757 for first and 0.763 for second IRT (p < 0.001, p < 0.001, respectively). PPV was calculated as 4.3%. Conclusion: Although sensitivity of CF NBS is high in our country, its PPV is significantly lower than expected from CF NBS programs. False-positive NBS results could have been overcome by revising NBS strategy. What is Known: • Although immunoreactive trypsinogen elevation is a sensitive test used in cystic fibrosis newborn screening, its specificity is low. • In countries although different algorithms are used, all strategies begin with the measurement of immunoreactive trypsinogen in dried blood spots. What is New: • In our study, it was shown that use of the IRT/IRT protocol for cystic fibrosis newborn screening is not sufficient for the cut-off values determined by the high number of patients. • Newborn screening strategy should be reviewed to reduce false positive newborn screening results.

Psychological status of mothers of children with cystic fibrosis and primary ciliary dyskinesia.

We aimed to investigate depression, burnout, attitude, and burden of caregivers of children with cystic fibrosis (CF), and especially caregivers of children with primary ciliary dyskinesia (PCD) due to limited number of studies on this topic, and to compare them according to their children's clinical status. Clinical features and demographic data of children and their families were asked from caregivers in four pediatric pulmonology centers. Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Parental Attitude Research Instrument were administered to caregivers in both groups. Results were compared between the two groups. In total, 131 children with CF and 39 with PCD and their caregivers were involved in the study. All primary caregivers were mothers in both groups. Depression, burnout, and burden scores of mothers of children with CF were significantly higher than mothers of children with PCD (p = 0.017, p = 0.024, p = 0.038, respectively). Burnout was higher in both CF and PCD groups with low family income (p = 0.022, p = 0.034). Number of hospital visits in the previous 6 months was correlated with burnout in both CF and PCD groups (r = 0.207, p = 0.034; r = 0.352, p = 0.044). Although mothers with children with CF have higher levels of depression, burnout, burden, and negative attitudes towardchildren than mothers with children with PCD, these are also significantly high in mothers with children with PCD. Psychological problems of mothers of children with CF and PCD may increase with frequent hospital visits, hospitalizations, low family income, number of children, and chronic disease in another child.

The associations between the clinical course of children with tracheostomy and their mothers' depression, burnout, burden, and self-esteem.

The aim of this study was to evaluate the clinical features of children with tracheostomy and the predictors of psychological status of their primary caregivers in order to determine the associations between the children's clinical course with their caregivers' psychological status. The caregivers of children with tracheostomy are responsible for providing basic tracheostomy care at home. All these responsibilities may be associated with significant changes in family members' lifestyles, daily routines and family dynamics. This study is a cross sectional study. Data of the family's socioeconomic status and clinical status of children with tracheostomy were noted in four paediatric pulmonology centers. The Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Rosenberg Self-esteem Scale were used for psychological measurements of the caregivers. The STROBE checklist was used for this study. Eighty-five children and their primary caregivers were enrolled in the study. The children's median age was 4.1 years. Thirty-eight of them were dependent on home ventilators. Twenty-one had bacterial colonisation. All children's primary caregivers were their mothers. Beck Depression Inventory scores of mothers of children with colonisation were higher. Number of hospitalizations in previous 6 months was related to mothers' emotional exhaustion and depersonalization scores. Duration of children's hospitalizations in previous 6 months was positively correlated to mothers' emotional exhaustion, depersonalization, and Beck Depression Inventory scores. Mothers of children with tracheostomy may experience psychological conditions, such as high levels of depression, burnout, burden and low levels of self-esteem. Frequent and long-term hospitalizations of children correlated with mother's depression and burnout. Therefore, primary caregivers should be evaluated and supported psychologically. Preventing mothers of children with tracheostomy from experiencing psychological conditions such as depression, burnout, burden and low self-esteem can also increase the quality of care for children.

Endosonography and endosonography guided needle aspiration for left adrenal gland assessment in lung cancer patients - 10 years' experience.

Lung cancer patients (LCP) require invasive evaluation of left adrenal glands (LAG) if distant metastases (M1b/1c) are suspected in CT or PET-CT. Only few studies showed utility of endosonography and particularly EUS-b-FNA as minimally invasive endoscopic method of LAG analysis. A retrospective study of consecutive LCP was conducted in two pulmonology centers between January 2010 and December 2019. Records of complete endosonographic staging with use of single ultrasound bronchoscope or two scopes were overviewed. The analysis included cases of enlarged LAG (body size or limbs > 10 mm) examined and sampled by EUS-b-FNA or EUS-FNA. 142 of 2596 LCP staged by complete endosonography (M: 88, F: 54 mean age 64.7) had enlarged LAG, which were biopsied by conventional EUS-FNA (52) and/or by EUS-b-FNA (90). Strong correlation with gland diameter (P < 0.001) was observed. The incidence of LAG metastases in analyzed group was 52.1% (74/142) and regarding histology: SCLC 76.9% (10/13), adenocarcinoma 66.7% (44/66), NSCLC 56.3% (9/16) and SCC 17.5% (7/40). A specificity and PPV for both methods were 100%. A sensitivity, accuracy and NPV for EUS-FNA were 91.7%, 96.2%, 93.3% and for EUS-b-FNA 88%, 93.3% and 87%, respectively and no significant differences for both methods were noted (P = 0.62, 0.44, 0.35). No severe complications afterall biopsies were observed. A six months clinical follow up included all negative LCP with enlarged LAG. After our study EUS-b-FNA seems to be a reasonable method of choice for LAG assesssment in LCP.

Pre- and post-COVID practice of interventional pulmonology in adults in Portugal

Introduction and objectivesThere is a lack of information regarding bronchoscopy practice in adults in Portugal. Our objective was to obtain an overview of the characteristics, resources, techniques and behaviors in national interventional pulmonology (IP) units, before and after SARS-CoV-2 outbreak. Materials and MethodsAn online survey was developed by an expert panel with a total of 46 questions comprising the specifications of each unit, namely physical space, equipment, staff, procedure planning, monitoring, technical differentiation, and numbers pre- and post-COVID-19. Forty-one interventional pulmonology centers were invited to participate between April and May 2021. Results37 units (90.2%) responded to the survey. The majority (64.9%) have a fully dedicated space with a weekly presence of ≥3 chest physicians (82.1%) and support of an anesthesiologist on specific days (48.6%). There is marked heterogeneity in the IP unit's equipment, and 56.8% acquired disposable bronchoscopes after COVID-19 pandemics. Pre-bronchoscopy hemogram, platelet count and coagulation tests are regularly asked by more than 90% of the units, even when deep sedation or biopsies are not planned. In 97.3% of cases, topical anesthesia and midazolam are utilized. Propofol (21.6%) and fentanyl (29.7%) are occasionally employed in some institutions. Most units use ancillary sampling techniques to diagnose central or peripheral lesions, with radial EBUS being used for guidance of distal procedures in 37.8% of centers, linear EBUS and EUS-B-FNA for mediastinal diagnosis and/or staging in 45.9% and 27.0% of units, respectively. Cryobiopsies are used by 21.6% of respondents to diagnose diffuse lung diseases. Rigid bronchoscopy is performed in 37.8% of centers. There was a decrease in the number of flexible (p < 0.001) and rigid (p = 0.005) bronchoscopies and an upscale of personal protective equipment (PPE) during the COVID-19 outbreak. ConclusionsIP units have variable bronchoscopic practices, but during the COVID-19 pandemic, they complied with most international recommendations, as elective procedures were postponed and PPE levels increased.

Нейтропенія в дітей: клінічні маски і лікарська тактика

Нейтропенічний синдром досить часто зустрічається в практиці педіатра. Природа його різноманітна, і кожен випадок нейтропенії потребує проведення ретельної диференціальної діагностики для визначення тактики ведення хворого. Тільки за диференційованого підходу до пацієнтів із нейтропенією можливо попередити розвиток серйозних ускладнень інфекційних процесів. Для ілюстрації наводимо клінічний випадок власного спостереження дитини 4 років із важким хронічним бронхолегеневим захворюванням, яка знаходилася на стаціонарному обстеженні та лікуванні в дитячому міському пульмонологічному центрі. Дитина поступила зі скаргами на постійний вологий малопродуктивний кашель, епізодичні дистанційні хрипи, зниження слуху. З анамнезу захворювання було встановлено, що з місячного віку у дитини спостерігалися повторні отити, рецидивуючі бронхіти з бронхообструктивним синдромом, неодноразові пневмонії різноманітної локалізації. Комплексне обстеження, що включало, окрім стандартних лабораторних досліджень, лікувально-діагностичну бронхоскопію, комп’ютерну томографію органів грудної клітини, виявило у дитини двосторонній дифузний катарально-гнійний ендобронхіт, КТ-ознаки дифузних інтерстиціальних змін у легенях і бронхомаляції. Ретельний динамічний аналіз наявних в амбулаторній картці гемограм дозволив запідозрити в пацієнта вроджену нейтропенію. Багаторазове виникнення нейтропенічних кризів зі зниженням числа нейтрофілів до 1000 кл/мкл і нижче на першому році життя дитини та їх періодичністю в 3–4 тижні підтвердило циклічний характер нейтропенії. Проведена стернальна пункція з оцінкою мієлограми не виявила виражених змін гранулоцитарного ряду, що, ймовірно, було обумовлено періодом ремісії циклічної нейтропенії на момент обстеження. Таким чином, важке хронічне захворювання бронхолегеневої системи у дитини стало клінічною маскою циклічної нейтропенії, що вимагала проведення ретельної діагностики. Нейтропенія у дітей повинна розглядатися практикуючим лікарем як лабораторний симптом, що потребує осмислення, зіставлення з клінічними проявами та обов’язкового динамічного аналізу.

Clinical spectrum of children with interstitial pneumonia with autoimmune features

BackgroundInterstitial pneumonia with autoimmune features (IPAF) is a term used to describe adult patients with interstitial lung disease (ILD) who display some clinical or serological features of autoimmune diseases and who do not meet the full criteria for a specific connective tissue disease (CTD). The aim of this study was to define the demographic, clinical, radiologic, serologic and histopathologic features and assess treatment in children with IPAF. MethodsThis retrospective cohort study was conducted at a tertiary referral pediatric pulmonology center between January 2010 and August 2020. Children with proven interstitial pneumonia with no known etiologies were evaluated for IPAF according to European Respiratory Society/American Thoracic Society research statement of IPAF. ResultsAmong 132 children with ILD, 17 patients were evaluated in detail for IPAF criteria and six patients were further diagnosed as having IPAF. The incidence of IPAF in our patients with ILD was 4.5%. Four of these patients were female. The median age at the time of ILD diagnosis was 10.5 years. The most common initial symptom was shortness of breath, and the most common physical examination sign was crackles in both lungs. Steroid therapy was given to all patients and four patients received other immunosuppressive agents for steroid sparing. Two of those patients died because of respiratory insufficiency during the follow-up. ConclusionChildren with interstitial pneumonia and certain clinical, serologic, and/or morphologic features should raise suspicion for the presence of an underlying systemic autoimmune disease. IPAF is also seen in children and should be categorized in chILD classifications.