Has the Coronavirus Disease 2019 Pandemic Played a Role in the Early Detection of Pulmonary Embolism in Children?

OBJECTIVE: Pulmonary embolism (PE) poses a significant threat to children, and nonspecific symptoms lead to delayed diagnosis. The emergence of coronavirus disease 2019 (COVID-19) has increased the complexity as it is associated with similar symptoms and increased risk of thrombotic complications. This study aimed to assess the risk factors, clinical presentations, and diagnostic features of PE in pediatric patients and to examine the impact of the COVID-19 pandemic on children with PE. MATERIALS AND METHODS: We conducted a retrospective descriptive study examining the clinical and diagnostic data of 44 pediatric patients with radiologically confirmed PE. The study compared and analyzed patients diagnosed before and during the COVID-19 pandemic. RESULTS: In the study, 21 of 44 pediatric patients were diagnosed in the 4 years before the COVID-19 pandemic, and 23 were diagnosed with PE during the COVID-19 pandemic. The mean time to diagnosis was 8 (2 to 14) days before the pandemic and 1 (1 to 2) days during the pandemic (P < 0.001). The most common associated condition in both groups was infection (65.9%). Dyspnea (65.9%) and tachypnea (50.0%) were common symptoms. Except for deep vein thrombosis, there were no significant differences according to associated conditions between the groups (P = 0.001). Pulmonary emboli were anatomically detected using computed tomography angiography, showing bilateral involvement in 45.4% of patients, segmental artery involvement in 38.6%, and main artery involvement in 15.9%. CONCLUSION: The COVID-19 pandemic heightened suspicion of pediatric PE and accelerated diagnosis. Standardized diagnostic guidelines are increasingly necessary to balance accurate diagnosis with avoiding excessive imaging.

Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.

BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.

Experience with flexible bronchoscopy for noncoronavirus disease of 2019 indications in pediatric patients during the coronavirus disease of 2019 pandemic.

BACKGROUND AND AIM: Flexible bronchoscopy (FB) poses a risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission due to aerosol generation. This study aimed to assess the utilization, indications, outcomes, and safety of FB in pediatric patients for noncoronavirus disease of 2019 (COVID-19) reasons during the pandemic. MATERIALS AND METHODS: We retrospectively analyzed pediatric patients who underwent FB for non-COVID-19 indications at a tertiary children's hospital's pulmonary clinic during the COVID-19 pandemic. Patients showed no COVID-19 symptoms and tested negative for SARS-CoV-2 by real-time polymerase chain reaction (PCR) of nasopharyngeal and throat swabs within 24 h before the procedure. FBs were conducted in the operating room, with healthcare professionals (HCPs) wearing personal protective equipment, including medical N95 masks, gloves, gowns, and eye protection. RESULTS: Between March 2020 and April 2022, 167 pediatric patients underwent FB for non-COVID-19 indications. Common indications included foreign body aspiration (22.7%), stridor (10.1%), and atelectasis (8.9%). No COVID-19 symptoms were observed in patients on the 1st and 10th days post-FB. During the 1-month follow-up, 52 patients underwent SARSCoV-2 PCR testing, and one patient tested positive in the third week after the procedure. None of the HCPs in the FB team experienced COVID-19 symptoms or tested positive for SARS-CoV-2. CONCLUSION: A bronchoscopy protocol with safety precautions minimized the risk of COVID-19 transmission, allowing safe FB performance for non-COVID-19 indications in pediatric patients during the pandemic. The experience gained in FB during COVID-19 is valuable for similar situations in the future.

Relationship between biologic modifying agents and development of latent tuberculosis in pediatrics.

INTRODUCTION: Biologic modifying agents are associated with an increased risk for infection with mycobacteria. The aim of this study is to document patients who received different biologic modifying therapies in our pediatric rheumatology department and the possibility of development of tuberculosis (TB). METHODOLOGY: This retrospective study was conducted in Ankara City Hospital. Pediatric patients who were treated with biologic modifying agents between 2010-2020 were documented. Development of TB and the risk factors were assessed in this patient group. RESULTS: There were 72 patients who were treated with different biologic modifying agents. Tuberculin skin test (TST) was positive in 7 (9.7%) patients during follow up. Three patients whose TST was positive had received canakinumab, 2 received etanercept, 1 received adalimumab and 1 received anakinra. Median duration of therapy was 43.5 (16.5-168) months for these patients and the duration was longer than patients who did not develop latent tuberculosis (p = 0.04). Patients who developed latent TB under treatment were significantly older than the patients who did not (p = 0.01). CONCLUSIONS: According to our findings, 9.7% of pediatric patients who received biologic modifying agent therapy developed latent TB. Patients who developed latent TB were older, and the duration of treatment was longer than patients who did not develop latent TB. Although not statistically significant, canakinumab, which is known as an agent less likely to cause TST conversion, was in fact the most common agent that caused TST conversion.

Macrophage activation syndrome induced by Staphylococcus Epidermidis in a pediatric patient with cystic fibrosis and familial Mediterranean fever.

Staphylococcus epidermidis (S. epidermidis) is the most frequently isolated member of the coagulase-negative staphylococci, which colonizes the skin and mucous membranes of the human body. Despite being a component of the normal flora, S. epidermidis can act as an opportunistic pathogen and is frequently implicated in both bacterial and nosocomial infections. Macrophage activation syndrome (MAS) is a rare but potentially fatal disease that occurs as a result of increased activation and proliferation of T lymphocytes and macrophages in response to infections and immune mediated diseases. It is very rare in patients with cystic fibrosis. Here we report a case of an 8-year-old girl with cystic fibrosis and familial Mediterranean fever who developed MAS after contracting a S. epidermidis infection.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data.

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.

Risk factors for complicated community-acquired pneumonia in children.

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

Evaluation of ciliary functions and ciliary beat frequency via cell culture method in patients with primary ciliary dyskinesia.

BACKGROUND: Cell culture increases both diagnostic specificity and sensitivity of primary ciliary dyskinesia (PCD) and the most important reason to use cell culture for definitive diagnosis in PCD is to exclude secondary ciliary defects. Here we aimed to evaluate the cilia functions and cilia ultrastructural abnormalities after ciliogenesis of cell culture in patients with definitive diagnosis of PCD. We also aimed to compare high speed videomicroscopy (HSVM) results of patients before and after ciliogenesis and to compare them with electron microscopy, genetic and immunofluorescence results in patients with positive diagnosis of PCD. METHODS: This study was conducted as a cross-sectional study in patients with PCD. HSVM, transmission electron microscopy (TEM) and immunofluorescence staining results of the nasal biopsy samples taken from patients with the definitive diagnosis of PCD were evaluated and HSVM findings before and after cell culture were described. RESULTS: Ciliogenesis and regrowth in the cell culture occurred in the nasal biopsy sample of eight patients with PCD. The mean age of the patients was 15.5±4.2 years (8.5-18 years). Mean beat frequency was found to be 7.54±1.01 hz (6.53-9.45 hz) before cell culture, and 7.36±0.86 hz (6.02-7.99 hz) after cell culture in the nasal biopsy of patients. There was no significant difference in the beat frequency of PCD patients before and after cell culture. Ciliary function analysis showed the similar beating pattern before and after cell culture in patients with PCD. CONCLUSIONS: This study showed us that there was no difference between cilia beat frequency and beat pattern before and after cell culture in patients with definitive diagnosis of PCD and repeated HSVM would be a useful diagnostic approach in patients who have no possibility to reach other diagnostic methods.

Interstitial Lung Disease in an Adolescent Girl with Lipopolysaccharide-Responsive Beige-Like Anchor Deficiency.

Background: Previously, lipopolysaccharide-responsive beige-like anchor (LRBA) deficiency was categorized as a subtype of common variable immune deficiency. Research shows that LRBA deficiency is caused by dysregulation of T cell activation and expansion; it is placed under the category of immune dysregulation with cytotoxic T lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency. Cohort studies have revealed a broad spectrum of clinical manifestations and variable phenotype expression, including immune dysregulation [enteropathy, autoimmune cytopenia, interstitial lung disease (ILD), etc.] on 1 hand and immune deficiency (hypogammaglobulinemia, recurrent infections, bronchiectasis, etc.) on the other hand. Chronic lung disease is frequently seen in LRBA deficiency and is associated with poor outcomes. Case Presentation: This case report evaluates a female who presented with recurrent pneumonia and bronchiectasis but did not respond to treatment; she was lastly diagnosed with ILD with detailed clinical, radiological, and pathological workup. Conclusions: The respiratory characteristics of patients with LRBA deficiency should be investigated, monitored, and treated from the time of its diagnosis. The awareness and involvement of pulmonologists to pulmonary morbidity of patients with LRBA deficiency in workup and clinical decision making are crucial.

Evaluation of Frequency and Characteristics of Drug Allergy in Pediatric Patients with Cystic Fibrosis.

Background: Previous studies reported that the prevalence of drug allergy is higher in patients with cystic fibrosis (CF) than in the general population. It is important to exclude or confirm the drug allergy diagnosis with detailed allergic evaluation for preventing drug allergy overdiagnosis. Our study aims to determine the actual frequency of drug allergy proven by diagnostic tests in children with CF and to compare it with the control group. Methods: Patients diagnosed with CF who were followed up in the Pediatric Pulmonology Clinic were included in the study group. Children with similar gender and age characteristics who did not have any chronic diseases and who applied to the Pediatric Polyclinics were included in the control group. We reviewed the medical data of patients with CF. Also, we evaluated the parents of the patients via phone conversation and/or during the control of the outpatient clinic and questioned them in terms of drug allergy. In addition, we assessed those with suspected drug allergies in the pediatric allergy clinic for diagnostic tests and compared it to the control group. Results: CF patients (n = 44) and control group (n = 100) were included in the study. Only 1 patient (2.2%) out of the 44 patients in the study group had a suspicion of drug-related hypersensitivity history. In the control group, 1 patient had a history of rash, provocation test was performed to rule out drug hypersensitivity reaction, and it was evaluated as a negative result. Conclusions: The result of our study showed that the frequency of drug allergy in children diagnosed with CF was not different from the control group. However, it will be useful to confirm the data of pediatric patients with CF in larger groups. In the presence of suspicion of drug allergy, a diagnostic evaluation can prevent unnecessary drug allergy diagnoses.

Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery.

INTRODUCTION: Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by partial obstruction and occurs at a rate of 1/20,000-30,000 live births. Here, we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature. METHODS: We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18 years at our center between 2013 and 2020. In addition, we examined the symptoms and findings recorded during the patients' follow-up in this retrospective descriptive study. RESULTS: The median age of 20 patients with CLE at diagnosis was 3.2 years (range 1 day-17 years). Respiratory distress and mediastinal shift were more prominent in patients who underwent surgery, and they were diagnosed at an earlier age compared with patients who were followed up conservatively (p = 0.001, 0.049, 0.001, respectively). Neither the pulmonary lobe involvement nor the bronchoscopy findings were found to be indicative of surgery. DISCUSSION: We observed that respiratory distress and mediastinal shift were more prominent in patients with a diagnosis of CLE who underwent surgery compared with patients who were conservatively followed up. Moreover, we observed that those who underwent surgery were diagnosed with CLE at an earlier age. In line with the literature, the pulmonary symptoms and CLE-related imaging findings in our study were reduced during conservative follow-up.

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care.

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients.

We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge, and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with forced vital capacity (FVC) (r = - 0.800, p = 0.014) and forced expiratory volume in 1 s (FEV1) (r = - 0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with FVC (r = - 0.697, p = 0.0082) and FEV1 (r = - 0.720, p = 0.006). CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology.

Clinical findings of patients with cystic fibrosis according to newborn screening results.

BACKGROUND: Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. METHODS: In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). RESULTS: In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). CONCLUSIONS: Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.

The controversy of drug hypersensitivity in patients with cystic fibrosis and review of the literature.

BACKGROUND: Cystic fibrosis (CF) is reported to be a risk factor for drug hypersensitivity. However, there are conflicting data about true prevalence of drug hypersensitivity in children with CF. METHODS: The suspicious drug hypersensitivity reactions (DHRs) of children with CF were enquired by the European Network for Drug Allergy (ENDA) questionnaire, and skin tests and/or drug provocation tests were performed according to the established guidelines. RESULTS: Two hundred and nineteen children (48.9% boys; median [IQR] age, 8.4 years [4.8-12.4 years]) with cystic fibrosis were included in the study, among which 22 patients with 24 suspected DHRs were evaluated. Most of the suspected DHRs were of non-immediate (n = 16, 66.6%) type, and the offending drugs were amoxicillin-clavulanic acid (n = 7), macrolides (n = 4), trimethoprim-sulfamethoxazole (TMP/SMX) (n = 2), piperacillin-tazobactam (n = 1), pancrelipase (n = 1), and ursodeoxycholic acid (n = 1). Eight (33.3%) of the DHRs were classified as immediate (ceftriaxone [n = 2], ceftazidime [n = 2], meropenem [n = 1], AmBisome [n = 2], and vancomycin [n = 1]). The main clinical presentations were maculopapular eruption (41.6%) and urticaria (37.5%), accompanied by angioedema (8.3%), flushing (12.5%), and vomiting (8.3%). Nine skin tests (with beta-lactam protocol in 6 patients) and 24 DPTs were performed, and none of the skin tests revealed a positive result; however, 2 DPTs with TMP/SMX were positive. CONCLUSION: Actual drug hypersensitivity was demonstrated in 2 of 219 patients (0.9%) with non-beta-lactam antibiotics. These results conflict with previous researches that showed higher drug hypersensitivity rates but are consistent with some recent studies. Allergological diagnostic workup is mandatory in patients with cystic fibrosis in case of a suspicious DHR.

Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula.

INTRODUCTION: Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center. METHODS: Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy. RESULTS: A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025). CONCLUSION: Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.

Diverse clinical characteristics of Aspergillus growth in patients with cystic fibrosis.

BACKGROUND AND OBJECTIVES: Patients with cystic fibrosis (CF) have a varying spectrum of clinically significant Aspergillus disease in addition to allergic bronchopulmonary aspergillosis (ABPA). Here we aimed to review the different clinical phenotypes related with Aspergillus growth on the airway culture of patients with CF, we also aimed to investigate the effect of Aspergillus growth on lung function tests. METHOD: The medical records of 100 patients with CF who had Aspergillus growth on airway culture within the period of April 2001 and June 2016 were retrospectively analyzed. Age, gender, symptoms, physical examination findings, pulmonary function tests, the diagnosis of ABPA, and airway culture results were recorded for every visit. Patients with Aspergillus growth on airway cultures were classified into different groups as ABPA, Aspergillus sensitization, Aspergillus colonization and Aspergillus bronchitis. RESULTS: Medical records of 83 patients and 147 sputum cultures were attained from 100 patients. The mean age of the patients was 17.6±7.6 years and the mean age of the first Aspergillus growth in sputum culture was 12.5±6.7 years. At first isolation, Aspergillus fumigatus SC was the most common Aspergillus SC in sputum (76.3%) and 14.5% of these patients required hospitalization. Aspergillus sensitization was diagnosed in 3.6% (n= 3) of the patients. Aspergillus colonization was diagnosed in 18.1% (n= 15) of all patients and led to a decline in FEV1%, FVC% and FEF25-75% which was not statistically significant, furthermore. ABPA was detected in 9.6% (n= 8) of all patients and led to a statistically significant decline in FEV1% (p= 0.02); nonsignificant decline in FVC% and FEF25-75%. Aspergillus bronchitis was detected in 43.4% (n= 36) of all patients and led to nonsignificant decline in FEV1%, FVC% and FEF25-75%. CONCLUSION: ABPA is recognized as the most common Aspergillus associated disorder in CF patients and is related to deteriorated pulmonary function tests; however Aspergillus colonization and bronchitis may also be associated with worsening lung function.

Variation in the bombesin staining of pulmonary neuroendocrine cells in pediatric pulmonary disorders-A useful marker for airway maturity.

OBJECTIVES: Pulmonary neuroendocrine cells (NEC) increase with age due to pulmonary maturity. The aim of this study was to determine whether open lung biopsies from patients with interstitial lung diseases have increased pulmonary NEC compared with neuroendocrine cell hyperplasia of infancy (NEHI). Our second aim was to assess pulmonary NECs in the lung autopsy of children without lung disease who died from different causes. METHODS: Lung tissue of 5 infants with NEHI; 21 patients with pediatric interstitial lung disease (chILD); 17 lung autopsies of infants at varying age without lung disease were included. The percentage of the airways containing neuroendocrine cells, the average percentage of neuroendocrine cells (NECs) per airway, and the number of neuroendocrine bodies (NEBs) in each case were analyzed. RESULTS: The mean percentage of the airways containing neuroendocrine cells were 95% in the NEHI group, 30% in the chILD group, 89% under Intrauterine 37 weeks, 70% between intrauterine 37 to 40 weeks, 52% at postnatal 4 days to 6 months of autopsy ages. In the NEHI group, diffuse NE cell distribution and large NEBs were noticed in the lung biopsy. In the chILD group, neuroendocrine cells were dispersed, did not form clusters and NE cells showed solitary distribution. In the lung autopsy group, linear NE cells were detected at younger aged fetuses and solitary distribution of NE cells was detected with the older increasing age. CONCLUSIONS: Our findings confirm that NECs are seen in many other childhood interstitial lung diseases; NE cell hyperplasia may be a marker of decreased pulmonary development and NE cells decrease with the increasing age of the fetus during Intrauterine life.

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

BACKGROUND: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. METHODS: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. RESULTS: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. CONCLUSION: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

BACKGROUND: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. METHODS: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. RESULTS: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). CONCLUSIONS: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.