Galectin-3 levels in children with cystic fibrosis.

Cystic fibrosis (CF) is a multisystemic disease in which airway obstruction, infection, and inflammation play a critical role in the pathogenesis and progression of CF lung disease. The carbohydrate-binding protein Galectin-3 is increased in several inflammatory and fibrotic diseases and has recently been forwarded as a biomarker in these diseases. We aimed to define the role of serum Galectin-3 in children with CF by comparison with healthy subjects. This is a cross-sectional, case-control study. 143 CF and 30 healthy subjects were enrolled in the study. Peripheral blood and sputum concentrations of Galectins-3, interleukin (IL)-17A, IL-8, and neutrophil elastase (NE) were determined with commercial ELISA kits. There was no significant difference between the groups in age and gender (p = 0.592, p = 0.613, respectively). Serum Galectin-3 and NE concentrations were higher in the patient group than in healthy controls (p = 0.002, p < 0.001, respectively). There were no significant differences between groups according to IL-17A and IL-8 concentrations. Serum Galectin-3 was correlated with age (r = 0.289, p < 0.001) and body mass index (BMI) (r = 0.493, p < 0.001) in children with CF. Sputum Galectin-3 levels are negatively correlated with percent predictive forced expiratory volume in 1 s (FEV1) (r = - 0.297, p = 0.029), FEV1 z-score, (r = - 0.316, p = 0.020), percent predictive forced vital capacity (FVC) (r = - 0.347, p = 0.010), and FVC z-score (r = - 0.373, p = 0.006).   Conclusion: The study shows that serum Galectin-3 levels increased in clinically stable CF patients, and serum Galectin-3 response may depend on age, gender, and BMI. The sputum Galectin-3 was found to be negatively correlated with patients' lung functions. What is known: • Galectin-3 is a key regulator of chronic inflammation in the lung, liver, kidney, and tumor microenvironment. What is new: • Children with cystic fibrosis (CF) have higher serum Galectin-3 concentrations than healthy children. • Serum Galectin-3 expression influenced by age, BMI, and gender in children with CF.

Evaluation of polysomnography findings in children with genetic skeletal disorders.

Children with genetic skeletal disorders have variable conditions that can lead to sleep-disordered breathing, and polysomnography is the gold standard for diagnosing this condition. We aimed to review polysomnography findings, to assess the severity of sleep apnea, and to investigate the clinical variables predictive of sleep-disordered breathing in these patients. We retrospectively collected the medical records of patients with genetic skeletal disorders who underwent polysomnography for 5 years. Twenty-seven children with various genetic skeletal disorders, including achondroplasia (14), Crouzon syndrome (3), acromesomelic dysplasia Maroteaux type (3), Apert syndrome (2), osteopetrosis (1), Jeune dysplasia (1), Desbuquois dysplasia (1), acrodysostosis (1), and spondyloepiphyseal dysplasia (1) were enrolled. The median age at the first polysomnography was 58 (1st-3rd quartile: 31-113) months. The overall sleep-disordered breathing results were: 19 (70.3%) had obstructive sleep apneas (OSA) (4 mild, 6 moderate, 9 severe), 2 (7.4%) had central apneas, 4 (14.8%) had nocturnal hypoventilation. There was a significant correlation between non-ambulatory status with both total AHI and OSA (p < 0.001, rho: -0.66/p = 0.04, rho: 0.38, respectively). Nine patients received positive airway pressure titration, and the oAHI values of all returned to the normal range. These patients were started with positive airway pressure treatment. Our cohort showed that the majority of the patients with skeletal dysplasia had sleep apnea syndrome characterised mainly by OSA, highlighting the importance of polysomnography screening for sleep disorders. Positive airway pressure therapy represents an effective treatment for sleep-disordered breathing in those patients.

Obstructive sleep apnea in children with Down syndrome: is it possible to predict severe apnea?

The objectives are to explore the demographic and polysomnographic features of children with Down syndrome and to determine the predictive factors associated with severe sleep apnea. A total of 81 children with Down syndrome referred for full-night polysomnography were analyzed. In addition, parental interviews were performed for each child. Data were available for 81 children, with a mean age of 4.8 years. Severe obstructive sleep apnea was determined in 53.1%. Age, sex, exposure to second-hand smoke, clinical findings, anthropometric features, and the presence of comorbidities were not predictors of severe obstructive sleep apnea. Children who were exposed to second-hand smoke had more sleep-related symptoms. Even in children without symptoms, the prevalence of severe obstructive sleep apnea was 40%. Moreover, 86% of parents had no previous information regarding possible sleep breathing disorders in their children. Clinically significant central apnea was present in 10 patients (12.3%).Conclusion: Our results demonstrate that severe obstructive sleep apnea is common in children with Down syndrome, even in children without a history of symptoms of sleep apnea. It is not possible to predict patients with severe apnea; thus, screening of children with Down syndrome beginning from young ages is very important. Central apneas could be a part of the spectrum of sleep abnormalities in Down syndrome.

Human bocavirus and human metapneumovirus in children with lower respiratory tract infections: Effects on clinical, microbiological features and disease severity .

BACKGROUND: We aimed to compare the clinical findings of human bocavirus (HBoV) and human metapneumovirus (HMPV) infections, and to analyze the effects of coinfections on clinical features and disease severity in children with HBoV and HMPV infections. METHODS: Data were collected from 125 children with lower respiratory tract infections due to HBoV or HMPV, detected from nasal swap by real-time polymerase chain reaction (PCR) during the period from January, 2013 to December, 2017. In total, there were 101 HBoV (group 1) and 23 HMPV (group 2) infections in our data. The patients were further divided into four subgroups according to the coinfection status: HoBV only (subgroup 1, n = 41), HMPV only (subgroup 2, n = 19), HBoV and coinfection with other respiratory viruses (subgroup 3, n = 60), and HMPV and coinfection with other respiratory viruses (subgroup 4, n = 4). RESULTS: The majority (88.8%) of the patients were aged 5 years or younger. Coinfections with other respiratory viruses were significantly more common in group 1 (P = 0.001). Among patients who had nosocomial pneumonia, patients with HBoV infections had significantly longer mean length of hospital stay (LOS) than those with HMPV infections (P = 0.032). The hospitalization and antibiotic requirements were significantly higher in subgroup 1 than subgroup 3 (P = 0.005, 0.039, resp.) According to the logistic regression analyses, the LOS increased by 21.7 times with HBoV infections (P = 0.006). CONCLUSIONS: Human bocavirus and HMPV infections are serious pathogens mostly seen in children and usually requiring hospitalization regardless of co-infection status. The HBoV infections caused longer LOS than the HMPV infections in patients with nosocomial infections.

The effect of Pseudomonas aeruginosa eradication regimens on chronic colonization and clinical outcomes in pediatric patients with cystic fibrosis.

BACKGROUND: Chronic Pseudomonas aeruginosa colonization (Pa-CC) affects cystic fibrosis (CF) progression, including pulmonary exacerbations and pulmonary function tests. There are few studies of the effects of eradication protocols on colonization time. Here, we aimed to evaluate the effect of eradication regimens on chronic colonization and assess the impact of Pa-CC on body mass index, lung functions, and pulmonary exacerbations. METHODS: A retrospective review was conducted of medical records, over a period of 11 years, of children aged under 18 years with CF who had Pa-CC in our tertiary care pediatric hospital. RESULTS: Pseudomonas aeruginosa was detected in 215 of our patients with CF during the study period. Forty-four patients with Pa-CC were recruited for the study. The eradication treatment for the initial acquisition of P. aeruginosa was inhaled antibiotics in 27 (61.4%) patients; the remainder were given intravenous antibiotics. It was observed that eradication treatment with either IV or inhaled antibiotics did not affect the time between the P. aeruginosa and the time of Pa-CC(P = 0.791). There was a non-significant decrease in the body mass index z-score from the Pa-IA to the last visit(P = 0.27), a significant decline in forced expiratory volume in 1 s (FEV1%) (P = 0.01) over time, and the annual number of exacerbations after colonization was significantly higher than before colonization (P = 0.03). CONCLUSIONS: There was no difference between eradication regimens in delaying the age at Pa-CC. Pseudomonas aeruginosa colonization in patients with CF was also associated with poorer lung functions, lower body mass index, and more pulmonary exacerbation regardless of mucoid type. Consequently, to slow the progression of lung disease, we must prevent Pa-CC, which we can achieve with early eradication. Despite conventional eradication protocols, future studies need to evaluate those who fail to clear P. aeruginosa.

Evaluation of sleep-disordered breathing and its relationship with respiratory parameters in children with mucopolysaccharidosis Type IVA and VI.

The aims of the study were to evaluate the prevalence of sleep-disordered breathing (SDB) by using polysomnography (PSG) in children with MPS IVA and MPS VI who underwent enzyme replacement therapy (ERT) and to analyze the effect on SDB of having upper airway surgery, pulmonary functions, and exercise capacity. A retrospective cross-sectional study was conducted on patients with MPS IVA (n:17) and MPS VI (n:11) aged under 19 years who underwent polysomnography. Descriptive and nonparametric analyses were performed for demographic, PSG, pulmonary function and exercise capacity variables. The frequency of sleep apnea in the study sample was 85.7% (24/28). Four patients (14.3%) had no sleep apnea, 15 (53.6%) had mild, and nine (32.1%) had moderate-to-severe sleep apnea. Two patients (7.1%) had central sleep apnea and 22 had obstructive sleep apnea (OSA) (78.6%). Forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were negatively correlated to apnea-hypopnea index (AHI) (r = -0.594, p = .009; r = -0.636, p = .005, respectively). Despite ERT and previous upper airway surgery, the prevalence of OSA was high in patients with MPS IVA-MPS IV, emphasizing the importance of PSG screening for sleep disorders. Pulmonary function tests may be useful for predicting sleep apnea in patients with MPS IVA and MPS VI.

Does cystic fibrosis make susceptible to celiac disease?

Patients with cystic fibrosis (CF) have a higher incidence of celiac disease (CD) than the healthy population; however, the actual incidence of coexisting CF and CD is unclear. In this report, we aimed to evaluate the frequency of CD and CF coexistence and to assess the clinical findings of affected patients during follow-up. We conducted a retrospective review of patients with CF to reveal the frequency of CD and also investigated the clinical characteristics and clinical response to gluten-free diet in patients with CD. The incidence of CD in 515 patients with CF was 1.4%. The median age at the time of CF diagnosis was 2 months (1-6 months). CD was diagnosed in six patients with poor weight gain, fatty stools, and low z score for BMI and one patient with poor weight gain despite a high protein and calorie diet and pancreatic enzyme replacement. The median age of CD diagnosis was 8 years (2-12 years). Except for one patient who was recently diagnosed, the other six patients gained weight and their accompanying symptoms resolved after starting a gluten-free diet.Conclusion: CD should be investigated in patients with CF in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment. What is Known: • CFTR dysfunction may be a risk factor for CD, due to increased intestinal permeability and intestinal inflammation, pancreatic exocrine insufficiency that results in higher antigen load and increased antibodies against to nutritional antigens such as anti-gliadin IgA antibodies. • Although coexistence of CF and CD are rare in the same patient; there is still no consensus on when children with CF should be screened for CD. What is New: • Physicians should consider the investigation of CD in patients with CF, in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment. • CFTR dysfunction has been emphasized to develop susceptibility to CD, and patients with CF who have persistent gastrointestinal symptoms despite appropriate and adequate nutritional and enzyme replacement treatment should be screened for CD.

The frequency and related factors of non-tuberculosis mycobacteria infections among patients with cystic fibrosis.

BACKGROUND: Non-tuberculous mycobacteria (NTM) can cause chronic lung infection particularly in patients who have structural lung disease such as cystic fibrosis (CF). We evaluated the incidence and management of NTM infections in patients with CF in our center. METHODS: A retrospective cohort study was carried out on CF patients having at least one positive NTM isolate between 2012 and 2020. RESULTS: Ten patients (2.1%) had at least one positive NTM culture from respiratory samples. All of them were vaccinated with Bacille Calmette-Guérin (BCG) vaccine, which is in the national vaccination program in our country. Eight patients had the Mycobacterium abscessus complex, one had Mycobacterium avium, and one had Mycobacterium szulgai growth in their respiratory samples. Three patients had transient NTM infection, two had persistent, and five had active NTM infection (NTM pulmonary disease). Patients with NTM pulmonary disease received antibiogram-directed antimycobacterial therapy. In patients with NTM pulmonary disease, the median ppFEV1 and BMI decreased by 17% and 1%, respectively, at the time of the first NTM isolation when compared with the values one year before the first NTM isolation. Culture conversion was not seen in any patient infected with Mycobacteriunm abscessus complex. CONCLUSIONS: The NTM infection incidence is lower in our country than in those countries where the BCG vaccine is not routinely applied. The BCG vaccine may be a protective factor for NTM infection. Further studies are needed about the prevalence of NTM infections, facilitating and protective factors, and appropriate management of NTM infections in patients with CF.

Fibrotic lung diseases in children.

In children, pulmonary fibrosis (PF) is an extremely unusual entity that can be observed in some types of interstitial lung disease (ILD). Defining whether ILD is accompanied by PF is important for targeted therapy. Algorithm for the diagnosis of PF in children is not clearly established. Besides, the clinical, radiological, and histological definitions commonly used to diagnose particularly the cases of idiopathic PF in adult patients, is not applicable to pediatric cases. However, a few studies conducted in children offer good exemplary diagnostic approach to fibrosing ILD. Thorax high resonance computed tomography and/or lung biopsy scanning can provide valuable information about PF. Another issue that has not been clearly established is when to start antifibrotic treatment in pediatric patients with PF. The objective of this current review is to provide a comprehensive overview of pediatric PF by drawing upon adult research, particularly focusing on the areas of uncertainty.

The airway microbiota in siblings with primary ciliary dyskinesia: Related factors and correlation with clinical characteristics.

OBJECTIVES-AIM: We aimed to show the composition and structure of and explore affecting factors on airway microbiota in primary ciliary dyskinesia (PCD) patients using culture-independent techniques. METHOD: A cross-sectional observational study was performed. We recruited 14 PCD patients (seven pairs of siblings) and nine parents. Bacterial rDNA was extracted from sputum and nasal samples. Sputum samples were also inoculated on suitable bacteriological media. RESULTS: Thirty-three separate genera were detected in sputum samples of PCD patients, and 41 were in nasal samples of parents. The detected genera were dominated by phyla Proteobacteria in PCD patients and their parents. Culture-dependent analyses could not detect many of the bacterial species detected with culture-independent analyses. There were no significant differences in alpha diversity between the siblings' pairs, and siblings' samples did not cluster together nearly as strongly as nonsiblings' samples. Patients who had no new complaints and no bacterial growth with the culture-dependent method at the time of study and patients who had no Haemophilus influenzae growth in the previous year had a significantly greater diversity (p < .05). Microbiota communities tended to cluster together by age, pulmonary exacerbation status, the existence of at least one H. influenzae growth with culture-dependent analyses in the previous year, and forced expiratory volume in 1 sec z and FEF25-75 z-scores. CONCLUSION: The airway microbiota of patients with PCD have presented more diverse bacterial communities than had been indicated with culture-dependent methods. The study identifies relationships between bacterial airway microbiota composition and the clinical measures of patients. Sibling pairs have no more community similarities than nonsibling PCD patients. Our results may indicate that the patients' clinical characteristics, which determine the disease severity, might affect the PCD microbiome.

Impact of interruption of CFTR modulator therapies.

Novel drug therapy targeting the defective cystic fibrosis transmembrane conductance regulator protein has the potential to significantly enhance the quality of life for numerous patients with cystic fibrosis. However, in some countries social insurance does not pay for modulators because these drugs are extremely expensive. This study sought to understand the impact on the health of children whose modulator treatments were interrupted because of legal procedures and delivery processes. Our study identified that the significant increase in percent-predicted forced expiratory volume levels (FEV1) and BMI z-score values associated with modulator therapies decreased sharply with their discontinuation. Significant worsening in FEV1, BMI z-scores, and BW z-scores were detected in the first follow-up visit after therapy discontinuation within 1 month. Eight patients had a reduction of FEV1 of more than 10%. The findings suggest that modulatory treatment continuation is important, and it is crucial that treatment is not interrupted.

Evaluation of Clinical Findings in Children with Chylothorax: A Descriptive Study.

OBJECTIVE: Chylothorax refers to the presence of chyle in the pleural space. There are multiple etiologies of chylothorax. Our aim in this study was to evaluate the clinical manifestations, causes, and treatment of chylothorax in childhood and also to show the differences between the 2 age groups admitted to a tertiary care children's hospital. The second aim was to evaluate the clinical and radiologic features of patients diagnosed as having Gorham-Stout disease via chylothorax. MATERIALS AND METHODS: The archives were reviewed for chylothorax documented in the last 31 years. Twenty-two patients (11 girls and 11 boys) were included. Patients were divided into 2 groups: the younger group aged under 24 months and the older group aged over 24 months. RESULTS: A total of 22 patients had chylothorax, and 10 were aged younger than 24 months. In the younger group, etiologies were in order congenital heart surgery, congenital chylothorax, and Gorham-Stout disease. In the older group, etiologies were Gorham-Stout disease, congenital heart surgery, heart failure, heart transplantation, thrombus, intestinal lymphangiectasia, and idiopathic. The most common treatment in the younger group was the medium-chain triglyceride diet (70%), and in the older group, it was sirolimus (50%). CONCLUSION: There is a wide variety of underlying etiologies in childhood, so a multidisciplinary approach is important to identify the underlying diagnosis. The common etiologies were postoperative and Gorham-Stout disease in our study. All patients with Gorham-Stout disease had a good prognosis. Gorham-Stout disease should be considered in patients of any age with a diagnosis of chylothorax who have bone lesions.

Plastic bronchitis during childhood: Diversity of presentation, etiology, treatment, and outcomes.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease in children, and reliable data are scarce. Here, we aimed to analyze the clinical features, management, and outcomes in children with PB. METHODS: The medical data of patients who were followed up with a diagnosis of PB between January 2010 and March 2022 were retrospectively analyzed. RESULTS: The median age of 15 patients was 9 (interquartile range: 4-10) years with a male/female ratio of 12/3. Initial symptoms included recurrent pneumonia (33.3%), persistent atelectasis (33.3%), cast expectoration (26.6%), and intense, persistent cough (6.6%). The most common underlying diagnosis was asthma (n = 12, 80%), and six of the patients were newly diagnosed. The most common radiological findings were atelectasis as a consequence of major airway obstruction on chest X-ray or computed tomography. Five patients, all diagnosed as having asthma, had recurrent PB and required multiple airway procedures for treatment and diagnosis. During a median 7-year follow-up of five patients, occasionally cast expectoration was observed in one patient with asthma who had poor compliance with inhaled corticosteroids. CONCLUSION: PB is a common reflection of the different underlying etiologies in the pediatric age group, and treatment and outcomes are closely related to these. It should be kept in mind that asthma can be a predisposing factor for the development of PB.

A case-control study of the effects of Aspergillus clinical phenotypes on pulmonary functions in patients with cystic fibrosis.

INTRODUCTION: There are no precise data about the effect of Aspergillus infection on lung function other than allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (pwCF). Here, we aimed to determine clinical phenotypes caused by Aspergillus spp. using laboratory and immunologic parameters and to compare Aspergillus phenotypes in terms of pulmonary function tests (PFT) prospectively. METHODS: Twenty-three pwCF who had Aspergillus isolation from respiratory cultures in the last year (case group) and 20 pwCF without Aspergillus isolation in sputum (control group) were included. Aspergillus immunoglobulin (Ig)-G, Aspergillus IgE, Aspergillus polymerase chain reaction (PCR), galactomannan, total IgE from blood samples, and Aspergillus PCR and galactomannan from sputum, and skin prick test reactivity to Aspergillus antigen were used to distinguish different Aspergillus phenotypes. Pulmonary functions and frequency of pulmonary exacerbations were evaluated during a 1-year follow-up. RESULTS: Of 23 pwCF, 11 (47.8%) had Aspergillus colonization, nine (39.1%) had Aspergillus bronchitis, and three (13%) had ABPA. Aspergillus infection was not associated with worse z-scores of forced expiratory volume in the first second (FEV1) (p = 0.612), forced vital capacity  (p = 0.939), and the median FEV 1% decline (0.0%/year vs. -4.7%/year, p = 0.626). The frequency of pulmonary exacerbations in the Aspergillus infected and noninfected groups was similar. CONCLUSION: Although Aspergillus spp. Isolation in pwCF was not associated with decreased lung function, a further decline was seen in the ABPA subgroup, and frequent pulmonary exacerbations during the 1-year follow-up.

The Importance of Flexible Bronchoscopy in Difficult-to-treat Asthma from a Pediatric Pulmonology Perspective.

OBJECTIVE: Asthma is the most common chronic lung disease in childhood. Difficult-to-treat asthma is defined as the continuation of symptoms or attacks of patients despite step 4 or 5 of Global Initiative for Asthma therapy. In the differential diagnosis of these patients, flexible fiberoptic bronchoscopy is recommended to exclude other lung diseases. In this study, we aimed to examine the clinical and radiologic features and flexible fiberoptic bronchoscopy findings of patients referred to our pediatric pulmonology department due to difficult-to-treat asthma and determine the effects of flexible fiberoptic bronchoscopy on the differential diagnosis and treatment. MATERIALS AND METHODS: The demographic characteristics and flexible fiberoptic bronchoscopy results of 62 patients who were diagnosed as having difficult-to-treat asthma in our pediatric pulmonology department between January 2015 and June 2020 were evaluated retrospectively. The symptoms, history, medications, physical examination findings, pulmonary function tests, and radiologic findings of patients who underwent flexible fiberoptic bronchoscopy were evaluated. RESULTS: The median age of the patients was 69 (interquartile range: 42-108 months). The most common reasons for the referral of these patients were chronic cough, recurrent pulmonary infections, and persistent wheezing. All patients had chest radiography and 37 (59.7%) had chest computed tomography at their first admission; 14 (37.8%) patients had abnormal findings on chest computed tomography. There was no significant difference in terms of age, physical examination findings, pulmonary function test results, and radiologic examinations between patients with and without pathologic bronchoscopy findings. None of the patients had complications during and after flexible fiberoptic bronchoscopy. The most common diagnoses of patients based on flexible fiberoptic bronchoscopy were persistent bacterial bronchitis in 19 (30.6%) patients, tracheomalacia and/or bronchomalacia in 12 (19.4%), and anatomic anomalies in 3 (4.8%) patients (separation of lingula into 3, separation of right upper lobe bronchus into 4, and tracheal dyskinesia). Mycobacterium tuberculosis growth was observed in the tuberculosis culture of 1 patient. According to the flexible fiberoptic bronchoscopy and bronchoalveolar lavage results, antituberculosis treatment was initiated in 1 patient and polypoid mass excision was performed in 1 patient. A proton pump inhibitor was started in 9 (15.5%) patients, physiotherapy in 5 (8.0%), antibiotics in 14 (22.5%), and ipratropium bromide in 7 (11.2%) patients. All patients were followed up with the diagnosis of asthma except for 2 patients. CONCLUSION: To date, there is no prospective study evaluating the importance of flexible fiberoptic bronchoscopy in difficult-to-treat asthma in childhood. In our small cohort, persistent bacterial bronchitis, airway tracheomalacia and/or bronchomalacia, gastroesophageal reflux, and other anatomic anomalies were successfully diagnosed using flexible fiberoptic bronchoscopy and treated without any complications, suggesting that flexible fiberoptic bronchoscopy is an important diagnostic tool with a low complication rate in children with difficult-to-treat asthma.

Telephone surveillance during the COVID-19 pandemic: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?

INTRODUCTION: The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease. METHODS: A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients. RESULTS: Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised. DISCUSSION: Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.

Comparison of inhaled mannitol/dornase alfa combination and daily dornase alfa alone in children with cystic fibrosis.

OBJECTIVES: Inhaled recombinant human deoxyribonuclease (dornase alfa) and osmotic agents such as inhaled mannitol are used for improving the clearance of secretions of cystic fibrosis (CF) patients. We aimed to evaluate the long-term clinical effects of adding dry powder inhaled (DPI) mannitol in subjects with CF who are taking daily dornase alfa. METHOD: We conducted a retrospective case-control study on subjects with CF. The effect of DPI mannitol was assessed by comparing DPI mannitol and dornase alfa combination with daily dornase alfa alone in children with CF during a 12-month period. The primary outcome measures of the study were absolute changes in percent predicted forced expiratory volume in 1 s (FEV1) and FEV1 z-scores and the secondary outcomes included other spirometry indices, body mass index, frequency of pulmonary exacerbations, SPO2 , and sputum microbiology. RESULT: Of a total of 28 patients who committed to use DPI mannitol treatments during the study period, five had a positive challenge with DPI mannitol and two were aged over 18 years. Therefore, the mannitol treatment group consisted of 21 patients. However, the effect of DPI mannitol was analyzed using 15 patients in the mannitol treatment group who received DPI mannitol for at least 12 months, and 18 patients who only used dornase alfa constituted the control group. The median absolute change in FEV1 between baseline and the third month; and baseline and the 12th month were significantly higher in the mannitol treatment group (p = 0.038, p = 0.004, respectively). When the groups are compared with respect to absolute z-score changes, all spirometry indices, except FVC at the end of 3 months, showed statistically significant improvements in the mannitol treatment group. Some secondary outcomes like pulmonary exacerbation frequency during the study year and median absolute body mass index z-score changes from baseline to the end of the study showed no significant differences between the groups (p = 0.735, p = 0.161, respectively). No colonization changes were observed in the treatment group. CONCLUSIONS: This study showed that in those patients who tolerated long-term (12 months) treatment with DPI mannitol and dornase alfa made greater improvements in FEV1, FVC, FEV1/FVC, FEF25-75 z-scores than treatment with dornase alfa alone in children with CF.

Clinical radiological and pathological staging of children with hypersensitivity pneumonitis.

OBJECTIVE: Hypersensitivity pneumonitis (HP) is rare in the pediatric population. To date, there are no studies defining a correlation between clinical, radiological, and pathological findings in children with HP. The objective of this study is to define the clinical, and radiological characteristics and prognosis of childhood HP and to examine the clinical, radiological, and pathological correlation between HP stages. METHODS: Patients with suspected HP and followed at two tertiary care hospitals between 2000 and 2020 were retrospectively evaluated. Computed tomography (CT) of the chest of patients was evaluated by a single radiologist. The interagreement between clinical and radiological severity of the patients was calculated with the κ test. RESULTS: Fourteen children with suspected HP were identified. The results of 10 patients with the definitive diagnosis were as follows: one patient (10%) had acute, five patients (50%) had subacute, and four patients (40%) had chronic HP. The most prominent findings in chest CT were hilar, or hilar and subcarinal lymphadenopathy (80%), centrilobular nodules (60%), patchy or diffuse ground-glass opacities (50%), and cysts (50%). The interagreement between clinical and radiological severity of the patients was 100% (approximate significance: 0.003). The diagnosis of four patients with suspected HP who were unresponsive to standardized medical treatments or developed multisystem involvement was diagnosed with other diseases. One patient (10%) with definitive chronic HP died due to respiratory failure during follow up. CONCLUSION: Similar to adult HP, the prognosis is worse in children with existing fibrotic equivalents in chest CT. Patients who are not responding to standard medical treatments or develop multisystem involvement should be evaluated for other lung diseases.

Antimycobacterial prophylaxis regarding Bacillus Calmette-Guérin -associated complications in children with primary immunodeficiency.

OBJECTIVE: Bacillus Calmette-Guérin (BCG) vaccine derived from Mycobacterium bovis can cause BCG vaccine associated complications (BCG-VAC) especially in patients with primary immunodeficiencies (PID). No consensus exists for antimycobacterial prophylactic therapy for patients with PID who receive the BCG vaccine. AIM: This study aimed to define the risk factors in the development of BCG-VAC and effect of antimycobacterial prophylaxis in PID patients vaccinated with BCG. METHODS: This is a retrospective cohort study. 104 patients diagnosed with PID who received the BCG vaccination were referred to pediatric pulmonology in a single center were enrolled. The demographic characteristics, type, dosage and duration of antimycobacterial prophylaxis regimen, treatment modalities for BCG-VAC were documented. Regression analysis was performed to evaluate the effect of covariates for predicting BCG-VAC in patients with PIDs. RESULTS: Among 104 patients 21 (21.2%) developed BCG-VAC. The frequency of BCG-VAC was highest in patients with Mendelian susceptibility to mycobacterial disease (46.2%) followed by patients with severe combined immunodeficiency (22.4%) and those with chronic granulomatous disease (9.5%). Prophylactic therapy against mycobacterium was initiated for 72 patients (69.2%). Among patients who received the antimycobacterial prophylaxis, BCG-VAC developed in only four patients (5.6%), whereas 17 patients (53.1%) developed BCG-VAC in the non-prophylaxis group and this difference was statistically significant (p < 0.001). Multivariable regression analysis with age at diagnosis, type of PID, receiving antimycobacterial prophylaxis, median T cell number at the time of PID diagnosis and HSCT status showed that not receiving antimycobacterial prophylaxis and lower median T cell number were predictors, with antimycobacterial prophylaxis having the highest odds ratio for BCG-VAC prediction in patients with PIDs (p:<0.001, R2:0.64). CONCLUSION: The lower frequency of BCG-VAC in our cohort can be explained by two main reasons; relatively late BCG vaccination schedule and receiving antimycobacterial prophylaxis. It is reasonable to begin antimycobacterial prophylaxis in patients with PIDs who are susceptible to BCG-VAC.