Browsing by Author "Sovtic, Aleksandar (16234625700)"

Escherichia coli is a Gram-negative bacterium and part of the intestinal microbiota. However, it can cause various diarrhoeal illnesses, i.e., traveller’s diarrhoea, dysentery, and extraintestinal infections when the bacteria are translocated from the intestine to other organs, such as urinary tract infections, abdominal and pelvic infections, pneumonia, bacteraemia, and meningitis. It is also an important pathogen in intensive care units where cross-infection may cause intrahospital spread with serious consequences. Within this study, four E. coli isolates from the tracheal aspirates of a tracheotomised paediatric patient on chronic respiratory support were analysed and compared for antibiotic resistance and virulence potential. Genomes of all four isolates (5381a, 5381b, 5681, 5848) were sequenced using Oxford Nanopore Technology. According to PFGE analysis, the clones of isolates 5681 and 5848 were highly similar, and differ from 5381a and 5381b which were isolated first chronologically. All four E. coli isolates belonged to an unknown sequence type, related to the E. coli ST131, a pandemic clone that is evolving rapidly with increasing levels of antimicrobial resistance. All four E. coli isolates in this study exhibited a multidrug-resistant phenotype as, according to MIC data, they were resistant to ceftriaxone, ciprofloxacin, doxycycline, minocycline, and tetracycline. In addition, principal component analyses revealed that isolates 5681 and 5848, which were recovered later than 5381a and 5381b (two weeks and three weeks, respectively) possessed more complex antibiotic resistance genes and virulence profiles, which is concerning considering the short time period during which the strains were isolated. © 2024 by the authors.

Escherichia coli is a Gram-negative bacterium and part of the intestinal microbiota. However, it can cause various diarrhoeal illnesses, i.e., traveller’s diarrhoea, dysentery, and extraintestinal infections when the bacteria are translocated from the intestine to other organs, such as urinary tract infections, abdominal and pelvic infections, pneumonia, bacteraemia, and meningitis. It is also an important pathogen in intensive care units where cross-infection may cause intrahospital spread with serious consequences. Within this study, four E. coli isolates from the tracheal aspirates of a tracheotomised paediatric patient on chronic respiratory support were analysed and compared for antibiotic resistance and virulence potential. Genomes of all four isolates (5381a, 5381b, 5681, 5848) were sequenced using Oxford Nanopore Technology. According to PFGE analysis, the clones of isolates 5681 and 5848 were highly similar, and differ from 5381a and 5381b which were isolated first chronologically. All four E. coli isolates belonged to an unknown sequence type, related to the E. coli ST131, a pandemic clone that is evolving rapidly with increasing levels of antimicrobial resistance. All four E. coli isolates in this study exhibited a multidrug-resistant phenotype as, according to MIC data, they were resistant to ceftriaxone, ciprofloxacin, doxycycline, minocycline, and tetracycline. In addition, principal component analyses revealed that isolates 5681 and 5848, which were recovered later than 5381a and 5381b (two weeks and three weeks, respectively) possessed more complex antibiotic resistance genes and virulence profiles, which is concerning considering the short time period during which the strains were isolated. © 2024 by the authors.

Patients with cystic fibrosis (CF) are repeatedly exposed to antibiotics, especially during the pulmonary exacerbations of the disease. However, the available therapeutic strategies are frequently inadequate to eradicate the involved pathogens and most importantly, facilitate the development of antimicrobial resistance (AMR). The evaluation of AMR is demanding; conventional culture-based susceptibility-testing techniques cannot account for the lung microenvironment and/or the adaptive mechanisms developed by the pathogens, such as biofilm formation. Moreover, features linked to modified pharmaco-kinetics and pulmonary parenchyma penetration make the dosing of antibiotics even more challenging. In this review, we present the existing knowledge regarding AMR in CF, we shortly review the existing therapeutic strategies, and we discuss the future directions of antimicrobial stewardship. Due to the increasing difficulty in eradicating strains that develop AMR, the appropriate management should rely on targeting the underlying resistance mechanisms; thus, the interest in novel, molecular-based diagnostic tools, such as metagenomic sequencing and next-generation transcriptomics, has increased exponentially. Moreover, since the development of new antibiotics has a slow pace, the design of effective treatment strategies to eradicate persistent infections represents an urgency that requires consorted work. In this regard, both the management and monitoring of antibiotics usage are obligatory and more relevant than ever. © 2023 by the authors.

Patients with cystic fibrosis (CF) are repeatedly exposed to antibiotics, especially during the pulmonary exacerbations of the disease. However, the available therapeutic strategies are frequently inadequate to eradicate the involved pathogens and most importantly, facilitate the development of antimicrobial resistance (AMR). The evaluation of AMR is demanding; conventional culture-based susceptibility-testing techniques cannot account for the lung microenvironment and/or the adaptive mechanisms developed by the pathogens, such as biofilm formation. Moreover, features linked to modified pharmaco-kinetics and pulmonary parenchyma penetration make the dosing of antibiotics even more challenging. In this review, we present the existing knowledge regarding AMR in CF, we shortly review the existing therapeutic strategies, and we discuss the future directions of antimicrobial stewardship. Due to the increasing difficulty in eradicating strains that develop AMR, the appropriate management should rely on targeting the underlying resistance mechanisms; thus, the interest in novel, molecular-based diagnostic tools, such as metagenomic sequencing and next-generation transcriptomics, has increased exponentially. Moreover, since the development of new antibiotics has a slow pace, the design of effective treatment strategies to eradicate persistent infections represents an urgency that requires consorted work. In this regard, both the management and monitoring of antibiotics usage are obligatory and more relevant than ever. © 2023 by the authors.

Rationale: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease is unknown. Objectives: To determine the prognostic value of CPET on the risk of death or lung transplant (LTX) within 2 years. Methods: We retrospectively collected data from 20 CF centers in Asia, Australia, Europe, and North America on patients with a forced expiratory volume in 1 second (FEV1) < 40% predicted who performed a cycle ergometer CPET between January 2008 and December 2017. Time to death/LTX was analyzed using mixed Cox proportional hazards regression. Conditional inference trees were modeled to identify subgroups with increased risk of death/LTX. Results: In total, 174 patients (FEV1, 30.9% 6 5.8% predicted) were included. Forty-four patients (25.5%) died or underwent LTX. Cox regression analysis adjusted for age, sex, and FEV1 revealed percentage predicted peak oxygen uptake (V_ O2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional 10% predicted were 0.60 (95% confidence interval, 0.43-0.90; P = 0.008) and 0.60 (0.48-0.82; P, 0.001). Tree-structured regression models, including a set of 11 prognostic factors for survival, identified Wpeak to be most strongly associated with 2-year risk of death/LTX. Probability of death/LTX was 45.2% for those with a Wpeak < 49.2% predicted versus 10.9% for those with a Wpeak. 49.2% predicted (P, 0.001). Conclusions: CPET provides prognostic information in advanced CF lung disease, and Wpeak appears to be a promising marker for LTX referral and candidate selection. Copyright © 2024 by the American Thoracic Society.

Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with cystic fibrosis may be an important determinant of maximal exercise capacity in cystic fibrosis. Previous studies on the relationship between CFTR genotype and maximal exercise capacity are scarce and contradictory. Objectives: This study was designed to explore factors influencing maximal exercise capacity, expressed as peak oxygen uptake (VO 2peak ), with a specific focus on CFTR genotype in children and adults with cystic fibrosis. Methods: In an international, multicenter, cross-sectional study, we collected data on CFTR genotype and cardiopulmonary exercise tests in patients with cystic fibrosis who were ages 8 years and older. CFTR mutations were classified into functional classes I-V. Results: The final analysis included 726 patients (45% females; age range, 8-61 yr; forced expiratory volume in 1 s, 16 to 123% predicted) from 17 cystic fibrosis centers in North America, Europe, Australia, and Asia, all of whom had both valid maximal cardiopulmonary exercise tests and complete CFTR genotype data. Overall, patients exhibited exercise intolerance (V O2peak , 77.3 6 19.1% predicted), but values were comparable among different CFTR classes. We did not detect an association between CFTR genotype functional classes I-III and either VO 2peak (percent predicted) (adjusted b = 20.95; 95% CI, 24.18 to 2.29; P = 0.57) or maximum work rate (Watt max ) (adjusted β = 21.38; 95% CI, 25.04 to 2.27; P = 0.46) compared with classes IV-V. Those with at least one copy of a F508del-CFTR mutation and one copy of a class V mutation had a significantly lower V O2peak (β = 28.24%; 95% CI, 214.53 to 22.99; P = 0.003) and lower Watt max (adjusted β = 27.59%; 95% CI, 214.21 to 20.95; P = 0.025) than those with two copies of a class II mutation. On the basis of linear regression analysis adjusted for relevant confounders, lung function and body mass index were associated with VO 2peak . Conclusions: CFTR functional genotype class was not associated with maximal exercise capacity in patients with cystic fibrosis overall, but those with at least one copy of a F508del-CFTR mutation and a single class V mutation had lower maximal exercise capacity. © Copyright 2018 by the American Thoracic Society.

Aims: We evaluated the impact of cystic fibrosis-related diabetes (CFRD) on lung disease and nutritional status. Study Design: The retrospective cohort study evaluated the subjects' medical records from 2004 to 2019. All participants older than 10 years diagnosed by a 30-minutely sampled OGTT formed OGTT-CFRD subgroup. The participants diagnosed with continuous glucose monitoring (CGM) (at least two peaks above 11.1 mmol/l and more than 10% of recorded time above 7.8 mmol/l) formed a CFRD-CGM subgroup. The participants without CFRD formed a non-CFRD group. The longitudinal follow-up was made 2 years before and 3 years after insulin therapy initiation. Results: Of 144 participants included, aged 10–55 years (44% males), 28 (19.4%) had CFRD. The HbA1c was significantly lower in the CGM-CFRD in comparison to the OGTT-CFRD subgroup (5.9 ± 0.62 and 7.3 ± 1.7% respectfully; p = 0.04). Subjects with CFRD were malnourished in comparison to non-CFRD, with significant improvements with insulin replacement therapy in regard to BMI Z-score (−1.4 ± 1.3 vs. −0.5 ± 1.2%, p = 0.04) and pulmonary exacerbation score (p = 0.02). In OGTT-CFRD subgroup there is an increase in FEV1 (62.7 ± 26.3 to 65.1 ± 21.7%, p = 0.7) and decrease in FVC (from 76.4 ± 24.2 to 71.2 ± 20%, p = 0.003) from diagnosis to second year of follow-up. In CGM-CFRD subgroup there was a decrease in FEV1 (from 58.2 ± 28.2 to 52.8 ± 25.9%, p = 0.2) and FVC-values (from 72.4 ± 26.5 to 67.4 ± 29.1%, p = 0.08).Chronic Pseudomonas aeruginosa infection was more prevalent in the CFRD group (p = 0.003). Conclusion: Continuous glucose monitoring is a useful tool for insight of glucose impairment and diagnosis of CFRD. Early recognition of CFRD and therapeutic intervention has favorable effects on clinical course of the disease. © Copyright © 2021 Gojsina, Minic, Todorovic, Soldatovic and Sovtic.

Rationale: Long-life ventilatory assistance is necessary for survival in pediatric patients with congenital central hypoventilation syndrome (CCHS). Invasive mechanical ventilation (IMV) through tracheostomy is the most used method, especially in the first years of life when the central nervous system is maturing. Nevertheless, IMV via tracheostomy is not ideal because tracheostomy in children is associated with impaired speech and language development, also frequent infections of the lower airway tract occur. Objective: Only few reports describe the transition from IMV to the noninvasive method, ending with decannulation in CCHS affected patients. We aim to provide our experience regarding decannulation program in CCHS affected children and to describe a proposal of an algorithm concerning transition from invasive to noninvasive ventilation (NIV) in CCHS patients. Methods: The study has been conducted retrospectively. Four children from two European centers underwent tracheostomy removal and decannulation, upon request of patients and their families. Results: All children were trained to carry out tracheostomy capping before decannulation and underwent endoscopic assessment of upper and lower airway. Subsequently they started training to NIV at mean age of 106.25 months (±40.7 SD). Decannulation occurred 12 months after and no patients needed the reintroduction of tracheal cannula in either short or long term follow up. Conclusions: our study shows that effective liberation from IMV, the transition to NIV and decannulation are possible in CCHS affected children and offers a proposal of an algorithm which can be applied in selected centers. © 2019 Wiley Periodicals, Inc.

The aim of the study was to compare the effects of high-intensity interval training (HIIT) and nutrition advice on cardiometabolic biomarkers, hormonal parameters, and cardiorespiratory fitness in adolescent girls with obesity. Adolescent girls with obesity (n = 44, aged 13–19 years) were randomized into a 12-week intervention as follows: (i) dietary advice and HIIT (n = 22), and (ii) dietary advice only (n = 22). The concentration of biomarkers of inflammation, biochemical and hormonal testing, oral glucose tolerance test, cardiorespiratory fitness, physical activity levels, and nutrition were assessed. After a 3-month intervention, the diet+HIIT group significantly increased insulin sensitivity index (–0.34 ± 1.52 vs. 1.05 ± 3.21; p = 0.001) and work load (0.6 ± 11.3 W vs. 14.6 ± 20.2 W; p = 0.024) and decreased glucose area under the curve (–0.29 ± 4.69 vs. −0.98 ± 4.06; p = 0.040), insulin area under the curve (−9.65 ± 117.9 vs. −98.7 ± 201.8; p = 0.003), and high-sensitivity C-reactive protein (hs-CRP) (0.12 ± 1.92 mg/L vs. −1.47 ± 3.67 mg/L; p = 0.039) in comparison with the diet group. Regarding within-group changes, both groups had significant improvements in body mass index (BMI), BMI-standard deviation score, body fat percentage, and systolic blood pressure. Positive impact on waist circumference, waist circumference/height ratio, diastolic blood pressure, hs-CRP, work load, maximal heart rate, and resting heart rate was observed only after the diet+HIIT intervention. No significant change was noted in peak oxygen uptake, lipid profile, and hormonal parameters between groups after intervention. Novelty • HIIT and nutrition advice increased insulin sensitivity and decreased BMI, bodyfat, systolicbloodpressure, and diastolicblood pressure. • Nutrition advice decreased BMI, body fat, and systolic blood pressure in adolescent girls with obesity. © 2020, Canadian Science Publishing. All rights reserved.

The aim of the study was to compare the effects of high-intensity interval training (HIIT) and nutrition advice on cardiometabolic biomarkers, hormonal parameters, and cardiorespiratory fitness in adolescent girls with obesity. Adolescent girls with obesity (n = 44, aged 13–19 years) were randomized into a 12-week intervention as follows: (i) dietary advice and HIIT (n = 22), and (ii) dietary advice only (n = 22). The concentration of biomarkers of inflammation, biochemical and hormonal testing, oral glucose tolerance test, cardiorespiratory fitness, physical activity levels, and nutrition were assessed. After a 3-month intervention, the diet+HIIT group significantly increased insulin sensitivity index (–0.34 ± 1.52 vs. 1.05 ± 3.21; p = 0.001) and work load (0.6 ± 11.3 W vs. 14.6 ± 20.2 W; p = 0.024) and decreased glucose area under the curve (–0.29 ± 4.69 vs. −0.98 ± 4.06; p = 0.040), insulin area under the curve (−9.65 ± 117.9 vs. −98.7 ± 201.8; p = 0.003), and high-sensitivity C-reactive protein (hs-CRP) (0.12 ± 1.92 mg/L vs. −1.47 ± 3.67 mg/L; p = 0.039) in comparison with the diet group. Regarding within-group changes, both groups had significant improvements in body mass index (BMI), BMI-standard deviation score, body fat percentage, and systolic blood pressure. Positive impact on waist circumference, waist circumference/height ratio, diastolic blood pressure, hs-CRP, work load, maximal heart rate, and resting heart rate was observed only after the diet+HIIT intervention. No significant change was noted in peak oxygen uptake, lipid profile, and hormonal parameters between groups after intervention. Novelty • HIIT and nutrition advice increased insulin sensitivity and decreased BMI, bodyfat, systolicbloodpressure, and diastolicblood pressure. • Nutrition advice decreased BMI, body fat, and systolic blood pressure in adolescent girls with obesity. © 2020, Canadian Science Publishing. All rights reserved.

Background/Objectives: Myotonic dystrophy type 1 (DM1) is a rare neuromuscular disorder characterized by respiratory dysfunction that significantly impacts quality of life and longevity. This study aimed to explore the outcomes of pulmonary function tests and sleep-disordered breathing (SDB) workups in children with DM1 and to identify the factors contributing to SDB. Methods: A retrospective study examined patients’ medical records, including genetic analyses, clinical characteristics, and noninvasive pulmonary function testing (PFT), when possible. The Pediatric Sleep Questionnaire (PSQ), arterial blood gases, polygraphy, and overnight transcutaneous capnometry (PtcCO2) were used to assess SDB. Results: The size of CTG expansion in the DMPK gene directly correlated with the severity of respiratory complications and the need for early tracheostomy tube insertion in 7/20 (35%) patients. A total of 13/20 (65%) children were available for respiratory evaluation during spontaneous breathing. While moderate/severe obstructive sleep apnea syndrome (OSAS) and hypoventilation were confirmed in 4/13 (31%) children, none of the patients had mixed or dominantly central sleep apnea syndrome. There was no correlation between apnea–hypopnea index (AHI) or PtcCO2 and the presence of SDB-related symptoms or the PSQ score. Although a significant correlation between AHI and PtcCO2 was not confirmed (p = 0.447), the oxygen desaturation index directly correlated with PtcCO2 (p = 0.014). Conclusions: While SDB symptoms in children with DM1 may not fully correlate with observed respiratory events or impaired gas exchange during sleep, a comprehensive screening for SDB should be considered for all patients with DM1. Further research into disease-specific recommendations encompassing the standardization of PFT, as well as overnight polygraphic and capnometry recordings, could help to guide timely, personalized treatment. © 2025 by the authors.

Background/Objectives: Myotonic dystrophy type 1 (DM1) is a rare neuromuscular disorder characterized by respiratory dysfunction that significantly impacts quality of life and longevity. This study aimed to explore the outcomes of pulmonary function tests and sleep-disordered breathing (SDB) workups in children with DM1 and to identify the factors contributing to SDB. Methods: A retrospective study examined patients’ medical records, including genetic analyses, clinical characteristics, and noninvasive pulmonary function testing (PFT), when possible. The Pediatric Sleep Questionnaire (PSQ), arterial blood gases, polygraphy, and overnight transcutaneous capnometry (PtcCO2) were used to assess SDB. Results: The size of CTG expansion in the DMPK gene directly correlated with the severity of respiratory complications and the need for early tracheostomy tube insertion in 7/20 (35%) patients. A total of 13/20 (65%) children were available for respiratory evaluation during spontaneous breathing. While moderate/severe obstructive sleep apnea syndrome (OSAS) and hypoventilation were confirmed in 4/13 (31%) children, none of the patients had mixed or dominantly central sleep apnea syndrome. There was no correlation between apnea–hypopnea index (AHI) or PtcCO2 and the presence of SDB-related symptoms or the PSQ score. Although a significant correlation between AHI and PtcCO2 was not confirmed (p = 0.447), the oxygen desaturation index directly correlated with PtcCO2 (p = 0.014). Conclusions: While SDB symptoms in children with DM1 may not fully correlate with observed respiratory events or impaired gas exchange during sleep, a comprehensive screening for SDB should be considered for all patients with DM1. Further research into disease-specific recommendations encompassing the standardization of PFT, as well as overnight polygraphic and capnometry recordings, could help to guide timely, personalized treatment. © 2025 by the authors.

Home mechanical ventilation (HMV) is a method of treatment in children with sleep-disordered breathing (SDB) and alveolar hypoventilation regardless of primary disease. The goal of the study was to describe the changes in the HMV program in Serbia during the last two decades. Cross-sectional retrospective study included data from the national HMV database from 2001 until 2019. HMV was initiated in clinically stable patients after the failure to wean from mechanical ventilation succeeded acute respiratory deterioration or electively after the confirmation of SDB and alveolar hypoventilation by sleep study or continuous transcutaneous capnometry and oximetry. The study included 105 patients (50 ventilated noninvasively and 55 ventilated invasively via tracheostomy). The median age at the time of HMV initiation was 6.2 years (range: 0.3–18 years). Invasive ventilation had been initiated significantly earlier than noninvasive ventilation (NIV) (p < 0.01), without difference in duration of ventilatory support (p = 0.95). Patients on NIV were significantly older (p < 0.01) than those ventilated invasively (13 and 1.5 years, respectively). Average waiting time on equipment had been shortened significantly—from 6.3 months until 2010 to 1 month at the end of the study (p < 0.01). Only 6.6% of patients had obstructive sleep apnea syndrome (OSAS) requiring HMV. During the study period, 24% patients died, mostly due to uncontrolled infection or progression of underlying disease. Availability and shortened waiting time for the equipment accompanied by advanced overall health care led to substantial improvements in the national HMV program. However, future improvements should be directed to systematic evaluation of SDB in patients with OSAS, early diagnosis of nocturnal hypoventilation, and subsequent timely initiation of chronic ventilation. © Copyright © 2020 Basa, Minic, Rodic and Sovtic.

Background and objectives: Persistent wheezing (PW) is defined as prolonged or recurrent episodes of wheezing despite regular treatment. Flexible bronchoscopy (FB) is recommended to determine the etiology of PW in children. This study aimed to determine the etiology of PW based on FB findings in a national pediatric center. Materials and Methods: Children presenting with PW that underwent flexible bronchoscopy from April 2016 to August 2019 at the Mother and Child Health Institute of Serbia were included in this observational study. After endoscopic evaluation, bronchoalveolar lavage fluid (BALF) samples were taken and further analyzed. Quantitative microbiology, cytological analysis and oil-red staining of specimens were performed to determine cellular constituents and presence of lipid laden macrophages (LLM). Upper gastrointestinal series were performed to exclude gastroesophageal reflux disease, swallowing dysfunction and vascular ring. Results: Pathological findings were revealed in 151 of 172 study participants, with bacterial lower airway infection (BLAI) (48.3%) and primary bronchomalacia (20.4%) as the most common. Younger participants were hospitalized for significantly longer periods (ρ = −0.366, p < 0.001). Study participants with BLAI and associated mucus plugging were notably younger (p < 0.001). Presence of LLM in BALF was not associated with findings of upper gastrointestinal series. All patients with confirmed BLAI were treated with oral antibiotics. Although FB is considered to be invasive, there were no complications associated with the procedure. Conclusions: Flexible bronchoscopy has an exceptional diagnostic value in evaluation of PW. In younger patients with BLAI, presence of mucus plugs may complicate the clinical course, so significant benefits can be achieved with therapeutic lavage during bronchoscopy. © 2020 by the authors. Licensee MDPI, Basel, Switzerland.

Background: The results of many national surveys on pediatric home mechanical ventilation (HMV) in developed countries have been presented elsewhere, but data from developing countries with low national incomes are scarce. Methods: Twenty-nine pediatric patients, treated in the Mother and Child Institute of Serbia, who had been receiving long-term ventilatory support at home, were surveyed. The major criterion for initiating HMV was hypercapnia, diagnosed by blood gas analysis, performed in the morning, after awakening. Other criteria were either symptoms of hypoventilation during the night associated with an apnea index of >5, or apnoea-hypopnoea index of >15, or nocturnal hypoxemia, defined as an oxygen saturation rate of <90% for >5% of total sleep time. Results: The mean age at initiation of HMV was 9.3 years (range 0.5-17.8 years). Patients waited for HMV initiation either in hospital or at home; the mean period was 6.3 months (range 1-18 months). The subjects received HMV for a mean of 25.06 months (range 3-119 months). There was a significant difference in the duration of HMV for different underlying diseases (P= 0.046), and mechanical malfunction was strongly dependent on the duration of HMV (P= 0.011). Eleven patients underwent invasive HMV via a tracheostomy, and 18 others received non-invasive ventilation, via nasal and full-face masks. Conclusion: HMV is feasible in developing countries. Valuable reimbursement policies as well as an organized and functional network are essential for its implementation, as a standard of care in leading national pediatric hospitals. © 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.

The modified Chrispin-Norman radiography score (CNS) is used in evaluation of radiographic changes in children with cystic fibrosis (CF). We evaluated the correlation of modified CNS with peak exercise capacity (Wpeak) and ventilatory efficiency (reflected by breathing reserve index-BRI) during progressive cardiopulmonary exercise testing (CPET). Thirty-six children aged 8-17 years were stratified according to their CNS into 3 groups: mild (<10), moderate (10-15), and severe (>15). CPET was performed on a cycle ergometer. Lung function tests included spirometry and whole-body plethysmogr aphy. Patients with higher CNS had lower FEV1 (p < .001), Wpeak predicted (%; p = .01) and lower mean peak oxygen consumption (VO2peak/kg; p = .014). The BRI at the anaerobic threshold and at Wpeak was elevated in patients with the highest CNS values (p < .001). The modified CNS correlates moderately with Wpeak (R = -0.443; p = .007) and BRI (R = -0.419; p = .011). Stepwise multiple linear regression showed that RV/TLC was the best predictor of Wpeak/pred (%; B = -0.165; b = -0.494; R2 = .244; p = .002). Children with CF who have high modified CNS exhibit decreased exercise tolerance and ventilatory inefficacy during progressive effort. © 2014 Human Kinetics, Inc.

Background N-terminal pro-brain natriuretic peptide (NT-proBNP) is used as a biomarker to differentiate congestive heart failure from lung disease in adults and children. The clinical significance of its use in term neonates has not yet been extensively studied. Methods NT-proBNP level was measured in 62 term neonates admitted for respiratory distress (RD): 38 with congenital heart disease (CHD) and 24 with pulmonary disease. The control group consisted of 28 healthy neonates. Findings of auscultation, chest radiography, Silverman-Anderson score and echocardiography were recorded for each patient. Blood samples for measuring NT-proBNP were collected on admission, when blood sampling was indicated for the clinical management of the newborn. Results In the control group NT-proBNP was significantly higher during the first week of life compared to the rest of the neonatal period (P < 0.001). The RD group, regardless of etiology, had significantly higher NT-proBNP than the control group (P < 0.001). Neonates with more severe RD had significantly higher NT-proBNP (P = 0.002). No significant difference was found between the RD group with CHD and those with pulmonary disease. Neonates with CHD and myocardial hypocontractility had significantly higher NT-proBNP than those with normal contractility (P = 0.022). Conclusion Term neonates with RD have significantly higher NT-proBNP than healthy neonates. A single measurement of NT-proBNP level cannot be used as the sole biomarker for distinguishing between cardiac and pulmonary cause of RD in term neonates. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

Generalized lymphatic anomaly (GLA) is a rare congenital disorder of lymphatic development, presenting with multiple lymphatic malformations in different organs and tissues. Here, we present a case of a female neonate prenatally diagnosed with foetal hydrops and a mediastinal cystic lymphatic malformation that showed postnatal expansive and infiltrative growth into the major airways, compromising mechanical ventilation and further management of the neonate. Complications that arose during surgical treatment of mediastinal structures led to the patient’s death. Lymphatic malformations were also noted in the skin at birth. Furthermore, a skin biopsy performed immediately after birth and the autopsy revealed an extremely rare diagnosis of combined macrocystic and microcystic forms of GLA with skin involvement. Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology

Introduction: Decreased respiratory muscle strength in patients with cystic fibrosis (CF) may cause progressive exercise intolerance during cardiopulmonary exercise testing (CPET), and may contribute to the development of chronic respiratory insufficiency. The aim of this study is to evaluate exercise tolerance during CPET of children and adults with clinically stable CF who exhibit different respiratory muscle strength. Methods: Sixty-nine clinically stable CF subjects aged 8-33 years underwent spirometry, body plethysmography, CPET, and respiratory muscle strength measurement. Respiratory muscle strength was measured using maximal inspiratory pressures (Pimax) and maximal expiratory pressures (Pemax). Participants were stratified into three groups according to Pimax values:below normal (≤80% predicted), normal (81-100% predicted), and above normal ( > 100% predicted). A similar stratification of participants was made according to Pemax values. The oxygen consumption on peak load (VO2peak) was expressed relative to BM (VO2peak/kg), relative to BM raised by the exponent of 0.67 (VO2peak/kg0.67) and as log-linear adjustment of VO2peak (VO2peak/kg-alo). Results: Participants with low Pemax values had a lower mean maximum load per kilogram/predicted (Wmax; p = 0.001) VO2peak/kg (p = 0.006), VO2peak/kg0.67 (p = 0.038) and VO2peak/kg-alo (p = 0.001). There were no significant differences in exercise tolerance parameters with regard to Pimax values. Stepwise multiple linear regressions confirmed that Pemax (B = 24.88, β = 0.48, p < 0.001) was the most powerful predictor of Wmax. There were no statistically significant differences in age, lung function parameters, exacerbation score, or respiratory muscle strength according to gender. Conclusions: In subjects with clinically stable CF, expiratory muscle strength is associated with a decrease in exercise performance during CPET and can predict exercise intolerance. Increase in expiratory muscle strength by patient specific rehabilitation protocols would result in improvement of exercise tolerance. © 2018 Sovtic, Minic, Markovic-Sovtic and Trajkovic.

This retrospective study aimed to investigate the clinical features and treatment of pediatric subglottic hemangioma (SH), identify risk factors for treatment-induced adverse effects, and identify a strategy for timely therapy discontinuation in children diagnosed with SH at the national pediatric center. Medical records of patients presented with stridor from 2010 to 2020 were retrieved and assessed, the diagnosis of SH was established via flexible bronchoscopy, and the patients were treated using propranolol with a subsequent gradual dose increase to 3 mg/kg body weight daily. A two-week oral steroids trial was added for those with circumferential lesions. Early indicators of a good therapeutic response included decreased stridor and primary lesion size on follow-up bronchoscopy performed one week after propranolol commencement. Duration of therapy, tailored individually based on bronchoscopy findings, and at least twelve months of treatment were the two main criteria for deciding therapy termination. Outpatient visits were arranged at least every three months. Our results showed that SH was the third most frequent cause of stridor (15/137 patients), and biphasic stridor was uniformly present as a typical symptom. Both clinical improvement and bronchoscopy findings confirmed the efficacy of the treatment. The mean therapy duration was 17 months. The only significant adverse event observed was hypoglycemic seizures in one infant. Contributory factors were all prematurity, high propranolol dose (3 mg/kg) and poor oral intake. Collectively, defining a safe and timely protocol for therapy cessation and avoidance of risk factors for adverse effects is the mainstay of SH treatment. © 2023 The Author(s).