ERS statement on paediatric long-term noninvasive respiratory support.

Long-term noninvasive respiratory support, comprising continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV), in children is expanding worldwide, with increasing complexities of children being considered for this type of ventilator support and expanding indications such as palliative care. There have been improvements in equipment and interfaces. Despite growing experience, there are still gaps in a significant number of areas: there is a lack of validated criteria for CPAP/NIV initiation, optimal follow-up and monitoring; weaning and long-term benefits have not been evaluated. Therapeutic education of the caregivers and the patient is of paramount importance, as well as continuous support and assistance, in order to achieve optimal adherence. The preservation or improvement of the quality of life of the patient and caregivers should be a concern for all children treated with long-term CPAP/NIV. As NIV is a highly specialised treatment, patients are usually managed by an experienced paediatric multidisciplinary team. This statement written by experts in the field of paediatric long-term CPAP/NIV aims to emphasise the most recent scientific input and should open up new perspectives and research areas.

Mycoplasma pneumoniae P1 Genotype Indicates Severity of Lower Respiratory Tract Infections in Children.

Mycoplasma pneumoniae strains can be classified into two major genetic groups, P1 type 1 (P1-1) and P1 type 2 (P1-2). It remains unknown if clinical manifestations of lower respiratory tract infections (LRTI) in children differ between the two genotypes. We aimed to determine if the M. pneumoniae P1 genotype is associated with severity of LRTI in children. Medical charts of 420 children (≤15 years old) with signs of acute LRTI who were PCR positive for M. pneumoniae from pharyngeal swabs in a recent M. pneumoniae epidemic were analyzed. We used a culture and pyrosequencing approach for genotyping PCR-positive samples. We compared epidemiological and clinical data of children with either P1-1 or P1-2 LRTI. P1-2-infected children presented with a significantly higher median baseline C-reactive protein level and were admitted to the hospital more often. The P1 genotype had a significant predictive value in a multiple linear regression model predicting C-reactive protein levels in our study sample. Moreover, the P1 genotype significantly affected the likelihood of hospital admission in a logistic regression model. Our modeling results were also confirmed on an additional independent sample of children with M. pneumoniae LRTI. Results from our large patient group indicate that the two M. pneumoniae P1 genotypes may have different pathogenic potential and that LRTI with P1-2 strains may have a more severe disease course than those with P1-1 strains in children. P1 genotyping is not routinely performed but could be used as a predictor of M. pneumoniae LRTI severity, enabling patient-tailored treatments.

Clinical characteristics of infections caused by Mycoplasma pneumoniae P1 genotypes in children.

Mycoplasma pneumoniae (M. pneumoniae) isolates can be classified into two major genetic groups, P1 type 1 (MP1) and P1 type 2 (MP2), based on the DNA sequence of the P1 adhesion protein gene. The aim of our study was to determine if M. pneumoniae P1 genotype is associated with disease manifestation and severity of acute M. pneumoniae infection. We compared epidemiological and clinical data of children infected with either MP1 or MP2. In addition, we separately analysed data of patients presenting with individual manifestations of M. pneumoniae infection. Data of 356 patients infected with MP1 were compared with those of 126 patients infected with MP2. MP2-infected children presented with higher median baseline C-reactive protein levels and were admitted to the hospital more often. The distribution of P1 genotype varied among groups of patients with different manifestations of M. pneumoniae infection. MP2 was more common than MP1 among patients with neurological and cardiovascular manifestations, whereas MP1 was more prevalent in other manifestations. The results from our large cohort indicate that the two P1 subtypes may have different pathogenic potential and that infections with MP2 strains could be more virulent than those with MP1 strains.

Assessment of serology and spirometry and the combination of both to complement microbiological isolation for earlier detection of Pseudomonas aeruginosa infection in children with cystic fibrosis.

BACKGROUND: The aim of this study was to assess whether serology and spirometry and the combination of both can complement culture-based detection for earlier recognition of Pseudomonas aeruginosa infection in children with cystic fibrosis. METHODS: A 4 year longitudinal prospective study that included 67 Slovenian children with cystic fibrosis with a mean age of 10.5 years was conducted. Serology, spirometry and a scoring system combining serology and spirometry were assessed and compared. Infection was confirmed with isolation of Pseudomonas aeruginosa from respiratory samples. RESULTS: There was a significantly positive correlation between serology and the combination of serology and spirometry and Pseudomonas aeruginosa isolation (P < 0.01 for both) and a significantly negative correlation between spirometry and Pseudomonas aeruginosa isolation (P < 0.05). An increase in serology for 1 ELISA unit increased the possibility of Pseudomonas aeruginosa isolation 1.6 times. A fall in FEV1% predicted for 10% increased the possibility of Pseudomonas aeruginosa isolation 9.8 times. Binary logistic regression analysis was used to determine the odds ratios and 95% confidence intervals for all three approaches. Serology had the highest specificity (0.80) and the combination of serology and spirometry the highest sensitivity (0.90). Both had a high negative predictive value (0.93 and 0.79 respectively). CONCLUSION: Using serology and the combination of serology and lung function measurement can be beneficial for earlier detection of infection with Pseudomonas aeruginosa in children with cystic fibrosis when done simultaneously with standard culture-based detection from respiratory samples.

First recovery of Rasamsonia argillacea species complex isolated in adolescent patient with cystic fibrosis in Slovenia--case report and review of literature.

We report the isolation of the emerging fungal pathogen Rasamsonia aegroticola, which belongs Rasamsonia argillacea species complex, from a respiratory sample of a patient with cystic fibrosis. This filamentous fungus, resembling members of a Penicillium and Paecilomyces spp., was identified by morphology and confirmed by DNA sequence analysis. Susceptibility pattern showed high minimal inhibitory concentration of voriconazole and amphotericin B but low minimal inhibitory concentration of caspofungin, micafungin and itraconazole.

Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry.

Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). We analysed forced expiratory volume in 1 s (FEV1) data of 14,732 patients registered in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database in 2007. We used linear and logistic regressions to investigate associations between FEV1 % predicted and clinical outcomes. Body mass index (BMI), chronic infection by Pseudomonas aeruginosa, pancreatic status and CF-related diabetes (CFRD) showed a statistically significant (all p<0.0001) and clinically relevant effect on FEV1 % pred after adjusting for age. Patients with a lower BMI experience a six-fold increased odds ratio (95% CI 5.0-7.3) of having severe lung disease (FEV1 <40% pred) compared to patients with normal BMI. Being chronically infected with P. aeruginosa increases the odds ratio of severe lung disease by 2.4 (95% CI 2.0-2.7), and patients with pancreatic insufficiency experience a 2.0-fold increased odds ratio (95% CI 1.6-2.5) of severe lung disease compared to pancreatic sufficient patients. Patients with CFRD have a 1.8-fold increased odds ratio (95% CI 1.6-2.2) compared to patients not affected. These potential risk factors for pulmonary disease in patients with CF are to some degree preventable or treatable. We emphasise the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of relevant therapies.

Influence of anti-asthmatic medications on dental caries in children in Slovenia.

OBJECTIVE. The study investigated the influence of exposure to anti-asthmatic medications and of various factors on the caries prevalence in children in Slovenia. METHODS. The study population consisted of children aged 2 to 17 years (n = 220) under treatment for asthma, who had used anti-asthmatic medications for at least 1 year; 220 controls were matched for age. Caries status was determined by the number of decayed, missing, and filled surfaces through clinical examination by two calibrated dentists using the International Caries Detection and Assessment System-II scoring criteria. Questionnaires completed by parents and data from the patients' medical records provided information on various confounding factors. RESULTS. Asthmatic children had significantly higher (P ≤ 0.01) prevalence of caries on primary and permanent teeth in all age groups, and the proportion of caries-free children was significantly smaller (P ≤ 0.05). In multivariate regression analysis, asthma diagnosis, child's age, daily use of inhaled glucocorticoids, length and frequency of medicine application, spacer use, mouth rinsing with water after medicine application, parents' education, frequent food and drink consumption, and frequency of toothbrushing were associated with caries experience of asthmatic children. CONCLUSION. Children with asthma who had used anti-asthmatic medications had higher caries experience in primary and permanent teeth.

Clinical, Laboratory, and Radiographic Features Can Help Predict Mycoplasma pneumoniae Lower Respiratory Tract Infection in Children.

Mycoplasma pneumoniae (Mp) is a common cause of lower respiratory tract infection (LRTI) in children that is difficult to distinguish from LRTI of other etiologies. We aimed to determine if a combination of clinical, laboratory, and chest radiographic features can help identify patients at higher risk of Mp LRTI. We reviewed medical charts of children referred to our tertiary hospital with suspected acute mycoplasmal LRTI. Pharyngeal swabs obtained from patients were tested by Mp PCR. We compared epidemiological and clinical data of children with positive and negative Mp PCR results. In addition, a multivariable logistic regression analysis was performed to predict Mp LRTI based on the patient's age, duration of symptoms, presence of extrapulmonary manifestations, laboratory findings, and chest radiographic findings. We included 65 children with Mp PCR-negative and 49 with Mp PCR-positive LRTI and no viral co-detection. Children with Mp LRTI were older (median age 5.8 vs. 2.2 years, p < 0.001), had a longer duration of symptoms on referral (median 7 vs. 4 days, p < 0.001), and lower median WBC (9.9 vs. 12.7 × 109/L, p < 0.001). On chest radiograph, unilateral infiltrates were more frequently observed in the Mp PCR-positive group (57.5% vs. 24.1%, p = 0.001). Age, duration of symptoms, and chest radiographic findings had the highest predictive value for Mp LRTI in a multivariable logistic regression model. Our analysis suggests that a combination of clinical, laboratory, and chest radiographic features can be used to assess the likelihood of Mp LRTI and assist in decision-making for which children need further tests or macrolide antibiotic treatment.

Physical fitness trajectories from childhood to adolescence in extremely preterm children: A longitudinal cohort study.

OBJECTIVE: Cohort studies on physical fitness (PF) in former extremely preterm children are scarce and yield conflicting results. Therefore, this study aimed to assess the effect of extremely preterm birth on PF in school-age with a focus on bronchopulmonary dysplasia (BPD). METHODS: Eighty school-aged children were enrolled in the longitudinal cohort study. Fifty were born extremely preterm (

Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry.

Aim: To examine the trajectory of forced expiratory volume in 1 s (FEV1) using data from the European Cystic Fibrosis Society patient registry (ECFPR) collected from 2008 to 2016, i.e. the era before highly effective modulator therapy (HEMT). We evaluated risk factors for FEV1 decline. Methods: The study population included patients with a confirmed diagnosis of cystic fibrosis recorded in the ECFPR (2008-2016). The evolution of FEV1 % predicted (%FEV1) with age, and the yearly change in %FEV1 were evaluated. Risk factors considered were cystic fibrosis transmembrane conductance regulator (-CFTR) mutation class, gender, age at diagnosis, neonatal screening, meconium ileus, sweat chloride concentration at diagnosis and country's income level. Results: We used 199 604 FEV1 recordings from 38 734 patients. The fastest decline was seen during puberty and in patients diagnosed before the age of 10 years. Males had a higher %FEV1, but a higher yearly %FEV1 loss between the ages of 15 and 25 years. We showed stabilisation and even improvement in %FEV1 over age in adults with a class III CFTR mutation, but a steady decline in patients homozygous for F508del or with both mutations of classes I/II. A faster decline in %FEV1 was found in patients from low-income countries compared to a similar %FEV1 evolution in patients from middle- and high-income countries. Conclusions: These longitudinal FEV1 data reflect the reality of cystic fibrosis across Europe in the era pre-HEMT, and can serve as baseline for comparison with the post-HEMT era. The similar evolution in middle- and high-income countries underlines opportunities for low-income countries.