Development of musculoskeletal deficits in children with cystic fibrosis in later childhood.

Cystic fibrosis (CF) is a genetic condition primarily affecting the respiratory system, with the associated progressive lung damage and loss of function resulting in reduced lifespan. Bone health is also impaired in individuals with CF, leading to much higher fracture risk even in adolescence. However, the development of these deficits during growth and the relative contributions of puberty, body size and muscular loading remain somewhat unexplored. We therefore recruited 25 children with CF (10 girls, mean age 11.3 ± 2.9y) and 147 children without CF (75 girls, mean age 12.4 ± 2.6y). Bone characteristics were assessed using peripheral quantitative computed tomography (pQCT) at 4 % and 66 % distal-proximal tibia. Muscle cross-sectional area (CSA) and density (an indicator of muscle quality) were also assessed at the latter site. Tibial bone microstructure was assessed using high-resolution pQCT (HR-pQCT) at 8 % distal-proximal tibial length. In addition, peak jump power and hop force were measured using jumping mechanography. Group-by-age interactions and group differences in bone and muscle characteristics were examined using multiple linear regression, adjusted for age, sex and pubertal status and in additional models, height and muscle force. In initial models group-by-age interactions were evident for distal tibial total bone mineral content (BMC) and trabecular volumetric bone mineral density (vBMD), with a lower rate of age-related accrual evident in children with CF. In assessments of distal tibial microstructure, similar patterns were observed for trabecular number and thickness, and cortical CSA. In the tibial shaft, group-by-age interactions indicating slower growth in CF were evident for total BMC and cortical CSA, whilst age-independent deficits in CF were observed for several other variables. Peak jump power and hop force also exhibited similar interactions. Group-by-age interactions for bone were partially attenuated at the distal tibia and fully attenuated at the tibial shaft by adjustment for muscle force. These results suggest that bone and muscle deficits in children with CF develop throughout later childhood, independent of differences in pubertal stage and body size. These diverging growth patterns appear to be mediated by differences in muscle function, particularly for bone characteristics in the tibial shaft. Given the high fracture risk in this population from childhood onwards, development of interventions to improve bone health would be of substantial clinical value.

Update on the Coordinated Efforts of Looking After the Health Care Needs of Children and Young People Fleeing the Conflict Zone of Ukraine Presenting to European Emergency Departments-A Joint Statement of the European Society for Emergency Paediatrics and the European Academy of Paediatrics.

This joint statement by the European Society for Emergency Paediatrics and European Academy of Paediatrics aims to highlight recommendations for dealing with refugee children and young people fleeing the Ukrainian war when presenting to emergency departments (EDs) across Europe. Children and young people might present, sometimes unaccompanied, with either ongoing complex health needs or illnesses, mental health issues, and injuries related to the war itself and the flight from it. Obstacles to providing urgent and emergency care include lack of clinical guidelines, language barriers, and lack of insight in previous medical history. Children with complex health needs are at high risk for complications and their continued access to specialist healthcare should be prioritized in resettlements programs. Ukraine has one of the lowest vaccination coverages in the Europe, and outbreaks of cholera, measles, diphtheria, poliomyelitis, and COVID-19 should be anticipated. In Ukraine, rates of multidrug resistant tuberculosis are high, making screening for this important. Urgent and emergency care facilities should also prepare for dealing with children with war-related injuries and mental health issues. Ukrainian refugee children and young people should be included in local educational systems and social activities at the earliest opportunity.

Use of the SpO2/FiO2 Diagram to Assess Gas Exchange in Horses Under General Anaesthesia.

The aim of this study was to evaluate the validity of the SpO2/FiO2 diagram in estimating gas exchange in horses under general anaesthesia. In this prospective, controlled clinical study were included 10 horses under general anaesthesia. FiO2 was progressively reduced with the following steps: 0.6, 0.4, 0.3 and 0.21; SpO2 was recorded at each step. An arterial blood sample was collected at the steps of 1.0 and 0.21, to calculate intrapulmonary shunt with the Fshunt formula. The Fshunt value calculated at 0.21 FiO2 was defined as "Fshunt 0.21", the one calculated at 1.0 FiO2 as "Fshunt 1.0". The FiO2 vs SpO2 data points were analyzed using a computer algorithm which uses the haemoglobin and a fixed value for arterial-venous oxygen difference of 3.5 mL/dL. The algorithm estimates a shunt value fitting the obtained data with an ideal SpO2/FiO2 curve. The value of shunt (Sshunt) was considered for the study. Correlation between "Fshunt 1.0", "Fshunt0.21" and SShunt was determined using the Spearman Rank Correlation Coefficient test, the analysis of the regression curve and the coefficient of determination (r2). Values of P < .05 were considered statistically significant. A significant and strong correlation (P = .0069; r = 0.839; r2=0.6194) and a significant and moderate correlation (P = .0443; r = 0.644; r2=0.2336) was found between Sshunt and "Fshunt 1.0" and between Sshunt and "Fshunt 0.21", respectively. The SpO2/FiO2 diagram proved to be a useful and non-invasive tool to characterize gas exchange in horses under general anaesthesia and mechanical ventilation.

Physical Activity in the Prevention of Childhood Obesity: The Position of the European Childhood Obesity Group and the European Academy of Pediatrics.

Obesity affects an increasing number of children and adolescents. Physical activity (PA) is a significant factor in the prevention of excessive body mass in the pediatric population. A significant percentage of pediatric population do not attain the public health recommendation for PA, and typically, those with higher levels of PA have lower content of body fat than less active peers. Although the development of childhood obesity is multifactorial, the decline in energy expenditure is considered as one of the most important determinants of excessive body weight. The lack of intervention causes that excess body weight to remain stable from birth through childhood and adolescence to adulthood. Accordingly, public health interventions are needed to increase the level of PA in the pediatric population. The task force from the European Childhood Obesity Group and the European Academy of Pediatrics reviewed English language meta-analyses, systematic reviews, randomized clinical trials, and observational studies from PubMed/MEDLINE, Cochrane Library, Science Direct, MEDLINE, and EBSCO databases, from 2018 to August 2020, and developed a consensus statement. This statement presents the role of PA in the prevention of excessive body weight and gives age-appropriate recommendations for PA and recommendations for school-based interventions, parents, and guardians.

Update of the European paediatric respiratory medicine syllabus.

The 10-year-old European syllabus for paediatric respiratory medicine (PRM; also known as paediatric pulmonology) was updated by a consensus-based method using an expert task force for redrafting, and a subsequent Delphi process to achieve consensus. There was a high degree of consensus for the final syllabus, which has been streamlined and made more relevant to current practice. All modules are now mandatory apart from the undertaking of research projects, which is optional. Although there are still a number of countries in Europe which do not recognise PRM as a separate subspecialty, there are paediatric respiratory physicians practising in every country in Europe, and a current and harmonised European syllabus in the subspecialty remains important for defining the training and areas of practice of PRM practitioners.

We strongly support childhood immunisation-statement from the European Academy of Paediatrics (EAP).

The eradication of smallpox and the elimination of several other infectious diseases from much of the world has provided convincing evidence that vaccines are among the most effective interventions for promoting health. The current scepticism about immunisation among members of the new US administration carries a risk of decreasing immunisation rates also in Europe. While only a small minority of the population are strongly anti-vaccine, their public activities have significantly influenced an uncertainty among the general population about both the safety of and the necessity for vaccination. Therefore, the EAP calls for greater publically available, scientifically supported information on vaccination, particularly targeted at health care providers, for the further development of electronically based immunisation information systems (IIS). We further call on all European countries to work together both in legislative and public health arenas in order to increase vaccination coverage among the paediatric population. In the interest of children and their parents, the EAP expresses its strong support for childhood immunisation and recommended vaccination schedules. We are prepared to work with governments and media and share the extensive evidence demonstrating the effectiveness and safety of vaccines.

Paediatric HERMES: European accreditation of training centres in paediatric respiratory medicine.

HERMES: European accreditation of training centres in paediatric respiratory medicine http://ow.ly/ZBmUg.

Correlation of radiographic thoracic area and oxygenation impairment in bronchopulmonary dysplasia.

We hypothesized that radiographically-assessed hyperinflation in bronchopulmonary dysplasia (BPD) is related to the degree of oxygenation impairment. Our objective was to explore the relation of chest radiographic thoracic area (CRTA) with right-to-left shunt, right shift of the oxyhemoglobin dissociation curve and ventilation/perfusion ratio (VA/Q) in infants with BPD. Twenty-two infants born at median (IQR) gestation of 26 (24-28) weeks with BPD were prospectively studied at 39 (30-69) days. Inspired oxygen (FiO2) was varied to obtain transcutaneous oxygen saturation (SpO2) values between 85 and 96%. Shunt, shift and VA/Q were derived by plotting and analysing pairs of SpO2 and FiO2. CRTA was measured by free hand-tracing the perimeter of the thoracic area in anterio-posterior chest radiographs. Median (IQR) shunt was 8 (1-14)%, shift was 13 (11-19)kPa and VA/Q 0.42 (0.30-0.48). Median (IQR) CRTA/kg was 2495 (1962-2838)mm(2) and was significantly related to shift (r=0.674, p<0.001), VA/Q (r=-0.633, p<0.001), weight at study (r=-0.457, p=0.003) and day of life (r=-0.406, p=0.009), but not to shunt. CRTA in BPD is significantly related to oxygenation impairment as quantified by shift and VA/Q. CRTA can be used as a simple radiographic test to quantify BPD severity.

Human papillomavirus vaccination crisis in Japan.

The European Academy of Paediatrics (EAP) is gravely concerned about the human papillomavirus (HPV) vaccination crisis in Japan and particularly about the negative position taken by governmental authorities. Given that the HPV vaccine is both safe and effective, there is no recognizable reason to date to withhold this lifesaving and cost effective public health measure from a population. Therefore, the EAP strongly encourages the Japanese health authorities to actively support HPV vaccination for the future health of their children and adolescents.

Using Measurements of Shunt and Ventilation-to-Perfusion Ratio to Quantify the Severity of Bronchopulmonary Dysplasia.

BACKGROUND: Classifying the severity of bronchopulmonary dysplasia (BPD) by continuous numerical variables would facilitate follow-up of disease progression and quantified analysis of disease determinants. OBJECTIVES: To non-invasively measure oxygenation impairment in BPD by the degree of right-to-left shunt, right shift of the oxyhaemoglobin dissociation curve and ventilation/perfusion (VA/Q) inequality and to explore their relation with clinical parameters. METHODS: Prospective cohort study of 24 infants with a median (interquartile range, IQR) gestation of 25 weeks (24-27) and a birth weight of 0.70 kg (0.63-0.93), studied at 36 days (30-66), at a postmenstrual age (PMA) of 33 weeks (29-36). Inspired oxygen (FIO2) was varied to obtain three to five transcutaneous oxygen saturation (SpO2) values between 85 and 96%. Values of shunt, shift and VA/Q were obtained by plotting the paired data of SpO2 against FIO2 for each infant using a unique program. Right-to-left shunt, right shift of the oxyhaemoglobin dissociation curve and VA/Q were measured in infants born <32 weeks PMA receiving oxygen at 28 days. RESULTS: The median (IQR) shunt was 8% (0.3-16.5), shift 14.5 kPa (10.9-19.4) and VA/Q 0.40 (0.30-0.48). Shunt, shift and VA/Q were significantly related to gestational age (GA) at birth, PMA at study, weight at study and weight gain per week. CONCLUSIONS: Severity of pulmonary oxygenation impairment in BPD can be quantified at the cot-side by non-invasive measurement of shunt, shift and VA/Q. Low GA at birth, low weight at birth and at the time of study and impaired weight gain are significantly associated with the severity of oxygen-exchange impairment in infants with BPD.

Paediatrics in Vienna.

The aim of this update is to describe, in the context of the current literature, major papers from the seven groups of the Paediatric Assembly (Respiratory Physiology; Asthma and Allergy; Cystic Fibrosis; Respiratory Infection and Immunology; Neonatology and Paediatric Intensive Care; Respiratory Epidemiology; and Bronchology) presented during the annual European Respiratory Society congress held in 2012 in Vienna, Austria.

Thirty-years of screening for cystic fibrosis in East Anglia.

BACKGROUND: Newborn screening for cystic fibrosis (CF) relies on the measurement of immunoreactive trypsinogen (IRT) originating from the pancreas. The Norfolk, Suffolk and Cambridgeshire screening programme initially exploited the persistent increase in IRT seen in CF (IRT-IRT protocol) and later changed to include mutation analysis as a second tier test (IRT-DNA-IRT protocol). RESULTS: During a 30 year period 582 966 babies have been screened by IRT-IRT and 147 764 by IRT-DNA-IRT (total 730730), resulting in 296 screen positive cases of CF and 29 false negatives (including 10 false negatives with meconium ileus). Ten missed CF cases were pancreatic insufficient, however all were diagnosed before their first birthday, suggesting that a false negative result did not forestall appropriate clinical investigation. The IRT-DNA-IRT protocol had a much improved positive predictive value (PPV) of 85.9% compared to 67.3% for IRT-IRT, excluding CF babies with meconium ileus. The PPVs increased to 82.2% and 98.2% respectively if only well, term babies were considered. The main factor to account for this improvement in PPV has probably been the incorporation of DNA analysis in the second tier testing. CONCLUSIONS: The diagnosis of screen-positive babies proved difficult in a minority of cases with the classification of some patients changing with evolving phenotype. Our results illustrate the importance of collecting outcome data over a long time period for accurate assessment of the screening programme. This study provides evidence that newborn screening for CF is a valid undertaking that detects 95% of unsuspected CF cases presenting before 3 years of age.

Branchio-oto-renal syndrome with obstructive sleep apnoea.

Branchio-oto-renal (BOR) syndrome is a rare autosomal dominant disorder characterised by branchial arch anomalies, otological and renal abnormalities. To the best of our knowledge, upper airway obstruction has not been hitherto reported in BOR. The authors report a 19-month-old girl with BOR syndrome with features of severe airway obstruction needing tracheostomy.

Paediatrics in Amsterdam.

The aim of this update is to describe the paediatric highlights from the 2011 European Respiratory Society (ERS) Annual Congress in Amsterdam, the Netherlands. Abstracts from all seven groups of the ERS Paediatric Assembly (Paediatric Respiratory Physiology, Paediatric Asthma and Allergy, Cystic Fibrosis, Paediatric Respiratory Infection and Immunology, Neonatology and Paediatric Intensive Care, Paediatric Respiratory Epidemiology, and Paediatric Bronchology) are presented in the context of current literature.

Report of neonatal meningitis due to Salmonella enterica serotype Agona and review of breast milk-associated neonatal Salmonella infections.

We present the first documented case of Salmonella enterica serotype Agona meningitis in a 6-day-old baby. S. enterica serotype Agona was isolated concurrently from infant cerebrospinal fluid and parental fecal samples, and Salmonella was isolated from breast milk. The role of breast milk in transmission of Salmonella enterica is discussed.

A prospective study of phrenic nerve damage after cardiac surgery in children.

OBJECTIVE: To gather detailed data on the incidence of phrenic nerve damage (PND) following cardiac surgery in children, the risk factors for its development, its effect on morbidity and its prognosis. DESIGN: Prospective electrophysiological measurement of phrenic nerve latency in 310 children before and after cardiac surgery. SETTING: Tertiary paediatric cardiac surgical centre. MEASUREMENTS AND RESULTS: Our findings were fourfold. Firstly, the incidence of PND in our group of patients was 20%, significantly higher than estimates using indirect methods of assessment. Secondly, PND increased the duration of ventilation by a median of 76 h (20 vs. 96 h; p<0.001), and late post-operative deaths (before hospital discharge) occurred in 12.9% of patients compared to 2.4% among patients with a normal post-operative phrenic latency. Thirdly, the risk factors that were independently predictive of the development of PND were the site of the surgery and the patient's age. Patients who required surgery at both the lung hilum and the pericardium were more likely to develop PND than patients with only one of those sites, or when neither was involved, and children less than 18 months old were more likely to develop PND than older children. Lastly, the natural history of PND following surgery appears to be good. In our follow-up to 3 months, approximately one third recovered within 1 month and a further third (overall) recovered by 3 months. CONCLUSIONS: We conclude that the incidence of PND is much higher than currently recognised, and has a very significant effect on post-operative morbidity and mortality. Most children who survive the post-operative period will recover nerve function within 3 months.