Evaluation of treatment and long-term management of anaphylaxis in pediatric departments of Greece: a 2-year nationwide survey.

BACKGROUND: Anaphylaxis proportions of incidence are increasing globally. However, limited data are available regarding anaphylaxis in the pediatric population of Greece. PURPOSE: The aim of the study was to evaluate management of anaphylaxis in Greek pediatric departments. METHODS: We performed a questionnaire-based study of children aged less than 16 years presenting with anaphylaxis in 10 national pediatric hospitals over a period of 2 years. Management of anaphylaxis was assessed prior to and after an informative intervention. RESULTS: In all, 127 cases of anaphylaxis were identified. Epinephrine was administered in almost half of all cases (51.2%), predominantly through intramuscular route (88.5%), while the majority of anaphylaxis patients were treated with antihistamines (92.9%) and corticosteroids (70.1%). Epinephrine was more likely administered by physicians if the elicitor was a drug (P < 0.003). Regarding long-term management, an epinephrine auto-injector was prescribed in 66.9% of patients. Follow-up information was available for most of the patients (92.9%), the majority of whom (76.3%) were referred to an allergist. More than half of these patients (63.6%) had a documented allergy follow-up, which identified a causative allergen in 53.3% of cases. No statistically significant differences were recorded prior to and after the intervention regarding management of anaphylaxis. CONCLUSIONS: This nationwide study highlighted the necessity of further improvement in terms of anaphylaxis treatment and secondary prevention measures. This presupposes appropriate education and training of healthcare professionals, thus contributing to proper and comprehensive care of the pediatric population.

"Antibiotic hypersensitivity reactions in Cystic Fibrosis: A thorough inspection on a stumbling block in patient care".

One hurdle in the management of CF, a disease characterized by progressive endobronchial infection, is the presence of hypersensitivity reactions to antimicrobials due to prolonged and repetitive treatment courses. The aim of this review is to compile existing data and provide insight to medical professionals on a long-debated topic for optimum patient care. Clinical studies were inducted from the last 15 years and filtered based on their relativity to drug hypersensitivity reactions (DHRs), antibiotics and CF. After completing the selection process, 10 clinical studies were thoroughly examined. The most frequent antibiotic group related to DHRs were beta-lactams. Frequency of the most common overall type of reaction (immediate or nonimmediate) differed among clinical studies. Although severe reactions seem rare comparatively, they do occur during and even after completion of treatment regimens. The prevalence of true drug allergies should be confirmed using a variety of tests available, however, should not be confused with overall DHR rates. Genetic mutations, gender and lifetime antibiotic dose were not related with an increased risk for DHR development. On the contrary, the most important factor according to most studies was the cumulative antimicrobial dose in a given period of time, especially when delivered parenterally. DHRs are an indisputable problem in the management of CF patients. Understanding possible risk factors and increased awareness is vital in both hospital and outpatient settings as early detection can decrease the severity of the reactions.

The Psychological Impact on Parents of Children who Receive an Inconclusive Diagnosis for Cystic Fibrosis following Newborn Screening: A Systematic Mini-Review.

Newborn screening (NBS) has been available for the diagnosis of cystic fibrosis (CF) over the last decades. Through the implementation of NBS, a new designation emerged, that of CF related metabolic syndrome (CRMS) or cystic fibrosis screen positive inconclusive diagnosis (CFSPID). As there is uncertainty regarding the clinical progression of these infants to CF, some studies have investigated the psychological impact of CRMS/CFSPID on their parents. This systematic narrative review aimed to describe the findings of the relevant studies. The number of studies is limited and the study samples are relatively small. It seems that there is a negative impact of CRMS/CFSPID on parental mental health. While some studies indicated similar levels of parental anxiety among those with infants diagnosed with CF and those with CRMS/CFSPID, not all studies reached the same conclusion. Parental uncertainty represents another mental dimension of the impact associated with the designation of CRMS/CFSPID. These observations suggest that parents of infants with CRMS/CFSPID should be provided with effective communication, and it may also be beneficial to consider parental mental screening. More robust and long-term studies are required to detect differences in parental emotional status between those with infants diagnosed with CF and those with CRMS/CFSPID.

How Different Are the Influences of Mediterranean and Japanese Diets on the Gut Microbiome?

The gut microbiome is a complex ecosystem, mainly composed of bacteria, that performs essential functions for the host. Its composition is determined by many factors; however, diet has emerged as a key regulator. Both the Mediterranean (MD) and Japanese (JD) diets have been associated with significant health benefits and are therefore considered healthy dietary patterns. Both are plant-based diets and although they have much in common, they also have important differences mainly related to total calorie intake and the consumption of specific foods and beverages. Thus, it has been hypothesized that they exert their beneficial properties through different nutrients and bioactive compounds that interact with gut microbes and induce specific changes on gut metabolic pathways. In this review, we present current data on the effects of the MD and JD on the gut microbiome. Furthermore, we aim to examine whether there are differences or shared effects on the gut microbiome of people who adhere to these dietary patterns.

A core outcome set for bronchiectasis in children and adolescents for use in clinical research: an international consensus study.

Improving the treatment of non-cystic fibrosis bronchiectasis in children and adolescents requires high-quality research with outcomes that meet study objectives and are meaningful for patients and their parents and caregivers. In the absence of systematic reviews or agreement on the health outcomes that should be measured in paediatric bronchiectasis, we established an international, multidisciplinary panel of experts to develop a core outcome set (COS) that incorporates patient and parent perspectives. We undertook a systematic review from which a list of 21 outcomes was constructed; these outcomes were used to inform the development of separate surveys for ranking by parents and patients and by health-care professionals. 562 participants (201 parents and patients from 17 countries, 361 health-care professionals from 58 countries) completed the surveys. Following two consensus meetings, agreement was reached on a ten-item COS with five outcomes that were deemed to be essential: quality of life, symptoms, exacerbation frequency, non-scheduled health-care visits, and hospitalisations. Use of this international consensus-based COS will ensure that studies have consistent, patient-focused outcomes, facilitating research worldwide and, in turn, the development of evidence-based guidelines for improved clinical care and outcomes. Further research is needed to develop validated, accessible measurement instruments for several of the outcomes in this COS.

Greater fruit and vegetables consumption, and adherence to a Mediterranean type of diet reduces the risk for asthma in children; a systematic review and meta-analysis.

The objective of this study was to explore the relationship between the consumption of fruits, vegetables and adherence to the Mediterranean diet with childhood asthma development and severity. A systematic literature search and synthesis of the results was performed throughout the last two decades. A total of 45 studies were analysed and 392,797 children were included. Greater adherence to a Mediterranean type of diet was inversely associated with asthmatic outcomes (OR:0.71,95% CI:0.54,0.88). Regarding fruits, vegetables consumption, a significant inverse association between increased fruits or vegetable intake and asthma was found (OR:0.82,95% CI:0.77,0.86; 0.84,95% CI:0.77,0.91, respectively). Fruits and vegetables consumption combined was also inversely related to all asthmatic outcomes (OR:0.65,95% CI:0.49,0.78). The level of heterogeneity was moderate-to-high (30%-97%). The present review and meta-analysis show a trend to an inverse association between adherence to the Mediterranean diet or a healthy diet rich in fruits and vegetables and the occurrence of childhood asthma but with a low level of certainty.

Technical Factors That May Influence mHealth Adherence in Children With Chronic Pulmonary Diseases: Scoping Review.

PURPOSE: To synthesize the technical factors influencing adherence to nonpharmacological treatment (NPhT) in children with chronic pulmonary diseases (CPDs), using mobile health (mHealth) technology. METHODS: Five electronic databases were searched from inception to October 12, 2022, with terms related to pediatrics, CPDs, adherence, NPhT, and mHealth. The methodological quality was assessed using the Critical Appraisal Skills Programme and the Mixed Methods Appraisal Tool checklist. RESULTS: Eleven articles were included. Six major technical themes were supported by the evidence that may influence adherence to NPhT: design and context, technical support/business model, connectivity, free availability, privacy and security, and cultural readiness. CONCLUSIONS: The design of mHealth applications (apps) should be done according to the needs of pediatric patients. This may mitigate any barriers and potentially foster adherence to the use of the apps. WHAT THIS ADDS TO THE EVIDENCE: Six major technical themes may influence adherence to NPhT in children with chronic respiratory diseases.Video Abstract: Supplemental digital content available at http://links.lww.com/PPT/A487 .

Children with cystic fibrosis are still receiving inconclusive diagnosis despite undergoing newborn screening.

AIM: We aimed to familiarise clinicians with the terms cystic fibrosis transmembrane conductance regulator related metabolic syndrome (CRMS) and cystic fibrosis screen positive inconclusive diagnosis (CFSPID). We also sought to highlight the monitoring and outcomes of children that match these designations. METHODS: A literature review was performed by searching PubMed from its inception until 30 November 2022. All relevant articles were included in this narrative review. RESULTS: Despite the implementation of newborn screening programmes for cystic fibrosis (CF), the diagnosis remains uncertain in some newborn infants with elevated immunoreactive trypsinogen. In 2016, a unified definition for CRMS/CFSPID was established to categorise these children. While many of them remain healthy, a portion of these children may develop CF. As a result, it is crucial to monitor them regularly. CONCLUSION: CRMS/CFSPID is a designation and not a diagnosis. Longer longitudinal studies are needed to shed light on the most appropriate follow-up of these children. Paediatricians need to be knowledgeable about this condition in order to administer proper care, and children should be in contact with their local CF centre.

Adherence to an Anti-Inflammatory Diet and Atopic Diseases' Prevalence in Adolescence: The Greek Global Asthma Network Study.

OBJECTIVE: Atopic diseases are among the most common morbidities in children and adolescents. The association between adherence to an anti-inflammatory dietary pattern and the prevalence of atopic diseases among adolescents was examined. METHODS: A total of 1934 adolescents (boys: 47.5%, mean age (standard deviation): 12.7 (0.6) years) were voluntarily enrolled. Participants completed a validated questionnaire on atopic disease status as well as one assessing dietary habits and other sociodemographic and lifestyle characteristics. A special Diet Anti-inflammatory Index (DAI) score was calculated for the evaluation of adherence to an anti-inflammatory dietary pattern. RESULTS: A total of 6.9% of the participants reported current asthma symptoms, while 25.3% reported rhinitis symptoms and 8.9% reported eczema. Adolescents with high adherence to an anti-inflammatory diet were 58% less likely to have asthma symptoms compared with those with low adherence when adjusted for multiple confounders (p < 0.01). No significant associations were observed between the level of adherence to an anti-inflammatory diet and the prevalence of allergic rhinitis and eczema. CONCLUSION: An anti-inflammatory diet seems to be independently associated with a lower prevalence of asthma in adolescents. Thus, pediatricians and other healthcare providers should promote anti-inflammatory dietary patterns as a preventive measure for atopic diseases from early stages of life.

The Association between CFTR Gene Mutation Heterozygosity and Asthma Development: A Systematic Review.

Asthma is caused by complex interactions between environmental and genetic factors. Various genes have been implicated as potential risk factors in the development of asthma; among them is cystic fibrosis transmembrane conductance regulator (CFTR) gene. The aim of this systematic review was to investigate the association of CFTR mutation heterozygosity with the development of asthma, by updating the existing data with recent studies' findings. Therefore, a systematic review of the literature was conducted on Pubmed, ESBCO (Cinahl) and Scopus Databases up to December 2022. After the eligibility assessment, 17 studies were included in this review. Nine of them supported a lack of relationship between CFTR mutation heterozygosity and asthma susceptibility, and eight reported a positive association. Consequently, more extensive research is needed through high-quality studies to provide valid evidence and highlight the clinical benefits of identifying CFTR mutations in asthma patients, their impact on asthma severity, or treatment perspectives.

Efficacy and Safety of Elexacaftor-Tezacaftor-Ivacaftor in the Treatment of Cystic Fibrosis: A Systematic Review.

Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) is a new CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) modulator treatment, used over the last few years, which has shown an improvement in different clinical outcomes in patients with cystic fibrosis (CF). The objective of this study was a systematic research of the literature on the efficacy and safety of this CFTR modulator on patients with CF. A search of Pubmed was conducted for randomized clinical trials and observational studies published from 2012 to September 2022. The included full manuscripts comprised nine clinical trials and 16 observational studies, whose participants were aged ≥12 years or were children 6-11 years old with at least one Phe508del mutation and/or advanced lung disease (ALD). These studies reported that ELX/TEZ/IVA has a significant positive effect on the lung function of patients with CF, by ameliorating parameters such as FEV1, LCI, pulmonary exacerbations or sweat chloride concentration, increasing BMI and improving quality of their life. Its role in cystic fibrosis-related diabetes (CFRD) is not yet clear. It was found that this new CFTR modulator has an overall favorable safety profile, with mild to moderate adverse events. Further studies are needed for a deeper understanding of the impact of CFTR modulators on other CF manifestations, or the possibility of treating with ELX/TEZ/IVA CF patients with rare CFTR mutations.

Assessment and management of asthma exacerbations in an emergency department unit.

BACKGROUND: The Pediatric Respiratory Assessment Measure (PRAM) score is a useful tool for the assessment of asthma exacerbations in children. This study aimed to estimate the risk of hospitalization in children, assessed with the PRAM score and having mild-moderate asthma exacerbation, who were treated with salbutamol delivered via a metered-dose inhaler and spacer (MDI/S). METHODS: The study population consisted of children aged 3-16 years with mild-moderate asthma exacerbations. All children received 1mg/kg prednisolone p.o. (max 40 mg) and 4-6 puffs of salbutamol via MDI/S. RESULTS: Fifty patients participated in the study. Admission was associated positively with the initial PRAM score (OR: 18.91, CI: 2.42-123.12, P = 0.005) and negatively with the improvement in PRAM score (OR: 0.52, CI: 0.01-0.78, P = 0.032). CONCLUSION: PRAM is a reliable tool that can be used effectively to estimate the asthma exacerbation severity.

COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.

Adult PAP patients experience similar #COVID19 rates to the general population, and high rates of hospitalisation and deaths, underscoring their vulnerability and the need for measures to prevent infection. The impact of iGM-CSF must be considered. https://bit.ly/3M0wKnZ.

Physicians' ability to recognize adventitious lung sounds.

BACKGROUND: Lung auscultation is an important tool for diagnosing respiratory diseases. However, the ability of observers to recognize respiratory sounds varies considerably and depends on the sound. The present study aimed to assess the auscultatory skills of healthcare professionals and medical students. METHODS: A total of 295 physicians (185 pediatricians, 69 pulmonologists, and 41 physicians of general/internal medicine and subspecialties), 55 residents, and 50 medical students participated in the survey. They listened to five audio-recorded respiratory sounds and described them in free-form answers. RESULTS: The rates of correct answers were 55.2% for fine crackles, 74.5% for coarse crackles, 72.2% for wheezes, 18.75% for squawks, and 11.25% for pleural friction rub. The medical specialty was correlated with the correct answers and both pediatricians and physicians of general/internal medicine and subspecialties recognized fewer sounds compared with respiratory physicians (odds ratio [OR]: 0.37; confidence interval [CI]: 0.22-0.62; p < 0.001 and, OR: 0.47; CI: 0.22-0.99, p = 0.048, respectively). Years of experience were negatively correlated with the number of correct answers (OR: 0.73; CI:0.62-0.84; p = 0.001). CONCLUSIONS: Gaps remain in both terminology and recognition of lung sounds among a wide population of Greek physicians. Less experienced physicians perform better on lung auscultation, indicating that continuing education with critical feedback should be offered.

Lung ultrasound systematic review shows its prognostic and diagnostic role in acute viral bronchiolitis.

AIM: Lung ultrasound (LUS) has not been included in the current guidelines for the diagnosis of bronchiolitis so far, even though data concerning its effectiveness have been published. METHODS: A systematic literature review was carried out to determine the role of LUS scores in the diagnosis and prognosis of patients aged 0-2 years with bronchiolitis, using MEDLINE, Scopus and ScienceDirect databases from their inception to December 2021. RESULTS: A total of 18 studies matching our eligibility criteria were analysed for the purposes of this review and 1249 patients with bronchiolitis were included. The sonographic and radiological findings were comparable and chest radiography was found to have a higher sensitivity in ruling out severe complications such as concomitant pneumonia. The LUS scores were correlated to the clinical course of bronchiolitis and it was able to predict the need of admission in paediatric intensive care unit, the duration of hospitalisation and the need for respiratory support. CONCLUSION: This review suggests that LUS could have both a diagnostic and a prognostic role in bronchiolitis during first evaluation in the emergency department and hospitalisation. Physicians could adjust management according to sonographic findings as a useful adjunct to the clinical ones.

Changes in Shear Wave Elastography after Lumacaftor/Ivacaftor Treatment in Children with Cystic Fibrosis.

BACKGROUND: Lumacaftor/Ivacaftor (LUM/IVA) is an approved combination therapy for cystic fibrosis (CF) patients homozygous for F508del. OBJECTIVE: This study aimed to detect changes in liver stiffness measurement (LSM) in patients under this treatment. METHODS: The study population consisted of CF patients homozygous for F508del, 6 to 11 years old, who had been treated for six months with LUM/IVA. Shear wave elastography (SWE) was performed in all of them, before and 6 months after the commencement of treatment. RESULTS: Thirty-one patients were included in the study. LSM values after treatment were significantly higher than the values before treatment (medians and interquartile ranges of LSM values before and after treatment: 5.6, 5.3-6.3 kPa and, 6.4, 6.0-7.6 kPa, respectively, p<0.001). CONCLUSION: SWE can detect early changes in LSM in some CF patients treated with LUM/IVA.

Clinical Course of Children with Chronic Suppurative Lung Disease or Bronchiectasis Infected with Pseudomonas aeruginosa.

Children with chronic wet cough and without cystic fibrosis (non-CF) may suffer from chronic suppurative lung disease (CSLD) or bronchiectasis. Pseudomonas aeruginosa (Pa) can be one of the offending microbes in these children. The present study aimed to describe the clinical course of children with the above two conditions who were infected with Pa. Data of 54 children with CSLD/bronchiectasis who were diagnosed and attended in our department were retrospectively analysed through a Cox proportional hazard model, with age, presence of bronchiectasis, use of inhaled colistin, azithromycin, inhaled hypertonic saline as the covariates. In 42 of the 54 patients, there was no identifiable cause or underlying chronic disorder. Microbiological clearance was defined as the absence of daily wet cough for four months along with four negative cultures taken during the last four consecutive follow-up visits. Multivariate analysis was performed with a Cox proportional hazard model with time to microbiological clearance as the outcome. Results are described as Hazard Ratios (HR) with 95% Confidence Intervals (95%CI). Nebulised antibiotics and the presence of bronchiectasis were statistically significant predictors of remission (HR: 3.99; 95%CI: 1.12-14.14; p = 0.032, and HR: 0.24; 95%CI: 0.08-0.71; p = 0.010). In conclusion, the rate of microbiological clearance increases with the use of inhaled colistin and decreases when there is established bronchiectasis.

The Differences Between RSV and no RSV Acute Bronchiolitis in Hospitalized Infants: A Cross-Sectional Study.

To identify the differences between the RSV and non-RSV bronchiolitis in hospitalized infants in a Greek tertiary pediatric unit and the possible risk factors related to severe forms of the illness. We performed a retrospective cross-sectional data analysis by reviewing medical records of patients that were hospitalized for acute bronchiolitis from 2012 to 2019. The patients with RSV bronchiolitis were found to require antibiotic treatment, IV fluids, adrenaline, and hypertonic saline inhalations more frequently than the non-RSV patients. They also required prolonged hospitalization, especially those that were admitted to PICU, and received oxygen therapy for longer periods. We searched risk factors for severe forms of the disease according to the need for admission to PICU, the supplemental oxygen and the extended length of hospital stay, concurrently. The patients with RSV bronchiolitis developed more severe illness in comparison with patients with bronchiolitis due to other respiratory viruses.

The Role of the Gut Microbiome in Cow's Milk Allergy: A Clinical Approach.

Cow's milk allergy (CMA) is the most prevalent food allergy (FA) in infancy and early childhood and can be present with various clinical phenotypes. The significant increase in FA rates recorded in recent decades has been associated with environmental and lifestyle changes that limit microbial exposure in early life and induce changes in gut microbiome composition. Gut microbiome is a diverse community of microbes that colonize the gastrointestinal tract (GIT) and perform beneficial functions for the host. This complex ecosystem interacts with the immune system and has a pivotal role in the development of oral tolerance to food antigens. Emerging evidence indicates that alterations of the gut microbiome (dysbiosis) in early life cause immune dysregulation and render the host susceptible to immune-mediated diseases later in life. Therefore, the colonization of the gut by "healthy" microbes that occurs in the first years of life determines the lifelong health of the host. Here, we present current data on the possible role of the gut microbiome in the development of CMA. Furthermore, we discuss how gut microbiome modification might be a potential strategy for CMA prevention and treatment.

The Intake of Antioxidant Capacity of Children Depends on Their Health Status.

The gastrointestinal digestion of food and further gut microbial activity render a myriad of different molecules that could be responsible for the biological activities that are classically assigned to their parent compounds. This has been previously shown for some phytochemicals whose antioxidant capacity was either increased or decreased after being metabolized by gut microbes. Whether a global antioxidant capacity that is extracted from food is determined by the gut microbial community structure is still not well described. In the present study, we in vitro digested and fermented 48 different foods that were submitted to different culinary treatments using the stools of lean children, obese children, celiac children and children with an allergy to cow's milk proteins. Their antioxidant capacities were assessed with the DPPH and FRAP assays, and the percentage that each food contributed to their daily antioxidant intake as well as their antioxidant capacity by portion size was inferred. Overall, cereals, fruits and vegetables displayed a higher contribution to their daily antioxidant intake, while tubers, fish and meat exhibited a higher antioxidant capacity by serving size. The food that was fermented in the lean children's and those children that were allergic to cow's milk protein's fecal material, showed a higher antioxidant capacity, which could imply that there is a larger role of the gut microbiota in this area.