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The traditional healthcare model is focused on diseases (medicine and natural science) and does not acknowledge patients' resources and abilities to be experts in their own lives based on their lived experiences. Improving healthcare safety, quality, and coordination, as well as quality of life, is an important aim in the care of patients with chronic conditions. Person-centered care needs to ensure that people's values and preferences guide clinical decisions. This paper reviews current knowledge to develop (1) digital care pathways for rhinitis and asthma multimorbidity and (2) digitally enabled, person-centered care. 1 It combines all relevant research evidence, including the so-called real-world evidence, with the ultimate goal to develop digitally enabled, patient-centered care. The paper includes (1) Allergic Rhinitis and its Impact on Asthma (ARIA), a 2-decade journey, (2) Grading of Recommendations, Assessment, Development and Evaluation (GRADE), the evidence-based model of guidelines in airway diseases, (3) mHealth impact on airway diseases, (4) From guidelines to digital care pathways, (5) Embedding Planetary Health, (6) Novel classification of rhinitis and asthma, (7) Embedding real-life data with population-based studies, (8) The ARIA-EAACI (European Academy of Allergy and Clinical Immunology) strategy for the management of airway diseases using digital biomarkers, (9) Artificial intelligence, (10) The development of digitally enabled, ARIA person-centered care, and (11) The political agenda. The ultimate goal is to propose ARIA 2024 guidelines centered around the patient to make them more applicable and sustainable., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

In recent years, non-small cell lung cancer treatment has been revolutionized. EGFR tyrosine kinase inhibitors and our improved understanding of its alterations have driven new diagnostic strategies. Liquid biopsies have emerged as a useful tool in these contexts, showing potential utility in early diagnosis combined with low-dose CT scans, as well as potential in monitoring treatment response and predicting the development of patients. We studied the circulating tumor DNA (ctDNA) of 38 EGFR -mutated non-small cell lung cancer patients at diagnosis in different moments of their disease by liquid biopsy techniques. Our results show that mean overall survival was significantly lower when a liquid biopsy was positive for the detection of EGFR mutations compared with wild-type patients in their liquid biopsy in both univariate (29 ± 4 vs. 104 ± 19 months; p = 0.004) and multivariate analysis ( p = 0.008). Taking this into consideration, liquid biopsies could be key to improving the control of this disease.

Asthma, rhinitis, and atopic dermatitis (AD) are interrelated clinical phenotypes that partly overlap in the human interactome. The concept of "one-airway-one-disease," coined over 20 years ago, is a simplistic approach of the links between upper- and lower-airway allergic diseases. With new data, it is time to reassess the concept. This article reviews (i) the clinical observations that led to Allergic Rhinitis and its Impact on Asthma (ARIA), (ii) new insights into polysensitization and multimorbidity, (iii) advances in mHealth for novel phenotype definitions, (iv) confirmation in canonical epidemiologic studies, (v) genomic findings, (vi) treatment approaches, and (vii) novel concepts on the onset of rhinitis and multimorbidity. One recent concept, bringing together upper- and lower-airway allergic diseases with skin, gut, and neuropsychiatric multimorbidities, is the "Epithelial Barrier Hypothesis." This review determined that the "one-airway-one-disease" concept does not always hold true and that several phenotypes of disease can be defined. These phenotypes include an extreme "allergic" (asthma) phenotype combining asthma, rhinitis, and conjunctivitis. Rhinitis alone and rhinitis and asthma multimorbidity represent two distinct diseases with the following differences: (i) genomic and transcriptomic background (Toll-Like Receptors and IL-17 for rhinitis alone as a local disease; IL-33 and IL-5 for allergic and non-allergic multimorbidity as a systemic disease), (ii) allergen sensitization patterns (mono- or pauci-sensitization versus polysensitization), (iii) severity of symptoms, and (iv) treatment response. In conclusion, rhinitis alone (local disease) and rhinitis with asthma multimorbidity (systemic disease) should be considered as two distinct diseases, possibly modulated by the microbiome, and may be a model for understanding the epidemics of chronic and autoimmune diseases., (© 2023 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

MASK-air ® , a validated mHealth app (Medical Device regulation Class IIa) has enabled large observational implementation studies in over 58,000 people with allergic rhinitis and/or asthma. It can help to address unmet patient needs in rhinitis and asthma care. MASK-air ® is a Good Practice of DG Santé on digitally-enabled, patient-centred care. It is also a candidate Good Practice of OECD (Organisation for Economic Co-operation and Development). MASK-air ® data has enabled novel phenotype discovery and characterisation, as well as novel insights into the management of allergic rhinitis. MASK-air ® data show that most rhinitis patients (i) are not adherent and do not follow guidelines, (ii) use as-needed treatment, (iii) do not take medication when they are well, (iv) increase their treatment based on symptoms and (v) do not use the recommended treatment. The data also show that control (symptoms, work productivity, educational performance) is not always improved by medications. A combined symptom-medication score (ARIA-EAACI-CSMS) has been validated for clinical practice and trials. The implications of the novel MASK-air ® results should lead to change management in rhinitis and asthma., (© 2023 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Background: Multisystem inflammatory syndrome in children (MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular dysfunction., Aim: To determine whether the age-adjusted N-terminal pro-brain natriuretic peptide (NT-proBNP) value (Z-log-NT-proBNP) is associated with severe MIS-C and myocardial dysfunction., Methods: A retrospective study was conducted which included children with MIS-C managed at our institution between April 1, 2020, and February 28, 2022. We divided the population into groups depending on severity based on pediatric intensive care unit (PICU) admission. We compared Z-log-NT-proBNP values across these groups and analyzed Z-log-NT-proBNP dynamics during the one-month follow-up., Results: We included 17 participants [median age 3 (2-9) years] and seven (41%) required PICU admission. All (100%) of these cases presented very high (Z-log > 4) levels of NT-proBNP at the time of admission compared to only 5 (50%) patients with non-severe MIS-C ( P = 0.025). NT-proBNP was significantly correlated with high-sensitive Troponin I levels ( P = 0.045), Ross modified score ( P = 0.003) and left ventricle ejection fraction ( P = 0.021)., Conclusion: Raised NT-proBNP, specifically very high values (Z-log-NT-proBNP > 4) could help in the early identification of MIS-C patients with myocardial dysfunction requiring inotropic support and PICU admission., Competing Interests: Conflict-of-interest statement: We have no financial relationships or any other conflict of interest to disclose., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

X-linked hypophosphatemia (XLH) is a rare genetic disorder with X-linked dominant inheritance. Mutations in the PHEX gene increase fibroblast growth factor 23 (FGF23) concentrations, causing loss of phosphorus at the proximal tubule. Most pediatric patients debut in the first two years with short stature and bowed legs. Conventional treatment consists of oral supplements with phosphorus and calcitriol. Since 2018, burosumab has been approved as a novel therapeutic option for XLH, with promising results. The purpose of this study was to share our experience with two cases of XLH treated with burosumab. These patients presented with a broad phenotypical differences. One had the most severe radiological phenotype and developed left ventricular hypertrophy (LVH) and left ventricular dysfunction with preserved ejection fraction. Treatment with burosumab was well-tolerated and was followed by radiological stability and a striking improvement in both blood biochemistry and quality of life. The LVH was stable and left ventricular function normalized in the patient with cardiac involvement. In recent years many studies have been carried out to explain the role of FGF23 in cardiovascular damage, but the exact pathophysiological mechanisms are as yet unclear. The most intensively studied populations are patients with XLH or chronic kidney disease, as both are associated with high levels of FGF23. To date, cardiovascular involvement in XLH has been described in patients treated with conventional treatment, so it would be of interest to investigate if early use of burosumab at the time of diagnosis of XLH would prevent the occurrence of cardiovascular manifestations.

We aimed to delineate cardiopulmonary interactions in acute bronchiolitis and to evaluate the capacity of a combined cardiopulmonary ultrasonography to predict the need for respiratory support. This was a prospective observational single-center study that includes infants <12 month of age admitted to a hospital due to acute bronchiolitis. All the included patients underwent clinical, laboratory and cardiopulmonary ultrasonographic evaluation at the same time point within 24 h of hospital admission. The existence of significant correlation between cardiac and respiratory parameters was the primary outcome. The association of different cardiopulmonary variables with the need of respiratory support higher than O 2 , the length of stay hospitalization, the PICU stay and the duration of respiratory support were a secondary outcome. We enrolled 112 infants (median age 1 (0.5-3) months; 62% males) hospitalized with acute bronchiolitis. Increased values of the pulmonary variables (BROSJOD score, pCO 2 and LUS) showed moderate correlations with NT-proBNP and all echocardiographic parameters indicative of pulmonary hypertension and myocardial dysfunction (Tei index). Up to 36 (32%) infants required respiratory support during the hospitalization. This group presented with higher lung ultrasound score ( p < 0.001) and increased values of NT-proBNP ( p < 0.001), the Tei index ( p < 0.001) and pulmonary artery pressures ( p < 0.001). All the analyzed respiratory and cardiac variables showed moderate-to-strong correlations with the LOS of hospitalization and the time of respiratory support. Lung ultrasound and echocardiography showed a moderate-to-strong predictive accuracy for the need of respiratory support in the ROC analysis, with the AUC varying from 0.74 to 0.87. Those cases of bronchiolitis with a worse pulmonary status presented with a more impaired cardiac status. Cardiopulmonary ultrasonography could be a useful tool to easily identify high-risk populations for complicated acute bronchiolitis hospitalization.

We present the rare case of lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone therapy in an infant with West syndrome, highlighting their relatively benign nature and good prognosis in children, and the relevance of the differential diagnosis with more dangerous cardiac masses in order to avoid aggressive diagnostic and therapeutic interventions.

Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin-angiotensin-aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration.

Background: Since the beginning of the pandemic, coronavirus disease-2019 (COVID-19) in children has shown milder cases and a better prognosis than adults. Although the respiratory tract is the primary target for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), cardiovascular involvement is emerging as one of the most significant and life-threatening complications of SARS-CoV-2 infection in adults., Aim: To summarize the current knowledge about the potential cardiovascular involvement in pediatric COVID-19 in order to give a perspective on how to take care of them during the current pandemic emergency., Methods: Multiple searches in MEDLINE, PubMed were performed using the search terms "COVID-19" or "SARS-CoV-2" were used in combination with "myocardial injury" or "arrhythmia" or "cardiovascular involvement" or "heart disease" or "congenital heart disease" or "pulmonary hypertension" or "long QT" or "cardiomyopathies" or "channelopathies" or "Multisystem inflammatory system" or "PMIS" or "MIS-C" or "Pediatric multisystem inflammatory syndrome" or "myocarditis" or "thromboembolism to identify articles published in English language from January 1st, 2020 until July 31st, 2020. The websites of World Health Organization, Centers for Disease control and Prevention, and the Johns Hopkins Coronavirus Resource Center were reviewed to provide up to date numbers and infection control recommendations. Reference lists from the articles were reviewed to identify additional pertinent articles. Retrieved manuscripts concerning the subject were reviewed by the authors, and the data were extracted using a standardized collection tool. Data were subsequently analyzed with descriptive statistics. For Pediatric multisystemic inflammatory syndrome temporally associated with COVID-19 (PMIS), multiple meta-analyses were conducted to summarize the pooled mean proportion of different cardiovascular variables in this population in pseudo-cohorts of observed patients., Results: A total of 193 articles were included. Most publications used in this review were single case reports, small case series, and observational small-sized studies or literature reviews. The meta-analysis of 16 studies with size > 10 patients and with complete data about cardiovascular involvement in children with PMIS showed that PMIS affects mostly previously healthy school-aged children and adolescents presenting with Kawasaki disease-like features and multiple organ failure with a focus on the heart, accounting for most cases of pediatric COVID-19 mortality. They frequently presented cardiogenic shock (53%), ECG alterations (27%), myocardial dysfunction (52%), and coronary artery dilation (15%). Most cases required PICU admission (75%) and inotropic support (57%), with the rare need for extracorporeal membrane oxygenation (4%). Almost all of these children wholly recovered in a few days, although rare deaths have been reported (2%). Out of PMIS cases we identified 10 articles reporting sporadic cases of myocarditis, pulmonary hypertension and cardiac arrythmias in previously healthy children. We also found another 10 studies reporting patients with pre-existing heart diseases. Most cases consisted in children with severe COVID-19 infection with full recovery after intensive care support, but cases of death were also identified. The management of different cardiac conditions are provided based on current guidelines and expert panel recommendations., Conclusion: There is still scarce data about the role of cardiovascular involvement in COVID-19 in children. Based on our review, children (previously healthy or with pre-existing heart disease) with acute COVID-19 requiring hospital admission should undergo a cardiac workup and close cardiovascular monitoring to identify and treat timely life-threatening cardiac complications., Competing Interests: Conflict-of-interest statement: Authors declare no conflict of interest for this article., (©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.)

We present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.

This study aims to systematically review the incidence of myocardial strain detected by echocardiography in previously healthy infants with acute bronchiolitis and its role as a predictor for adverse outcomes in this setting., Methods: Pubmed/Medline, Excerpta Medica Data Base (EMBASE), and Cochrane Library were searched in April 2020 to identify original observational prospective studies that systematically performed echocardiography for the screening of myocardial strain in healthy infants with acute bronchiolitis. Pooled estimates were generated using random-effects models. Heterogeneity within studies was assessed using Cochran's Q and I 2 statistics. Funnel plots and Egger´s regression method were constructed to evaluate publication bias. Sensitivity analyses were also conducted to evaluate potential sources of heterogeneity., Results: After a detailed screening of 305 articles, a total of 10 studies with 395 participants (mean of 40 participants per study) was included. Five of them were classified as high-quality studies. Up to 28% of cases presented adverse outcomes. The echocardiographic screening for myocardial strain was performed within the first 24 h of admission in 92% cases. Tissue Doppler imaging and Speckle-Tracking echocardiography were performed only in 20% of cases. The presence of pulmonary hypertension was evaluated with methods different from the tricuspid regurgitation jet in 64% of cases. Seven studies found some grade of myocardial strain with a pooled incidence of 21% (CI 95%, 11-31%), in the form of pulmonary hypertension (pooled incidence of 20% (CI 95%, 11-30%)), and myocardial dysfunction (pooled incidence of 5% (CI 95%, 1-9%)). The presence of these echocardiographic alterations was associated with adverse outcomes (pooled relative risk = 16; CI 95%, 8.2-31.5). After a subgroup analysis based on the echocardiographic techniques used, no significant heterogeneity across the studies was observed. There was no evidence of publication bias when assessed by Egger´s test. Cardiac biomarkers to assess myocardial strain were used in five studies. Only N-terminal-pro-brain natriuretic peptide accurately predicted the presence of myocardial strain by echocardiography., Conclusions: Myocardial strain is not infrequent in previously healthy infants with acute bronchiolitis, and it could be present at the early stages of the disease with prognostic implications. There is a need for sufficiently powered prospective studies with a similar methodology, preferably employing advanced imaging techniques, to conclusively address the usefulness of the assessment of myocardial strain in this setting.

Asymptomatic VPE refers to the presence of this abnormal ECG pattern in the absence of any symptoms. The natural history in these patients is usually benign, and most children (60%) with VPE are usually asymptomatic. However, Sudden Cardiac Death (SCD) has been reported to be the initial symptom in many patients too. The increased risk of SCD is thought to be due to the rapid conduction of atrial arrhythmias to the ventricle, via the AP, which degenerates into Ventricular Fibrillation (VF). The best method to identify high-risk patients with asymptomatic VPE for SCD is the characterization of the electrophysiological properties of the AP through an Electrophysiological Study (EPS). Also, catheter ablation of the AP with radiofrequency as definitive treatment to avoid SCD can be performed by the same procedure with high rates of success. However, the uncertainty over the absolute risk of SCD, the poor positive predictive value of an invasive EPS, and complications associated with catheter ablation have made the management of asymptomatic VPE challenging, even more in those children younger than 8-year-old, where there are no clear recommendations. This review provides an overview of the different methods to make the risk stratification for SCD in asymptomatic children with, as well as our viewpoint on the adequate approach to those young children not included in current guidelines., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Exposure of developing rats to noise has shown to induce hippocampal-related behavioral alterations that were prevented after a week of housing in an enriched environment. However, neither the effect of repeated exposures nor its impact on key endogenous antioxidants had been studied yet. Thus, the aim of the present work was to reveal novel data about hippocampal oxidative state through the measurement of possible age-related differences in the levels of hippocampal thioredoxins in rats exposed to noise at different developmental ages and subjected to different schemes and housing conditions. In addition, the possibility that oxidative changes could underlie hippocampal-related behavioral changes was also analyzed. Developing male Wistar rats were exposed to noise for 2 h, either once or for 5 days. Upon weaning, some animals were transferred to an enriched cage for 1 week, whereas others were kept in standard cages. One week later, auditory and behavioral assessments, as well as measurement of hippocampal thioredoxin, were performed. Whereas no changes in the auditory function were observed, significant behavioral differences were found, that varied according to the age, scheme of exposure and housing condition. In addition, a significant increase in Trx-1 levels was found in all noise-exposed groups housed in standard cages. Housing animals in an enriched environment for 1 week was effective in preventing most of these changes. These findings suggest that animals become less susceptible to undergo behavioral alterations after repeated exposure to an environmental challenge, probably due to the ability of adaptation to an unfavorable condition. Moreover, it could be hypothesized that damage to younger individuals could be more easily prevented by a housing manipulation.

Aim: To investigate whether the presence of left ventricular myocardial dysfunction (LVMD) assessed by Tei index (LVTX) impacts the outcomes of healthy infants with Respiratory Syncytial Virus Bronchiolitis (RSVB). To explore whether N-terminal pro-B-type natriuretic peptide (NT-proBNP) increases the accuracy of traditional clinical markers in predicting the outcomes., Methods: A single-centre, prospective, cohort study including healthy infants aged 1-12 months old admitted for RSVB between 1 October 2016 and 1 April 2017. All patients underwent clinical, laboratory and echocardiographic evaluation within 24 h of admission. Paediatric intensive care unit (PICU) admission was defined as severe disease., Results: We enrolled 50 cases of RSVB (median age of 2 (1-6.5) months; 40% female) and 50 age-matched controls. We observed higher values of LVTX in infants with RSVB than in controls (0.42 vs. 0.36; p = 0.008). Up to nine (18%) children presented with LVMD (LVTX > 0.5), with a higher incidence of PICU admission (89% vs. 5%; p < 0.001). The diagnostic performance of NT-proBNP in predicting LVMD was high (area under the receiver operator characteristic curve (AUC) 0.95, CI 95% 0.90-1). The diagnostic yield of the predictive model for PICU admission that included NT-proBNP was excellent (AUC 0.945, CI 95% 0.880-1), and significantly higher than the model without NT-proBNP (p = 0.026)., Conclusions: LVMD could be present in healthy infants with RSVB who develop severe disease. NT-proBNP seems to improve traditional clinical markers for outcomes., Competing Interests: The authors declare no conflict of interest.

Aim: To explore NT-proBNP as biomarker for pulmonary hypertension (PH) in infants with respiratory syncytial virus infection (RSVI). Patients & methods: We prospectively enrolled 93 healthy infants with RSVI aged 1-12 months. NT-proBNP determination and echocardiography were performed at admission. Results: PH was found in 22% of patients and associated with a severe course of the disease. NT-proBNP >1635 pg/ml resulted an independent predictor for PH (odds ratio: 16.46 [95% CI: 4.10-66; p < 0.001]). The diagnostic performance of NT-proBNP to detect PH in RSVI was high (area under receiver operator curve of 0.932 [95% CI: 0.883-0.981; p < 0.001]). Conclusions: The presence of PH in healthy infants with RSVI is associated with worse outcomes. NT-proBNP resulted an accurate biomarker for PH in this setting.

Background: Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. The initial diagnosis of myocarditis is usually based on clinical presentation, but this widely ranges from the severe sudden onset of a cardiogenic shock to asymptomatic patients. Early recognition is essential in order to monitor and start supportive treatment prior to the development of severe adverse events. Of note, many cases of fulminant myocarditis are usually misdiagnosed as otherwise minor conditions during the weeks before the unexpected deterioration., Aim: To provide diagnostic clues to make an early recognition of pediatric myocarditis. To investigate early predictors for poor outcomes., Methods: We conducted a retrospective cross-sectional single-center study from January 2008 to November 2017 at the Pediatric Department of our institution, including children < 18-years-old diagnosed with myocarditis. Poor outcome was defined as the occurrence of any of the following facts: death, heart transplant, persistent left ventricular systolic dysfunction or dilation at hospital discharge (early poor outcome), or after 1 year of follow-up (late poor outcome). We analyzed different clinical features and diagnostic test findings in order to provide diagnostic clues for myocarditis in children. Multivariable stepwise logistic regression analysis was performed using all variables that had been selected by univariate analysis to determine independent factors that predicted a poor early or late outcome in our study population., Results: A total of 42 patients [69% male; median age of 8 (1.5-12) years] met study inclusion criteria. Chest pain (40%) was the most common specific cardiac symptom. Respiratory tract symptoms (cough, apnea, rhinorrhea) (38%), shortness of breath (35%), gastrointestinal tract symptoms (vomiting, abdominal pain, diarrhea) (33%), and fever (31%) were the most common non-cardiac initial complaints. Tachycardia (57%) and tachypnea (52%) were the most common signs on the initial physical exam followed by nonspecific signs of respiratory tract infection (44%) and respiratory distress (35%). Specific abnormal signs of heart failure such as heart murmur (26%), systolic hypotension (24%), gallop rhythm (20%), or hepatomegaly (20%) were less prevalent. Up to 43% of patients presented an early poor outcome, and 16% presented a late poor outcome. In multivariate analysis, an initial left ventricular ejection fraction (LVEF) < 30% remained the only significant predictor for early [odds ratio (OR) (95%CI) = 21 (2-456), P = 0.027) and late [OR (95%CI) = 8 (0.56-135), P = 0.047) poor outcome in children with myocarditis. LVEF correlated well with age ( r = 0.51, P = 0.005), days from the initiation of symptoms ( r = -0.31, P = 0.045), and N-terminal pro-brain natriuretic peptide levels ( r = 0.66, P < 0.001), but not with troponin T ( r = -0.05, P = 0.730) or C-reactive protein levels ( r = -0.13, P = 0.391). N-terminal pro-brain natriuretic peptide presented a high diagnostic accuracy for LVEF < 30% on echocardiography with an area under curve of 0.931 (95%CI: 0.858-0.995, P < 0.001). The best cut-off point was 2000 pg/mL with a sensitivity of 90%, specificity of 81%, positive predictive value of 60%, and negative predictive value of 96%., Conclusion: The diagnosis of myocarditis in children is challenging due to the heterogeneous and unspecific clinical presentation. The presence of LVEF < 30% on echocardiography on admission was the major predictor for poor outcomes. Younger ages, a prolonged course of the disease, and N-terminal pro-brain natriuretic peptide levels could help to identify these high-risk patients., Competing Interests: Conflict-of-interest statement: All authors declare no conflicts-of-interest related to this article.

Kawasaki disease (KD) is a systemic childhood vasculitis with peculiar tropism for the heart. Coronary artery aneurysms are the primary cause of morbidity and mortality in these patients. The timely administration of gammaglobulin decreases the risk for development of coronary artery aneurysms, highlighting the importance of early KD recognition. However, the most significant dilemma in the management of KD is the diagnosis itself. In this article, we review the recent literature focusing on the diagnostic utility of N-terminal probrain natriuretic peptide as a biomarker for diagnosis of KD. The main conclusion is that N-terminal probrain natriuretic peptide is an useful biomarker for KD diagnostic that represents a valuable addition to the current diagnostic workup of patients with suspected KD, increasing the diagnostic accuracy.

In approximately 5% of patients with idiopathic recurrent pericarditis, the disease usually follows a chronic relapsing course, and children can develop dependence and side effects of prolonged high-dose corticosteroid regimens. In this setting anakinra, a recombinant human interleukin-1 competitive receptor antagonist that blocks the biologic effects of interleukin-1, thereby reducing systemic inflammatory responses, appears to be one of the most promising strategies. We report an adolescent with steroid-dependent idiopathic recurrent pericarditis that was successfully treated with anakinra, highlighting that this therapeutic option seems to be an effective, rapidly acting, steroid-sparing, and relatively safe agent for the treatment of this entity in children.

Background: Multimorbidity in allergic airway diseases is well known, but no data exist about the daily dynamics of symptoms and their impact on work. To better understand this, we aimed to assess the presence and control of daily allergic multimorbidity (asthma, conjunctivitis, rhinitis) and its impact on work productivity using a mobile technology, the Allergy Diary., Methods: We undertook a 1-year prospective observational study in which 4 210 users and 32 585 days were monitored in 19 countries. Five visual analogue scales (VAS) assessed the daily burden of the disease (i.e., global evaluation, nose, eyes, asthma and work). Visual analogue scale levels <20/100 were categorized as "Low" burden and VAS levels ≥50/100 as "High" burden., Results: Visual analogue scales global measured levels assessing the global control of the allergic disease were significantly associated with allergic multimorbidity. Eight hypothesis-driven patterns were defined based on "Low" and "High" VAS levels. There were <0.2% days of Rhinitis Low and Asthma High or Conjunctivitis High patterns. There were 5.9% days with a Rhinitis High-Asthma Low pattern. There were 1.7% days with a Rhinitis High-Asthma High-Conjunctivitis Low pattern. A novel Rhinitis High-Asthma High-Conjunctivitis High pattern was identified in 2.9% days and had the greatest impact on uncontrolled VAS global measured and impaired work productivity. Work productivity was significantly correlated with VAS global measured levels., Conclusions: In a novel approach examining daily symptoms with mobile technology, we found considerable intra-individual variability of allergic multimorbidity including a previously unrecognized extreme pattern of uncontrolled multimorbidity., (© 2018 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Background: Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1-30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%)., Case Report: We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD. The initial diagnosis was not made during the first 10 days of illness (therapeutic window for immunoglobulin treatment) as he was suspected to have hand-mouth-foot disease. We believe that both the delay in diagnosis and treatment of an atypical presentation of KD, combined with the more severe vasculitis and inflammatory burden reported in these cases, contributed to the development of CAA in our patient. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the importance of considering KD diagnosis in children with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP. Atypical cases like this may be at increased risk of CAA because of delayed diagnosis and a higher inflammatory burden; therefore, a more aggressive treatment approach may be necessary., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Coronary vasospasm can result from silent myocardial ischaemia to sudden death. There are many precipitant factors including different pharmacological agents. Kounis syndrome is defined by acute coronary syndromes associated with anaphylactic or anaphylactoid reactions. We report, to the best of our knowledge, the first paediatric case of Kounis syndrome due to intravenous atropine.

Mobile technology has been used to appraise allergic rhinitis control, but more data are needed. To better assess the importance of mobile technologies in rhinitis control, the ARIA (Allergic Rhinitis and its Impact on Asthma) score ranging from 0 to 4 of the Allergy Diary was compared with EQ-5D (EuroQuol) and WPAI-AS (Work Productivity and Activity Impairment in allergy) in 1288 users in 18 countries. This study showed that quality-of-life data (EQ-5D visual analogue scale and WPA-IS Question 9) are similar in users without rhinitis and in those with mild rhinitis (scores 0-2). Users with a score of 3 or 4 had a significant impairment in quality-of-life questionnaires., (© 2017 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Increasing bioactivity and mechanical properties of polymers to produce more suitable scaffold for tissue engineering is a recurrent goal in the development of new biomedical materials. In this study, collagen-functionalized poly (lactic acid), PLA, was obtained by means of a simple grafting route, and electrospun scaffolds were produced to grow cells in vitro ; their bioactivity was compared with scaffolds made of physical blends of PLA and collagen. Grafting was verified via nuclear magnetic resonance, attenuated total reflection-Fourier transform infrared and X-ray photoelectron spectroscopy. The cell adhesion performance of the scaffolds was studied using macrophages. Elastic modulus (74.7 megapascals) and tensile strength (3.0 megapascals) of the scaffold made from PLA grafted with collagen were substantially higher than the scaffolds made from physical blends of collagen and PLA: 32 and 2.16 megapascals, respectively, implying a more resistant material because of the chemical bond of the polypeptide to PLA. Besides, the fibers had more uniform diameter without defects. Scaffolds made from PLA grafted with collagen presented four-fold increase in cell adhesion than those of PLA blended with collagen. Furthermore, cell spreading within the scaffolds occurred only when collagen-functionalized poly (lactic acid) was used. These results open a new option for the easy tailoring of nanofiber-based scaffolds in three dimensions for tissue engineering.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo-8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.

Solitary pleuro-pulmonary fibrous tumours are relatively uncommon neoplasms that are difficult to manage therapeutically and which, cytogenetically, have been poorly studied. The aim of the present work was to analyse the characteristics of a series of consecutive operated solitary pleural fibrous tumours in an attempt to discover a malignant pattern of evolution. This was a retrospective observational study of 19 cases. Samples were studied for clinical, histological, immunohistochemical and cytogenetic characteristics (aCGH, FISH). Descriptive statistics were used: the Kapplan-Meyer log-rank test and the Cox-regression model for survival analysis. Analysis of malignant evolution was achieved using 2x2 tables; significant factors were included in a binary logistic regression model. Parietal pleural implantation of the primary tumour, high mib1 expression, and low p53 expression were seen to be statistically significant factors for survival. We recommend a close follow-up for patients with a malignant primary tumour and low p53 expression and a regular long-term follow-up for benign primary tumours with a high mib1 index, high positive p53, and deletions. These findings need confirmation in more extensive series.