X-linked lymphoproliferative disease (XLP) is a rare inherited X-linked primary immunodeficiency diseases (PID). One such disease, X-linked inhibitor of apoptosis protein (XIAP) deficiency, is characterized by Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). However, EBV-HLH with coronary artery dilation and acute renal injury (AKI) in children is unusual.We report the case of a young boy aged 17 months […]
Ninety percent of intrapartum-related neonatal deaths are attributable to respiratory depression, with the vast majority of these deaths occurring in low- and lower-middle-income countries. Neonatal resuscitation training with Helping Babies Breathe (HBB) decreases mortality from respiratory depression. Cardiorespiratory monitoring in conjunction with HBB can provide valuable resuscitation feedback for both training and bedside purposes. In […]
Bystander training in cardiopulmonary resuscitation (CPR) is crucial to improve the victims’ survival and quality of life after sudden cardiac arrest. This quasi-experimental study aimed to determine the success rate of two different programs of CPR-training for children, adolescents, and adults in school communities. We assessed the development and acquisition of the following CPR-skills: checking […]
Childhood obesity represents a serious global health crisis. Apelin and its receptor system are widely distributed throughout the central nervous system and have been demonstrated to serve a role modulating feeding behaviour and energy homeostasis. The purposes of this study were to examine apelin concentrations and anthropometric-cardiometabolic parameters in obese and non-obese children and to […]
Cardiac T2* MRI (T2*CMR), for accurate estimation of myocardial siderosis, was introduced as part of a QI collaborative to optimise chelation therapy in order to improve cardiac morbidity in transfusion dependent thalassaemia (TDT) patients. We report the impact of this QI initiative from two thalassaemia centres from this collaborative.A key driver based quality initiative was […]
Young children and those with chronic medical conditions are at risk for complications of influenza including cardiopulmonary compromise. Here we aim to examine risks of mortality, clinical complications in children with congenital heart disease (CHD) hospitalized for influenza.We analyzed data from in-hospital pediatric patients from 2003, 2006, 2009, 2012 and 2016 using the nationally representative […]
The Paediatric Admission Guidance in the Emergency Department (PAGE) score is an assessment tool currently in development that helps predict hospital admission using components including patient characteristics, vital signs (heart rate, temperature, respiratory rate and oxygen saturation) and clinical features (eg, breathing, behaviour and nurse judgement). It aims to assist in safe admission and discharge […]
The identification of life-threatening infection in febrile children presenting to the emergency department (ED) remains difficult. The quick Sequential Organ Failure Assessment (qSOFA) was only derived for adult populations, implying an urgent need for pediatric scores. We developed and validated a novel, adapted qSOFA score (Liverpool quick Sequential Organ Failure Assessment [LqSOFA]) and compared its […]
Low socioeconomic status (SES) has emerged as an important risk factor for higher short-term mortality and neurodevelopmental outcomes in children with hypoplastic left heart syndrome and related anomalies; yet little is known about how SES affects these outcomes over the long-term.We linked data from the Single Ventricle Reconstruction trial to US Census Bureau data to […]
Haemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child.A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation […]
Twenty-four patients with bi-allelic familial hypercholesterolemia (FH) commencing chronic lipoprotein apheresis (LA) at a mean age of 8.5 ± 3.1 years were analysed retrospectively and in part prospectively with a mean follow-up of 17.2 ± 5.6 years. Mean age at diagnosis was 6.3 ± 3.4 years. Untreated mean LDL-C concentrations were 752 mg/dl ± 193 […]
International guidelines for resuscitation recommend using positive end-expiratory pressure (PEEP) during ventilation of preterm newborns. Reliable PEEP-valves for self-inflating bags have been lacking, and effects of PEEP during resuscitation of term newborns are insufficiently studied. The objective was to determine if adding a new PEEP valve to the bag-mask during resuscitation of term and near-term […]
To describe neurodevelopment and follow-up services in preschool children with heart disease (HD).Secondary analysis of a prospectively collected multicentre dataset.Three London tertiary cardiac centres.Preschool children<5 years of age: both inpatients and outpatients.We analysed results of Mullen Scales of Early Learning (MSEL) and parental report of follow-up services in a representative convenience sample evaluated between January […]
Severe pulmonary hypertension (PH) causing right heart failure can occur due to thiamine deficiency in exclusively breastfeeding infants. This study describes the clinical profile and management of thiamine-responsive acute pulmonary hypertension.A prospective observational study of infants presenting with severe PH without any other significant heart or lung disease. History of symptoms, clinical examination, echocardiography and […]
Achondroplasia is associated with foramen magnum stenosis (FMS) and significant risk of morbidity and sudden death in infants. A sensitive and reliable method of detecting infants who require decompressive surgery is required. This study aims to describe the incidence and severity of FMS in an unselected, sequential series of infants using a novel MRI score […]
Congenital heart defect is the leading malformation in China. There may have been changes in congenital heart defect incidence because of birth policy shift in China over past years. This study aimed to investigate the epidemiology, prenatal diagnosis, and outcomes of congenital heart disease to improve medical and policy decisions.Data on cases of congenital heart […]
Among children with congenital heart disease (CHD), there is often neurodevelopmental and behavioral impairment with unclear implications regarding adult socioeconomic achievements. We aimed to compare economic self-sufficiency and educational attainment in CHD adults with sibling and general population controls. Using Danish population-based nationwide registries this cohort study aimed to include all CHD subjects greater than […]
To evaluate the effectiveness of two palivizumab programmes targeting high-risk infants, defined by prematurity, diagnosis of comorbidities and geography, and assess potential disparities by neighbourhood income.Controlled, interrupted time series.Ontario, Canada.We used linked health and demographic administrative databases to identify all children born in hospitals 1 January 1993 through 31 December 2016. Follow-up ended at the […]
Improved clinical care has led to an increase in the number of adults with congenital heart disease (CHD) engaging in leisure time and competitive sports activities. Although the benefits of exercise in patients with CHD are well established, there is a low but appreciable risk of exercise-related complications. Published exercise recommendations for individuals with CHD […]
Improved clinical care has led to an increase in the number of adults with congenital heart disease (CHD) engaging in leisure time and competitive sports activities. Although the benefits of exercise in patients with CHD are well established, there is a low but appreciable risk of exercise-related complications. Published exercise recommendations for individuals with CHD […]
Kawasaki disease (KD) causes coronary artery lesions (CAL) and is the leading cause of acquired heart disease in children. The aim of this study is to evaluate the risk factors and set-up a scoring system for predicting CAL of KD.We retrospectively reviewed a total of 478 patients diagnosed with KD. We compared age, gender, laboratory […]
In this report, we determine the cumulative incidence of symptomatic cardiac ischaemia and its risk factors among European 5-year childhood cancer survivors (CCS) participating in the PanCareSurFup study.Eight data providers (France, Hungary, Italy (two cohorts), the Netherlands, Slovenia, Switzerland and the UK) participating in PanCareSurFup ascertained and validated symptomatic cardiac events among their 36 205 […]
An 11-week-old unvaccinated, term Amish boy initially presented with poor feeding, microcephaly, failure to thrive, and developmental delays. His physical examination was significant for both weight and head circumference being less than the third percentile, and he was noted to have micrognathia, truncal hypotonia, and head lag. He was admitted to the pediatric hospital medicine […]
To test the effect of a 4-month telehealth home monitoring program (REACH), layered on usual care, on postdischarge outcomes in parents of infants recovering from cardiac surgery and their infants.Randomized trial of infants discharged from the hospital after cardiac surgery for congenital heart disease. Consecutive infants with complex congenital heart disease undergoing cardiac surgery within […]
Extracorporeal life support (ECLS) has been used for >30 years as a life-sustaining therapy in critically ill patients for a variety of indications. In the current study, we aimed to examine trends in use, mortality, length of stay (LOS), and costs for pediatric ECLS hospitalizations.We performed a retrospective cohort study of pediatric patients (between the […]
To identify and prioritise technical procedures that should be integrated in a curriculum of simulation-based procedural training in paediatrics using the Delphi method.National general needs assessment using a Delphi process was completed among 93 key opinion leaders in paediatrics in Denmark. Delphi round 1 identified technical procedures. Round 2 explored frequency of procedures, number of […]
The International Liaison Committee on Resuscitation Neonatal Life Support Task Force reviewed evidence for the duration of cardiopulmonary resuscitation (CPR) for newborns immediately after birth.To summarize evidence for ongoing CPR on the outcomes of survival, neurodevelopment, and the composite of survival without moderate or severe neurodevelopmental impairment (NDI).Medline, Embase, Evidence-Based Medicine Reviews, Cumulative Index to […]
The optimal approach to screening and diagnosis of prediabetes and diabetes in youth is uncertain.We conducted a cross-sectional analysis of 14 119 youth aged 10 to 19 years in the 1999-2016 NHANES. First, we examined the performance of American Diabetes Association risk-based screening criteria. Second, we evaluated the performance of current clinical definitions of prediabetes and […]
Atrial flutter (AFL) is a supraventricular tachyarrhythmia. In the ECG tracing, it is marked by a fast, irregular atrial activity of 280-500 beats per minute. AFL is known to be a rare and also life-threatening rhythm disorder both at the fetus and neonatal period. AFL may result in circulatory failure, and in a more severe […]
We aimed to evaluate the feasibility of interventional treatment of atrial septal defect (ASD) in low weight infants under 2-year-old. Seven hundred and ninety-three secundum ASD patients were divided into 2 groups: 665 were above 2-year-old and 128 were under 2-year-old. The basic conditions before the operation, postoperative complications within 24 hours, and adverse outcomes […]
Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult […]
Congenital diaphragmatic hernia is a deficiency of the fetal diaphragm resulting in herniation of the abdominal viscera into the thoracic cavity. The best method of respiratory management of congenital diaphragmatic hernia is unclear, but high frequency oscillatory ventilation is often used as the initial ventilator mode for severe congenital diaphragmatic hernia. When it becomes impossible […]
Death is defined biologically as the irreversible loss of the functioning of the organism as a whole, which typically occurs after the loss of cardiorespiratory function. In 1968, a Harvard committee proposed that death could also be defined neurologically as the irreversible loss of brain function. Brain death has been considered to be equivalent to […]
Background Increasing numbers of neonates are undergoing painful procedures in low-income and middle-income countries, with adequate analgesia seldom used. In collaboration with a multi-disciplinary team in Kenya, we aimed to establish the first evidence-based guidelines for the management of routine procedure-related neonatal pain that consider low-resource hospital settings.We did a systematic review by searching MEDLINE, […]
Cardiopulmonary exercise test (CPET) provides accurate evaluation of physical capacity and disease severity in children with congenital heart disease (CHD). However, full participation to obtain optimal measure of VO2max may be difficult. As an alternative, the oxygen uptake efficiency slope (OUES) is a reproducible and reliable parameter measured during CPET, which does not require a […]
Background Increasing numbers of neonates are undergoing painful procedures in low-income and middle-income countries, with adequate analgesia seldom used. In collaboration with a multi-disciplinary team in Kenya, we aimed to establish the first evidence-based guidelines for the management of routine procedure-related neonatal pain that consider low-resource hospital settings.We did a systematic review by searching MEDLINE, […]
Cardiopulmonary exercise test (CPET) provides accurate evaluation of physical capacity and disease severity in children with congenital heart disease (CHD). However, full participation to obtain optimal measure of VO2max may be difficult. As an alternative, the oxygen uptake efficiency slope (OUES) is a reproducible and reliable parameter measured during CPET, which does not require a […]
We aimed to assess the risk factors for left ventricle (LV) enlargement in children with idiopathic frequent ventricular premature complexes (VPC) and discuss the clinical features and treatment strategies. Children diagnosed with idiopathic frequent VPC at Xinhua Hospital affiliated to the Shanghai Jiao Tong University during 2013 to 2019 were retrospectively evaluated. Gender, age, body […]
We sought to evaluate the association between low birth weight (LBW) and outcomes following neonatal cardiac surgery in a low-income and middle-income country setting where LBW prevalence is high and its impact on surgical outcomes is undefined.Single-centre retrospective cohort study.Referral paediatric hospital in Southern India PATIENTS: All neonatal cardiac surgical cases between January 2011 and […]
Paediatric Early Warning Scores (PEWSs) are being used increasingly in hospital wards to identify children at risk of clinical deterioration, but few scores exist that were designed for use in emergency care settings. To improve the prioritisation of children in the emergency department (ED), we developed and validated an ED-PEWS.The TrIAGE project is a prospective […]
Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. Despite early detection of CHD and major advances in paediatric cardiac surgery, ES is still prevalent and requires a multidisciplinary approach by adult CHD experts in tertiary centres. […]
Heart rate (HR) assessment is crucial in neonatal resuscitation, but pulse oximetry (PO) and electrocardiography (ECG) are rarely accessible in low-resource to middle-resource settings. This study evaluated a free-of-charge smartphone application, NeoTap, which records HR with a screen-tapping method bypassing mental arithmetic calculations.This observational study was carried out during three time periods between May 2015 […]
Cardiorespiratory and pulse oximetry monitoring in children who are hospitalized should balance benefits of detecting deterioration with potential harms of alarm fatigue. We developed recommendations for monitoring outside the ICU on the basis of available evidence and expert opinion.We conducted a comprehensive literature search for studies addressing the utility of cardiorespiratory and pulse oximetry monitoring […]
Hereditary hemorrhagic telangiectasia (HHT) can be clinically diagnosed, but children often lack characteristic features. We report a family with homozygous growth differentiation factor 2 (GDF2)-related HHT diagnosed by genetic testing. A boy aged 5 years and 2 months presented with isolated hypoxemia. He was the product of a consanguineous marriage; his parents were second cousins. […]
To assess and compare associations between childhood maltreatment and cardiovascular disease (CVD) in men and women in the UK. In secondary analyses, we also explored possible age differences and associations with early onset CVD (<50 years).We included 157 311 participants from the UK Biobank who had information on physical, sexual or emotional abuse, emotional or physical […]
In April, 2020, clinicians in the UK observed a cluster of children with unexplained inflammation requiring admission to paediatric intensive care units (PICUs). We aimed to describe the clinical characteristics, course, management, and outcomes of patients admitted to PICUs with this condition, which is now known as paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 […]
Highly reliable identification of adults with hypertrophic cardiomyopathy (HC) at risk for sudden death (SD) has been reported. A significant controversy remains, however, regarding the most reliable risk stratification methodology for children and adolescents with HC. The present study assesses the accuracy of SD prediction and prevention with prophylactic implantable cardioverter-defibrillators (ICDs) in young HC […]
Currently, car seat tolerance screens (CSTSs) are recommended for all infants born prematurely in the United States. Although many late-preterm infants are cared for exclusively in newborn nurseries (NBNs), data on implementation of CSTS in nurseries are limited. Our objective for this study was to determine management strategies and potential variation in practice of CSTS […]
Preterm birth is associated with incident heart failure in children and young adults.To determine the effect size of preterm birth on cardiac remodeling from birth to young adulthood.Data sources include Medline, Embase, Scopus, Cochrane databases, and clinical trial registries (inception to March 25, 2020).Studies in which cardiac phenotype was compared between preterm individuals born at […]
To examine how overweight and obesity at specific ages and overall BMI growth patterns throughout childhood predict cardiometabolic phenotypes at 11 to 12 years.In a population-based sample of 5107 infants, BMI was measured every 2 years between ages 2 to 3 and 10 to 11 years. We identified 5 BMI trajectories using growth curve models. […]
13q33-q34 microdeletions are rare chromosomal aberrations associated with a high risk of developmental disability, facial dysmorphism, cardiac defects and other malformation of organs. It is necessary to collect and report evidence of this rare chromosome mutation to improve the prognosis of this rare disease.We report a patient harboring an 11.56 Mb microdeletion at 13q33.1-34 region, which […]
More than 50% of newborns with congenital heart disease (CHD) are unrecognized at birth; however, the use of echocardiogram (Echo) for diagnosing CHD in newborns with asymptomatic, non-syndromic cardiac murmurs (ANCM), has not been systematically reviewed yet. We aimed to identify the incidence of CHD diagnosed with Echo and systematically review whether Echo should be […]
Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on […]
Despite substantial variation of streptococcal antibody titres among global populations, there is no data on normal values in sub-Saharan Africa. The objective of this study was to establish normal values for antistreptolysin O (ASO) and antideoxyribonuclease B (ADB) antibodies in Uganda.This was an observational cross-sectional study.This study was conducted at Mulago National Referral Hospital, which […]
Understanding the epidemiology and clinical course of multisystem inflammatory syndrome in children (MIS-C) and its temporal association with coronavirus disease 2019 (Covid-19) is important, given the clinical and public health implications of the syndrome.We conducted targeted surveillance for MIS-C from March 15 to May 20, 2020, in pediatric health centers across the United States. The […]
A multisystem inflammatory syndrome in children (MIS-C) is associated with coronavirus disease 2019. The New York State Department of Health (NYSDOH) established active, statewide surveillance to describe hospitalized patients with the syndrome.Hospitals in New York State reported cases of Kawasaki’s disease, toxic shock syndrome, myocarditis, and potential MIS-C in hospitalized patients younger than 21 years […]
Sleep-disordered breathing (SDB) is a common syndrome in children, related to their immune responses, cardiovascular function, and neurocognitive function. This study aimed to determine the prevalence of SDB among children in Wuxi, China, and to evaluate the protective and risk factors of SDB in children.A cross-sectional study was conducted on children attending different schools across […]
The prevalence of child and adolescent obesity and severe obesity continues to increase despite decades of policy and research aimed at prevention. Obesity strongly predicts cardiovascular and metabolic disease risk; both begin in childhood. Children who receive intensive behavioral interventions can reduce body mass index (BMI) and reverse disease risk. However, delivering these interventions with […]
Corticosteroids are widely used in pediatric cardiac surgery to blunt systemic inflammatory response and to reduce complications; nevertheless, their clinical efficacy is uncertain.To determine whether intraoperative administration of dexamethasone is more effective than placebo for reducing major complications and mortality during pediatric cardiac surgery.The Intraoperative Dexamethasone in Pediatric Cardiac Surgery was an investigator-initiated, double-blind, multicenter […]
Flecainide acetate is a Vaughan-Williams class IC antiarrhythmic drug prescribed for the treatment of supraventricular arrhythmias. It has a narrow therapeutic index and proarrhythmic effects even at therapeutic doses. Flecainide is metabolized by a CYP2D6 enzyme that exhibits polymorphism. In this case report, we present, to our best knowledge, the first case of toxicity contributed […]
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