Because of the absence of a specific diagnostic test and pathognomonic clinical features, physicians must rely on the presence of specific clinical criteria and laboratory data that support the diagnosis of KD. To help clinicians distinguish KD, KDSS, septic shock, and TSS earlier, we suggest differential diagnosis and treatment guideline.Medical records of immunocompetent patients who […]
Current nutritional management of infants born very preterm results in significant deficiency of the essential fatty acids (FAs) arachidonic acid (ARA) and docosahexaenoic acid (DHA). The impact of this deficit on brain maturation and inflammation mediated neonatal morbidities are unknown. The aim of this study is to determine whether early supply of ARA and DHA […]
Upper endoscopy is an essential tool for diagnosing pediatric gastrointestinal issues. This study aimed to assess the indications, diagnostic yields, concordance between histopathological and endoscopic findings and suitability of upper endoscopies performed at a tertiary university hospital in Jordan.Hospital records of children who underwent upper endoscopy were retrospectively reviewed. Demographics, endoscopic details (e.g., indications, findings […]
N-methyl-D-aspartate (NMDA) receptors are ligand-gated ion channels that mediate excitatory synaptic transmission in the central nervous system. The functional NMDA receptors are heterotetramers consisting mainly of two GluN1 and two GluN2 subunits. GluN2 is encoded by the GRIN2D gene. A few case series have shown that GRIN2D variants are linked to developmental and epileptic encephalopathy. […]
Data on anxiety, depression, and attention-deficit/hyperactivity disorder (ADHD) are lacking for youth with congenital heart disease (CHD), particularly those with simple CHD. This study aims to characterize these disorders in youth with CHD compared to those without CHD.A comparative cross-sectional study was conducted by using the electronic medical records of a large tertiary care hospital […]
Granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody-associated vasculitis that primarily affects small vessels. The disease typically affects the respiratory tract and kidneys but has also been known to involve the gastrointestinal tract, genitourinary tract, thyroid, and liver. Cardiac involvement is rare. Coronary artery aneurysms (CAAs) are an extremely uncommon finding, with only 1 reported […]
Bicuspid aortic valve (BAV) aortopathy is defined by dilation of the aortic root (AoRt) and/or ascending aorta (AsAo), and increases risk for aortic aneurysm and dissection. The effects of medical prophylaxis on aortic growth rates in moderate to severe bicuspid aortopathy have not yet been evaluated. This was a single-center retrospective study of young patients […]
Aortic Z-score (Z-score) is utilized in clinical trials to monitor the effect of medications on aortic dilation rate in Marfan (MFS) patients. Z-scores are reported in relation to body surface area (BSA) and therefore are a function of height and weight. However, an information void exists regarding natural, non-pharmacological changes in Z-scores as children age. […]
It is proven that adverse intrauterine environment results in ‘early life programming,’ alterations in metabolism and physiological development of the fetus, often termed as ‘Developmental Origins of Health and Disease’ (DOHaD) resulting in a smaller size at birth, greater non-communicable diseases (NCD) risk factors during childhood and adolescence, and cardiometabolic disorders in adulthood. Nevertheless, very […]
Bazett formula is frequently used in paediatric screening for the long QT syndrome (LQTS) and proposals exist that using standing rather than supine electrocardiograms (ECG) improves the sensitivity of LQTS diagnosis. Nevertheless, compared to adults, children have higher heart rates (especially during postural provocations) and Bazett correction is also known to lead to artificially prolonged […]
Pulmonary hypertension is a deadly complication of bronchopulmonary dysplasia, the most common pulmonary morbidity of prematurity. Despite these catastrophic consequences, no evidence-based therapies are available for the prevention of pulmonary hypertension in this population. Sildenafil is a potent pulmonary vasodilator approved by the US Food and Drug Administration for the treatment of pulmonary hypertension in […]
To establish novel facial characteristics unique to congenital rubella syndrome (CRS) as prediagnostic criteria to supplement disease diagnosis in patients with or without a history of maternal rubella infection.An analysis of 115 CRS case series (2018-2020) based on the presence of any of the triad features.Outpatient department of a tertiary care referral cardiac hospital in […]
Necrotizing enterocolitis (NEC) is a devastating disease that is relatively frequently diagnosed in term infants with congenital heart disease (CHD), compared with term infants without CHD, in whom NEC is rare. The exact pathogenesis of NEC in term infants with CHD is unknown, but it is hypothesized that ischemia of the intestines plays a pivotal […]
Kangaroo mother care (KMC) is an effective intervention for preterm and low birth weight infants. Effective implementation of KMC relies on a multidisciplinary team centering on the newborn’s caregiver, who delivers care with support from health care workers. This study explored the experiences of caregivers on the implementation of KMC.We conducted a descriptive qualitative study […]
Cardiovascular anomalies are the largest group of congenital anomalies and the major cause of death in young children, with various data linking rising atrial septal defect incidence (ASDI) with prenatal cannabis exposure. Objectives / Hypotheses. Is cannabis associated with ASDI in USA? Is this relationship causal?Geospatiotemporal cohort study, 1991-2016. Census populations of adults, babies, congenital […]
Although continuous subcutaneous insulin infusion therapy (ie, insulin pump therapy) is associated with improved metabolic control compared with multiple daily insulin injections in children with type 1 diabetes, it is unclear when it is best to start it after diagnosis. In this study, we aimed to compare the outcomes between early and delayed start of […]
Hemiscrotal agenesis (HSA) is an exceedingly rare congenital anomaly in scrotal development. It is characterized by unilateral absence of scrotal skin with intact midline raphe. In the English literature, only seven patients were diagnosed with HSA. Herein, we report a 14-month-old boy with HSA, unilateral cryptorchidism and a perineal skin tag. Additionally, the patient had […]
Galectin-3 is a new biomarker, which plays an important role in tissue inflammation, cardiac remodeling, and fibrosis. It can be readily measured in the circulation to detect early heart failure (HF). This study aimed to assess the value of galectin-3 assay in early diagnosis of children with heart failure secondary to congenital heart disease (CHD) […]
Parents of adolescents and young adults (AYAs) with serious illness experience enormous stress as they navigate their child’s illness. In this study, we aimed to elucidate AYA parental perspectives on the advanced cancer experience, including what parents find challenging and their sources of strength.Parents of AYAs aged 14 to 24 years old being treated for […]
Changes in left ventricular structure and function have been previously described in children with obstructive sleep apnea (OSA). We aimed to determine if these structural and functional cardiac changes are reversible after treatment of OSA with adenotonsillectomy. Children aged 5-13 years with OSA and matched healthy controls were recruited. Adenotonsillectomy occurred within 1 month after […]
Noonan syndrome is a multiorgan system disorder mediated by genetic defects along the rat sarcoma-mitogen-activated protein kinase (RAS-MAPK) pathway known as RASopathies. It is the second most common syndromic cause of congenital heart disease and, in ∼20% of the cases, is associated with severe lymphatic disorders, including chylothorax and protein-losing enteropathy. Recently, we reported on […]
With improvements in survival rates in newborns with congenital heart defects (CHDs), focus has now shifted toward enhancing neurodevelopmental outcomes across their life span.To systematically review the prevalence and extent of motor difficulties in infants, children, and adolescents with CHD requiring open-heart surgery.Data sources included Embase, Medline and the Cumulative Index to Nursing and Allied […]
Multisystem inflammatory syndrome in children (MIS-C) has spread through the pediatric population during the coronavirus disease 2019 pandemic. Our objective for the study was to report the prevalence of conduction anomalies in MIS-C and identify predictive factors for the conduction abnormalities.We performed a single-center retrospective cohort study of pediatric patients <21 years of age presenting […]
Prevalence of hypertension (both primary and secondary) in children and adolescents in the US ranges from 3% to 4%. Primary hypertension in children and adolescents occurs primarily in children older than 13 years and has no known cause but is associated with several risk factors, including family history and higher body mass index. Secondary hypertension […]
We describe characteristics and outcomes of contemporary pediatric hypertrophic cardiomyopathy (PHC) patients. We studied 398 consecutive pediatric HC patients (<18 years, median 14 years, 65% boys) seen at our center between 2002-2018. Baseline clinical and pediatric echocardiographic data was collected. Left ventricular outflow tract gradient (LVOTG), LV fractional shortening and Z-score for left ventricular (LV) […]
Our aim was to assess the risk of cardiovascular disease (CVD) mortality in US 5-year survivors of adolescent and young adult (AYA) cancer compared with those of the general population and contemporaneous 5-year survivors of childhood cancer.A total of 160 834 5-year AYA cancer survivors (aged 15-39 years at diagnosis) were included, representing 2 239 […]
Women with Turner syndrome (TS) have high prevalence of cardiovascular anomalies. Literature suggests pregnancy is associated with a higher dissection risk, presumably preceded by aortic dilatation. Whether the aortic diameter truly changes during pregnancy in TS is not well investigated. This study aims to evaluate ascending aortic diameter change during pregnancy and reports on cardiac […]
Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled […]
A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort.To describe the associations, symptoms and impact of prenatally diagnosed DAA.Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment […]
This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention.This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 […]
Postnatal ductal closure is stimulated by rising oxygen tension and withdrawal of vasodilatory mediators (prostaglandins, nitric oxide, adenosine) and by vasoconstrictors (endothelin-1, catecholamines, contractile prostanoids), ion channels, calcium flux, platelets, morphologic maturity, and a favorable genetic predisposition. A persistently patent ductus arteriosus (PDA) in preterm infants can have clinical consequences. Decreasing pulmonary vascular resistance, especially […]
Acute flaccid myelitis (AFM) is characterised by rapid onset of limb weakness with spinal cord grey-matter abnormalities on MRI scan. We aimed to assess whether detection of enterovirus in respiratory or other specimens can help predict prognosis in children with AFM.In this nationwide, longitudinal study, we evaluated the significance of detection of enterovirus in any […]
To determine ifVery low dose mydriatic eye microdrop regimen sufficiently dilates the pupil (above 4.1 mm) compared with the currently used low dose mydriatic eye microdrop regimen.Cardiovascular, gastrointestinal and respiratory adverse effects occur following eye drop instillation.Seventeen premature infants were recruited into this prospective, randomised controlled pilot trial in January 2017 to November 2018. Data were […]
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 […]
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 […]
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 […]
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 […]
Evolocumab, a fully human monoclonal antibody directed against proprotein convertase subtilisin-kexin type 9, is widely used in adult patients to lower low-density lipoprotein (LDL) cholesterol levels. Its effects in pediatric patients with heterozygous familial hypercholesterolemia are not known.We conducted a 24-week, randomized, double-blind, placebo-controlled trial to evaluate the efficacy and safety of evolocumab in pediatric […]
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 […]
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