Multicenter prospective clinical study to evaluate children short-term neurodevelopmental outcome in congenital heart disease (children NEURO-HEART): study protocol.

Abstract: Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. While advances in early diagnosis and postnatal management have increased survival in CHD children, […]

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Integration of Pediatric Palliative Care Into Cardiac Intensive Care: A Champion-Based Model.

Integration of pediatric palliative care (PPC) into management of children with serious illness and their families is endorsed as the standard of care. Despite this, timely referral to and integration of PPC into the traditionally cure-oriented cardiac ICU (CICU) remains variable. Despite dramatic declines in mortality in pediatric cardiac disease, key challenges confront the CICU community. Given increasing comorbidities, technological dependence, lengthy recurrent hospitalizations, and interventions risking significant morbidity, many patients in the CICU would benefit from PPC involvement across the illness trajectory. Current PPC delivery models have inherent disadvantages, insufficiently address the unique aspects of the CICU setting, place significant burden on subspecialty PPC teams, and fail to use CICU clinician skill sets. We therefore propose a novel conceptual framework for PPC-CICU integration based on literature review and expert interdisciplinary, multi-institutional consensus-building. This model uses interdisciplinary CICU-based champions who receive additional PPC training through courses and subspecialty rotations. PPC champions strengthen CICU PPC provision by (1) leading PPC-specific educational training of CICU staff; (2) liaising between CICU and PPC, improving use of support staff and encouraging earlier subspecialty PPC involvement in complex patients’ management; and (3) developing and implementing quality improvement initiatives and CICU-specific PPC protocols. Our PPC-CICU integration model is designed for adaptability within institutional, cultural, financial, and logistic constraints, with potential applications in other pediatric settings, including ICUs. Although the PPC champion framework offers several unique advantages, barriers to implementation are anticipated and additional research is needed to investigate the model’s feasibility, acceptability, and efficacy.

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Neonatal meningitis, endocarditis, and pneumonitis due to Streptococcus gallolyticus subsp. pasteurianus: a case report.

Streptococcus pasteurianus is a rare cause of neonatal infection, with only 3 cases reported in the USA and 18 cases reported in other countries within the past decade. Neonatal S. pasteurianus infection typically presents as meningitis. This case report describes the first neonatal case of S. pasteurianus endocarditis in the literature, in addition to a neonatal case of S. pasteurianus infection presenting as pneumonitis without meningitis. The S. pasteurianus infections in these two cases are unusual not only because of how rare this particular pathogen is, but also because of the atypical clinical manifestations.The first patient is a full-term male infant admitted to NICU at 20?h of life due to respiratory distress. He was empirically started on ampicillin and gentamicin for presumed sepsis. Laboratory analysis of cerebral spinal fluid obtained after initiation of antibiotics was suggestive of partially treated meningitis. Blood cultures came back positive for S. pasteurianus. The neonate was transitioned from ampicillin to cefepime, while gentamicin was continued. Echocardiograph showed a possible tricuspid valve vegetation concerning for endocarditis. Due to the unusual complication of endocarditis, the patient remained on IV cefepime for 28?days rather than the more conventional duration of 14-21?days reported in the literature. The baby clinically improved with no evidence of thrombi or vegetations on repeat cardiac echo. The second patient is a full-term male infant who required intubation at birth for respiratory distress. Chest X-ray findings were concerning for meconium aspiration with pneumonitis. The baby went into septic shock and was empirically started on ampicillin and gentamicin. Blood cultures came back positive for S. pasteurianus, while cerebral spinal fluid and urine cultures were negative. Ampicillin and gentamicin were discontinued after 3?days and the baby was started on cefepime and clindamycin for a total 14-day course. The baby clinically recovered and was discharged from NICU without any sequelae.These two cases highlight the importance of recognizing S. pasteurianus as a potential cause of neonatal sepsis and the importance of recognizing endocarditis and pneumonitis as possible clinical manifestations of this infection.

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Marfan syndrome in childhood: parents’ perspectives of the impact on daily functioning of children, parents and family; a qualitative study.

Marfan syndrome (MFS) is a heritable connective tissue disease caused by a defect in FBN1. The diagnosis is based on the revised Ghent criteria. The main features involve the cardiovascular, musculoskeletal, ophthalmic, pulmonary systems and facial features. Although the clinical manifestations of MFS in children are thoroughly addressed in several studies, literature on the impact of MFS on daily functioning is restricted to pediatric advice on sports and leisure participation. Therefore, the full impact of MFS on daily functioning remains unclear. The aim of this qualitative study was to explore parents’ perspectives on the impact of MFS on daily functioning of children with MFS aged 4-12?years, themselves and family regarding functional performance, activities, participation, personal and environmental factors, and disease burden.In this qualitative study parents participated in individual semi-structured interviews (n =?10) and 3 focus groups (n =?5, n =?5 and n =?6). Meetings were transcribed, and data were analyzed using thematic analysis. Meaningful concepts were coded, and concepts concerning children with MFS were linked to the International Classification of Functioning, Disability and Health for Children and Youth. Thereafter themes were identified and interpreted.Parents reported their children could not keep up with peers because of fatigue, pain and physical impairments. Children experienced participation restrictions in school, sports, play and other leisure activities. Parents reported their child as being different due to physical appearance, which provoked unsupportive attitudes. Parental burden was caused by high care needs, lack of support, a limited social life, and concerns about the child’s development. Family burden was caused by adjusted and complex family schedules, other family members with MFS, and reproductive planning decision-making, whereas family cohesiveness and caring were positively perceived factors.Parents perceived a large impact of MFS on daily functioning of their children with MFS, themselves and their family. More awareness among all professionals involved in the care of children with MFS and their families is needed so that professionals can address their support needs and provide tailored interventions, rehabilitation and/or educational programs to empower and improve daily functioning of the children, parents and family.

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A Framework for Evaluation of the Higher-Risk Infant After a Brief Resolved Unexplained Event.

In 2016, the American Academy of Pediatrics published a clinical practice guideline that more specifically defined apparent life-threatening events as brief resolved unexplained events (BRUEs) and provided evidence-based recommendations for the evaluation of infants who meet lower-risk criteria for a subsequent event or serious underlying disorder. The clinical practice guideline did not provide recommendations for infants meeting higher-risk criteria, an important and common population of patients. Therefore, we propose a tiered approach for clinical evaluation and management of higher-risk infants who have experienced a BRUE. Because of a vast array of potential causes, the initial evaluation prioritizes the diagnosis of time-sensitive conditions for which delayed diagnosis or treatment could impact outcomes, such as child maltreatment, feeding problems, cardiac arrhythmias, infections, and congenital abnormalities. The secondary evaluation addresses problems that are less sensitive to delayed diagnosis or treatment, such as dysphagia, intermittent partial airway obstruction, and epilepsy. The authors recommend a tailored, family-centered, multidisciplinary approach to evaluation and management of all higher-risk infants with a BRUE, whether accomplished during hospital admission or through coordinated outpatient care. The proposed framework was developed by using available evidence and expert consensus.

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Measles infection causing Bacillus Calmette-Guérin reactivation: a case report.

Reactivation of the Bacillus Calmette-Guérin (BCG), manifesting as erythema, induration, ulceration or crust formation at a previous BCG inoculation site, is a common and highly specific feature of Kawasaki disease (KD). We report the unusual finding of BCG reactivation in an infant with laboratory-confirmed measles.A previously healthy 7-month old infant presented initially with fever, cough and coryza, and subsequently developed Koplik’s spots followed by a typical morbilliform skin rash. There was significant contact history with a household relative who had recently been diagnosed with measles. On examination, a 2.5?cm area of erythema and induration was seen at the previous BCG inoculation site, in addition to the widespread maculopapular rash. No other clinical features of KD were present. Measles virus was isolated from the throat swab and measles antibodies (IgM) were present in the serum. The patient recovered completely with oral vitamin A and supportive therapy, and had normal echocardiography examination on follow up.This case report highlights the rare finding of BCG reactivation in a child with confirmed measles infection, and suggests that this clinical manifestation may occasionally occur in children with infections or conditions other than KD.

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Anatomical patterns of cleft lip and palate deformities among neonates in Mekelle, Tigray, Ethiopia; implication of environmental impact.

Cleft lip and palate deformities are considered one of the most common birth defects of the head and neck that pose significant medical, psychosocial and financial burdens on the affected individuals and families, especially in low income communities. The etiology and pathogenesis of cleft lip and palate is complex and is known to involve genetic and/or environmental factors.To assess the patterns of anatomical cleft lip and palate deformities among neonates in Mekelle and Ayder Comprehensive Specialized hospitals, Tigray, Northern Ethiopia.A hospital-based retrospective study was conducted from May 2017 to June 2017 at Mekelle and Ayder Comprehensive Specialized hospitals, both in Mekelle city. Data was collected from all medical charts of neonates registered from 2011 to 2016 and analyzed using SPSS version 21.0 and OpenEpi software. Results were presented using tables and graphs; Chi-square test was used to look for an association between variables, odds ratio to determine the strength of association of selected variables using multinomial logistic regression model, while Fisher Exact (Clopper-Pearson) was used to compare yearly prevalence.Of 37,152 neonatal charts analyzed, 119 (0.32%) cases were identified as having cleft deformities. 38.7, 17.6, and 43.7% of this figure had cleft lips, cleft palates and both cleft lip and palate respectively. 46 (38.7%) neonates had lateral patterns of cleft lip deformities with 56.5% located unilaterally on the right and 43.5% unilaterally on the left. Of 52 (43.7%) neonates with cleft lip and palate deformities, 40.4% were located bilaterally while 38.5 and 21.2% were located unilaterally on the left and right, respectively. Associated malformations were: cardiac (3.4%), central nervous system (1.7%) and limb deformities (5.9%). The overall prevalence of cleft deformities was found to be 3.11 per 1000 live births.The study showed a higher prevalence of cleft deformities than that reported in Addis Ababa and some other African countries. A higher occurrence of left unilateral pattern of cleft lip and palate was observed whereas a higher right unilateral pattern of cleft lip was identified. The higher prevalence of cleft lip and palate recorded in this region of Ethiopia may reflect an environmental impact.

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Cardiometabolic Risk and Body Composition in Youth With Down Syndrome.

Whether BMI captures adiposity and cardiometabolic risk in Down syndrome (DS), a condition associated with obesity, short stature, and altered body proportions, is not known. We compared cardiometabolic risk measures in youth with DS and typically developing matched controls.Youth with (n = 150) and without (n = 103) DS of comparable age (10-20 years), sex, race, ethnicity, and BMI percentile underwent whole-body dual-energy X-ray absorptiometry, fasting glucose, insulin, lipids, lipoprotein particles, inflammatory factors, and when BMI percentile ?85, an oral glucose tolerance test.Sixty-four percent of youth with DS had BMI percentile ?85. Among these, no difference in glucose, insulin, or insulin resistance was detected, but prediabetes was more prevalent with DS (26.4% vs 10.3%; P = .025) after adjustment for demographics, pubertal status, and BMI z score (odds ratio = 3.2; P = .026). Among all participants, those with DS had higher low-density lipoprotein cholesterol (median 107 [interquartile range 89-128] vs 88.5 [79-103] mg/dL; P < .00005), triglycerides (89.5 [73-133] vs 71.5 [56-104] mg/dL; P < .00005), non-high-density lipoprotein cholesterol (non-HDL-C; 128 [104-153] vs 107 [92-123] mg/dL; P < .00005), and triglycerides/HDL-C (2.2 [1.6-3.4] vs 1.7 [1.1-2.5] mg/dL; P = .0003) and lower levels of HDL-C (41 [36.5-47] vs 45 [37-53] mg/dL; P = .012). DS youth had higher high-sensitivity C-reactive protein, interleukin-6, small low-density lipoprotein particles (LDL-P), and total LDL-P, but similar LDL-P size. Youth with DS had less visceral fat (VFAT), fat mass, and lean mass for BMI z score, but greater VFAT at higher fat mass. However, VFAT did not fully explain the increased prevalence of dyslipidemia or prediabetes in youth with DS.Despite similar insulin resistance, youth with DS had greater prevalence of dyslipidemia and prediabetes than typically developing youth, which was not fully explained by VFAT.

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Congenital anomalies prevalence in Addis Ababa and the Amhara region, Ethiopia: a descriptive cross-sectional study.

During the first three months of pregnancy, the developing embryo may be susceptible to external and internal factors, which may lead to structural and functional congenital anomalies. The main objective of this study was to determine the prevalence of congenital anomalies in Addis Ababa and the Amhara region, Ethiopia.A descriptive cross-sectional study was conducted on children 0-17?years of age who visited the 16 selected hospitals in Addis Ababa and the Amhara Region between January 1 and July 5, 2015. The proportions of neonates, infants, and children with external and internal congenital anomalies whether the anomalies were major or minor were estimated.Out of 76,201 children, 1518 of whom 57.6% were male identified with congenital anomalies. The overall proportion of congenital anomaly was 1.99% (95% CI: 1.89-2.091) i.e., 199 per 10,000 children. The proportion of neural tube defects, orofacial clefts, masculo-skeletal system anomalies, syndrome disorders, and cardiovascular system problems were 40.3% 37.7-43, 23.3% 21.3-25.4, 23.1% 20.9-25.2, 8% 6.7-9.4, and 2.6% 1.8-3.4, with a 95% CI, respectively. The majority (72.5%) of the mothers were multigravidae; 38(2.5%) of the mothers and 32(2.1%) of the fathers had history of other children with congenital anomalies. Similarly, 20(1.3%) of the participant children’s mothers and 17(1.1%) of the fathers had familial history of congenital anomaly. Iron folate and multivitamin use by mothers during preconception and early pregnancy was found to be low.Neural tube defects, orofacial clefts, and musculoskeletal anomalies were the observed prevalent problems. Maternal illness, viral infections, and malnutrition were seen in a significant number of the mothers. Iron folate/folic acid and multivitamin use by the mothers during and before pregnancy was very low.

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Toxicity of Bupropion Overdose Compared With Selective Serotonin Reuptake Inhibitors.

Adolescent depression and attempted and completed suicide are increasing in the United States. Because suicide is often impulsive, the means of self-harm are frequently items of convenience like medication. Authors of a recent study compared tricyclic antidepressant overdose to bupropion overdose. Fluoxetine and escitalopram are the only agents with Food and Drug Administration approval for pediatric depression, but off-label bupropion prescriptions are common. We sought to compare the effects of selective serotonin reuptake inhibitors (SSRIs) and bupropion in overdose.This was an analysis of the National Poison Data System from June 2013 through December 2017 for adolescent (ages 10-19) exposures to SSRIs or bupropion coded as “suspected suicide.” Demographics, clinical effects, therapies, and medical outcome were analyzed.There were 30?026 cases during the study period. Sertraline and fluoxetine accounted for nearly 60%, whereas bupropion was reported in 11.7%. Bupropion exposure was significantly associated with death (0.23% vs 0%; P < .001) or serious outcome (58.1% vs 19%; P < .001) as well as the 10 most common clinical effects, including seizures (27.0% vs 8.5%; P < .001) and hallucinations (28.6% vs 4.3%; P < .001). Bupropion exposure was significantly associated with the need for cardiopulmonary resuscitation (0.51% vs 0.01%; P < .001), intubation (4.9% vs 0.3%; P < .001), vasopressors (1.1% vs 0.2%; P < .001), and benzodiazepines (34.2% vs 5.5%; P < .001). There was a significant increase in all exposures and in proportion of serious outcomes over time.Adolescents who attempt self-harm are at higher risk for serious morbidity and poor outcomes with bupropion than with SSRIs. These risks, and the patient's propensity for self-harm, should be evaluated when therapy with bupropion is considered.

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Exon sequencing of the alpha-2-globin gene for the differential diagnosis of central cyanosis in newborns: a case report.

Cyanosis is usually associated with serious conditions requiring urgent treatment in the neonatal intensive care unit (NICU). Hemoglobin M (Hb M) disease is one type of congenital methemoglobinemia characterized by cyanosis. Among these variants, ?-globin chain mutations such as Hb M Boston present cyanosis from birth while other variants usually manifest later in life.We report a case of a male newborn with cyanosis apparent since birth. Surprisingly, his respiratory and hemodynamic status including normal arterial blood oxygen saturation was stable, but oxygen saturation on pulse oximetry did not increase after 100% supplemental oxygen was started. In addition to routine pulmonary and cardiologic evaluation, further evaluation for dyshemoglobin was conducted; ?2-globin gene sequencing showed a single-point variant causing Hb M Boston. Methemoglobin (MetHb) level estimated by co-oximetry was normal. After a 14-day stay in the NICU, the patient remained respiratory and hemodynamically stable without supplemental oxygen except for cyanosis.Hb M disease is a benign disease and does not require any treatment whereas acquired methemoglobinemia is a potentially fatal condition. Neonatologists should be aware that low oxygenation status on pulse oximetry in the face of normal arterial blood saturation values might indicate the possibility of Hb M disease in early neonatal cyanosis, irrespective of MetHb value.

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The relationship between payer type and lipid outcomes in response to clinical lifestyle interventions in youth with dyslipidemia.

Payer-type (government-sponsored health coverage versus private health insurance) has been shown to influence a variety of cardiovascular disease outcomes in adults. However, it is unclear if the payer-type impacts the response to a lifestyle intervention in children with dyslipidemia.We analyzed data prospectively collected from patients under the age of 25?years who were referred to a large regional preventive cardiology clinic from 2010 to 2016 in Massachusetts. We compared baseline high density lipoprotein cholesterol (HDL-C), triglycerides (TG), non-HDL-C, and low density lipoprotein cholesterol (LDL-C) by payer-type. Further, we analyzed the change in lipid values in response to a clinic-based multidisciplinary intervention over a nearly six-year period by payer-type with multi-variable adjusted linear regression models. We also tested for effect modifications by age, sex, race, and body mass index (BMI) category.Of the 1739 eligible patients (mean age 13?years, 52% female, 60% overweight and obese, 59% White), we found that patients with government-sponsored coverage (n?=?354, 20%) presented to referral lipid clinic with lower HDL-C (-?3.5?mg/dL [1.0], p? » Read more

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