Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982–2007)

Surgical treatment of congenital heart disease represents a major cause of pediatric hospitalization and healthcare resource use. Larger centers may provide more efficient care with resulting shorter length of postoperative hospitalization (LOH). Data from 46 centers over 25 years were used to evaluate whether surgical volume was an important determinant of LOH using a competing risk regression strategy that concurrently accounted for deaths, transfers, and discharges with some time interactions. Earlier discharge was more likely for infants and older children compared to neonates [subhazard ratios at postoperative day 6 of 1.64 (99 % confidence interval (CI) 1.57, 1.72) and 2.67 (99 % CI 2.53, 2.80), respectively], but less likely for patients undergoing operations in Risk Adjustment for Congenital Heart Surgery categories 2, 3, 4, and 5/6 compared to category 1 [subhazard ratios at postoperative day 6 of 0.66 (99 % CI 0.64, 0.68), 0.34 (95 % CI 0.33, 0.35), 0.28 (99 % CI 0.27, 0.30), and 0.10 (99 % CI 0.09, 0.11), respectively]. There was no difference by sex [non-time-dependent subhazard ratio 1.019 (99 % CI 0.995, 1.040)]. For every 100-operation increase in center annual surgical volume, the non-time-dependent subhazard for discharge was 1.035 (99 % CI 1.006, 1.064) times greater, and center-specific exponentiated random effects ranged from 0.70 to 1.42 with a variance of 0.023. The conditional discharge rate increased with increasing age and later era. No sex-specific difference was found. Centers performing more operations discharged patients sooner than lower volume centers, but this difference appears to be too small to be of clinical significance. Interestingly, unmeasured institutional characteristics estimated by the center random effects were variable, suggesting that these played an important role in LOH and merit further investigation.     

Social Skills and Problem-solving Training for Children with Early-onset Conduct Problems: Who Benefits?

Families of 99 children with early-onset conduct problems, aged 4-8 years, were randomly assigned to a child training treatment group (CT) utilizing the Incredible Years Dinosaur Social Skills and Problem Solving Curriculum or a waiting-list control group (CON). Post-treatment CT children had significantly fewer externalizing problems at home, less aggression at school, more prosocial behavior with peers, and more positive conflict management strategies than CON children. Significantly more CT than CON children showed clinically significantly improvements on reports and independent observations of aggressive and noncompliant behavior. The differential treatment response was evaluated according to child comorbidity with attention deficit hyperactivity disorder (ADHD), parenting discipline practices. and family risk factors. The only risk factor related to failure to make improvements in child conduct problems after treatment was negative parenting (i.e., maternal critical statements and physical force). The long-term follow-up 1 year later indicated that most of the significant post-treatment changes were maintained.     

Cardiac Remodeling After Enzyme Replacement Therapy with Acid α-Glucosidase for Infants with Pompe Disease

Background  Infantile Pompe disease (glycogen storage disease type 2) is a fatal disorder caused by deficiency of acid α-glucosidase. This deficiency results in glycogen accumulation in the lysosomes of many tissues including cardiac muscle. The disease is characterized by profound hypotonia, poor growth, organomegaly, and cardiomegaly. Severe hypertrophic cardiomyopathy often is present in early infancy, and most patients die of cardiac or respiratory failure in the first year of life. This report describes the cardiac response of infants with Pompe disease to a phase 2 trial of enzyme replacement therapy (ERT). Methods  Eight patients with classical infantile Pompe disease were given intravenous recombinant human GAA (rhGAA) for 1 year. Cardiac monitoring included echocardiography, electrocardiograms (ECGs), chest radiographs, and clinical cardiac evaluation at 4, 8, 12, 24, 36, and 52 weeks. At 52 weeks, 6 patients were alive. Results  Most of the treated patients had rapid regression of ventricular hypertrophy in response to ERT, with near normalization of posterior wall thickness, ventricular mass, and ventricular size. Systolic ventricular function was preserved despite rapid changes in ventricular mass and size. Concomitantly, ECGs documented lengthening of the PR interval and decreased ventricular voltages, whereas chest radiographs documented a decreased cardiothoracic ratio. Symptoms of pulmonary congestion were diminished, and survival was improved. Conclusion  The cardiovascular system responds quickly and strikingly to ERT with rhGAA, suggesting rapid reversal of excessive glycogen storage in cardiac muscle cells. Changes in ventricular mass and function are maintained throughout 1 year of follow-up evaluation and associated with decreased morbidity and prolonged survival.     

Parents’ Experiences with Services and Treatment for Their Children Diagnosed with Cerebral Palsy

ABSTRACT

While there is a considerable body of knowledge investigating the efficacy of constraint-induced movement therapy (CIMT), there is a need for information focusing on parents’ experiences when their children receive these services. This qualitative research study initially explored the experiences of four parents with children who are diagnosed with cerebral palsy (CP) and who participated in CIMT using a group format. Additionally, the richness of the information shared by parents provided insights on other important issues related to family supports when raising a child and the availability of resources for children diagnosed with CP. Response analysis from in-depth interviews with parents resulted in three themes: (a) Everything in my family changed when my child was diagnosed with CP and the support I feel from my family makes all the difference in how I cope. (b) It was so hard to get the information and support that I needed for my child. (c) The group format enhanced CIMT for my child and provided social benefits as well. Findings from this study suggest there are benefits of using CIMT in group formats.     

Prevalence and Spectrum Diseases Predisposing to Sudden Cardiac Death: Are They the Same for Both the Athlete and the Nonathlete?

Sudden cardiac death (SCD) is a rare but devastating event among young people. The precise scale of the event remains undefined. This uncommon but catastrophic event usually is proved to be the consequence of varied unsuspected congenital or acquired cardiovascular diseases. Hypertrophic cardiomyopathy and coronary artery anomalies are the two most frequent causes of SCD. Most commonly, SCD resulting from these diseases occurs during or immediately after exercise. This report highlights the prevalence and spectrum of SCD-predisposing diseases and how they are affected by athletic participation. In addition, disease-specific guidelines for sports participation are addressed.     

Community Health Centers: Medical Homes for Children?

ObjectiveTo explore medical home attributes of community health centers (CHCs) that provide care to low-income children nationwide compared to other providers for the poor.MethodsCross-sectional study of children aged 0 to 17 years in the Medical Expenditure Panel Survey (MEPS; 2003 to 2009) who resided in families living at <200% of the federal poverty level (FPL) and had visits to a primary care setting. CHC visits were defined as a visit to a neighborhood/family health center, rural health clinic, or community health center. Independent measures included provider type, age, gender, race/ethnicity, insurance, FPL, number of parents at home, language, maternal education, health status, and special health care need. Dependent measures included 4 medical home attributes: accessibility, and family-centered, comprehensive, and compassionate care.ResultsCHCs typically serve low-income children who are publicly insured or uninsured, come from racial/ethnic minority groups, and have poorer health status. Eighty percent to 90% of parents visiting both CHCs and other primary care providers rated high levels of family-centered, comprehensive, and compassionate care. However, CHCs had a 10% to 18% lower rating of accessibility (after-hours care, telephone access) even after controlling for sociodemographic characteristics. Racial/ethnic disparities existed at both settings, but these patterns did not differ between CHCs and other settings.ConclusionsOn the basis of parental reports, CHCs received similar ratings to other primary care providers for family-centered, comprehensive, and compassionate care, but lower ratings for accessibility. Further studies should examine strategies for practice transformation in CHCs to improve patient satisfaction and accessibility to optimize child health outcomes.     

Social–Emotional Screening Protocol Implementation: A Trauma-Informed Response for Young Children in Child Welfare

Adverse childhood experiences in young children result in negative outcomes as trauma affects brain development. In child welfare services, early recognition of delayed social–emotional skills and treatment referral is essential in reducing the effects of trauma. This quality improvement pilot project implemented an evidence-based social–emotional screening protocol using the Ages and Stages Questionnaire: Social Emotional-2 screening tool for very young children placed in out-of-home care through the county's child welfare system. Findings showed significant improvement in identification of children younger than 3 years with social–emotional concerns (p < .0001) and significant improvement in referral of those children positively identified (p = .0130). Ongoing use of the protocol was recommended, because it showed improved identification and referral for young children in the child welfare system with social–emotional concerns that were potentially trauma related. Further collaboration between child protective services, pediatric medical systems, and pediatric mental health systems is needed to facilitate trauma-informed care for children in the child welfare system.     

Children with moderately elevated lead levels: is chelation with DMSA helpful?

This study evaluates the effectiveness (use under routine circumstances) of DMSA (2,3 dimercaptosuccinic acid) and environmental remediation as compared with placebo and environmental remediation on children with blood lead (BPb) levels of 30-45 micrograms/dL (1.45-2.17 mumol/L). The endpoints were BPb at 1 month and 6 months after study entry. This double-blind placebo-controlled trial involved 39 children aged 2-5 years, who were randomized to one course of DMSA or placebo. The mean BPb levels of the two groups at study entry were similar, placebo group 33.0 micrograms/dL (1.59 mumol/L) and the DMSA group 34.9 micrograms/dL (1.68 mumol/L). At 1 month (the end of treatment) the mean BPb levels of the two groups were: placebo group 33.2 micrograms/dL (1.60 mumol/L) and the DMSA group 27.4 micrograms/dL (1.32 mumol/L), p = 0.16. At 6 months, the mean BPb levels were 25.1 micrograms/dL (1.21 mumol/L) for the placebo group and 28.8 micrograms/dL (1.39 mumol/L) for the DMSA-treated group, p = 0.06. Neither of these differences is statistically significant. All children with BPb, in the range studied here, should receive environmental evaluation and remediation; DMSA does not improve long-term blood lead levels.     

Call for a Sudden Cardiac Death Registry: Should Reporting of Sudden Cardiac Death be Mandatory?

There is currently no central mandatory pediatric sudden cardiac arrest (SCA) registry in the United States. Perhaps the time is right to actively endorse and develop a pediatric SCD registry, in collaboration with other agencies, advocacy groups, and organizations. This registry, if well constructed, thorough, and validated, would serve not only important SCD epidemiologic purposes but also provide the seed bed for closer collaboration with medical examiners and coroners and enhance critical cascade testing for identification of genotypically and/or phenotypically affected family members.     

The Dimensions of Mastery Questionnaire in School-Aged Children with Congenital Hemiplegia: Test–Retest Reproducibility and Parent–Child Concordance

ABSTRACT

Aim: To examine internal consistency, test–retest reproducibility, and parent–child concordance of the Dimensions of Mastery Questionnaire 17.0 (DMQ) in school-aged children with congenital hemiplegia. Method: Forty-two children (8.24 ± 2.38 years, Manual Ability Classification System (MACS) I = 23, MACS II = 19) and their parents completed the DMQ, and a subset on two occasions 2–30 days apart (n = 27). Cronbach's alpha (α), intraclass correlation coefficients (ICCs), standard error of measurement (SEM), and 95% limits of agreement were calculated. Results: Internal consistency for child self-report was variable (α = 0.57–0.90). Cronbach's alphas for parent proxy report were good (α = 0.69–0.86). Test–retest reproducibility for instrumental aspect (ICC = 0.86) and total motivation (ICC = 0.84) were excellent with subscales ranging from 0.70 to 0.91. The SEM for total motivation was 0.23 points. Parent–child concordance was poor across all scores (ICC = ?0.04 to 0.42) with a large SEM (0.50–0.91). Interpretation: The DMQ parent report has good test–retest reproducibility for subscales, instrumental, and total motivation scores in school-aged children with congenital hemiplegia. Parent–child concordance was low highlighting differences in individual and contextual perspectives.     

Home-based DIR/Floortime™ Intervention Program for Preschool Children with Autism Spectrum Disorders: Preliminary Findings

ABSTRACT

Improving parent–child interaction and play are important outcomes for children with autism spectrum disorder (ASD). Play is the primary occupation of children. In this pilot study conducted in Taiwan, we investigated the effects of the developmental, individual difference, and relationship-based (DIR)/Floortime? home-based intervention program on social interaction and adaptive functioning of children with ASD. The participants were 11 children with ASD, ages from 45–69 months, and their mothers. Mothers were instructed the principles of the approach by an occupational therapist. All 11 children and their mothers completed the 10-week home-based intervention program, undergoing an average of 109.7 hr of intervention. Children made significant changes in mean scores for emotional functioning, communication, and daily living skills. Moreover, the mothers perceived positive changes in their parent-child interactions. The findings of this pilot study contribute to knowledge regarding the effects of home-based DIR/Floortime? intervention program on increasing the social interaction and adaptive behaviors of children with ASD in Taiwan.     

Children followed with difficulty: How do they differ?

OBJECTIVE: To determine if very low birthweight children followed with ease differ in any perinatal or sociodemographic characteristics, or outcomes, compared with children followed with more difficulty. METHODOLOGY: Consecutive children of birthweight < 1000 g or with gestational ages < 28 weeks born in 1991 (n = 51) or of birthweight < 1500 g born in 1992 (n = 166) at the Royal Women's Hospital, Melbourne, surviving to 5 years of age, were assessed at 5 years of age, corrected for prematurity. Those who attended on the first mutually agreed appointment without substantial reluctance were considered to have been followed with ease. The remainder were considered to have been followed with difficulty. Outcomes included impairments such as cerebral palsy, blindness, deafness, and low IQ. Children had a disability if they had any of cerebral palsy, blindness, deafness requiring amplification, or an IQ more than 1 SD below the mean. RESULTS: Of the 217 survivors, 204 (94%) were assessed fully at 5 years of age. Of the 204 children assessed, 153 (75%) were followed with ease, and 51 (25%) with difficulty. Of data available in the perinatal period, significantly fewer children followed with more difficulty came from intact families, and more of their mothers had fewer than 12 years of schooling. More children followed with difficulty had a disability (41% compared with 19%), as they predominantly had lower IQ scores (mean difference in IQ - 12.7, 95% confidence interval - 18.0, - 7.4). The association between difficulty of assessment and both higher rates of disability and lower IQ scores remained after adjustment for significant perinatal and sociodemographic variables. CONCLUSIONS: Children followed with difficulty can partly be recognized on several sociodemographic characteristics in the perinatal period, and have substantially worse sensorineural outcomes than those followed with ease. In any longitudinal study, the more incomplete the follow up, the lower will be the rate of adverse sensorineural outcome.