Health-Related Quality of Life in Adolescents with Thalassemia

Children and adolescents with thalassemia suffer from chronicity of the disease and its treatment, including transfusion dependence and complications of iron overload. This study aimed to assess the health-related quality of life of adolescents with thalassemia compared with healthy controls. Sixty-four adolescents with thalassemia aged 13 to18 years and their parents were enrolled in this cross-sectional study, as well as their age- and gender-matched those of the healthy controls (64 participants and their parents). The Pediatric Quality of Life Inventory 4.0 Scales (PedsQL 4.0) self-report form was administered to the adolescents in both groups. Parents were also asked to complete the PedsQL 4.0, parent proxy-report form. The self-reported total, psychosocial, and school functioning scores of the thalassemia patients were significantly lower than those of the healthy controls (p = 0.03, 0.04, and <0.001, respectively). The parent-reported psychosocial and school functioning scores of the thalassemia group were also significantly lower than those of the controls (p = 0.03 and 0.003, respectively). Among adolescents with thalassemia, the serum ferritin level and comorbidity were the only variables associated with quality of life scores. This study showed that thalassemia negatively affected quality of life. For a better quality of life, thalassemia patients should be monitored for serum ferritin levels and treated for comorbidity as part of their comprehensive health care.     

Quality of Life Experienced by Adolescents and Young Adults With Congenital Heart Disease

This study aimed to assess the quality of life (QOL) experienced by adolescents and young adults with congenital heart disease (CHD) and to determine which factors negatively affect adjustment and which factors increase resilience. The participants in the study were 74 patients with CHD (41 males and 33 females) ranging in age from 12 to 26 years (mean age, 18.76 ± 3.86 years). Demographic information and a complete clinical history were obtained. The participants were interviewed regarding topics such as social support, family educational style, self-image, and physical limitations. They responded to questions in a standardized psychiatric interview (SADS-L) and completed a self-report questionnaire (WHOQOL-BREF) for assessment of QOL. Compared with the Portuguese population as a whole, the study patients had better QOL, especially with regard to the environmental dimension (t = 3.754; P = 0.000) and social relationships (t = 2.333; P = 0.022). Patients who had undergone surgery experienced poorer QOL in the physical dimension (t = −1.989; P = 0.050), in social relationships (t = −2.012; P = 0.048) and overall (Mann–Whitney U = 563.000; P = 0.037). Social support played a positive role in the QOL of the patients, both in the physical dimension (t = 3.287; P = 0.002) and in social relationships (t = 3.669; P = 0.000). A higher school achievement also was associated with higher levels of QOL overall (Mann–Whitney U = 457.000; P = 0.046) as well as in the physical (t = 2.045; P = 0.045) and environmental (t = 2.413; P = 0.018) dimensions. Physical limitations had a detrimental impact on general QOL (Mann–Whitney U = 947.500; P = 0.001) and on the physical (t = −2.910; p = 0.005) and psychological (t = −2,046; P = 0.044) dimensions. Patients with CHD tended to perceive QOL as better when their social networks were supportive.     

Parental Understanding of Neonatal Congenital Heart Disease

Background This study aimed to evaluate the impact of prenatal diagnosis on parental understanding of congenital heart disease (CHD) in newborns. Methods Consenting parents of newborns with CHD answered questions about the cardiac lesion, surgical repair, follow-up management, risk for CHD in future children, and maternal education before neonatal intensive care unit (NICU) discharge. A total understanding score was calculated (0–10) as the sum of five subscores: physician score, CHD score, surgery score, follow-up score, and reproduction score. Each category was scored as 0 (none correct), 1 (some correct), or 2 (all correct). The prenatal and postnatal diagnoses scores were compared. Results From June 2006 to November 2006, 50 families completed the questionnaire. Of these 50 families, 26 reported a prenatal diagnosis. The mean infant age when the parents were approached was 17.3 ± 13.3 days. The summary understanding score for the entire group was 6.3 ± 2.4 of 10. Multivariate regression analysis demonstrated a difference in scores between prenatal and postnatal diagnosis groups (p = 0.02) when control was used for maternal education. Prenatal diagnosis and maternal education (p < 0.01) had independent effects on the score. Conclusion Prenatal diagnosis increases parental understanding of neonatal CHD. Nevertheless, parental understanding remains suboptimal.     

Health-Related Quality of Life Experienced by Children With Chromosomal Abnormalities and Congenital Heart Defects

Long-term outcomes are fundamental in advising parents about the potential future of their children with congenital heart disease (CHD). No published reports have described the health-related quality of life (HRQL) experienced by children with chromosomal abnormalities who had surgery in early infancy for CHD. A study was undertaken to assess HRQL among children with chromosomal abnormalities and CHD. The authors hypothesized that these children have a worse HRQL than healthy children or a cohort of children matched for CHD diagnosis. Infants with chromosomal abnormalities undergoing cardiac surgery for CHD at 6 weeks of age or younger at the Stollery Children’s Hospital between July 2000 and June 2005 were included in the study. The HRQL of these infants was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales completed by their parents at a 4-year follow-up evaluation. The study compared the scores for 16 children with normative data. The children with chromosomal abnormalities and CHD had significantly lower mean total PedsQL (71.3 vs. 87.3; p < 0.0001), Psychosocial Summary (70.3 vs. 86.1; p < 0.0001), and Physical Summary (74.3 vs. 89.2; p = 0.0006) scores. Compared with the matched children, those with chromosomal abnormalities had a significantly lower median total PedsQL (75.0 vs. 84.6; p = 0.03), Physical Summary (79.5 vs. 96.9; p = 0.007), and School Functioning (68.5 vs. 83.0; p = 0.03) scores. A better understanding of the mechanisms and determinants of HRQL in these children has the potential to yield important implications for clinical practice including clarity for treatment decision making as well as determination of targeted supports and services to meet the needs of these children and their families differentially.     

Health-Related Quality of Life in Egyptian Children and Adolescents with Hemophilia A

Quality of life (QoL) in hemophilia is an important area in hemophilia outcome assessment. The Haemo-QoL instrument is a set of questionnaires to measure QoL in those children. The objectives of this study was to assess health-related quality of life (HRQoL) in Egyptian hemophilic children and adolescents using an Arabic version of the Haemo-QoL questionnaire. Sixty patients with severe hemophilia A were recruited from 2 hemophilia treating centers in Egypt. Assessment of quality of life was done using the Haemo-QoL questionnaire. The scores of HRQoL were found to be for all dimensions widely above 50. It was highly significant in the 3 dimensions (physical health–family–treatment) in different age groups, but it was impaired in the dimension of “physical health” for 2 groups, and in the dimension of “family” for the oldest group, whereas the youngest group had highly impaired scores concerning the “treatment.” The HRQoL in this study was not affected by the presence of factor VIII (FVIII) inhibitors. The QoL in hemophilic patients in Egypt needs strenuous efforts from hemophilia care–integrated teams of pediatric hematologists and psychiatrists in order to properly assess and improve QoL.     

Health-Related Quality of Life in Children With Sickle Cell Disease Using the Child Health Questionnaire

IntroductionThis study sought to determine if changes in parent-reported health-related quality of life (HRQOL) in children with sickle cell disease (SCD-SS) occurred after participation in a nutritional supplementation study and to compare HRQOL responses with normative scores from non-White children.MethodParents of children with SCD-SS between the ages of 5 and 13 years completed the Child Health Questionnaire (CHQ-PF50) at baseline and at 12 months.ResultsFor the 47 children (8.6 ± 2.4 yrs, 43% female), baseline Child Health Questionnaire scale scores were significantly lower than normative scale scores for parental emotional impact, general health, and overall physical health, but they were higher for mental health. After the nutritional supplementation study, overall physical health and parental emotional impact improved to normative levels. Furthermore, physical role functioning significantly improved.DiscussionParticipation in a nutritional study had a positive impact on parent-reported HRQOL physical scores in children with SCD-SS. More research is necessary to develop care providers' awareness and adequate HRQOL interventions for this population.     

Impact of Exercise Training in Aerobic Capacity and Pulmonary Function in Children and Adolescents After Congenital Heart Disease Surgery: A Systematic Review with Meta-analysis

The aim of the study was to examine the effects of exercise training on aerobic capacity and pulmonary function in children and adolescents after congenital heart disease surgery. We searched MEDLINE, Cochrane Controlled Trials Register, EMBASE, (from the earliest date available to January 2015) for controlled trials that evaluated the effects of exercise training on aerobic capacity and pulmonary function (forced expiratory volume in 1 s and forced vital capacity) in children and adolescents after congenital heart disease surgery. Weighted mean differences and 95 % confidence intervals (CIs) were calculated,, and heterogeneity was assessed using the I ² test. Eight trials (n = 292) met the study criteria. The results suggested that exercise training compared with control had a positive impact on peak VO₂. Exercise training resulted in improvement in peak VO₂ weighted mean difference (3.68 mL kg^?1 min^?1, 95 % CI 1.58–5.78). The improvement in forced expiratory volume in 1 s and forced vital capacity after exercise training was not significant. Exercise training may improve peak VO₂ in children and adolescents after congenital heart disease surgery and should be considered for inclusion in cardiac rehabilitation. Further larger randomized controlled trials are urgently needed to investigate different types of exercise and its effects on the quality of life.     

Incidence and Etiology of Mental Retardation in Patients with Congenital Heart Disease

Of 1,235 patients (609 male; 626 female) with congenital heart disease for whom types, etiology and associated congenital anomalies were determinable, 129 patients (10.4%) had mental retardatin. Patients with congenital heart disease complicated by mental retardation included 70 (54.3%) with chromosome aberrations, three(2.3%) with single gene disorders, two (1.6%) caused by environmental insult, and two (1.6%) with other recognized syndromes. Among the remaining 52 patients, asphyxia at birt was noted in 16, including 12 complicated by multiple malformations, and 4 in whom mental retardedation was presumed to be due to the asphyxia. In the remaining 36 patients, the incidence of the complication of mental retardation in cyanotic congenital heart disese was significantly higher than that in acyanotic congenital heart disease was signicantly higher than that in acyanotic congenital heart disease. Patients with congenital heart disease of unknown etiology associated with mental retardation included those from two families considered to have new pedigree syndromes.     

先天性心脏病患儿日常生活的标准化管理

近年来小儿心脏外科迅猛发展,我国几个大的小儿心脏外科中心的诊治水平已处于或接近国际领先水平,但是对于先天性心脏病(先心病)的认识目前还存在较大差异,不同中心、不同地区间还有分歧,探讨先心病患儿的标准化管理很有必要,有利于我国小儿心脏外科的规范发展.现从临床的角度提供一些标准化管理经验,探讨如何更好地对先心病患儿日常生活进行标准化管理,以提高临床治疗效果,有效改善先心病的预后.     

Cardiac Rehabilitation Improves Heart Rate Recovery Following Peak Exercise in Children with Repaired Congenital Heart Disease

We assessed heart rate (HR) recovery following peak exercise before and after a 12-week cardiac rehabilitation program in 14 children, 12.1 ± 1.8 years of age, with repaired complex congenital heart disease (CHD; 11 with Fontan surgery) and impaired exercise performance. Exercise testing using bicycle ergometry was performed at baseline, after completion of the rehab program and 1.0 ± 0.2 years after the baseline test. These data were compared to HR recovery in 15 controls (age, 12.7 ± 2.4 years) with CHD (13 with Fontan surgery) with two serial exercise tests at an interval of 1.1± 0.3 years. There was no change in peak HR between the two serial tests in either group. Peak VO₂ improved in the rehab group (26.3 ± 9.6 ml/kg/min at baseline vs 30.9 ± 9.6 ml/kg/min after rehab, p = 0.01) but remained unchanged in controls on serial testing. One-minute HR recovery (in beats per minute) improved significantly following completion of the rehab program (27 ± 15 at baseline vs 40 ± 23 after rehab, p = 0.01). Partial improvement in 1-minute HR recovery in the rehab group persisted 1 year later (1-minute HR recovery, 35 ± 19; p = 0.1 compared to baseline). There was no change in 1-minute HR recovery over time in the control group (37 ± 16 vs 40 ± 13, p = not significant). In conclusion, HR recovery following peak exercise improves in children with CHD after participation in a cardiac rehab program.     

Mood and Health-Related Quality of Life Among Pediatric Patients With Heart Failure

Adult patients with heart failure (HF) commonly experience depression, with morbid and mortal consequences. However, mood disorders in pediatric patients with HF are poorly understood. This study examined mood and health-related quality of life (HRQOL) in children with HF and compared them cross-sectionally with those of healthy control subjects and heart transplant (Htx) recipients with good heart function. The 62 participants in this study were divided into three groups: HF subjects (n = 15), Htx subjects (n = 23), and healthy control subjects (n = 24). The HF subjects all had chronic HF with a left ventricular ejection fraction lower than 35 %. All the participants completed the Mini-Mental State Examination (MMSE), the Childhood Depression Inventory (CDI), and the Pediatric Quality-of-Life Inventory Cardiac Module (PedsQL CM). Overall, the MMSE scores and CDI subscale scores were similar for all the groups. The HF and Htx participants scored similarly on the PedsQL CM subscales for HRQOL, treatment anxiety, perceived physical appearance, cognitive function, and communication. However, the HF group had a significantly lower HRQOL related to heart problems and treatment than the Htx group. The prevalence of depression among children with HF is not as high as reported in the adult HF literature. However, certain aspects of HRQOL experienced by pediatric HF patients still suffer, especially those related to heart problems and treatment. Health-related QOL tended to be better for the Htx participants than for the HF participants. Exploring developmental and psychosocial outcomes is critical for patients with HF, especially because it has an impact on vital developmental, academic, and social outcomes.     

Health-Related Quality of Life Compared With Cardiopulmonary Exercise Testing at the Midterm Follow-up Visit After Tetralogy of Fallot Repair: A Study of the German Competence Network for Congenital Heart Defects

This nationwide study aimed to evaluate health-related quality of life (QoL) experienced by children after tetralogy of Fallot repair and to compare self-reported physical ability with objective exercise performance. This prospective nonrandomized, government-funded multicenter study enrolled 168 patients (70 girls; ages 8–16 years) after tetralogy of Fallot repair at eight German heart centers. Health-related QoL was analyzed by the self-reported KINDL-R quality-of-life questionnaire. The patients’ actual exercise capacity was evaluated by a cardiopulmonary exercise test. Health-related QoL and cardiopulmonary exercise capacity were compared with those of an age-matched German standard population. Correlation of health-related QoL with self-estimated physical rating and cardiopulmonary exercise capacity were analyzed. Health-related QoL in children and adolescents after tetralogy of Fallot repair is without limitation. Compared with the standard population, all the items evaluated by the KINDL-R questionnaire showed better or similar values, whereas objective exercise capacity compared with that of the standard population was impaired. Peak oxygen uptake correlated significantly with the physical well-being (p = 0.002) and the total score (p = 0.01) of the KINDL-R questionnaire. Health-related QoL experienced by children and adolescents after tetralogy of Fallot repair is comparable with that of the healthy standard population. However, closer inspection shows that self-estimated physical functioning is significantly overestimated compared with actual exercise capacity. Quality-of-life instruments and exercise tests, therefore, should be used in a complementary manner with children to avoid eventually fatal misinterpretation of patient-estimated physical ability.     

Health-Related Quality of Life,Depression, and Metabolic Parameters in Overweight Insulin-Resistant Adolescents

IntroductionThe purpose of this study was to examine the incidence and severity of depression and health-related quality of life (HRQoL) in youth with insulin resistance (IR) who are overweight/obese and to examine the impact on making lifestyle changes.MethodNew patients presenting for treatment in an IR clinic were screened for depression and HRQoL and reassessed twice during a 1-year treatment period. Metabolic and growth parameters were obtained for each participant.ResultsElevated symptoms of depression were reported in 51% of the sample, and these symptoms were stable over time. Approximately 10% of these youth reported moderate or severe symptoms of depression. HRQoL scores indicated a good quality of life overall with slight improvement in some areas over time. Depression scores were not associated with demographic variables or metabolic parameters.DiscussionMore than 50% of adolescents with IR and obesity reported elevated symptoms of depression. These results provide sufficient evidence for the need to conduct routine screening of depression for all youth with IR so that appropriate mental health referrals can be made.     

Chromosomal Anomalies Influence Parental Treatment Decisions in Relation to Prenatally Diagnosed Congenital Heart Disease

This study aimed to identify the variables that influence parental treatment decisions after a prenatal diagnosis of congenital heart disease (CHD). The authors reviewed all cases of prenatally diagnosed structural CHD from August 1998 to December 2006 at their center. The following variables were studied as potential predictors of parental intent to treat: maternal age, race, insurance status, obstetric history, fetal gender, univentricular versus biventricular cardiac physiology, and fetal chromosomal abnormality. Uni- and multivariable logistic regression analyses were performed. In the review, 252 consecutive cases of prenatally diagnosed CHD were identified. Of these, 204 women pursued full medical treatment, whereas 25 women sought termination of pregnancy or comfort care. Parental intent to treat was unknown for 23 cases. Multivariable logistic regression analysis identified fetal chromosomal abnormality as the only variable that influenced parental intent to treat (odds ratio [OR], 14.33; 95% confidence interval [CI], 3.28–62.66; p = 0.0006). An associated chromosomal abnormality rather than the severity of the heart defect influences the decision to choose termination of pregnancy or comfort care for a fetus with prenatally diagnosed CHD. Women were 14 times more likely to terminate a pregnancy or seek comfort care for a fetus with CHD if a chromosomal abnormality was present.