Risk factors for impaired health-related quality of life in a cohort of pediatric patients with inborn metabolic diseases.

In the last decade, health-related quality of life (HrQoL) has become an increasingly important outcome parameter in children and adolescents with chronic health conditions; among them are pediatric patients with inborn metabolic diseases (IMDs). Hence, knowledge on this topic is increasing, but findings on non-medical influences on the HrQoL of IMD patients are still scarce. In the present study, we retrospectively evaluated the self-reported generic HrQoL of a cohort of pediatric patients (ages 7 to 17 years) with diverse IMDs (n = 204) and explored associations between HrQoL and psychosocial and medical characteristics of the patients. We aimed to identify risk factors for impaired HrQoL to improve and tailor support for the patients and economize resources. Generic HrQoL was assessed with the KINDL-R questionnaire. We compared the HrQoL scores to published German normative data and analyzed the impact of demographic variables and intellectual and psychosocial functioning on the HrQoL. Moreover, we examined the influence of the diagnostic category and the health impairment (as judged by the physicians) on our patients' HrQoL. Overall, the HrQoL of the adolescent patients was comparable to the HrQoL of the norm group. Disorders of intellectual development, impaired psychosocial functioning, and a severe health impairment were associated with lower HrQoL scores.Conclusion: We recommend evaluating these factors in children and adolescents with IMDs to identify patients at risk for impaired HrQoL. What is Known: • Studies on HrQoL in pediatric patients with IMDs mainly focused on subgroups with specific diagnoses and found normal HrQoL in some of those subgroups. • In healthy children and adolescents as well as in pediatric patients with various chronic diseases, associations between psychosocial factors and HrQoL are well known. What is New: • Impaired psychosocial functioning, disorders of intellectual development, and a significant disease and/or treatment burden are risk factors for impaired HrQoL in pediatric patients with IMDs. • Evaluating these factors in children and adolescents with IMDs can help identify patients and families in need of enhanced psychological support.

Less ready for adulthood?-Turner syndrome has an impact on transition readiness.

OBJECTIVE: Young women with Turner syndrome (TS) are known to be at risk for loss to medical follow-up. Recent literature indicates that there are disparities regarding transition readiness between different chronic conditions. So far, studies in young women with TS investigating their transition readiness compared to youths with other chronic conditions with no or minor neurocognitive challenges have not been reported. METHODS: Patients (n = 52), 26 patients with Turner syndrome (mean age 17.24 ± 2.10) and 26 controls with type 1 diabetes or a rheumatic disease (mean age 17.41 ± 2.44), were recruited from specialized paediatric endocrine outpatient clinics. The Transition Readiness Assessment Questionnaire TRAQ-GV-15 was used to compare transition readiness scores between TS and controls. In addition, information on individual handling of the questionnaire was obtained. Descriptive statistics and nonparametric methods were used to analyse the data. RESULTS: Significant differences for transition readiness scores were found between the two study groups. The global TRAQ-GV-15 score was significantly lower for females with TS. In particular, subscale 1 'autonomy' of the TRAQ-GV-15 showed lower scores in patients with TS. Patients with TS needed significantly more help and more time to complete the questionnaire. CONCLUSION: Special attention should be given to young women with Turner syndrome in the preparation for the transitional phase. By incorporating the assessment of transition readiness specialists will find it easier to identify underdeveloped skills and knowledge gaps in their patients. Unless a multidisciplinary young adult clinic is established, an older age than 18 years at transfer to adult endocrine care might be beneficial.

Cochrane corner: psychological interventions for individuals with cystic fibrosis and their families.

Psychological issues associated with cystic fibrosis may arise from the patients' lifelong disease- and treatment-related burden. This Cochrane Review aimed to determine psychosocial and physical outcomes of psychological interventions. Trial registries, databases and professional networks were used to identify relevant studies. Altogether, 16 studies involving 556 participants were included. They were heterogeneous in their methods, design, target groups, and outcomes. Overall, the current evidence for psychological interventions is insufficient. Preliminary evidence was available for interventions targeting specific aspects of the treatment regimen, such as behavioural nutrition interventions.

A web-based psychological support program for caregivers of children with cystic fibrosis: a pilot study.

BACKGROUND: Parents caring for a child with Cystic Fibrosis (CF) are at high risk for psychological distress and have limited access to psychological care. Therefore, a web-based psychological support program for severely distressed parents of children with CF (WEP-CARE) was developed and evaluated for its feasibility and efficacy. METHODS: A clinical expert panel developed WEP-CARE based on principles of cognitive-behavioral therapy. This web-based writing therapy comprises nine sessions, tailored for the specific needs of caregivers. The pilot study was conducted as a single-group intervention with pre-post-follow-up design. Out of 31 participants, 23 parents completed the intervention (21 female; mean age 37 years; SD = 6.2 years, range 25 - 48 years). Psychological symptoms and quality of life were assessed online by self-report measures at pre- and post-treatment and were followed up three months later. RESULTS: On average, the caregivers' symptoms of anxiety decreased statistically significant and clinical relevant about five points from an elevated (M = 11.4; SD =2.6) to a normal level (M = 6.7; SD = 2.6; p < .001) between pre and post treatment. Fear of disease progression (p < .001) and symptoms of depression (p = .02) significantly decreased as well. Quality of life significantly improved (p = .01). The effects were maintained at the 3-months follow-up assessment. CONCLUSIONS: WEP-CARE is feasible and promising regarding its efficacy to improve parental mental health and quality of life.

Psychological interventions for individuals with cystic fibrosis and their families.

BACKGROUND: With increasing survival estimates for individuals with cystic fibrosis, long-term management has become an important focus. Psychological interventions are largely concerned with adherence to treatment, emotional and social adaptation and health-related quality of life. We are unaware of any relevant systematic reviews. OBJECTIVES: To determine whether psychological interventions for people with cystic fibrosis provide significant psychosocial and physical benefits in addition to standard medical care. SEARCH METHODS: Studies were identified from two Cochrane trials registers (Cystic Fibrosis and Genetic Disorders Group; Depression, Anxiety and Neurosis Group), Ovid MEDLINE and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search of the Cystic Fibrosis and Genetic Disorders Group's register: 19 December 2013.Most recent search of the Depression, Anxiety and Neurosis Group's register: 12 November 2013. SELECTION CRITERIA: Randomised controlled studies of a broad range of psychological interventions evaluating subjective and objective health outcomes, such as quality of life or pulmonary function, in individuals of all ages with cystic fibrosis and their immediate family. We were interested in psychological interventions, including psychological methods within the scope of psychotherapeutic or psychosomatic mechanism of action (e.g. cognitive behavioural, cognitive, family systems or systemic, psycho-dynamic, or other, e.g. supportive, relaxation, or biofeedback), which were aimed at improving psychological and psychosocial outcomes (e.g. quality of life, levels of stress or distress, psychopathology, etc.), adaptation to disease management and physiological outcomes. DATA COLLECTION AND ANALYSIS: Three authors were involved in selecting the eligible studies and two of these authors assessed their risk of bias. MAIN RESULTS: The review includes 16 studies (eight new studies included in this update) representing data from 556 participants. Studies are diverse in their design and their methods. They cover interventions with generic approaches, as well as interventions developed specifically to target disease-specific symptoms and problems in people with cystic fibrosis. These include cognitive behavioural interventions to improve adherence to nutrition or psychosocial adjustment, cognitive interventions to improve adherence or those associated with decision making in lung transplantation, a community-based support intervention and other interventions, such as self-hypnosis, respiratory muscle biofeedback, music therapy, dance and movement therapy, and a tele-medicine intervention to support patients awaiting transplantation.A substantial proportion of outcomes relate to adherence, changes in physical status or other specific treatment concerns during the chronic phase of the disease.There is some evidence that behavioural interventions targeting nutrition and growth in children (4 to 12 years) with cystic fibrosis are effective in the short term. Evidence was found that providing a structured decision-making tool for patients considering lung transplantation improves patients' knowledge of and expectations about the transplant, and reduces decisional conflict in the short term. One study about training in biofeedback-assisted breathing demonstrated some evidence that it improved some lung function measurements. Currently there is insufficient evidence for interventions aimed at other aspects of the disease process. AUTHORS' CONCLUSIONS: Currently, insufficient evidence exists on psychological interventions or approaches to support people with cystic fibrosis and their caregivers, although some of the studies were promising. Due to the heterogeneity between studies, more of each type of intervention are needed to support preliminary evidence. Multicentre studies, with consequent funding implications, are needed to increase the sample size of these studies and enhance the statistical power and precision to detect important findings. In addition, multicentre studies could improve the generalisation of results by minimizing centre or therapist effects. Psychological interventions should be targeted to illness-specific symptoms or behaviours to demonstrate efficacy.

Symptoms of depression impact the course of lung function in adolescents and adults with cystic fibrosis.

BACKGROUND: Epidemiological studies report high rates of depression among patients with cystic fibrosis (CF). Assuming a causal relationship between depression and the progression of CF, our hypothesis is that elevated symptoms of depression would be a predictor of worse lung function after two years. METHODS: In the context of the TIDES study, 473 German patients with CF (age 12-53 years, FEV1% predicted M = 66.2, range 13-137) completed the Hospital Anxiety and Depression Scale (HADS). Lung function (FEV1% predicted) was assessed at baseline and followed up two years later. Repeated measures analysis was performed involving the level of FEV1% and the level of depressive symptoms at baseline as independent factors and FEV1% at the 2-year follow-up as the dependent variable. RESULTS: Interaction between lung function and depression at baseline significantly affected the change in lung function at the 2-years observation interval. The largest decline in FEV1% occurred in depressed patients with good lung function at baseline. In contrast, patients without any clinically relevant depressive symptoms and with poor lung function at baseline showed a slight increase two years later. CONCLUSION: The findings emphasise the need to screen patients with CF for symptoms of depression and to treat co-morbid depression.

Parents' understanding of medication at discharge and potential harm in children with medical complexity.

OBJECTIVE: Children with medical complexity (CMC) are among the most vulnerable patient groups. This study aimed to evaluate their prevalence and risk factors for medication misunderstanding and potential harm (PH) at discharge. DESIGN AND SETTING: Cross-sectional study at a tertiary care centre. STUDY POPULATION: CMC admitted at Medical University of Vienna between May 2018 and January 2019. INTERVENTION: CMC and caregivers underwent a structured interview at discharge; medication understanding and PH for adverse events were assessed by a hybrid approach. MAIN OUTCOME MEASURES: Medication misunderstanding rate; PH. RESULTS: For 106 included children (median age 9.6 years), a median number of 5.0 (IQR 3.0-8.0) different medications were prescribed. 83 CMC (78.3%) demonstrated at least one misunderstanding, in 33 CMC (31.1%), potential harm was detected, 5 of them severe. Misunderstandings were associated with more medications (r=0.24, p=0.013), new prescriptions (r=0.23, p=0.019), quality of medication-related communication (r=-0.21, p=0.032), low level of education (p=0.013), low language skills (p=0.002) and migratory background (p=0.001). Relative risk of PH was 2.27 times increased (95% CI 1.23 to 4.22) with new medications, 2.14 times increased (95% CI 1.10 to 4.17) with migratory background. CONCLUSION: Despite continuous care at a tertiary care centre and high level of subjective satisfaction, high prevalence of medication misunderstanding with relevant risk for PH was discovered in CMC and their caregivers. This demonstrates the need of interventions to improve patient safety, with stratification of medication-related communication for high-risk groups and a restructured discharge process focusing on detection of misunderstandings ('unknown unknowns').

The Benefit of Detecting Reduced Intracellular B12 Activity through Newborn Screening Remains Unclear.

Vitamin B12 (B12) deficiency (B12D) can have detrimental effects on early growth and development. The Austrian newborn screening (NBS) program targets inborn errors of cobalamin metabolism and also detects B12D. Of 59 included neonates with B12D suspected by NBS, B12D was not further investigated in 16 (27%) retrospectively identified cases, not confirmed in 28 (48%), and confirmed in 15 (25%) cases. NBS and recall biomarkers were recorded. Age at sampling of the dried blood spots for NBS and the 1st-tier methionine/phenylalanine ratio were the strongest parameters to predict B12D (67.4% correct allocations). No differences between cases with confirmed, unconfirmed, or unknown B12D or differences to norms were observed for growth and psychomotor development (Vineland III scales, phone interviews with parents of children between months 10 and 14 of life). B12 intake was below recommendations in most mothers. NBS can detect reduced intracellular B12 activity. No advantage of NBS detection and treatment regarding infant cognitive development or growth could be proven. Since conspicuous NBS findings cannot be ignored, and to prevent exposing newborns to invasive diagnostics, assessment of maternal B12 status during pregnancy seems advisable.

Effects of alcohol mixed with energy drink and alcohol alone on subjective intoxication.

Recent studies suggest that the combination of caffeine-containing drinks together with alcohol might reduce the subjective feelings of alcohol intoxication-the so-called "masking effect". In this study, we aimed to review the effects of alcohol in combination with caffeine or energy drink with special focus on the "masking effect". Fifty-two healthy male volunteers were analysed concerning breath alcohol concentration and subjective sensations of intoxication using a 18 item Visual Analogue Scale in a randomised, double-blinded, controlled, four treatments cross-over trial after consumption of (A) placebo, (B) alcohol (vodka 37.5% at a dose of 46.5 g ethanol), (C) alcohol in combination with caffeine at a dose of 80 mg (equivalent to one 250 ml can of energy drink) and (D) alcohol in combination with energy drink at a dose of 250 ml (one can). Primary variables were headache, weakness, salivation and motor coordination. Out of four primary variables, weakness and motor coordination showed a statistically significant difference between alcohol and non-alcohol group, out of 14 secondary variables, five more variables (dizziness, alterations in sight, alterations in walking, agitation and alterations in speech) also showed significant differences due mainly to contrasts with the non-alcohol group. In none of these end points, could a statistically significant effect be found for the additional ingestion of energy drink or caffeine on the subjective feelings of alcohol intoxication. This within-subjects study does not confirm the presence of a "masking effect" when combining caffeine or energy drink with alcohol.

Cultural aspects in the management of inborn errors of metabolism.

European Health Care Systems have not yet accommodated both previous and current migration waves. Children from immigrant families, especially children with chronic conditions, are particularly affected from the shortcomings in medical care. One condition, phenylketonuria (PKU), is an inborn error of metabolism (IEM) which results in intellectual disability unless treated with a lifelong phenylalanine (Phe) restricted diet. In our PKU clinic, patients from families who previously had emmigrated from the geographic area of Turkey to Austria, exhibited worse blood Phe control and cognitive development than comparable patients from native Austrian families. Using structured and semi-structured interviews, questionnaires, and illness narratives, we identified language, psychosocial, economic, educational and cultural barriers as factors influencing adherence to treatment. Our findings led us to conclude that access to interpreter services, exploration of the socio-cultural background and of family ecology, as well as bi-directional communication and medical decision making according to the "best interest of the child" principle, may improve outcomes in patients requiring complex treatment and care.

Does immigration background influence outcomes after renal transplantation?

Migration implies differences in lifestyle, dietary and health behavior practice, and adherence, all of which are relevant factors in terms of disease outcome. However, renal transplantation in immigrant groups has been rarely studied in Europe. We have investigated the effect of immigration on outcomes in all children who underwent renal transplantation (RTx) at the Medical University of Vienna. From 1978 to 2007, 196 children underwent 236 RTx. In comparison to native recipients, immigrant recipients (31 boys, 17 girls) tended to be younger and male, with a higher rate of congenital renal diseases. The percentage of adolescent immigrant recipients tended to be lower, and living donation tended to be higher. In both the immigrant and native groups, RTx outcomes at 1, 5, and 10 years, including acute rejection rate (34 vs. 44, 55 vs. 62, 74 vs. 78%, respectively) and patient (98 vs. 92, 88 vs. 91, 80 vs. 82%, respectively) and graft survival (83 vs. 82, 79 vs. 65, 66 vs. 51%, respectively) were similar. All outcomes improved over time. In conclusion, this study demonstrates that outcomes in RTx are equivalent in immigrants and native recipients. Potential barriers to success among the Austrian immigrant recipient population may have been overcome by protective factors. These results should serve as a catalyst to retrieve data from larger databases to verify these single-center results.

A retrospective study on disease management in children and adolescents with phenylketonuria during the Covid-19 pandemic lockdown in Austria.

BACKGROUND: In classical phenylketonuria (PKU) phenylalanine (Phe) accumulates due to functional impairment of the enzyme phenylalanine hydroxylase caused by pathogenic variants in the PAH gene. PKU treatment prevents severe cognitive impairment. Blood Phe concentration is the main biochemical monitoring parameter. Between appointments and venous blood sampling, Austrian PKU patients send dried blood spots (DBS) for Phe measurements to their centre. Coronavirus disease-19 (COVID-19), caused by the SARS CoV-2 virus, was classified as a pandemic by the World Health Organization in March 2020. In Austria, two nationwide lockdowns were installed during the first and second pandemic wave with variable regional and national restrictions in between. This retrospective questionnaire study compared the frequency of Phe measurements and Phe concentrations during lockdown with the respective period of the previous year in children and adolescents with PKU and explored potential influencing factors. RESULTS: 77 patients (30 female, 47 male; mean age 12.4 [8-19] years in 2020) from five centres were included. The decline of venous samples taken on appointments in 2020 did not reach significance but the number of patients with none or only one DBS tripled from 4 (5.2%) in 2019 to 12 (15.6%) in 2020. Significantly more patients had a decline than a rise in the number of DBS sent in between 2019 and 2020 (p < 0.001; Chi2 = 14.79). Especially patients ≥ 16 years sent significantly less DBS in 2020 (T = 156, p = 0.02, r = 0.49). In patients who adhered to DBS measurements, Phe concentrations remained stable. Male or female sex and dietary only versus dietary plus sapropterin treatment did not influence frequency of measurements and median Phe. CONCLUSION: During the COVID pandemic, the number of PKU patients who stopped sending DBS to their metabolic centre increased significantly, especially among those older than 16 years. Those who kept up sending DBS maintained stable Phe concentrations. Our follow-up system, which is based on DBS sent in by patients to trigger communication with the metabolic team served adherent patients well. It failed, however, to actively retrieve patients who stopped or reduced Phe measurements.

High Rate of Living Kidney Donation to Immigrant Children Despite Disparities-An Epidemiological Paradox?

Background: Kidney transplantation is the preferred treatment modality for children with end-stage renal disease. In the adult population, migration-related modifiable factors were associated with low living donation rates; no such data are available on the pediatric population. This pilot study therefore compares donation modality, communication, knowledge, and attitudes/beliefs between families of immigrant and non-immigrant descent. Methods: Demographic and clinical characteristics of a cohort of children from 77 families of immigrant (32; 42%) and non-immigrant (45; 58%) descent who had undergone renal transplantation were assessed and related to donation modality at the Medical University of Vienna. In a representative subset, modifiable migration-related factors were assessed in a questionnaire-based study. Results: In immigrant families, information delay, limited communication, low knowledge levels, and self-reported conflicting beliefs were significantly more prevalent than in non-immigrants. The living kidney donation rate to children was high in both populations (immigrants: 63%, non-immigrants: 44%; p = 0.12). Living donation to children on dialysis was even significantly higher in immigrant families (immigrants: 13 out of 20; 57%, non-immigrants: 9 out of 33; 27%; p = 0.03). Conclusion: Contrary to expectations, migration-related disparities did not translate into decreased living donation rates in immigrant families, in particular to children on dialysis. Certain factors might therefore be less important for the living donation process in pediatric care structures and/or might be overcome by yet undefined protective factors. Larger pediatric studies including qualitative and quantitative methods are required to validate and refine current conceptual frameworks integrating the perspective of affected families.

Total homocysteine, B-vitamins and genetic polymorphisms in patients with classical phenylketonuria.

Hyperhomocysteinemia has occasionally been reported in patients with phenylketonuria (PKU) and B-vitamin deficiency. In our study total homocysteine (tHcy) and B-vitamins were measured in treated PKU patients and healthy controls. In the patients, dietary parameters and genetic polymorphisms affecting the Hcy pathway were investigated to identify parameters modulating tHcy. A case control study including 37 PKU patients and 63 healthy controls was conducted. t-Tests for independent samples were used to test between groups. Multiple regressions with tHcy as dependent variable were calculated. Hardy-Weinberg expectations were tested against the observed distribution of genotypes applying the Chi-square goodness-of-fit method. THcy concentrations were not significantly different (p=0.059) while folate and cobalamin (Cbl) concentrations were significantly higher in PKU patients compared to controls. However, 29.7% of patients had tHcy concentrations >97th centile. THcy did not vary with age nor correlate with folate and Cbl concentrations probably due to high saturatory levels. The presence of genetic polymorphisms had no impact on tHcy. In conclusion, in PKU patients treated with amino acid mixtures enriched with B-vitamins, tHcy is not significantly higher than in healthy controls, but tHcy concentrations exceed the 97th centile in about one third of patients. Even higher B-vitamin saturation may be required to further decrease tHcy concentrations and factors generally influencing tHcy such as betaine are to be investigated in PKU patients in the future.

Long-term follow-up of patients with congenital hyperinsulinism in Austria.

AIM: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria. PATIENTS AND METHODS: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2-25.5 years) in a follow-up study in the year 2004. RESULTS: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up. CONCLUSION: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

Transcultural pediatrics: compliance and outcome of phenylketonuria patients from families with an immigration background.

Living in a foreign country with a different lifestyle and a different orientation is a many-faceted challenge for immigrants. A considerable percentage (30-50%) of patients with metabolic disease come from immigrant families from Turkey and the Middle East. Phenylketonuria is one example of metabolic disease in which severe mental retardation can be entirely prevented by early detection via newborn screening and consistent dietary treatment. We report 7 phenylketonuria patients from 3 Turkish families who had considerable difficulty in coping with the diagnosis and adherence to the diet. Blood phenylalanine levels beyond recommended limits and IQ values below average, clearly demonstrate the risks arising from language as well as psychological and cultural communication barriers, despite standardized follow-up care structures and the observance of continuity by medical caregivers. To propose a basis for systematic improvement in the care of patients from immigrant families we suggest that a) the services of professional interpreters be used in case of language barriers; b) social workers with appropriate sociocultural and language competence should accompany the family in a professional manner; c) it would be meaningful to introduce treatment contracts that clearly establish the limits of the client's rights and duties as well as those of the care-givers. From the viewpoint of legislation, providing medical information is duty of the hospital and the use of translator is mandatory with patients from foreign countries and with foreign languages.

Fear of disease progression questionnaire for parents: Psychometric properties based on a sample of caregivers of children and adolescents with cystic fibrosis.

BACKGROUND: Parents caring for a child with a chronic somatic condition are at risk of increased distress and impaired quality of life. Fear of disease progression (FoP) is known to be an important source of distress in patients and their partners, and may be of relevance for parents as well. Existing measures are not applicable to parents. This study describes the adaptation of the FoP questionnaire for parental caregivers and investigated its psychometric properties. METHODS: Sixteen items appropriate for parents were derived from existing measures and from interviews with clinical experts in family-oriented psychosocial care. Factor structure, internal consistency, validity and sensitivity to change were analyzed in a clinical sample of 162 caregivers (M(age)=42.07 years, SD=6.0 years, 87.8% female) of a child with cystic fibrosis. RESULTS: The exploratory factor analysis reveal ed a two-factor structure, which was not supported by confirmatory analysis. Cronbach's α was examined for total score (.91) and significant positive correlations of the total score with anxiety (HADS: r=.70) and depression (CES-D: r=.60, BDI-II: r=.59), and a significant negative correlations with quality of life (r=-.66) could be demonstrated. A significant decrease in FoP was found (d=1.11) in a group of highly distressed caregivers undergoing web-based cognitive behavioral intervention. CONCLUSIONS: The FoP questionnaire is a reliable and valid instrument for parents of children with CF. Further studies in larger samples are needed to clarify dimensionality and validity among parents of children with other chronic conditions.

Self-concept in male and female adolescents with congenital heart disease.

Cognitive achievement, behavioural problems, and various dimensions of personality were assessed in 48 male and female patients with congenital heart disease (CHD) aged from 12 to 16 years in comparison to a control group. The CHD group showed a lower speed of cognitive processing but seemed to have less state-anxiety and to possess a higher superego strength. Male adolescents with CHD presented with a reduced perceived capacity and self-esteem. This was not true for adolescent girls with CHD. The negative self-concept of boys with CHD may be partly explained by reduced physical ability interfering with peer relationships.