Do children with asymptomatic ventricular preexcitation have similar quality of life as healthy children?

BACKGROUND: To our knowledge, no studies have assessed quality of life (QoL) in asymptomatic children with a preexcitation electrocardiogram pattern. AIM: To evaluate the QoL of children with asymptomatic Wolff-Parkinson-White (WPW) syndrome. METHODS: This study involved QoL assessment of 31 children with asymptomatic preexcitation and 82 healthy children using the WHOQOL-BREF and the Pediatric Arrhythmia Related Score (PARS), a specific questionnaire that we have developed, which is related to patients' feelings and observations concerning arrhythmia. RESULTS: There were no significant differences between the two groups in all the measured domains; however, there were significant differences regarding general satisfaction with their health condition (P = 0.01). There were no differences in general satisfaction with the QoL, but WPW children more often experienced palpitations than the control group (P <0.001) and were more likely to feel sad (P = 0.046) and nervous (P = 0.04) compared to healthy children. CONCLUSIONS: The children with WPW were more dissatisfied with their health compared to healthy children. Although both groups of children had similar levels of satisfaction with their QoL, some areas of physical and psychological parameters of QoL were worse in WPW children. The PARS questionnaire is a useful tool as a disease-specific QoL instrument, which supplements the general questionnaire and aids in clinical practice and decision-making.

Subjective Psychophysical Experiences in the Course of Inflammatory Bowel Disease-A Comparative Analysis Based on the Polish Pediatric Crohn's and Colitis Cohort (POCOCO).

No gold standard is available to evaluate subjective psychophysical experiences in pediatric inflammatory bowel disease (IBD). We aimed to assess pain, anxiety, and limitations in social activities at diagnosis and the worst flare of the disease in relation to clinical expression, treatment and IBD severity. A total of 376 children completed the survey (Crohn's disease (CD) n = 196; ulcerative colitis (UC) n = 180). The questionnaire included 12 questions regarding pain, anxiety, and social activity, all assessed at recruitment and retrospectively at diagnosis and worst flare using a numeric rating scale. Patients that had ever been treated with systemic glucocorticosteroids scored higher in pain (p < 0.001), anxiety (p = 0.015), and social activity domains (p < 0.016) at worst flare, and the answers correlated with the number of steroid courses (p < 0.0392). The perception of social activity limitations also correlated independently with the number of immunosuppressants (p < 0.0433) and biological agents (p < 0.0494). There was no difference in retrospective perception of pain, anxiety and social activity limitations between CD and UC patients at diagnosis and the worst flare. The level of limitations in social activity correlated with hospitalisations due to relapse, days spent in the hospital, number of relapses, and severe relapses with the strongest association of rho = 0.39 (p = 0.0004). Subjective and retrospective perception of pain, anxiety, and limitations in social activity differs depending on therapy, correlates with treatment modalities, and severity measures such as hospitalisations.

Overweight in classical phenylketonuria children: A retrospective cohort study.

PURPOSE: This cohort study aimed to determine the frequency of overweight and obesity in classical phenylketonuria children and to identify the possible influence of metabolic control on the BMI of the studied patients. PATIENTS AND METHODS: The study group included 63 classical phenylketonuria patients (40 girls and 23 boys; aged 5-16 years). Their z-score BMI, metabolic control, educational level of parents and socioeconomic status were determined. RESULTS: Twenty children were overweight or obese and only three were underweight. The percentages of overweight and obese children were 31.7% for the whole group, 21.7% (5 out of 23) for boys and 37.5% (15 out of 40) for girls. Overweight and obesity in these phenylketonuria patients was statistically significantly more frequent when compared to national reference studies (p = 0.0031). The five-year index of dietary control and the percentage of spikes exceeding 6 and 12 mg/dl (Spikes 6 and 12) indicated better metabolic control in the case of normal weight children than those who were overweight and obese (p < 0.049, p < 0.041 and p < 0.011, respectively). The odds ratio of being overweight or obese for those having poorer metabolic control (values higher vs lower than mean) was statistically significantly higher than for the remaining patients (for Spikes 12: 6.926 < 95%CI: 2.011-23.854 > ; p < 0.002). These results strongly suggest a link between overweight and diet non-compliance. CONCLUSIONS: Children with classical phenylketonuria presented higher odds of being overweight or obese as compared with reference national studies, with girls only having a higher frequency of overweight.

Therapy compliance in children with phenylketonuria younger than 5 years: A cohort study.

BACKGROUND: Phenylketonuria (PKU) is a metabolic disease. It is manifested by a complete or partial inability to convert phenylalanine (Phe) to tyrosine and leads to increased concentrations of Phe in the blood and in other tissues, including the brain, causing irreversible neurological damage if left untreated. Low-phenylalanine diet is a key component of classical PKU therapy. OBJECTIVES: The objective of this study was to assess the effectiveness of classical phenylketonuria therapy and compliance with doctors' recommendations in the first 5 years of life. MATERIAL AND METHODS: Data was collected from all diagnosed and treated patients (n = 57) born 1999-2010. Phenylalanine blood levels, the number of visits to a specialist outpatients' center, the number of blood tests, as well as socioeconomic status (SES) and parents' education level have been analyzed, and potential relationships have been assessed. RESULTS: In the 1st year of life patients visited their doctors (odds ratio (OR) = 6.8267; 95% confidence interval (95% CI) = 2.827-16.5163; p < 0.0001) and had their blood collected (OR = 2.7875; 95% CI = 1.0467-7.4234; p < 0.0402) significantly more frequently than in the 2nd year. This tendency persisted into subsequent years. Similarly, in infancy they had statistically significantly lower odds of exceeding more than 40% of their Phe levels over therapeutic range than 1 year later (OR = 3.6078; 95% CI = 1.4859-8.7599; p < 0.0046). No PKU child had more than 70% of Phe levels over the therapeutic range in the 1st year of life, whereas 4 years later there were 18 such children. Phe levels were correlated with the number of visits to a specialist (ρ = 0.39) and the number of Phe blood tests with index of dietary control (ρ = -0.33). The effectiveness of therapy and compliance with the doctor's recommendations seem to depend neither on the level of education of the patient's parents nor on their SES. CONCLUSIONS: Therapy effectiveness and patients' compliance in PKU is very good in infancy. However, both deteriorate in subsequent years. Moreover, they do not seem to depend on the family background.

Evaluation of medical and psychological parameters of quality of life in supraventricular tachyarrhythmia children. A comparison with healthy children.

INTRODUCTION: There are only a few available studies evaluating quality of life (QoL) in pediatric patients with cardiac arrhythmia. The aim of the study was to evaluate medical and psychological parameters of the QoL in children with a diagnosed supraventricular tachyarrhythmia (SVT) and to compare the obtained data with a group of healthy children (HC). MATERIAL AND METHODS: Inclusion criteria: children aged 7-18 with SVT, treated at Poznan University of Medical Sciences, Department of Pediatric Cardiology. The evaluation tools were the WHOQOL-BREF instrument and a questionnaire related to the patient's feelings and observations concerning arrhythmia (Pediatric Arrhythmia Related Score - PARS), developed by the authors and adjusted to the group of arrhythmia patients. RESULTS: The study included 180 SVT children and 83 HC. On the basis of WHOQOL-BREF the SVT group was found to have lower assessment values of QoL within the physical domain (Phd) (mean ± SD: 65.7 ±15.8 vs. 81.6 ±12.8; p < 0.0001) and psychological domain (Psd) (mean ± SD: 75.8 ±15.2 vs. 81.3 ±14.1; p < 0.005). No significant differences were found within the social relationships domain or the environment domain. On the basis of PARS in the SVT group the patients reported significantly increased symptoms within Phd (mean ± SD: 2.3 ±0.7 vs. 1.6 ± 0.3; p < 0.0001) as well as increased negative feelings within Psd (mean ± SD: 2.3 ±0.7 vs. 2.1 ± 0.6; p < 0.005). CONCLUSIONS: Medical and psychological parameters of the QoL in SVT children are significantly lower in comparison with HC. A diagnosis of SVT has no influence on the social and environmental areas of QoL. The PARS appears to be a useful tool to supplement the generic questionnaire for QoL evaluation in SVT children.