An Exercise Program in Youngsters with Complex Congenital Heart Disease: does it improve Health Related Quality of Life and Psychosocial Functioning? A randomized controlled trial

__Abstract__ A 12-week standardized exercise program improved self-reported cognitive functioning, and parent-reported social functioning in children and adolescents with complex congenital heart disease. Furthermore, it resulted in a decrease in passive leisure time spending. In contrast, it had no effect on emotional and behavioural problems, on sports enjoyment, or on active leisure time spending. Parental mental health moderated changes in social functioning: adolescents in the exercise group whose parents’ overall mental health was worse, reported a pre-post decrease in social functioning, compared with controls

Association between nutritional status and subjective health status in chronically ill children attending special schools

Purpose: In hospitalized children with a chronic disease, malnutrition was associated with a lower subjective health status. In outpatient children with a chronic disease attending special schools, this association has never been studied. The aim of this study was to assess the association between nutritional status and subjective health status in chronically ill children attending special schools. Methods: Overall, 642 children, median age 9.8 years (IQR 7.7–11.5), 60 % male, 72 % Caucasian, were included in this prospective study in nine special schools for chronically ill children in the Netherlands. Overall malnutrition was assessed as: acute malnutrition (<−2 SDS for weight for height (WFH)) and chronic malnutrition (<−2 SDS for height for age). The malnutrition risk was assessed with the nutritional risk-screening tool STRONGkids. Subjective health status was assessed with EQ-5D. Results: Overall, 16 % of the children had overall malnutrition: 3 % acute and 13 % chronic malnutrition. Nurses reported ‘some/severe problems’ on the health status dimensions mobility (15 %), self-care (17 %), usual activities (19 %), pain/discomfort (22 %), and anxiety/depression (22 %) in chronically ill children. Their mean visual analogue scale score (VAS) was 73.0 (SD 11.1). Malnutrition, medication usage, and younger age explained 38 % of the variance of the VAS score. Conclusions: The presence of overall malnutrition in chronically ill children attending special schools was associated with lower subjective health status, especially in younger children and in those with chronic medication usage. Therefore, it is important to develop and use profile-screening tools to identify these children

Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents

Background: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated. Objective: To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents. Methods: A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires. Results: Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients. Conclusion: Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage

The eXpectations of Parents regarding Anesthesiology Study (XPAS) from a parental perspective:a two-phase observational cross-sectional cohort study

Background: Uncertainty concerning anesthetic procedures and risks in children requiring anesthesia may cause concerns in parents and caregivers. Aims: To explore parental expectations and experiences regarding their child's anesthesia using questionnaires designed with parental input. Methods: This observational cross-sectional cohort study included parents (including caregivers) of children undergoing anesthesia in a tertiary pediatric referral university hospital. The study consisted of two phases. In Phase 1, we developed three questionnaires with parental involvement through a focus group discussion and individual interviews. The questionnaires focused on parental satisfaction, knowledge, concerns, and need for preparation regarding their child's anesthesia. In Phase 2, independent samples of parents completed the questionnaires at three time points: before the preanesthesia assessment (T1), 2 days after the preanesthesia assessment (T2), and 4 days after the anesthetic procedure (T3). Results: In Phase 1, 22 parents were involved in the development of the questionnaires. The three questionnaires contained 43 questions in total, of which 10 had been proposed by parents. In Phase 2, 78% (474 out of 934) parents participated at T1, 36% (610 out of 1705), at T2 and 34% (546 out of 1622) at T3. Parental satisfaction scores were rated on a visual analogue scale for the preanesthesia assessment with a median of 87/100, and with a median of 90/100 for the anesthetic procedure (0: not satisfied and 100: satisfied). Parental concerns were rated with a median of 50/100 (0: no concerns and 100: extremely concerned). Parental answers from the questionnaire at T2 revealed significant knowledge deficits, with only 73% reporting that the anesthesiologist was a physician. Parents preferred to receive more information about the procedure, especially regarding the intended effects and side effects of anesthesia. Conclusions: Overall, parental satisfaction scores regarding the pediatric anesthesiology procedure were high, with a minority expressing concerns. Parents indicated a preference for their child's anesthesiologist to visit them both before and after the anesthetic procedure. Parental expectations regarding anesthesia did not completely correspond with the information provided; more information from the clinician about the intended effects and side effects of anesthesia was desired.</p

Health-related quality of life of children and their parents 2 years after critical illness

Background: Pediatric intensive care unit (PICU) survivors are at risk for prolonged morbidities interfering with daily life. The current study examined parent-reported health-related quality of life (HRQoL) in former critically ill children and parents themselves and aimed to determine whether withholding parenteral nutrition (PN) in the first week of critical illness affected children’s and parents’ HRQoL 2 years later. Methods: Children who participated in the pediatric early versus late parenteral nutrition in critical illness (PEPaNIC) trial and who were testable 2 years later (n = 1158) were included. Their HRQoL outcomes were compared with 405 matched healthy controls. At PICU admission, childre

Predictors of subjective health status 10 years post-PCI

Background: Subjective health status is an increasingly important parameter to assess the effect of percutaneous coronary intervention (PCI) in clinical practice.Aim of this study was to determine medical and psychosocial predictors of poor subjective health status over a 10 years' post-PCI period. Methods: We included a series of consecutive PCI patients (n = 573) as part of the RESEARCH registry, a Dutch single-center retrospective cohort study. Results: These patients completed the 36-item Short-Form Health Survey (SF-36) at baseline and 10 years post-PCI. We found 6 predictors of poor subjective health status 10 years post-PCI: SF-36 at baseline, age, previous PCI, obesity, acute myocardial infarction as indication for PCI, and diabetes mellitus (arranged from most to least numbers of sub domains). Conclusions: SF-36 scores at baseline, age, and previous PCI were significant predictors of subjective health status 10 years post-PCI. Specifically, the SF-36 score at baseline was an important predictor. Thus assessment of subjective health status at baseline is useful as an indicator to predict long-term subjective health status. Subjective health status becomes better by optimal medical treatment, cardiac rehabilitation and psychosocial support. This is the first study determining predictors of subjective health status 10 years post-PCI

Impact of critical illness and withholding of early parenteral nutrition in the pediatric intensive care unit on long-term physical performance of children:a 4-year follow-up of the PEPaNIC randomized controlled trial

Background: Many critically ill children face long-term developmental impairments. The PEPaNIC trial attributed part of the problems at the level of neurocognitive and emotional/behavioral development to early use of parenteral nutrition (early-PN) in the PICU, as compared with withholding it for 1 week (late-PN). Insight in long-term daily life physical functional capacity after critical illness is limited. Also, whether timing of initiating PN affects long-term physical function of these children remained unknown. Methods: This preplanned follow-up study of the multicenter PEPaNIC randomized controlled trial subjected 521 former critically ill children (253 early-PN, 268 late-PN) to quantitative physical function tests 4 years after PICU admission in Leuven or Rotterdam, in comparison with 346 age- and sex-matched healthy children. Tests included handgrip strength measurement, timed up-and-go test, 6-min walk test, and evaluation of everyday overall physical activity with an accelerometer. We compared these functional measures for the former critically ill and healthy children and for former critically ill children randomized to late-PN versus early-PN, with multivariable linear or logistic regression analyses adjusting for risk factors. Results: As compared with healthy children, former critically ill children showed less handgrip strength (p &lt; 0.0001), completed the timed up-and-go test more slowly (p &lt; 0.0001), walked a shorter distance in 6 min (p &lt; 0.0001) during which they experienced a larger drop in peripheral oxygen saturation (p ≤ 0.026), showed a lower energy expenditure (p ≤ 0.024), performed more light and less moderate physical activity (p ≤ 0.047), and walked fewer steps per day (p = 0.0074). Late-PN as compared with early-PN did not significantly affect these outcomes. Conclusions: Four years after PICU admission, former critically ill children showed worse physical performance as compared with healthy children, without impact of timing of supplemental PN in the PICU. This study provides further support for de-implementing the early use of PN in the PICU. Trial registration ClinicalTrials.gov, NCT01536275; registered on February 22, 2012.</p

Outcomes of Delaying Parenteral Nutrition for 1 Week vs Initiation Within 24 Hours Among Undernourished Children in Pediatric Intensive Care

_IMPORTANCE_ Undernourishment has been associated with poor outcomes of critical illness in children. The effects of withholding parenteral nutrition (PN) for 1 week in undernourished critically ill children are unknown. _OBJECTIVE_ To assess the outcome effects of withholding PN for 1 week in undernourished critically ill children. _DESIGN, SETTING, AND PARTICIPANTS_ This is a subanalysis of the randomized clinical trial Pediatric Early vs Late Parenteral Nutrition in Intensive Care Unit (PEPaNIC; N = 1440), which focused on the subgroup of pediatric intensive care unit (PICU) patients identified as undernourished on admission. Children included in the PEPaNIC trial were enrolled between June 18, 2012, and July 27, 2015. Undernourishment was defined as weight-for-age z score less than −2 in children younger than 1 year, and body mass index–for-age z score less than −2 in children 1 year or older. Data analysis was conducted from August 3, 2017, to July 6, 2018. _INTERVENTIONS_ Patients were randomized to initiation of supplemental PN within 24 hours (early PN) or after 1 week (late PN) when enteral nutrition was insufficient. _MAIN OUTCOMES AND MEASURES_ Primary end points were risk of new infections acquired in the PICU and time to live PICU discharge, assessed via multivariable logistic regression and Cox proportional hazard analyses, adjusted for risk factors. _RESULTS_ A total of 289 of 1440 children (20.1%), term newborn to age 17 years, were identified as undernourished, of whom 150 of 717 patients (20.9%) were in the late PN group and 139 of 723 patients (19.2%) were in the early PN group. On admission, characteristics were similar among the treatment groups. Mean (SD)weight z scoreswere −3.33 (1.18) in the late PN group and −3.21 (1.09) in the early PN group. Compared with well-nourished PICU patients, undernourishment on admission was associated with lower likelihood of an earlier live PICU discharge. Among undernourished PICU patients, late PN reduced the risk of new infections by 11.0%, and shortened the duration of PICU stay by a median of 2 days. The safety outcomes mortality, incidence of hypoglycemia during the first week, and incidence of weight deterioration during PICU stay were similar between the treatment groups. _CONCLUSIONS AND RELEVANCE_ In undernourished critically ill children, withholding PN for 1week was clinically superior to early PN