Long-term neurodevelopmental outcome after selective feticide in monochorionic pregnancies.

OBJECTIVE: To assess the incidence of and risk factors for adverse long-term neurodevelopmental outcome in complicated monochorionic pregnancies treated with selective feticide at our centre between 2000 and 2011. DESIGN: Observational cohort study. SETTING: National referral centre for fetal therapy (Leiden University Medical Centre, the Netherlands). POPULATION: Neurodevelopmental outcome was assessed in 74 long-term survivors. METHODS: Children, at least 2 years of age, underwent an assessment of neurologic, motor and cognitive development using standardised psychometric tests and the parents completed a behavioural questionnaire. MAIN OUTCOME MEASURES: A composite outcome termed neurodevelopmental impairment including cerebral palsy (GMFCS II-V), cognitive and/or motor test score of <70, bilateral blindness or bilateral deafness requiring amplification. RESULTS: A total of 131 monochorionic pregnancies were treated with selective feticide at the Leiden University Medical Centre. Overall survival rate was 88/131 (67%). Long-term outcome was assessed in 74/88 (84%). Neurodevelopmental impairment was detected in 5/74 [6.8%, 95% confidence interval (CI), 1.1-12.5] of survivors. Overall adverse outcome, including perinatal mortality or neurodevelopmental impairment was 48/131 (36.6%). In multivariate analysis, parental educational level was associated with cognitive test scores (regression coefficient B 3.9, 95% CI 1.8-6.0). Behavioural problems were reported in 10/69 (14.5%). CONCLUSIONS: Adverse long-term outcome in survivor twins of complicated monochorionic pregnancies treated with selective feticide appears to be more prevalent than in the general population. Cognitive test scores were associated with parental educational level. TWEETABLE ABSTRACT: Neurodevelopmental impairment after selective feticide was detected in 5/74 (6.8%, 95% CI 1.1-12.5) of survivors.

Neurodevelopmental outcome in twin anemia-polycythemia sequence after laser surgery for twin-twin transfusion syndrome.

OBJECTIVE: To evaluate the long-term neurodevelopmental outcome in children who developed twin anemia-polycythemia sequence (TAPS) after fetoscopic laser surgery for twin-twin transfusion syndrome (TTTS). METHODS: Neurological, motor and cognitive development was assessed in a consecutive cohort of TTTS survivors treated with laser surgery between 2004 and 2011 and complicated by post-laser TAPS. Primary outcome was neurodevelopmental impairment, a composite outcome including any of the following: cerebral palsy, bilateral deafness, blindness, severe motor and/or cognitive developmental delay (>2 SD below the mean). A risk analysis on cognitive outcome was performed. RESULTS: During the study period, 33/306 (11%) monochorionic twin pairs developed TAPS after laser surgery for TTTS. Survival was 53/66 (80%). Long-term outcome was assessed in 47/53 (89%) children. The incidence of neurodevelopmental impairment was 4/47 (9%), occurring in one donor (1/20; 5%) and three recipients (3/27; 11%) (P=0.63). Mild-to-moderate cognitive delay, i.e. scores below 85, was detected in 8/47 (17%) children. Risk factors for low cognitive scores were low gestational age at birth (P=0.02) and low birth weight (P<0.01). The lowest cognitive scores were detected in the subgroup of TAPS survivors treated with intrauterine transfusion (median score, 82.5). CONCLUSIONS: Neurodevelopmental impairment and cognitive delay were found in almost one in five children surviving post-laser TAPS. Better treatment and, ideally, prevention of this complication after laser treatment for TTTS is urgently needed.

Managing a dual role--experiences and coping strategies of parents donating haploidentical G-CSF mobilized peripheral blood stem cells to their children.

UNLABELLED: Hematopoietic stem cell transplantation is an effective therapy for life-threatening hematological diseases. Parents may be asked to donate hematopoietic stem cells for their child when no compatible related or unrelated donor is available. OBJECTIVE: Parents donating G-CSF mobilized peripheral blood stem cells simultaneously and uniquely fulfill the dual role of donor and caregiver for their ill child. The experiences of both sibling and unrelated stem cell donors have been extensively reported but not those of parental donors. METHODS: We therefore undertook a study specifically to investigate the experiences and coping strategies of parental stem cell donors. In-depth qualitative interviews were conducted with 13 parental donors, which were subsequently transcribed and subjected to thematic analysis. In addition, parental coping was assessed utilizing the Utrecht Coping List. RESULTS: Qualitative analyses revealed four main thematic categories describing the way parental stem cell donation was experienced, namely 'Hope and Fear', 'Need for Information', 'Do Anything for your Child' and 'Transplant Outcome' In addition parents noted similar difficulties which were unrelated to their specific role as a donor, for example they felt socially isolated. CONCLUSIONS: Individual information for the parents needs to address not only the transplantation procedure but particularly those aspects related to the donation process. We feel there is a need for a protocol specifically designed to support and coach parental donors.

Psychological functioning and disease-related quality of life in pediatric patients with an implantable cardioverter defibrillator.

The objective of this multicenter study was to evaluate psychological functioning and disease-related quality of life (DRQoL) in pediatric patients with an implantable cardioverter defibrillator (ICD) in The Netherlands. Thirty patients were investigated; the mean age was 16.3 years, and the mean duration of implantation was 3.6 years. To assess psychological problems, three domains of the Symptom Checklist (SCL-90-R) were administered to the 25 patients[13 years old. DRQoL was assessed with a disease-specific pediatric questionnaire, the short-form 11-item Worries About (WA)ICDs Scale. Patients C13 years old scored significantly higher than the reference group on the domains of anxiety, depression, and sleeping problems of the SCL-90-R (T = 7.5, p\0.001; T = 5.4, p\0.001; and T = 7.8, p\0.001, respectively). Patients who had received an (in)appropriate shock reported more depressive symptoms (T = 2.1, p\0.03). Patients with [2 years implant duration (N = 19) or who had received an (in)appropriate shock (N = 13) showed lower DRQoL scores on the modified WAICD (T = 2.1, p\0.04; T = 2.1, p\0.5, respectively). Age at implantation or underlying disease did not influence psychological problems or DRQoL. Young ICD patients showed more anxiety, depression, and sleeping disorders. Worries were increased among patients with ICD shocks and in those who had their ICD implanted for[2 years. To determine psychological problems and help children to learn to cope with shocks, proper guidance and monitoring of young ICD patients are recommended.

Health-related quality of life after completion of successful treatment for childhood cancer.

BACKGROUND: Previous studies have assessed health-related quality of life (HRQOL) during several treatment stages in children with cancer, but there is limited knowledge about HRQOL shortly after completing therapy. This study determined HRQOL of children with cancer shortly after the end of successful treatment compared with normative values. PROCEDURE: Several age-specific HRQOL questionnaires were administered: the ITQOL (generic, proxy-report, 0-4 years), CHQ PF 50 (generic, proxy-report, 5-7 years), Kidscreen (generic, self-report, 8-18 years) and Disabkids (chronic generic, self-report, 8-18 years). RESULTS: Children with cancer (N = 191, mean age 9.25, SD 5.06, 47.1% female) participated. Physical well-being was affected for all ages. Compared to normative values 0- to 7-year-olds were rated significantly lower on the majority of the scales. In addition, 12- to 18-year-olds had significantly better HRQOL than the norm on social scales. Compared to chronically ill norms, 8- to 18-year-olds demonstrated no differences, except for 12- to 18-year-olds who experienced significantly more physical limitations. Additionally, we found that HRQOL of parents of 0- to 7-year-olds was poorer than the norm. CONCLUSION: HRQOL in children with cancer and their parents can be impaired compared with the norm. Therefore, HRQOL should be monitored in clinical practice to make paediatric oncologists aware of these problems. For young children, we recommend checking whether certain HRQOL problems can be explained by parental worries. For older children and adolescents, paediatric oncologists need to consider social desirability and the child's adaptive style.

Parental stress before, during, and after pediatric stem cell transplantation: a review article.

GOALS OF WORK: Pediatric stem cell transplantation (SCT) is a stressful treatment for children with relapsed or high-risk malignancies, immune deficiencies and certain blood diseases. Parents of children undergoing SCT can experience ongoing stress related to the SCT period. The aim of this article was to present a literature review of articles on parental distress and adaptation before, during, and after SCT and to identify risk and protective factors. MATERIALS AND METHODS: The review was conducted systematically by using PubMed, Web of Science, PsychInfo, and Picarta databases. Eighteen articles met our inclusion criteria: publishing date between January 1, 1990 and January 1, 2009; studies concerning parents of children undergoing SCT; studies examining the psychological adjustment and/or stress reactions of parents as primary outcomes and studies available in English. MAIN RESULTS: Highest levels of parental stress are reported in the period preceding SCT and during the acute phase. Stress levels decrease steadily after discharge in most parents. However, in a subgroup of parents, stress levels still remain elevated post-SCT. Parents most at risk in the longer term display highest levels of stress during the acute phase of the SCT. CONCLUSIONS: Psychosocial assessment before SCT, during the acute phase and in the longer term, is necessary to identify parents in need for support and follow-up care.

[Fraud in medicine with the child as victim. Munchausen syndrome by proxy. A review of the literature]. / Bedrog in de geneeskunde met het kind als inzet. Het Münchhausen syndroom bij proxy. Een overzicht van de literatuur.

In a review of the literature on the subject 'Münchhausen syndrome by proxy' the authors try to give an impression of the history of diagnosis and treatment of this special kind of child abuse. Particularly aspects of confrontation are discussed. They consider features of the chief actors in this very dramatic play. The authors point to problems in differential diagnosis in the framework of other subjects like 'non-accidental poisoning', 'doctor-shopping' and 'filicide'. They discuss ethical and legal consequences.

[Ritalin: a stimulating drug in the treatment of children with minimal brain dysfunction (MBD)]. / Ritalin: een stimulans bij de behandeling van kinderen met minimal brain dysfunction (MBD).

Children with Minimal Brain Dysfunction (MBD) are vulnerable in society. Therapy consists of a combination of behaviour modification techniques, function training, psychotherapy, family therapy and counseling of parents and teachers. This way of treatment is not appropriate for all children with MBD. Some of them need additional therapy with a psychotropic drug e.g. methylphenidate (Ritalin). We describe a procedure to establish, whether Ritalin improves the therapeutic impact in a specific child. Eight children enrolled the program. In seven Ritalin improved the therapeutic possibilities for a longer period of time. The dose for an optimal therapeutic effect varied individually, and was relatively low (0.4-0.8 kg/kg/dg). The hypothesis concerning the effect of psychotropic drugs in MBD and the pharmacological properties of Ritalin are discussed. We believe this procedure to be applicable in any multidisciplinary setting, experienced with behavioural and learning problems in childhood.

Biomechanical conceptual design of a passive transfemoral prosthesis.

In this study, we present the conceptual design of a fully-passive transfemoral prosthesis. The proposed design is inspired by the analysis of the musculo-skeletal activity of the healthy human leg. In order to realize an energy efficient device, we introduce three storage elements, which are responsible of the energetic coupling between the knee and the ankle joints. Simulation results show that the power storage of the designed conceptual prosthesis is comparable with the human gait.

Parental stress and perceived vulnerability at 5 and 10 years after pediatric SCT.

With the aim of assessing parental stress after SCT, 73 parents of children and adolescents who underwent SCT 5 or 10 years ago responded to questionnaires on general distress (General Health Questionnaire (GHQ)), disease-related stress (Pediatric Inventory for Parents-short form (PIP-SF)) and perceptions of child vulnerability (Child Vulnerability Scale (CVS)). General distress scores were comparable with the reference groups, but 40% of the mothers at 5 years after SCT reported increased stress levels as compared with 26% in the community-based reference group. Disease-related stress was comparable with the reference group of parents of children who were just off cancer treatment, 5 years after SCT. At 10 years after SCT, scores were lower than the reference group. Perceived child vulnerability did diminish over time, but remained high in parents of SCT survivors, compared with parents of healthy children: 96% of the parents at 5 years after SCT and 76% of the parents at 10 years after SCT scored above the cutoff point. Perceived vulnerability was found to be a predictor for parental disease-related stress. To conclude, although most parents of SCT survivors are resilient, the majority of parents perceive their child to be much more vulnerable as compared with parents of healthy children. This perception is associated with disease-related stress and may induce overprotective parenting.

Health-related quality of life problems of children aged 8-11 years with a chronic disease.

In paediatric research, Health-Related Quality-of-Life (HRQoL) has received increasing recognition as an important health outcome. This study aimed to investigate the nature and prevalence of HRQoL problems in children with different chronic diseases. Data were available on 318 children aged 8-11 years with different diseases: congenital heart disease (n = 50); coeliac disease (n = 105); asthma (n = 32); cancer (n = 23); juvenile chronic arthritis (n = 45); children with capillary haemangioma (n = 25) and severe meningococcal disease (n = 38). They all answered a validated generic instrument [TNO-AZL Children's Quality of life questionnaire] (TACQoL), in the outpatient clinic or at home. Analyses of variance were performed to investigate differences in mean scores for children with chronic conditions in comparison to healthy children. Prevalence of children at risk for substantial HRQoL problems was based on the 25th percentile in the norm population. In comparison to healthy children, only a small number of differences were found in mean scores of children studied. In contrast, prevalence of HRQoL problems in children with chronic diseases was higher in several domains. It is concluded that using an indicator variable of the norm 25th percentile seems important in identifying at-risk children with chronic disease.

Psychometric properties of the TACQOL-asthma, a disease-specific measure of health related quality-of-life for children with asthma and their parents.

The disease-specific-TACQOL-asthma questionnaire measures health-status and appraisal of health-status. The TACQOL-asthma evaluates the personal feelings about problems in the domains, 'complaints, situations, emotions, treatment and medication'. The TACQOL-asthma can be used alone or in combination with the generic TACQOL. Our objective was to study the psychometric properties of the TACQOL-asthma-questionnaire. Responses of 298 parents and children with asthma (age eight to 16 years) in four paediatric practices in the northern part of The Netherlands were studied. The factor-analysis and item-domain correlation analysis show a moderate to strong correlation between the different items and their hypothesised domains. For all items, the correlation of the separate item with the hypothesized domain is stronger than with any other domain. The internal consistency (Cronbach's alpha) of the domains is moderate to good. Concurrent correlation with the Paediatric-Asthma-Quality-of-Life-Questionnaire-(PAQLQ) was significant. Effect sizes of differences between asthma-severity classes in TACQOL-asthma and PAQLQ-scores were similar and of clinical importance. This study validates the TACQOL-asthma as a new disease-specific questionnaire. The TACQOL-asthma ensures a measurement of health status as well as appraisal of health problems. The TACQOL-asthma has good reliability and validity properties to serve as an evaluative and discriminate disease-specific health-related-quality-of-life questionnaire.

Clinicians' perspective on quality of life assessment in paediatric clinical practice.

AIM: This study was undertaken to investigate paediatric clinicians' views on and use of quality of life (QoL) assessment in clinical practice. METHODS: A survey was conducted among members of the Dutch Paediatric Association via e-mail. RESULTS: Over half of the 303 respondents (57%) believed that it was possible to use QoL questionnaires in clinical practice. The majority indicated that assessing QoL was beneficial and that it was especially necessary to assess QoL in children with a chronic disease (82%). Although only a minority (17%) currently used QoL questionnaires, most respondents would want to use QoL questionnaires in the future (76%). Obstacles that prevent the use of QoL questionnaires are the extra time needed for assessment, the unavailability of standardized questionnaires and insufficient knowledge about QoL. CONCLUSION: This survey shows that paediatric clinicians are positive towards QoL assessment, but that certain obstacles prevent the use of questionnaires. Thus, to facilitate future use, QoL questionnaires need to be developed from the perspective of the paediatrician. This means that they need to be developed for clinical use and brought to the attention of the paediatric health care community, demonstrating their validity to child health care.

Health-related quality of life in children with celiac disease.

We have investigated the health-related quality of life of children with celiac disease (n = 133) using two generic and one disease-specific questionnaires. In general, the children reported an adequate quality of life, similar to that of the reference sample (n = 1183).

Quality of life in preschool children born preterm.

The relationship of preterm birth to health-related quality of life (HRQoL) was examined for children aged 1 to 4 years. Three gestational age groups with a NICU history were selected, <32 weeks (n=65), 32 to 36 weeks (n=41), 237 weeks (n=54), and a reference group from the open population (n=50). The main instrument was the TNO-AZL Preschool Quality Of Life (TAPQOL) questionnaire, which was completed by the parents. In addition, other outcome measures obtained from parents or neonatologists were investigated. Children born <32 weeks had significantly lower HRQoL than the reference group in the scales for lungs, stomach, eating disorders, motor functioning, communication, and anxiety. Parental feelings towards the child were related to the child's HRQoL. We found differences between the neonatologists' and parents' perceptions of the children's situation, which can have clinical consequences (e.g. different opinions about what needed treatment). Neonatal intensive care after birth has HRQoL implications for all children, particularly in children born at <32 weeks of gestation.

Quality of life in paediatric inflammatory bowel disease measured by a generic and a disease-specific questionnaire.

UNLABELLED: This study assessed the impact of inflammatory bowel disease (IBD) on the health-related quality of life (HRQoL) of children and adolescents, using both a generic and a disease-specific instrument. Three questionnaires were sent to all patients (8-18 y old) from the database of two large secondary/tertiary hospitals in the western part of The Netherlands. In total, 83 (66%) children responded, 18 were between 8 and 12 y old and the remaining 65 were older. HRQoL was measured using a generic instrument, the TNO-AZL Children's Quality of Life questionnaire (TACQOL), assessing seven domains, and the Impact-II (NL), a disease-specific instrument assessing six domains. Disease activity was measured by a five-item symptom card. Compared with scores from a large reference population (n= 1810), younger children with IBD had a comparable HRQoL (measured by the TACQOL) on six domains, and better cognitive functioning, although they did not have severely active disease. Adolescent patients with IBD had a significantly impaired HRQoL on four domains (body complaints, motor functioning, autonomy and negative emotions). The Impact-II discriminated well between patients with varying disease activity states on all domains. CONCLUSION: Adolescents with IBD have a severely affected HRQoL. Impairment on motor functioning and autonomy is a threat to gaining independence from caregivers, and a high occurrence of negative emotions places patients at risk for depressive and behavioural disorders. The Impact-II is recommended for clinical use because of its high discriminative validity.

Development and psychometric evaluation of the TAPQOL: a health-related quality of life instrument for 1-5-year-old children.

The 43-item TNO-AZL Preschool Children Quality of Life (TAPQOL) questionnaire was developed to meet the need for a reliable and valid instrument for measuring parent's perceptions of health-related quality of life (HRQoL) in preschool children. HRQoL was defined as health status in 12 domains weighted by the impact of the health status problems on well-being. The aim of this study was to evaluate the psychometric performance of the TAPQOL. A sample of 121 parents of preterm children completed the TAPQOL questionnaire (response rate 88%) as well as 362 parents of children from the general population (response rate 60%). On the base of Cronbach's alpha, item-rest correlation, and principal component analysis, the TAPQOL scales were constructed from the data for the preterm children sample. The psychometric performance of these scales was evaluated for both the preterm children sample and the general population sample. Cronbach's alpha ranged from 0.66 to 0.88 for the preterm children sample and from 0.43 to 0.84 for the general population sample. The unidimensionality of the separate scales was confirmed by principal component analysis for both the preterm children sample and the general population sample. Spearman's correlation coefficients between scales were, on average, low. T-tests showed that the very preterm children, the children with chronic diseases, the less healthy and the less happy children had lower mean scores on the TAPQOL scales than healthy children, indicating a worse quality of life. This study shows that the TAPQOL is a reliable and valid parent's perception of HRQoL in preschool children. More research is needed to evaluate the psychometric performance of the TAPQOL in different clinical populations.

Health status development in a cohort of preterm children.

OBJECTIVE: To determine the impact of preterm birth on health status (HS) development at the ages of 5 and 10 years in a cohort of children born before term. SAMPLE: Six hundred eighty-eight children, born in 1983 with a gestational age of <32 weeks and a birth weight of <1500 g. DESIGN: Prospectively collected HS variables, obtained from the parents, were analyzed in a longitudinal perspective by using principal component analyses. RESULTS: One third of the sample had minor to severe HS problems at both ages of measurement. One third had problems on one assessment only. The remainder of the sample had no HS problems at either age. The analyses grouped the HS variables into 3 combinations. Problems in basic functioning, such as mobility or speech, decreased with age. Negative moods substantially increased, and concentration problems increased slightly. Specifically at risk were preterm born children with handicaps, boys, and children who were small for gestational age. CONCLUSION: According to the parents, one third of the cohort had no HS problems at either age. The pattern of HS problems of the preterm born children changed between 5 and 10 years of age.

Measuring health-related quality of life in children: the development of the TACQOL parent form.

Health-related quality of life (HRQoL), conceptualized as patients' own evaluations of their health status, is an important criterion in evaluation health and health care and in the treatment of individual patients. Until now, few systematic attempts have been made to develop instruments to assess the HRQoL of children using such a conceptualization. This article describes the conceptualization and results of a study aiming to develop such an instrument for children aged 6-15 years using their parents as a proxy. The feasibility and psychometric performance of the instrument were evaluated in a study of 77 patients of the paediatric out-patient clinic of Leiden University Hospital. For each of the a priori-defined domains, a parent form scale could be constructed with satisfactory reliability and moderate correlations with the other scales. Only some of the parents indicating health status problems also signalled negative reactions to these problems. This is, in our view, a strong argument for the distinction between health status and quality of life (QoL). The correlation coefficients between the parent form and a children's questionnaire were low. Overall, the psychometric performance of the TACQOL parent form looks promising, which suggests that this instrument--with some modifications--can indeed be used to assess group differences in HRQoL in children. The results, however, should be replicated in larger samples, currently under study. The relation between parents' proxy reports on the HRQoL of their children and children's self-reports needs further investigation.

The proxy problem: child report versus parent report in health-related quality of life research.

This study evaluates the agreement between child and parent reports on children's health-related quality of life (HRQoL) in a representative sample of 1,105 Dutch children (age 8-11 years old). Both children and their parents completed a 56 item questionnaire (TACQOL). The questionnaire contains seven eight-item scales: physical complaints, motor functioning, autonomy, cognitive functioning, social functioning, positive emotions and negative emotions. The Pearson correlations between the child and parent reports were between 0.44 and 0.61 (p < 0.001). The intraclass correlations were between 0.39 and 0.62. On average, the children reported a significantly lower HRQoL than their parents on the physical complaints, motor functioning, autonomy, cognitive functioning and positive emotions scales (paired t-test: p < 0.05). Agreement on all of the scales was related to the magnitude of the HRQoL scores and to some background variables (gender, age, temporary illness and visiting a physician). According to multitrait-multimethod analyses, both the child and parent reports proved to be valid.