The use of Patient Reported Outcomes (PROs) in daily clinical practice receives increasing attention. Former studies suggest that discussing PROs improves communication between physicians and patients and facilitates early recognition of HRQOL problems. Especially in paediatrics, there is a need to address HRQOL in daily clinical practice in the context of a child’s development; repeated measurement of HRQOL in different developmental stages can be valuable. With the use of PROs, HRQOL problems can be detected early and tailored intervention can be provided to the child, before the HRQOL problems increase. However research of PROs in paediatrics is still scarce. Children with Juvenile Idiopathic Arthritis (JIA) are at risk for HRQOL problems. Therefore, it is important to monitor their HRQOL during doctor’s visits. The aim of this study is to investigate the effectiveness of a web-based PRO about HRQOL (KLIK PROfile) in paediatric rheumatology practice.

Context: Long-term follow-up data on cognitive and motor functioning in adult patients with congenital hypothyroidism, diagnosed by neonatal screening, are scarce. Hence, it is still unclear whether the frequently reported cognitive and motor deficits observed during childhood persist in adulthood.Objective: The objective of this study was to examine cognitive and motor functioning in young adults with congenital hypothyroidism, born in the first 2 yr after the introduction of the Dutch neonatal screening program.Design/Setting/Patients: Seventy patients were tested (mean age, 21.5 yr); 49 of them were previously tested at 9.5 yr. The median age at the start of treatment was 28 d (range, 4-293 d). Congenital hypothyroidism was classified as severe, moderate, or mild, according to pretreatment T(4) concentrations.Main Outcome Measurement: The main outcome measurement was the influence of the severity of congenital hypothyroidism and age at which T(4) supplementation was started on cognitive and motor outcome.Results: Patients, particularly those with severe congenital hypothyroidism, had significantly higher (i.e. worse) motor scores (total score, 7.8; ball skills, 2.0; balance, 4.1) compared with controls (total score, 3.2; ball skills, 0.7; balance, 1.1), and lower full-scale (95.8), verbal (96.4), and performance (95.6) intelligence quotient (IQ) scores than the normal population. No significant change in IQ from childhood to adulthood was found, and for the majority of patients, motor score classification remained the same. The severity of congenital hypothyroidism, but not the starting day of treatment, was correlated with IQ and motor scores.Conclusions: It is concluded that the severity of congenital hypothyroidism, but not the timing of treatment initiation, is an important factor determining long-term cognitive and motor outcome. Clearly, detrimental effects on developmental outcome in patients with congenital hypothyroidism persist over time.

Objectives: As a result of advances in the treatment of childhood cancer many patients who may previously have had a limited life expectancy, are now surviving into adulthood. More insight is needed into the long‐term adjustment of young adult survivors of childhood cancer. The purpose of this study was to (1) assess health‐related quality of life (HRQoL), and (2) to explore the role of cognitive coping in relation to HRQoL.

Objective To describe employment achievement and social independence of adults with childhood end-stage renal disease (ESRD) and to explore determining factors.Study design Employment, occupational level, living arrangements, social engagements, and subjective health perception were cross-sectionally established between 1998 and 2000 in 144 of all living 187 adult Dutch patients with ESRD with an onset at age 0 to 15 years between 1972 and 1992. Potential clinical determinants were established by means of a review of all medical charts.Results Compared with age-matched Dutch citizens, patients were more often involuntarily unemployed (19.4% vs 11.1%), had a lower occupational level, more often still lived with their parents, and more often had no partner. A low occupational level was associated with a dialysis duration > 8 years (OR, 9.6; 95% Cl, 1.9-47.6); living at the parental home was associated with the male sex (OR, 3.4; 95% Cl, 1.5-7.8) and with a dialysis duration > 8 years (OR, 3.7; 95% Cl, 1.3-10.2).Conclusion Prolonged dialysis during childhood may decrease the ability to gain high-skilled professions and social independence. Unemployment is twice as high in adult patients with childhood ESRD than in healthy persons, but more than twice as low as compared with young ESRD patients with an adult onset of the disease. according to previous reports.

Background The aim of the present study is to describe the quality of life (QoL) of siblings of children with cancer and to predict it according to their health before the diagnosis of cancer in the ill child and their ways of coping with the illness.Methods Participants were 83 siblings from 56 families - 46 girls and 37 boys, aged 7-18. The assessment took place 1 month to 8 weeks after the diagnosis of cancer in the ill child. The siblings' QoL was assessed with the TNO-AZL children's quality of life questionnaire (TACQOL). Coping strategies were assessed with the Cognitive Coping Strategies Scale for siblings (CCSS-s). Physical problems and eating and sleeping problems that existed before the ill child was diagnosed were determined in a structured interview with the parents.Results A substantial number of siblings reported impaired cognitive and emotional QoL compared to the reference group. School-aged siblings (7-11 years) reported more trouble with motor functioning than peers. The coping strategy 'predictive control' (maintaining positive expectations regarding the illness) positively predicted siblings' QoL. The presence of health problems before diagnosis was negatively associated with siblings' QoL. Older siblings reported more negative emotions, while girls reported lower social QoL and reliance on 'interpretative control' (trying to understand the illness) was associated with fewer positive emotions.Conclusions During the first 2 months after the diagnosis of cancer in a brother or sister, siblings have relatively lower QoL than peers. Health problems that existed before diagnosis may be a predictor of later adjustment problems. Positive expectations about the course of the illness appear to protect siblings from distress. Information about the illness is a delicate issue that requires parental guidance.

Assessment of quality of life (QoL) has thus far been a neglected approach in describing psychological adaptation in siblings of seriously ill children. The present results concern differences and correspondences between parent- and child-reported QoL in siblings of paediatric cancer patients, at 1 month and 2 years after the diagnosis in the ill child. A total of 83 Siblings aged 7-18 participated in the study at 1 month after the diagnosis, 57 of these siblings (69%) participated in follow-up assessment 24 months later. The parent and child form of the TNO-AZL Children's Quality of Life questionnaire (TACQOL) and the Child Behaviour Check List (CBCL) and Youth Self-Report (YSR) were used to assess QoL and behavioural problems in siblings. The General Health Questionnaire (GHQ) was used to assess parent mental health. Mean differences, correspondences between informants and partial correlations with parent mental health were assessed at both measurement occasions. Correspondence between parent and child was low to moderate for most domains at both assessments. Low agreement was observed on several domains in our study group compared to reference data. Young siblings (ages 7-11) reported significantly more physical and motor problems at 1 and 24 months and less positive emotions at 24 months than their parents. Adolescent siblings reported more physical complaints at 1 month and more emotional and behaviour problems (YSR) at both assessments, but also reported higher social QoL than their parents at 24 months. Parent psychological distress was negatively correlated with parent-reported physical QoL in the sibling. The findings suggest that siblings of children with cancer experience a more serious burden from the illness than is perceived by the parents. Physical complaints and emotional problems remain mostly unnoticed, although distressed parents are more focused on the child's physical health. These results imply that assessment of self-reported well-being is especially relevant in siblings of a critically ill child, in order to obtain a realistic image of siblings' QoL. Further studies on sibling QoL are needed.

OBJECTIVEClassical galactosemia (McKusick 230400) is an autosomal recessive disorder of galactose metabolism caused by a deficiency of galactose-1-phosphate uridyltransferase (EC 2.7.712). Treatment, consisting of a severe restriction of dietary galactose, is life saving, but most patients develop abnormalities despite this diet. The aim of this study was to study the influence of galactosemia on the patients' health-related quality of life (HRQoL), on educational levels, and on the specific galactosemia-related concerns of these families.METHODSAge-specific HRQoL questionnaires, a classical galactosemia-specific questionnaire designed by the authors, and a list of questions regarding educational attainment were handed out or sent to all 75 members of the Dutch Galactosemia Society and their families.RESULTSSixty-three (84%) patients with classical galactosemia from 58 families returned the questionnaire. Concerning HRQoL, significant differences between patients aged 1 to 5 and healthy children were found on the domains of abdominal complaints and communication. Patients aged 8 to 15 years differed from their healthy peers on the domain of cognitive function. Mothers of patients aged 6 to 15 reported a significantly lower HRQoL on the domains of motor and cognitive function. Patients 16 years and older had significant lower scores on the domains of cognitive and social function. The percentage of patients who attend special schools is significantly higher than in the general population, and the educational attainment is significantly lower in patients with classical galactosemia.CONCLUSIONSThis is the first study to describe the HRQoL of patients with classical galactosemia using well-developed and validated instruments in different age groups. The results of the present study indicate that having galactosemia negatively influences the HRQoL. Early and regular evaluation and support of possible cognitive problems should be a major part of the protocol for the follow-up of patients with classical galactosemia.

OBJECTIVE:To assess associations of coping and family functioning with psychosocial adjustment in siblings of pediatric cancer patients at 1, 6, 12, and 24 months after diagnosis.METHODS:Eighty-three siblings (ages 7-19 years) participated. Effects on anxiety, quality of life, behavioral-emotional problems, and emotional reactions to the illness were investigated. Data-analysis was performed with multilevel mixed modeling.RESULTS:Psychosocial functioning was impaired at 1 month but ameliorated over time. Adjustment problems were associated with high family adaptation and cohesion, older age, and female gender. Lower anxiety, insecurity, loneliness, and illness involvement were related to siblings' ability to remain optimistic. Insecurity and illness involvement were positively related to reliance on the medical specialist and a tendency to seek information about the illness.CONCLUSIONS:Siblings of pediatric cancer patients are most affected by the illness in the first months. Children at risk may be identified according to sibling age and gender and according to long-term family adaptation processes and sibling coping abilities.

Previous research suggests that posttraumatic stress disorder (PTSD) is present in survivors of childhood cancer. The aim of the current study was to explore posttraumatic stress symptoms in a sample of young adult survivors of childhood cancer. In addition, the impact of demographic, medical and treatment factors on survivors' posttraumatic stress symptoms was studied.

Background: Constipation and encopresis frequently cause problems with respect to emotional wellbeing, and social and family life. Instruments to measure Health Related Quality of Life (HRQoL) in these disorders are not available. Methods: A disease specific HRQoL instrument, the “Defecation Disorder List” (DDL) for children with constipation or functional non-retentive faecal soiling (FNRFS) was developed using accepted guidelines. For each phase of the process, different samples of patients were used. The final phase of development included 27 children. Reliability was assessed in two ways: internal consistency of domains with Cronbach’s alpha, and test-retest reliability with intra-class correlation coefficients (ICC). To assess validity, comparable items and domains were correlated with Tacqol, a generic HRQoL instrument for children (TNO-AZL). Results: In the final phase of the development, 27 children completed the instrument. It consisted of 37 items in four domains. The response rate was 96%. Reliability was good for all domains, with Cronbach’s alpha values ranging from 0.61 to 0.76. Measures of test-retest stability were good for all four domains with ICCs ranging from 0.82 to 0.92. Validity based on comparison with the Tacqol instrument was moderate. Conclusion: The DDL is promising as a measure of HRQoL in childhood defecation disorders.

The developmental consequences in adulthood of growing up with childhood cancer are not well understood. The Course of life questionnaire was developed to assess the attainment of developmental milestones retrospectively and socio-demographic outcomes in young adulthood. The aim of this study was to assess the course of life and socio-demographic outcomes in young adult survivors of childhood cancer. Knowledge about possible gaps in the course of life could enable health care providers to aim for the most favourable course of life.A total of 353 Dutch survivors and a comparison group of 508 peers without a history of cancer, all aged between 18 and 30. filled in the Course of life questionnaire. The course of life of the survivors was found to be hampered. The young adult survivors of childhood cancer in the Netherlands turned out to have achieved fewer milestones than their peers with respect to autonomy development, social development, and psycho-sexual development, or to have achieved the milestones when they were older than their peers. In addition, survivors displayed less risk behaviour than the comparison group. The survivors and the comparison group also differed on some socio-demographic issues. A considerably lower percentage of survivors than peers in the comparison group were married or living together, and/or employed. Their educational level, on the other hand, was as high as that of their peers. Copyright (c) 2004 John Wiley & Sons, Ltd.

This study assessed quality of life, self-esteem and worries in young adult survivors of childhood cancer compared to a group of young adults with no history of cancer. The impact of demographic, medical and treatment factors and self-esteem on survivors' quality of life and worries was studied.Participants were 400 long-term survivors (LTS) of childhood cancer (age range 16-49 years, 45% female) who had completed treatment an average of 16 years previously and 560 persons (age range 16-53 years, 55% female) with no history of cancer. All participants completed the MOS-24 (Medical Outcome Study Scale), a Worry questionnaire consisting of three scales (cancer-specific concerns, general health concerns, present and future concerns). and the Rosenberg Self-Esteem Scale.Small to moderate differences were found in mean MOS-24 scores between the LTS group and controls (range effect sizes -0.36-0.22). No significant difference was found in the mean self-esteem scores between LTS and controls. Female LTS had more cancer-specific concerns than male LTS. In several related areas of general health, self-image and dying, the LTS group reported less worries than controls, but LTS worried significantly more about their fertility, getting/changing a job and obtaining insurance's. Multiple linear regression analysis revealed that female gender, unemployment, severe late effects/health problems and a low self-esteem were predictors of worse quality of life in survivors. In addition, age at follow-up, unemployment, years since completion of therapy and a low self-esteem were associated with a higher degree of survivors' worries.Quality of life and the level of self-esteem in LTS of childhood cancer is not different from their peers. Although many LTS worried not more or even less about health issues than their peers, they often are concerned about some present and future concerns. The investigated factors could explain poor quality of life and worries only to a limited extent. Further research exploring determinants and indices of quality of life and worries in LTS is warranted. Copyright (C) 2004 John Wiley Sons, Ltd.

This paper investigated educational achievement, employment status, living situation, marital status and offspring in 500 Dutch long‐term young adults survivors of childhood cancer (age range, 16–49 years, 47% female). The results were compared with a reference group of 1092 persons with no history of cancer (age range, 15–33 years, 55% female). The impact of demographic and medical characteristics on psychosocial adjustment was studied. All participants completed a self–report questionnaire. The results showed that, although many survivors are functioning well and leading normal lives, a subgroup of survivors were less likely to complete high‐school, to attain an advanced graduate degree, to follow normal elementary or secondary school and had to be enrolled more often on learning disabled programs. The percentage of employed survivors was lower than the percentage of employed controls in the comparison group, but more survivors were student or homemaker. Survivors had lower rates of marriage and parenthood, and worried more about their fertility and the risk of their children having cancer. Survivors, especially males, lived more often with their parents. Cranial irradiation dose ≤25 Gy was an important independent prognostic factor of lower educational achievement. Survivors with a history of brain/CNS tumours had a higher risk of being single than survivors with a diagnosis of leukaemia/non‐Hodgkin lymphoma. These results indicate that important aspects of life are affected in a substantial number of persons who have been diagnosed with cancer during childhood or adolescence. Copyright © 2002 John Wiley & Sons, Ltd.

staodaM error SE); 13(SE 6) per 24 hr vs 52(SE 6), p < 0.01), and high-amphtude propagating sequences 0"tAPS )(5 (SE 2) per 24 hr vs 99 (SE 1.4), p < 0.05), The amplitude of FL4PS pressure waves was less than controls (94 (SE 10) mmHg vs 117 (SE 3), p<0.01), but the amplitude ot retrograde propagating sequences was greater (43 (SE 6) mmHg vs 27 (SE 1), p<0 01).The distance travelled by ~PS was less than that of controls.Meal ingestion and waking did not provoke an increase in the fi'equency of HAPS or nonpropagating colonic activity that occurred in controls.Conclusions: Children with slow transit constipation show significant abnormalities in pre-and post-prandial colonic motor function Many of these changes are similar to those seen in adults with slow transit constipation.We speculate that Slow transit constipation in childhood occurs secondarily to the abnormal nlaturation of enteric neurotransmitters, T1339

Several studies have been conducted on sibling psychosocial adaptation to cancer in a brother or sister, but little is known on how the long‐term adaptation of siblings to the illness develops. The concept quality of life has primarily been applied in research on the effects of chronic illness on the affected patient, but has not yet been studied in siblings. Aims: To investigate the prevalence of self‐reported psychosocial problems in siblings of pediatric cancer patients, 2 years after the onset of the illness. Measurements: Two Dutch quality of life questionnaires, the TACQOL and the DUCATQOL are used, as well as generic non‐health‐related measures of emotional and behaviour problems (CBCL‐YSR) and anxiety (STAI‐C). Participants: Participants were 103 siblings aged 7–18 years old. Fifty seven Siblings participated in a prospective and 46 in a retrospective study group. Results: Siblings aged 7–11 report lower overall quality of life than children in the norm group. No differences in mean scores were found on any of the other domains that were investigated. When the prevalence of problems was investigated, however, relatively more siblings compared to normative data had scores in the impaired group based on the 20th percentile norm. A relatively high number of siblings aged 7–11 reported impaired emotional (42%), social (34%) and total quality of life (47%) (DUCATQOL) and physical problems (26%) (TACQOL). Relatively many adolescent siblings (26%) reported significant internalising problems on the CBCL‐YSR. Conclusions: Although acute emotional distress reactions seem to have normalised in most siblings as has been suggested in the literature, emotional distress of having a brother or sister with cancer may continue beyond diagnosis for a subgroup of children. Young siblings seem to be affected in their quality of life, whereas a subgroup of adolescent siblings experience clinically relevant internalising problems. The results support the use of quality of life measures for siblings. Predictors of long‐term adaptation in siblings need to be investigated. Copyright © 2003 John Wiley & Sons, Ltd.