Search

Background/objectives: Children treated for cancer are at risk to develop cognitive problems. Insight in underlying associations with emotional functioning and fatigue can be used to optimize interventions. We therefore aim to study emotional functioning, fatigue, and cognitive functioning in children postcancer treatment and investigate whether fatigue mediates the relationship between emotional and cognitive functioning., Design/methods: Emotional functioning, fatigue, and cognitive functioning were assessed in children post-cancer treatment using subscales of the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scales, Multidimensional Fatigue Scale and Cognitive Functioning Scale. A one sample t-test was used to compare outcomes with general population peers and mediation analysis was used to address the effect of fatigue on the relationship between emotional and cognitive functioning., Results: A total of 137 children (mean age: 13.6, SD ± 3.3 years; mean time since end of treatment: 7.1 months, SD ± 5.9) participated. Lower scores on emotional functioning (Cohen's d [D]: 0.4), fatigue (D: 0.8) and cognitive functioning (D: 0.6) were found (p < .001) in children post-cancer treatment than in peers. A medium association was found between emotional and cognitive functioning (standardized regression coefficient [β]: 0.27, p < .001), which was mediated by fatigue (β = 0.16)., Conclusions: Outcomes on emotional and cognitive functioning are decreased and fatigue is increased in children postcancer treatment. Fatigue mediates the relationship between emotional and cognitive functioning. Our results show the importance to focus on fatigue amongst stress as a target for intervention to improve cognitive functioning., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

The survival of childhood brain tumors has improved in the past 30 years, but acquired brain injury due to damage caused by tumor invasion and side effects of different treatment modalities frequently occurs. This study focused on residual impairments, health-related quality of life (HRQoL), and emotional and behavioral problems in 2 cohorts of survivors diagnosed and treated for various types of brain tumors. Survivors in the 2004 cohort visited the Erasmus Medical Centre for standardized follow-up between 2003 and 2004, and in the 2014 cohort, between 2012 and 2014. Data of neurologically impairments of all children were extracted from medical records. Parents and survivors filled out questionnaires on quality of life and emotional and behavioral problems. In both cohorts, approximately 55% of the survivors displayed neurologic impairments. In comparison with the healthy reference group, a reduced parent-reported quality of life was found on the Motor, Cognition, and Autonomy (Cohort 2004) scales. Comparison between the cohorts showed that parents in the 2004 cohort reported a higher HRQoL on the Motor and Cognitive functioning scales. In the 2014 cohort, children reported less negative emotions than healthy children. No increase in emotional or behavioral problems were reported by children in both cohorts, whereas parents reported problems in social functioning and isolation related to a delay in emotional development. Children surviving brain tumor treatment have a reduced quality of life. The authors therefore recommend regular screening of HRQoL and emotional and behavioral problems and referral to specific aftercare.

The objective of this study is to determine the long-term outcome and consequences of Guillain-Barré syndrome (GBS) in children. This is an observational cross-sectional cohort study of children diagnosed with GBS (0-18 years old) at the Sophia Children's Hospital in Rotterdam from 1987 to 2009. All patients were invited for a structured interview, questionnaires, and full neurologic exam to record their current clinical condition focused on complaints and symptoms, neurological deficits, disabilities, behavior, and quality of life. Thirty-seven patients participated, 23 were now adults, with a median age of 20 years (range 4-39 years) and a median follow-up time of 11 years (range 1-22 years). Residual complaints were reported by 24 (65%) patients, including paresthesias (38%), unsteadiness of gait in the dark (37%), painful hands or feet (24%), and severe fatigue (22%). Four patients had severe neurological deficits, including facial diplegia and limb weakness. Two patients had had a recurrence of GBS. In 10 patients (26%), GBS had a negative impact on their school career. Questionnaires identified a wide range of behavioral problems. Quality of life was below normal on the subscale vitality, and above normal on the subscales social functioning and positive emotions in the adult group. Most children show good recovery of neurological deficits after GBS, but many have persisting long-term residual complaints and symptoms that may lead to psychosocial problems interfering with participation in daily life., (© 2014 Peripheral Nerve Society.)

Aim: To study functional outcome in children aged 1 month to 18 years after paediatric arterial ischaemic stroke (PAIS) and to identify risk factors influencing their quality of life., Method: In a consecutive series of 76 children (35 males 41 females, median age at diagnosis 2y 6mo, range 1mo-17y 2mo; median length of follow-up 2y 4mo, range [7mo-10y 6mo]) with PAIS diagnosed at the Erasmus Medical Centre Sophia Children's Hospital between 1997 and 2006, we collected clinical, biochemical, and radiological data prospectively. In 66 children surviving at least 1 year after PAIS, functional outcome could be evaluated with the World Health Organization's International Classification of Impairments, Disabilities and Handicaps., Results: Significant risk factors at presentation for a poor neurological outcome were young age, infarction in the right middle cerebral artery territory, and fever at presentation. Fifty-four % of children had severe neurological impairments at 12 months after PAIS, and at last follow-up more than half needed remedial teaching, special education, or institutionalization. Health-related quality of life (HRQOL) questionnaires showed a significantly lower HRQOL in all age groups. Children with a longer follow-up had a lower HRQOL in the cognitive functioning domain., Interpretation: Our study shows significant morbidity and mortality and a reduced HRQOL after PAIS depending on age, fever at presentation, and infarction in the right middle cerebral artery territory.

Background: Kinsbourne syndrome or opsoclonus myoclonus syndrome (OMS) is characterized by rapid, involuntary, irregular conjugate eye movements (opsoclonus), myoclonic jerking of the limbs and trunk, ataxia, and behavioral disturbances. In general, the outcome of neurologic and behavioral symptoms is poor. Studies on quality of life (Qol) and recovery after very long-term follow-up of children with OMS are lacking., Methods: We studied long-term cancer survival, neurologic recovery, and Qol of a consecutive series of eight patients with OMS that were treated in our center. Two cross-sectional follow-up evaluations were conducted at a 3-year interval (T1 and T2)., Results: In four out of eight children with OMS a neuroblastoma (NBL) was diagnosed and surgically removed completely. All children received immuno-modulatory therapy, consisting of prednisone (three children), prednisone and ACTH (four children), or prednisone, ACTH, and gammaglobulin (one child). At T1, median follow-up time was 9.5 years (range 3-14 years) after OMS onset and at T2, 11.6 years (range 6-17 years). Neurologic functioning improved in all children, reflected by a significant improvement of the mean Z-score in the motor domain of the Qol questionnaire at T2 as compared to T1. In contrast, seven children continued to have a severe developmental delay at T2. No significant difference in any of the variables was found between NBL survivors and OMS patients without NBL., Conclusion: Cognitive and behavioral impairments and not a motor impairment, such as ataxia, appear to be predominant at long-term in children with OMS.

Objective: To investigate health-related quality of life (HR-QOL) in children with neurofibromatosis type 1 (NF1) with parental reports and children's self-reports, and to investigate the potential contribution of demographic factors, disease-specific factors, and problems in school performance or behavior., Study Design: In a prospective observational study, parents of 58 children with NF1 (32 boys, 26 girls, age 12.2 +/- 2.5 years) visiting a university clinic, and their 43 children 10 years or older were assessed with the Child Health Questionnaire (CHQ). Potential determinants of domain scores were assessed in 3 explorative regression models., Results: Parents reported a significant impact of NF1 on 9/13 CHQ scales, with moderate effect sizes on 8 (general health perceptions, physical functioning, general behavior, mental health, self esteem, family activities, role functioning emotional/behavioral, and parent emotional impact). Children report an impact on bodily pain, and an above average general behavior. Multiple CHQ scales were sensitive to demographic factors and behavioral problems, and 1 to NF1 severity. NF1 visibility and school problems did not influence HR-QOL., Conclusions: Parents, but not the children with NF1, report a profound impact of NF1 on physical, social, behavioral, and emotional aspects of HR-QOL. Multiple HR-QOL domains were most sensitive to behavioral problems, which points to an exciting potential opportunity to improve HR-QOL in children with NF1 by addressing these behavioral problems.

Background: The relatively high survival rate of patients with low-grade astrocytoma necessitates increasing attention to physical and psychosocial outcomes. The objective of the current study was to investigate functional outcomes among children who were treated for low-grade or pilocytic astrocytoma in different areas of the brain., Methods: Functional outcomes were evaluated in the following domains: impairments, disabilities, handicaps, and quality of life (QOL). In a consecutive series, 38 children were included. Follow-up ranged from 3 years and 7 months to 11 years and 4 months after diagnosis., Results: Approximately 61% of children had impairments and 10% had a severe disability. Handicaps were found in the domains of relationships, school, and behavior. Children who were treated for supratentorial tumors required significantly more special education, and children who were treated for infratentorial tumors had significantly more behavioral and social problems. QOL was decreased significantly in all domains except emotions. Children who had a diagnosis in adolescence reported a lower QOL in social functioning compared with younger children. Data analysis revealed that some deficits suddenly became apparent years after diagnosis., Conclusions: At long-term follow-up, children who had low-grade or pilocytic astrocytomas were found to have poor functional outcomes, depending on tumor site, age, and recurrence. Children without deficits may develop severe cognitive, social, and behavioral deficits years after diagnosis, because of the phenomenon of "growing into deficit." Therefore, the authors suggest a long-term follow-up of children who are treated for low-grade or pilocytic astrocytomas at a young age to detect and subsequently offer support focused on the medical and cognitive impairments as well as on the behavioral and social consequences of their disease.

Aim: This study prospectively evaluated neuropsychological functioning in 8-year-old patients with anorectal malformation (ARM) and Hirschsprung's disease (HD).Methods: School functioning and behaviour were assessed in a standardised interview. Intelligence, attention, self-esteem and quality of life were evaluated with validated tests and questionnaires. The following predictors were assessed: socio-economic status, number of episodes of general anaesthesia, laxative treatment and premature birth. Severely intellectually disabled patients were excluded.Results: In total, twelve of the 23 (52%) patients with ARM and 11 (55%) of the 20 patients with HD received special education or remedial teaching. The intelligence quotient was normal: mean (standard deviation or SD) was 98 (17) and 96 (17), respectively. However, sustained attention was below the norm: mean (SD) Z-score was -1.90 (1.94) and -1.43 (1.98) for ARM and HD patients; both p < 0.01. Self-esteem was normal: mean (SD) Z-score was 0.10 (1.29) and -0.20 (1.11) for ARM and HD patients. Quality of life was normal in ARM patients and slightly impaired in HD patients. No predictors for neuropsychological outcome were identified.Conclusion: Despite normal intelligence, more than half of these patients received special education or remedial teaching. In addition, problems with sustained attention were found. These findings are important for long-term care. [ABSTRACT FROM AUTHOR]

Abstract: Background: Fatigue is an important symptom in adult multiple sclerosis (MS) and it is likely to occur in children with MS. It is currently unknown whether children who experienced a monophasic inflammatory demyelinating event of the central nervous system in the past also suffer from fatigue. Methods: We studied the presence and severity of fatigue in 32 children (18 boys, 14 girls) between 11–17 years old (mean: 14 years, 10 months) with a monophasic inflammatory demyelinating disease (n =22) or definite MS (n =10). This was measured with the Checklist Individual Strength. A score of ≥40 on the severity of fatigue subscale indicated the presence of severe fatigue. We also examined the relation between fatigue and depression (assessed by the Child Depression Inventory). Additionally we measured the health-related quality of life (HRQoL), using the TNO-AZL Child Quality of Life child form. We compared the scores of the MS and monophasic patients with the scores of healthy Dutch children. Results: The highest scores on the fatigue scales subjective fatigue and physical activity were found in the children with MS. Only 1 of the monophasic patients suffered from severe fatigue in contrast to 4 of the MS patients. In the MS group fatigue and depression were correlated. MS patients experienced a lower HRQoL on the scales locomotor functioning, cognitive functioning and interaction with peers. Conclusion: The occurrence of fatigue is very rare after a monophasic inflammatory demyelinating event in the past. As expected, fatigue occurs more frequent in paediatric MS patients. [Copyright &y& Elsevier]

Advances in antiretroviral treatment improved the life expectancy of perinatally HIV-infected children. However, growing up with HIV provides challenges in daily functioning. This cross-sectional cohort study investigated the neuropsychological and psychosocial functioning of a group of perinatally HIV-infected children in the Netherlands and compared their outcomes with Dutch normative data and outcomes of a control group of uninfected siblings. The children's functioning was assessed with internationally well-known and standardized questionnaires, using a multi-informant approach, including the perspectives of caregivers, teachers, and school-aged children. In addition, we explored the associations of socio-demographic and medical characteristics of the HIV-infected children with their neuropsychological and psychosocial functioning. Caregivers reported compromised functioning when compared to Dutch normative data for HIV-infected children in the areas of attention, sensory processing, social-emotional functioning, and health-related quality of life. Teachers reported in addition compromised executive functioning for HIV-infected children. A comparison with siblings revealed differences in executive functioning, problems with peers, and general health. The concurrent resemblance between HIV-infected children and siblings regarding problems in other domains implies that social and contextual factors may be of influence. A family-focused approach with special attention to the child's socio-environmental context and additional attention for siblings is recommended. [ABSTRACT FROM AUTHOR]