Impaired Neurocognitive Performance in Children after Liver Transplantation.

OBJECTIVES: To assess longitudinal neurocognitive development after liver transplantation and evaluate factors associated with neurocognitive performance. STUDY DESIGN: Data from neurocognitive testing of 65 children (aged <18 years) who underwent liver transplantation at Oslo University Hospital between 1995 and 2018 were collected from the testing program after transplantation. The parent-reported version of the Behavior Rating Inventory of Executive Function was used to assess executive function. RESULTS: A total of 104 neurocognitive tests were conducted on 65 patients. At the first test, conducted at a median of 4.1 years (IQR, 1.5-5.3 years) after transplantation and at a median age of 6.7 years (IQR, 5.4-10.5 years), the mean full-scale IQ (FSIQ) was 91.7 ± 14, and the mean verbal comprehension index was 92.0 ± 14.5. In the 30 patients tested more than once, there was no significant difference in FSIQ between the first test at a median age of 5.8 years (IQR, 5.2-8.5 years) and the last test at a median age of 10.8 years (IQR, 9.8-12.9 years) (87.4 ± 12.9 vs 88.5 ± 13.2; P = .58). Compared with the patients who underwent transplantation a age >1 year (n = 35), those who did so at age <1 year (n = 30) had a lower FSIQ (87.1 ± 12.6 vs 96.6 ± 13.8; P = .005) and lower verbal comprehension index (87.3 ± 13.8 vs 95.4 ± 13.0; P = .020). Age at transplantation (P = .005; adjusted for cholestasis: P = .038) and transfusion of >80 mL/kg (P = .004; adjusted for age at transplantation: P = .046) were associated with FSIQ. CONCLUSIONS: Young age at transplantation and large blood transfusions during transplantation are risk factors for poor neurocognitive performance later in life. Children who undergo transplantation before 1 year of age have significantly lower neurocognitive performance compared with those who do so later in childhood. Cognitive performance did not improve over time after transplantation.

Social attainment in physically well-functioning long-term survivors of pediatric brain tumour; the role of executive dysfunction, fatigue, and psychological and emotional symptoms.

The purpose of this cross-sectional study was to investigate long-term social attainment in physically well-functioning adult survivors of pediatric brain tumour (PBT) and identify demographic, medical, and psychological factors related to poor social outcomes, with a special focus on the significance of executive dysfunction. One hundred and fourteen PBT survivors and a healthy control group provided personal data on social outcomes, i.e., education, work, and government benefits, and completed questionnaires on executive function (EF), psychological and emotional difficulties, and fatigue. A significantly higher number of survivors compared to healthy controls reported having received educational adjustments and substantial government benefits, and significantly more survivors than controls were currently not engaged in regular employment/training. PBT survivors and healthy controls did not differ on educational level or living situation. The factors most strongly associated with poor social outcomes were self-reported executive dysfunction, difficulties with adaptive functioning, and fatigue. The findings show that physically well-functioning PBT survivors are at risk of poorer social outcomes and financial dependence in adulthood compared to their healthy peers, and underline the importance of investigating EF in short- and long-term follow-ups. Future rehabilitation efforts should focus more on compensatory strategies for executive dysfunction and improving EF skills.

Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus-long-term follow-up.

INTRODUCTION: Benign external hydrocephalus (BEH) is characterized by too rapidly increasing head circumference in infants, combined with typical neuroimaging findings. Psychomotor developmental delay is typically seen during the first few years of life; after that, the children's development assumedly normalizes. However, little is known about the long-term effects of BEH. METHODS: In this retrospective population-based study, children diagnosed with BEH during the years 1994-2003 in Southern Norway were asked to participate. Included patients (age 8-18 years old) and their parents answered the PedsQL questionnaire. The patient's family physicians contributed by giving information from medical records, with special emphasis on developmental, cognitive, and social function. RESULTS: One hundred seventy-six children were identified with BEH. One hundred three patients and 86 parents completed the PedsQL questionnaire. Supplemental medical information for 142 of the patients was received, mainly from their family physicians. Children and adolescents with BEH score themselves better than the normative mean on health-related quality of life, while the parents score their BEH children within the normative mean, except for the school functioning subgroup, where they score significantly lower. Various developmental, physical, and social problems are reported, like mental retardation, speech problems, epilepsy, motor impairment, psychiatric disorders, and cognitive difficulties. Among these patients, there is a discrepancy in some areas between the child-reported and parent-reported quality of life. CONCLUSIONS: Children and adolescents who were diagnosed with BEH during infancy generally do well. However, for some patients, there appear to be various developmental, social, and cognitive problems, and they seem to struggle more in school than their healthy peers.

Neurocognitive and psychosocial function in children with benign external hydrocephalus (BEH)-a long-term follow-up study.

INTRODUCTION: Long-term impact of benign external hydrocephalus (BEH) on cognition is largely unknown, and indication for neurosurgical CSF diversion procedure is debated. This study reports neuropsychological and psychosocial function in operated and non-operated BEH children. METHODS: Eighty-six children (76 males) between 8 and 18 years (mean 13.9) diagnosed with BEH before 12 months were included, of whom 30.2 % were operated. Participants completed neuropsychological tests and questionnaires covering quality of life (PedsQL) and executive function (BRIEF). RESULTS: Both operated and non-operated BEH children performed significantly below normative means on several neuropsychological tests. The children scored themselves higher than the norm average on PedsQL; however, the parents reported life quality comparable to other children. Operated children performed poorer compared with non-operated children on tests of psychomotor speed, attention span, executive function, motor speed and coordination, and on the BRIEF subscale Monitoring. Operated children, but not their parents, reported more problems on PedsQL subscale School than non-operated children. DISCUSSION: Children with BEH display long-term subtle neurocognitive difficulties. Non-operated children performed significantly better on some neuropsychological measures and reported less psychosocial problems. This difference may be caused by a selection bias: neurosurgical intervention was more likely in children with clinically more pronounced symptoms.

Cognitive function, behaviour and quality of life in children with myotonic dystrophy type 1 in South - Eastern Norway.

BACKGROUND: Cognitive and behavioural problems may be predominant in the clinical picture of myotonic dystrophy (DM1) in childhood. This can lead to a diagnostic delay and thus prevent optimal therapeutic measures. OBJECTIVE: To obtain an overview of children with DM1 in our health region and study their cognitive and behavioural function, quality of life and neurological status. METHODS: Patients diagnosed with DM1 were recruited to this cross-sectional study through local habilitation teams of our health region. Neuropsychological testing and physical examination were performed for the majority. For some patients information was retrieved from medical records and through telephone interviews. A questionnaire was administered regarding quality of life. RESULTS: 27 subjects <18 years diagnosed with DM1 were identified, giving a frequency of DM1 of 4.3/100 000 in this age group. Twenty consented to participate. Five had congenital DM1. Most of the participants had only mild neurological deficits. Two with congenital type had hydrocephalus requiring a shunt. Ten, whereof none with congenital DM1, had a cognitive function within normal range. Three were diagnosed with an autism spectrum disorder, and additional three were reported with autistic traits. Many parents reported social and school problems for their child. CONCLUSIONS: Intellectual disability and varying degrees of autistic behaviour were quite common. Motor deficits were most often mild. A strong focus regarding support at school and in social communication is needed for children growing up with DM1.

Metacognitive strategy training versus psychoeducation for improving fatigue in children and adolescents with acquired brain injuries: A randomized controlled trial.

OBJECTIVE: The present study aims to explore the relative effectiveness of two group-based cognitive rehabilitation programs for reducing fatigue in pediatric acquired brain injury (pABI). METHOD: This is an exploratory study of secondary endpoints in a blinded, parallel-randomized controlled trial with children and adolescents (ages 10-17 years) with pABI and reported executive dysfunction. It investigates the effectiveness of a metacognitive program (pediatric goal management training, n = 36) compared to a psychoeducational program (pediatric brain health workshop, n = 37) for reducing fatigue (Pediatric Quality of Life Inventory, Multidimensional Fatigue Scale), 8 weeks and 6 months postintervention. RESULTS: Seventy-three participants completed the allocated interventions, and 71 attended the 6-month follow-up. The results showed a significant decrease in parent-reported fatigue for both interventions from baseline to the 6-month follow-up. Forty percent of the total sample had a reliable change. There was no significant difference between the intervention groups, but a tendency in favor of the psychoeducational approach. Only subscales cognitive and sleep/rest fatigue showed significant reductions. In regression analyses, several factors predicted fatigue at 6 months follow-up, but only better global outcome and executive attention predicted a decrease in fatigue symptoms after 6 months. CONCLUSIONS: Group-based cognitive rehabilitation in the chronic phase of pABI, including education of parents and teachers, may be helpful in reducing fatigue. Global outcome and executive attention at baseline predicted fatigue improvement. Developmental factors are important to consider when tailoring pediatric interventions, as well as modifiable factors associated with fatigue. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Self-Reported Executive Dysfunction, Fatigue, and Psychological and Emotional Symptoms in Physically Well-Functioning Long-Term Survivors of Pediatric Brain Tumor.

Long-term executive function (EF), psychological and emotional difficulties, and fatigue among adult survivors of pediatric brain tumors (PBTs) are sparsely studied. One hundred and fourteen adult PBT survivors, 89 PBT survivor informants, and a healthy control group completed questionnaires assessing these domains. Significant differences between PBT survivors and controls were found on all self-reported measures, most strongly on measures reflecting EF. Patients reported significantly more difficulties with metacognitive aspects of EF compared to behavioral aspects. PBT survivors reported more psychological symptoms and executive dysfunction than their informants. These findings should be taken into consideration in long-term neuropsychological and psychosocial follow-ups.

An extensive long term follow-up of a cohort of patients with hypoplasia of the left heart.

We report the results of follow-up of the complete cohort of Norwegian children born in the period from 1987 through 1998 in whom there was the intention to treat surgically hypoplasia of the left heart using the Norwood sequence of operations. Of the 54 children, 21 are alive. Of these, 15 have been extensively studied, while the medical state of all the remaining survivors is known from reports from other hospitals. Of the survivors, the majority have reasonably acceptable cardiac and haemodynamic function, but significant neurological and neuropsychological morbidity is identified within the group as a whole, which requires special attention from qualified personnel of various kinds.

Clinical and psychosocial follow-up study of children treated with extracorporeal membrane oxygenation.

BACKGROUND: Serious heart and pulmonary failure may be treated with extracorporeal membrane oxygenation (ECMO). The aim of this follow-up study was a multimodal assessment of outcome in children surviving ECMO at our hospital from 1991 to 2004. METHODS: Twenty-two children were, on average, 1.18 years old (SD = 1.92; range, 0.1 to 7.8) when ECMO was initiated. Average age at follow-up was 7.2 years (range, 1.8 to 13.9). Thirteen children were treated for cardiac and nine for pulmonary failure. Venoarterial ECMO was performed in 21 patients and venovenous ECMO in one patient. The protocol included a review of patients records, clinical, neurologic and neuropsychologic, and radiological examinations, electroencephalogram, and interviews with the parents. RESULTS: Moderate or severe impairment in at least two clinical assessments were found in 16 (72.7%) children. Five (22.7%) children had cerebral palsy and 15 (68.2%) had moderate or severe cognitive impairment. Eight (36%) children had pathological radiologic findings. Pathological electroencephalograms were found in 11 (50%) patients including four (18.2%) with epileptic activity. Children with radiologic findings had a slightly worse cognitive outcome. There was no association between the neurophysiologic findings and the neuropsychologic performance or the radiologic findings. Based on parental assessment, only four children had pathological scores. CONCLUSIONS: All children, except for two, came walking to the follow-up visit. All parents were positive to the ECMO treatment, and reported a good or slightly reduced quality of life for their child. Cerebral sequelae were frequently found in children treated with ECMO. The need for treatment and support should be assessed in order to optimize their physical and psychosocial functioning.