Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series.

MSUD is a complex metabolic disorder that has been associated with central nervous system damage, developmental delays, and neurocognitive deficits. Although liver transplantation provides a metabolic cure for MSUD, changes in cognitive and adaptive functioning following transplantation have not been investigated. In this report, we present data from 14 patients who completed cognitive and adaptive functioning testing pre- and one yr and/or three yr post-liver transplantation. Findings show either no significant change (n=8) or improvement (n=5) in IQ scores pre- to post-liver transplantation. Greater variability was observed in adaptive functioning scores, but the majority of patients evidenced no significant change (n=8) in adaptive scores. In general, findings indicate that liver transplantation minimizes the likelihood of additional central nervous system damage, providing an opportunity for possible stabilization or improvement in neurocognitive functioning.

Friendships and social interactions of school-aged children with migraine.

We set out to evaluate the friendships and social behaviour of school-aged children with migraine. Concern exists regarding the impact of paediatric migraine on daily activities and quality of life. We hypothesized that children with migraine would have fewer friends and be identified as more socially sensitive and isolated than comparison peers. Sixty-nine children with migraine participated in a school-based study of social functioning. A comparison sample without migraine included classmates matched for gender, race and age. Children with migraine had fewer friends at school; however, this effect was limited to those in elementary school. Behavioural difficulties were not found. Middle-school students with migraine were identified by peers as displaying higher levels of leadership and popularity than comparison peers. Concern may be warranted about the social functioning of pre-adolescent children with migraine; however, older children with migraine may function as well as or better than their peers.

Complex visual hallucinations and cyclosporine.

Insidious loss of visual acuity and simultaneous onset of complex visual hallucinations were observed in a patient with acute lymphoblastic leukemia who had recently received bone marrow transplantation and was being treated with prednisone and cyclosporine to suppress graft-v-host disease. Problems with visual acuity and visual hallucinations spontaneously ameliorated with reduction and termination of cyclosporine treatment. The complex visual hallucinations caused considerable psychological distress for the patient. These hallucinatory phenomena were placed within the framework of loss of control and decreased competence, often experienced by children with chronic illness. Recommendations for prompt therapeutic intervention were made.

Follow-up of language and cognitive development in patients with mannosidosis.

Three brothers with mannosidosis were assessed both biochemically by levels of enzyme activities and developmentally by serial testing of language and cognitive development. The findings indicated that while the leukocyte enzyme activity of alpha-mannosidase was exceptionally low, only mild intellectual deficits were present that did not progress during a two-year follow-up. These results do not substantiate the expected relationship between the severities of enzyme deficiency and developmental delays. Language and cognitive deficits appeared uniform with no areas of strengths or weaknesses. Deficits in development did not progress during a two-year follow-up.

Long-term follow-up of biochemical and cognitive functioning in patients with mannosidosis.

Longitudinal assessments of three brothers with alpha-mannosidosis were performed biochemically by determining levels of leukocyte enzyme activity, and neurodevelopmentally by testing of general intelligence, language, visual spatial skills, and overall adaptive abilities. During the follow-up examination, enzyme activity was assessed in fibroblasts to evaluate the uniformity of biochemical deficits. The biochemical findings demonstrated profound deficits of leukocyte alpha-mannosidase that remained remarkably stable over time and were very similar to levels of the same enzyme activity in fibroblasts. The cognitive findings showed that the patients manifested mild cognitive deficits. Cognitive deficits were generally uniform with no signs of progressive deterioration, except receptive language abilities. Suggestions are made for careful follow-up of auditory abilities in patients with mannosidosis.

Neuropsychological effects of cranial irradiation in young children with acute lymphoblastic leukemia 9 months after diagnosis. The Children's Cancer Group.

STUDY OBJECTIVE: To determine the effects of cranial irradiation on neuropsychological test performance evident 9 months after diagnosis. DESIGN: A companion study to a randomized clinical trial (CCG-105). SETTING: Institutions participating in Childrens Cancer Group cooperative treatment trials. PATIENTS: Seventy-four children aged 3.0 to 6.5 years with average-risk acute lymphoblastic leukemia. Children with central nervous system leukemia at the time of diagnosis, preexisting mental retardation, or Down's syndrome or for whom English was not the primary language were not eligible for study. INTERVENTIONS: Children were randomized to receive treatment with one of four systemic chemotherapy regimens and either intrathecal methotrexate sodium during induction and consolidation plus 18 Gy of cranial irradiation or intrathecal methotrexate during induction, consolidation, and maintenance as central nervous system prophylaxis. MEASUREMENT AND RESULTS: The groups were comparable with regard to chronologic age, sex, and family socioeconomic status. Children who received cranial irradiation plus intrathecal methotrexate scored significantly lower on the McCarthy Motor Scale (P < .05) and the Token Test (P < .05) than children who received intrathecal methotrexate alone. The groups did not differ significantly on the McCarthy General Cognitive Index, Developmental Test of Visual Motor Integration, or Peabody Picture Vocabulary Test-Revised. CONCLUSIONS: Findings suggest that the combined effects of cranial irradiation and intrathecal methotrexate therapy on neuropsychological performance may be evident in young children as early as 9 months after diagnosis. Follow-up assessment of these children will reveal whether these effects remain constant, intensify, or resolve.

Mealtime interactions and family relationships of families with children who have cancer in long-term remission and controls.

This study investigated reports of parents (mothers and fathers) of 25 children with cancer in long-term remission and matched neighborhood control families on issues related to the quality of mealtime interactions, and the relationship between parental reports of overall family functioning and the quality of mealtime interactions. Results showed positive correspondence for both groups between fathers' and mothers' reports of favorable family environments (greater cohesion and expressiveness, less conflict) and positive mealtime interactions. No significant differences were found between parents of children with cancer and control parents with regard to mealtime conflicts, current concerns about their child's eating, or satisfaction with the child's food intake. The data demonstrate a moderate relationship between overall family functioning and the quality of mealtime interactions, but suggest a minimal relationship between children with cancer in long-term remission who are at low nutritional risk and the quality of mealtime interactions.

Pre- and post-stroke MRI and neuropsychological studies in sickle cell disease: a case study.

The case of a patient with sickle cell disease is presented in which neuropsychological and magnetic resonance imaging studies were completed prior to and after a right hemispheric stroke. The contribution of a new MR perfusion technique in understanding the neurological complications in this patient is discussed. This case illustrates the complex pathophysiology of neuropsychological deficits in SCD and underscores the need to develop models that better reflect this complexity.

Hypnotherapy of a child with warts.

Hypnosis was used to treat a 7-year-old female with 82 common warts. The lesions had been present for 12-18 months and were refractory to routine dermatologic treatment. Hypnotic suggestions were given for the facial warts to disappear before warts from the rest of the body. After 2 weeks, eight of 16 facial warts were gone, with no other changes. After three additional biweekly sessions, all 82 warts were gone. To the author's knowledge, this is the first reported case of systematic wart removal in children. Discussion focuses on the relationship between psychological mechanisms and the immune system.

Psychosocial adjustment of children with chronic illness: an evaluation of three models.

This study was designed to assess social, emotional, and behavioral functioning of children with chronic illness and to evaluate three models addressing the impact of chronic illness on psychosocial functioning: discrete disease, noncategorical, and mixed. Families of children with cancer, sickle cell disease, hemophilia, and juvenile rheumatoid arthritis participated, along with families of classroom comparison peers without a chronic illness who had the closest date of birth and were of the same race and gender (COMPs). Mothers, fathers, and children provided information regarding current functioning of the child with chronic illness or the COMP child. Child Behavior Checklist and Children's Depression Inventory scores were examined. Results provided support for the noncategorical model. Thus, the mixed model evaluated in this study requires modifications before its effectiveness as a classification system can be demonstrated.

Social interactions between children with cancer or sickle cell disease and their peers: teacher ratings.

We evaluated the social reputation of children with cancer (nonprimary brain tumors) (n = 26) children with a primary malignancy involving the central nervous system (n = 15), and children with sickle cell disease (SCD) (n = 33) using a standardized measure of social reputation, the Revised Class Play (RCP). Each child's classroom teacher completed the RCP, an instrument designed to assess sociability-leadership, aggressive-disruptive, and sensitive-isolated interpersonal qualities. Matched-pairs analyses comparing the child with chronic illness to one peer in each class who was the same gender/race and the closest date of birth showed children with cancer were nominated more often for sociability-leadership roles and less often for aggressive-disruptive roles. Brain tumor survivors were nominated more often for sensitive-isolated roles. Children with SCD were not significantly different from peers. Findings suggest that the stressful life events associated with cancer or SCD did not have a significant negative impact on social adjustment for school-aged children with these diseases. The data demonstrate the critical need for appropriate controls when evaluating psychosocial morbidity and chronic illness. Better understanding of the processes of resilience for these children who are routinely exposed to heightened stress provides an opportunity to improve the competence of other high-risk youth.

Long-term neurobehavioral outcome in pediatric brain-tumor patients: review and methodological critique.

This paper provides an overview of pediatric brain tumors and reviews the literature on long-term neurobehavioral outcomes for these children. Progress in this area has been slow due to numerous methodological and practical complications. While some general conclusions can be drawn, the development of comprehensive models awaits more research into the multiplicity of biological and nonbiological events, and their interactions, potentially affecting outcome. Increased theoretical and methodological sophistication will be needed to address this complex classification of diseases. Barriers to research in this area are enumerated as are suggestions for future efforts.

Social functioning of children surviving bone marrow transplantation.

OBJECTIVE: To evaluate the behavioral reputation and peer acceptance of pediatric bone marrow transplant (BMT) survivors. METHODS: Forty-eight BMT survivors (8-16 years of age) were compared to 48 nonchronically ill, same-classroom, same-gender comparison peers (COMP). Peer, teacher, and self-report data were collected. RESULTS: Relative to COMP, BMT survivors had fewer friends and were described by peers, but not teacher or self-report, as more socially isolated. In addition, peers described BMT survivors as being less physically attractive and athletically skilled. Further analyses suggested that these nonsocial attributes (physical appearance and athletic ability) and treatment variables (whether cranial irradiation was received) mediated the social difficulties of BMT survivors. CONCLUSIONS: These data are suggestive of an unremitting pattern of difficulties with peers that has the potential to disrupt normal social and emotional development. Differences between peer, teacher and self-reports highlight the need for multiple informants in future work.

Identification of alcohol by smell among preschoolers: evidence for early socialization about drugs occurring in the home.

This study tested preschoolers' ability to provide accurate verbal associations to alcoholic beverage odors and whether this ability was related to parental drinking patterns and motivations. Older preschoolers performed better than younger preschoolers; photographic cues improved performance; children who correctly identified a substance by smell had socially appropriate knowledge of the culturally appropriate users of the substance; children reported liking substances that are used mainly by children and adults, and generally reported disliking substances whose use is legally limited to adults only; children were better at identifying substances they commonly use, but success at recognition of alcoholic beverages was related to heavier parental drinking and use of alcohol for escape reasons. Findings have implications for theories of socialization to drug use and for models of prevention.

Potential bias in classroom research: comparison of children with permission and those who do not receive permission to participate.

Compared both the social reputation and preference/acceptance of children who received (n = 4,073) and did not receive parental consent (n = 469) to participate in classroom-based sociometric research. Peers and teachers described nonparticipants as less sociable. Peers described nonparticipants as being lower on social acceptance, more aggressive, and less academically competent. No significant differences were obtained for sensitive/isolated characteristics, appearance, or athleticism. There were no significant interactions between consent status and sex or age. Associations between social reputation and social preference scores were similar for participants and nonparticipants. The findings suggest that children who do not return consent forms are systematically different from classmates who participate, although these differences were modest. Further work is needed to determine why children do not participate.

Social interactions between children with cancer and their peers: teacher ratings.

Compared children (ages 8-18) with cancer (n = 24) and matched classroom control children (n = 24) using a modified version of the Revised Class Play (RCP). A wide variety of malignancies were represented, except brain tumors. Childrens' classroom teachers completed the RCP, an instrument modified to obtain teachers' impressions of three fundamental dimensions of interpersonal style: sociability-leadership, aggressive-disruptive, sensitive-isolated. Relative to the matched controls, children with cancer were perceived by teachers as (a) less sociable and prone towards leadership and (b) more socially isolated and withdrawn. These findings suggest a need for long-term psychosocial interventions oriented towards peer relationships of children with cancer.

Peer relationships and adjustment in children with cancer.

Evaluated the psychosocial adaptations of children with cancer (n = 24, ages 8-18) as compared with same classroom controls on indices of (a) peer- and self-perceptions of sociability, aggression, and social isolation; (b) overall popularity; (c) mutual friendships; (d) feelings of loneliness; and (e) self-concept in multiple domains. Although the peer report data showed that children with cancer had a social reputation as significantly more socially isolated, no significant differences were found for their popularity, number of mutual friends, loneliness, or self-worth. Findings suggest that children with cancer have a reputation as more socially isolated, but differences were not found on measures of acceptance by peers, self-concept, or loneliness.

Differences in the child-rearing practices of parents of children with cancer and controls: the perspectives of parents and professionals.

Obtained self-reports of parenting practices from fathers and mothers of 24 children with cancer, and 24 controls using the Child-Rearing Practices Report (CRPR). Cancer patients were primarily in long-term remission and represented a typical pattern of childhood malignancies. Same age/sex controls were recruited from the classrooms of the children with cancer. In addition, CRPR ratings were obtained from experts in pediatric oncology based upon their prediction of how a parent of a child with cancer would respond. The experts predicted differences in the areas of overinvolvement, discipline, worry about the child, nutritional concerns, and use of supernatural explanations. Results from parents showed surprising similarity between the parents of children with cancer and control parents, and disagreement with the experts. Discussion focuses on explanations for this apparent contradiction.

Social, emotional, and behavioral functioning of children with juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the hypothesis that children with juvenile rheumatoid arthritis (JRA) would have more social and emotional problems than case-control classmates. METHODS: Using a case-control design, children with JRA (n = 74), ages 8-14, were compared with case-control classmates (n = 74). Peer relationships, emotional well-being, and behavior, based on peer-, teacher-, parent-, and self-report scores on common measures, were compared using analysis of variance. RESULTS: Relative to case-control classmates, children with JRA were similar on all measures of social functioning and behavior. Mothers reported more internalizing symptoms in the child with JRA, but child self reports and father reports showed no differences. Scores on all standardized measures were in the normal range for both the JRA and the case-control groups. CONCLUSION: Children with JRA were remarkably similar to case-control children on measures of social functioning, emotional well-being, and behavior. These findings are not supportive of disability/stress models of chronic illness in childhood and suggest considerable psychological hardiness among children with JRA.

Sibling adjustment to pediatric traumatic brain injury: a case-controlled pilot study.

OBJECTIVE: To assess depressive symptoms, self-concept, and behavior in non-affected siblings of children with severe pediatric traumatic brain injury (TBI). DESIGN: Cross-sectional study with case controls. SETTING: Children's hospital tertiary care center. PARTICIPANTS: Twelve siblings of children consecutively admitted to an inpatient rehabilitation unit after a severe TBI. Case controls were randomly selected from the sibling's classmates. MAIN OUTCOME MEASURES: The Child Behavior Checklist (CBCL), the Teacher's Report Form of the CBCL (TRF-CBCL), the Self-Perception Profile for Children and the Children's Depression Inventory (CDI). RESULTS: No statistical differences were found in depressive symptoms, self-concept, or behavior between the siblings and their classmates 3 to 18 months after injury. Poorer functional outcomes in the child with a TBI were found to correlate significantly with lower self-concept and more symptoms of depression in the siblings. CONCLUSIONS: Further research is needed to evaluate the potential impact on sibling adjustment after pediatric TBI.