Association of 1800 cGy cranial irradiation with intellectual function in children with acute lymphoblastic leukaemia

Cranial radiation therapy in childhood acute lymphoblastic leukaemia has been associated with adverse neuropsychological effects, such as low intelligence. However, records show that these associations usually occur when the dose of radiation used is 2400 cGy. We investigated whether a lower dose of 1800 cGy had the same adverse effects on long-term survivors and whether high doses of methotrexate but no radiation therapy would have a more beneficial effect. We evaluated 203 children for six years in a multi-centre European study. The patients were divided into two groups: 129 children treated with 1800 cGy of cranial radiation therapy and 74 children who received high-dose methotrexate but no radiation therapy. We used full scale intelligence quotient, verbal, and performance IQ tests to assess the patient's intelligence. We found a significant decline in full scale intelligence quotient in the irradiated group that increased with the length of time from diagnosis. Younger age at diagnosis was associated with lower full scale intelligence quotient in the radiated group. Our results indicate that a radiation dose of 1800 cGy can have negative effects on neurocognitive function and we continue to question the benefit of low-dose cranial radiation therapy

Experimental Chemotherapy in Children with Cancer—A Parent's View

Parental attitude and the parents' perceptions of a child's responsibilities were measured by mailing a questionnaire to 156 parents of cured children of the Pediatric Oncology Center, University of Groningen. Simplistically, the questionnaire concerned parental judgment on (1) the role of the child in decisions about experimental therapy, (2) what information should be related to the child and by whom, (3) parental attitudes toward experimental therapy, and (4) the parents' opinions about ethical aspects of proposing experimental therapy. A high response rate (87.8%) mas achieved. A majority of the respondents would allow a child take responsibility in deciding about experimental therapy. In their opinion the median age (16 years) at which a child should be allowed to give consent was higher than the median age (12 years) at which a child should merely be involved in the decision. Parents were more likely to overrule the child's decision if the child decided against experimental therapy than if the child chose the therapy (p < 0.001). Parents found it more difficult to talk about death than about experimental therapy, and parents would be more willing to involve the physician in discussing experimental therapy than in discussing imminent death with a child (p < 0.001). Many respondents (68%) felt that the child should be given both altruistic reasons and reasons of self-interest for participating in experimental therapy. About half of all of the respondents believed that a pediatric oncologist should always advise experimental therapy for the benefit of similarly afflicted patients

Brain function in classic galactosemia, a galactosemia network (GalNet) members review.

Classic galactosemia (CG, OMIM #230400, ORPHA: 79,239) is a hereditary disorder of galactose metabolism that, despite treatment with galactose restriction, affects brain function in 85% of the patients. Problems with cognitive function, neuropsychological/social emotional difficulties, neurological symptoms, and abnormalities in neuroimaging and electrophysiological assessments are frequently reported in this group of patients, with an enormous individual variability. In this review, we describe the role of impaired galactose metabolism on brain dysfunction based on state of the art knowledge. Several proposed disease mechanisms are discussed, as well as the time of damage and potential treatment options. Furthermore, we combine data from longitudinal, cross-sectional and retrospective studies with the observations of specialist teams treating this disease to depict the brain disease course over time. Based on current data and insights, the majority of patients do not exhibit cognitive decline. A subset of patients, often with early onset cerebral and cerebellar volume loss, can nevertheless experience neurological worsening. While a large number of patients with CG suffer from anxiety and depression, the increased complaints about memory loss, anxiety and depression at an older age are likely multifactorial in origin