Quality of survival assessment in European childhood brain tumour trials, for children below the age of 5 years.

The highest incidence rate of childhood brain tumours is in children below the age of five years, who are particularly vulnerable to the effects of treatments. The assessment of quality of survival (QoS) in multiple domains is essential to compare the outcomes for different tumour types and treatment regimens. The aim of this position statement is to present the domains of health and functioning to be assessed in children from birth to five years, to advance the collection of a common QoS data set in European brain tumour trials. The QoS group of the European Society of Paediatric Oncology (SIOP-E) Brain Tumour group conducted consensus discussions over a period of six years to establish domains of QoS that should be prioritised in clinical trials involving children under 5 years. The domains of health and functioning that were agreed to affect QoS included: medical outcomes (e.g. vision, hearing, mobility, endocrine), emotion, behaviour, adaptive behaviour, and cognitive functioning. As for children aged five years and older, a 'core plus' approach is suggested in which core assessments are recommended for all clinical trials. The core component for children from birth to three years includes indirect assessment which, in this age-group, requires proxy assessment by a parent, of cognitive, emotional and behaviour variables and both direct and indirect endocrine measures. For children from four years of age direct cognitive assessment is also recommended as 'core'. The 'plus' components enable the addition of assessments which can be selected by individual countries and/or by, age-, treatment-, tumour type- and tumour location-specific trials.

Differences in attentional functioning between preterm and full-term children underline the importance of new neuropsychological detection techniques.

AIM: The aim of this study was to investigate specific attentional components in preterm born children who had not yet started school. METHODS: Between January and December 2011, we assessed 52 preterm and 52 full-term children aged between five years five months and six years two months, of comparable age and gender, at the Medical University of Vienna. Different attentional components were evaluated through selected subtests of the Test of Attentional Performance and the German version of the Wechsler Intelligence Scale for Children. Each child's behaviour was also evaluated using parental ratings and descriptive item-based evaluation during neuropsychological assessment. RESULTS: Children born preterm showed poor attentional performance in sustained attention, focused attention and distractibility, as well as reductions in processing speed in divided attention and flexibility tasks. Children born preterm also showed decreased volitional attention compared with automatic attention. No problems were detected in alertness or inhibition. In addition, a higher rate of aborted tests, decreased motivation and poorer parental ratings were detected among the preterm population compared with full-term born children. CONCLUSION: Our results highlighted differences in attentional functioning between preterm and full-term children, indicating the importance of new neuropsychological techniques for the detection of specific attentional disorders.

Sarcoidosis and lymphoma: a comparative study.

AIM: To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis. DESIGN: Multicentre retrospective cohort study. METHODS: Retrospective chart review. RESULTS: Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24-68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin's lymphoma and 18 patients with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma (DLBCL) (n = 11), follicular lymphoma (n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 2), anaplastic large cell lymphoma ALK + (n = 1), angioimmunoblastic T-cell lymphoma (n = 1) and T-cell prolymphocytic leukemia (n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups (P = 0.685). CONCLUSIONS: We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.

Bioactivity of cedarwood oil and cedrol against arthropod pests.

Heartwood samples from Juniperus virginiana L. were extracted with liquid carbon dioxide, and the bioactivity of carbon dioxide-derived cedarwood oil (CWO) toward several species of ants and cedrol toward ticks was determined. Repellency was tested for ants, and toxicity was tested for ticks. Ants in an outdoor bioassay were significantly repelled by the presence of CWO on a pole leading to a sugar-water solution. Similarly, CWO was a significant repellent barrier to red imported fire ants and prevented them from finding a typical food source. Black-legged tick nymphs exhibited dosage-dependent mortality when exposed to cedrol and at the highest dosage (i.e., 6.3 mg/ml) tested, the cedrol killed 100% of the ticks. These repellency and toxicity results together demonstrate a clear potential for the use of CWO as a pest control agent.

Synthesis and stereochemical elucidation of a 14-membered ring phosphonate.

[structure: see text]. We report the synthesis and stereochemical elucidation of a 14-membered ring phosphonate. The key step in the synthesis of the macrolide phosphonate was the cyclization of the acyclic precursor using the Mitsunobu reaction, a mild reaction for the preparation of mixed phosphonates.

Quantitative neuropathological changes in presymptomatic Huntington's disease.

Morphometric studies of the tail of the caudate nucleus, the site where the pathology is first seen, were performed on 16 brain specimens collected from individuals at risk for inheriting Huntington's disease (HD). Medical records and information obtained from immediate family members indicated that all had died without symptoms of HD. Six individuals had 37 or more CAG repeats and were designated HD gene carriers, whereas 10 were determined to be non-carriers. Cell counts of the tail of the caudate nucleus revealed an increased density of oligodendrocytes among the presymptomatic HD gene carriers (mean cells/field: carriers = 40.0, noncarrier = 21.3; age, sex, repeated measure adjusted F[126] = 11.7, p = 0.0008). No statistically significant differences were found between HD carriers and noncarriers in the density of neurons (carriers = 16.9, noncarriers = 15.5), astrocytes (carriers = 27.8, noncarriers = 21.3) or microglial cells (carriers = 7.9, noncarriers = 5.6). Ubiquitin immunostaining performed in 3 gene carriers revealed intranuclear inclusions in all 3 cases, including 1, with 37 repeats, who died 3 decades before the expected age for onset of the clinical syndrome. Normal densities of other cell types and careful macroscopic examination suggest that the increase in oligodendroglial density is not a consequence of atrophy and may instead reflect a developmental effect of the HD gene.