Neuroblastoma diagnosed antenatally: a treatment dilemma with implications for screening.
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Biomedical subjects
Publications and source records attributed to M C Stevens.
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The purpose of this study is to describe the incidence and survival of childhood cancer in the West Midlands for the period 1980-1984. Proportional breakdown by Asian subgroup is also considered. A total of 587 patients were registered, 49 of them of Asian origin. Breakdown to Asian versus non-Asian subgroups by diagnosis revealed comparatively high rates for Hodgkin's disease, retinoblastoma and neuroblastoma in the Asian patients. However, a deficit of cases was seen for CNS tumours. Comparison of overall age-standardized rates (ASR) for all cancers revealed a substantially lower value compared to that reported for the USA white population but a similar value to the USA black and UK white populations. Diagnostic breakdown revealed that the major difference between the West Midlands Regional Children's Tumour Research Group (WMRCTRG) and the USA white ASR was in the leukaemia and lymphoma group. Overall survival for the series was 56% at 5 years. The poorest prognosis was found in acute myeloid leukaemia, with only 23% of patients surviving at 5 years, against 62% in acute lymphoblastic leukaemia. CNS tumours also had a poor outcome, with an overall survival rate of 47%, although certain individual diagnoses were more favourable. We observed a 100% survival rate in Hodgkin's disease up to 5 years from diagnosis, and both Wilms' tumour and retinoblastoma had 90% survival rates.
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The incidences of human disease vary from place to place, and some show distinct patterns. Patterns in rare diseases, such as childhood cancer, are not readily discernible, however, and this makes it difficult to relate their distribution to factors in the environment in seeking possible causes of the disease. Provided cases are geographically indexed, data on a disease can be analysed geostatistically. Variograms can be computed to determine the strength and spatial scale of any pattern, and to summarize the variation, and then the risk of developing the disease can be estimated by kriging. This has been done for the incidence rates of childhood cancer from 1980 to 1984 in the West Midlands Health Authority Region of England. A novel modification was made to compute the variogram of the risk from that of the frequencies, taking into account the binomial nature of the data. The incidence of the disease appears patchy. The results show that this patchiness is spatially correlated and not purely random. The risk appears greatest in the rural south west of the Region and in some of the suburbs around the conurbation. This approach using geostatistics seems very promising and will be developed further as more data for this and other diseases become available.
A prospective, controlled study of nutritional status in 100 newly diagnosed paediatric oncology patients is reported. Results showed that although patients' height-for-age and weight-for-height were normal, mid-upper arm circumference (MUAC) and triceps skinfold thickness (TSFT) were significantly less than reference and control values. Moreover, 20% of patients had MUAC below the 5th percentile (P less than 0.001) and 23% had TSFT more than 2 SD below the mean (P less than 0.001). The discrepancy between these assessments is attributed to the effect of large tumour masses masking diminished true body weight. Children with intra-abdominal solid tumours had significantly lower MUAC and TSFT than those with either extra-abdominal solid tumours or leukaemia, and were more likely to be malnourished based on arm anthropometric criteria. Of all subjects, 44% were consuming less than 80% of their recommended daily allowance of energy at diagnosis, compared with 0% of controls (P = 0.001).
This study was undertaken to investigate the incidence, outcome and referral patterns of central nervous system tumours in a defined childhood population over a recent 5-year period. The study incorporated pathological review of all available diagnostic material and follow-up assessed survival at a minimum of 5 years from diagnosis. One hundred and forty-seven cases were reviewed, representing an annual incidence of 26.5 per million children aged less than 15 years. The distribution of individual diagnoses by age, sex and anatomical site was similar to comparable data collected previously in the United Kingdom and North America, but the inclusion of 28 cases (19%) without biopsy suggested that the wider use of computed tomography might account for a small increase in incidence over previous estimates. Analysis of referral to the Regional Paediatric Oncology Unit showed that the patients referred were younger than those not referred and were over-represented amongst the diagnoses of medulloblastoma, ependymoma and brain stem glioma, which carry the worst prognosis. Survival for all diagnoses together was 51% at 5 years, ranging from 13% for unbiopsied brain stem gliomas to 100% for juvenile astrocytomas. Referral to the Regional Unit appeared to have some survival advantage for children with medulloblastoma, although this was not statistically significant. Accurately reviewed data such as these are essential in order to assess current workload and treatment success, in addition to enabling investigation of future diagnostic and treatment strategies.
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The West Midlands Regional Children's Tumour Registry collects detailed information on all cases of childhood cancer in the West Midlands Health Authority Region (WMHAR). The distribution by electoral ward of all cases diagnosed in the WMHAR between 1980 and 1984 has been determined. Analysis has also been performed for leukaemias/non-Hodgkin's lymphomas alone. We suggest that this latter grouping should be universally employed, owing to the difficulty of accurately separating out cases of leukaemia. Both spatial analyses showed several wards with significantly excessive rates on the basis of their cumulative Poison probability. Observed/expected ratios of 3-35 were seen for cases in significant wards, which are similar to the ratios seen in analysis of incidence around nuclear installations. However, further detailed consideration of these individual significance levels in the light of the number of statistically significant wards which would occur by chance alone, due to the multiple use of the test, accounted completely for the number of wards obtained in each of the groups considered. Thus, apparent 'clustering' of cases could be mere statistical artefact. In the WMHAR, therefore, using the technique of probability mapping, no true spatial pattern of incidence was found, other than that which would occur by chance alone. This, in a large area without nuclear installations and an even mix of rural and industrialised regions, could be seen as control data for those studies which have considered cases of childhood leukaemia around nuclear facilities, where the observation of single point clusters associated with suspected sites restricts assessments of spatial pattern in the rest of the area.(ABSTRACT TRUNCATED AT 250 WORDS)
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Male patients with SS disease had significantly lower T3 and higher TSH levels than a comparison group. Stimulation with TRH in 10 male sibling pairs showed highly significant increases in T3 and TSH in both patients and sibling controls although the increase in TSH was significantly greater in SS disease. The interpretation of these findings is unclear although the thyroid indices indicate an abnormal pituitary-thyroid axis most consistent with a modest primary thyroid failure.
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Platelets from patients with Bernard-Soulier syndrome (BSS) are larger than normal and demonstrate characteristic aggregation abnormalities. A molecular defect involving platelet membrane glycoproteins has been established as the primary abnormality, and the major defect is described as an absence of GPIb, the receptor for von Willebrand factor, which prevents the adhesion of BSS platelets to damaged endothelium. Associated deficiencies of GPV and GPIX are also recognized. The overall clinical effect is a haemorrhagic tendency which, although variable, is severe at times in all patients. We report a child who presented with the typical morphological and aggregation abnormalities associated with BSS, but who had negligible bleeding problems. Further investigation revealed a significant but incomplete deficiency of GPIb and of GPV and GPIX. This case suggests that BSS may be a heterogeneous disorder with clinical consequences dependent on the extent and complexity of the platelet membrane glycoprotein deficiency.
As the prospects of cure for children with cancer increase, the long term consequences of treatment and the quality of life experienced by survivors assume greater importance. Strategies to encourage early return to school after diagnosis are of considerable importance both educationally and socially, but teachers require information to help them facilitate the child's return. The extent to which professional contact is fostered between children's cancer centres and the education service is the subject of this short survey.