26 research outputs found

    Blueberry muffin baby: the pathogenesis of cutaneous extramedullary hematopoiesis

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    Two neonates exhibited the clinical picture of the "blueberry muffin baby" at delivery. The integument manifested petechiae and purpuric magenta-colored macules, papules, and plaques,as well as, blueberry-colored ecchymoses. These findings led to the diagnosis of a connatal cytomegalovirus infection and fetal erythroblastosis, respectively. The hemorrhagic-purpuric looking skin lesions reflected extramedullary hematopoiesis with ultrastructural study disclosing evidence of both erythro- and granulopoietic lineage. For the first time, we were able to demonstrate that complexes of red cells in various stages of maturation can occur in the skin,similarly to, the erythroblastic islands of the bone marrow. In the pathogenesis of extramedullary hematopoiesis, mechanisms underlying the reconstitution of blood cells must be considered. These may reactivate hematopoiesis in organs where it previously occurred in embryonic and fetal life. Possible causative factors may be great compensatory demand, deficient replacement, or loss or dysfunction of corpuscular blood elements. This. would explain the occurrence of this disease entity in conjunction with etiologically completely heterogeneous systemic diseases

    Sprint training in preadolescent soccer players

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    Young soccer players are usually trained with adult-training methods, even though the physiological adaptations are likely to be very different compared with adults. In contrast, some have suggested training preadolescents only with coordination training. The purpose of this study was to investigate whether coordination or repeatedsprint training better improved speed over 20 m, with and without the ball. Sixteen soccer players (mean age 11 ± 0.5 y) were randomly assigned to a sprint-training group (STG = 7) or a coordination-training group (CTG = 9). The STG trained twice a week for 12 wk and performed 20 repetitions of 20- and 10-m sprints; the CTG performed coordination training (eg, speed ladder running) for the same training duration. Maximal jump height, anthropometric measures, and 20-m sprint time, with and without ball, were evaluated before and after the training period. Statistical significance was determined using two-way ANOVA with repeated measure and Pearson test for correlation. Both groups improved speed without the ball: STG = 3.75 ± 0.10 s to 3.66 ± 0.09 s (P < .05); CTG = 3.64 ± 0.13 s to 3.56 ± 0.13 s (P < .05), with no difference between groups. Sprint time with the ball pre- and posttraining was 4.06 ± 0.11 s and 4.05 ± 0.19 s (P > .05) for STG and 4.04 ± 0.12 s and 3.82 ± 0.15 s (P < .05) for CTG, with a significant difference between groups posttraining (P < .05). There were significant correlations between sprint time without ball, CMJ, and SJ. These data suggest that coordination training increases the speed with the ball more than typical repeated-sprint training. It can be hypothesized that running speed with ball improved more in CTG because this particular action requires improvements in coordination

    Production yields of noble-gas isotopes from ISOLDE UCx/graphite targets

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    Yields of He, Ne, Ar, Kr and Xe isotopic chains were measured from UCx/graphite and ThCx/graphite targets at the PSB-ISOLDE facility at CERN using isobaric selectivity achieved by the combination of a plasma-discharge ion source with a water-cooled transfer line. The delay times measured for a UCx/graphite target allow for an extrapolation to the expected yields of very neutron-rich noble gas isotopes, in particular for the "NuPECC reference elements" Ar and Kr, at the next-generation radioactive ion-beam facility EURISOL. (C)2003 Elsevier Science B.V. All rights reserved

    HLA-B*5801 allele as a genetic marker for severe cutaneous adverse reactions caused by allopurinol

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    Allopurinol, a commonly prescribed medication for gout and hyperuricemia, is a frequent cause of severe cutaneous adverse reactions (SCAR), which include the drug hypersensitivity syndrome, Stevens–Johnson syndrome, and toxic epidermal necrolysis. The adverse events are unpredictable and carry significant morbidity and mortality. To identify genetic markers for allopurinol–SCAR, we carried out a case-control association study. We enrolled 51 patients with allopurinol–SCAR and 228 control individuals (135 allopurinol-tolerant subjects and 93 healthy subjects from the general population), and genotyped for 823 SNPs in genes related to drug metabolism and immune response. The initial screen revealed strong association between allopurinol–SCAR and SNPs in the MHC region, including BAT3 (encoding HLA-B associated transcript 3), MSH5 (mutS homolog 5), and MICB (MHC class I polypeptide-related sequence B) (P < 10(–7)). We then determined the alleles of HLA loci A, B, C, and DRB1. The HLA-B*5801 allele was present in all (100%) 51 patients with allopurinol–SCAR, but only in 20 (15%) of 135 tolerant patients [odds ratio 580.3 (95% confidence interval, 34.4–9780.9); corrected P value = 4.7 × 10(–24)] and in 19 (20%) of 93 of healthy subjects [393.51 (23.23–6665.26); corrected P value = 8.1 × 10(–18)]. HLA alleles A*3303, Cw*0302, and DRB1*0301 were in linkage disequilibrium and formed an extended haplotype with HLA-B*5801. Our results indicated that allopurinol–SCAR is strongly associated with a genetic predisposition in Han Chinese. In particular, HLA-B*5801 allele is an important genetic risk factor for this life-threatening condition
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