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1.
JM Martín† L Calduch† C Monteagudo‡ I Molina† D Ramón† V Alonso† E Jordᆠ《Journal of the European Academy of Dermatology and Venereology》2006,20(4):428-431
Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobulinaemia. We present the case of a 55-year-old Caucasian man who suffered from a cutaneous plasmacytosis associated with two different carcinomas. Cutaneous plasmacytosis seems to be a reactive process because most cases reported are not associated with any apparent underlying disease. Nevertheless, because few reported cases were associated with malignancies, screening of additional neoplasms would be justified. 相似文献
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JM Vilanova J Figueras-Aloy J Roselló G Gómez E Gelpí R Jiménez 《Acta paediatrica (Oslo, Norway : 1992)》1998,87(5):588-592
The aim of this study was to evaluate the cerebral synthesis of eicosanoids in the asphyctic newborn and to investigate the relation between the prostanoid profiles in cerebrospinal fluid (CSF) and the appearance and severity of hypoxic-ischaemic encephalopathy (HIE). Levels of 6-keto-PGF 1-α, TXB2 , PGE2 and PGF2-α in CSF were measured in 40 full term newborns during the first day of life. Thirty of these newborns had birth asphyxia and were divided into three groups: 10 without HIE, 12 with mild HIE and 8 with moderate-severe HIE. They were compared to a control group of 10 non-hypoxic newborns. Determinations of the metabolites in CSF were performed by RIA and expressed as pg/ml (mean ± SD). The CSF TXB2 (thromboxane A2 metabolite) in asphyxiated newborns was always higher than in the control group (28.12 ± 10.6), and related to the severity of HIE ( p = 0:005): without HIE (50.84 ± 16.4; p = 0:02), mild HIE (80.65 ± 12.64; p ± 0:01) and moderate-severe HIE (178.14 ± 20.5; p < 0:01). The CSF 6-keto-PGF 1-α (prostacyclin metabolite) in asphyxiated newborns was always higher than in the control group (80.55 ± 12.56), but indirectly related to the severity of HIE: without HIE (240.95 ± 28.12; p < 0:01), mild HIE (183.65 ± 30.1; p < 0:01) and moderate-severe HIE (140.55 ± 25.12; p < 0:01). In the moderate-severe HIE group, the increase in TXB2 was higher than the rise in 6-keto-PGF 1-α . 相似文献
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Vigo T Schenone A Mancardi G Abruzzese M Timmerman V Van Hummelen P Nobbio L . 《Journal of the peripheral nervous system : JPNS》2004,9(2):111-111
CMT1A patients bear the same genetic defect but often present with a wide range of clinical disability. Knowing the relationship between the phenotypic variability and other parameters, such as electrophysiological findings, age, gender, disease duration and environmental factors may be important for understanding the pathogenetic mechanisms underlying CMT1A. We studied 15 families and 7 sporadic cases affected by CMT1A (27 adults and 9 children) from the clinical, neurophysiological and genetic standpoint. A detailed patient history included: disease onset and progression, distribution of weakness, additional symptoms, life habits, genealogical tree, exposure to toxic substances, geographic provenance, instruction grade, job, use of drugs, and concurrent diseases. A questionnaire about diet was administered. The disability was evaluated by modified Rankin scale, deambulation index, functional independence measure and Barthel index. As previously reported, disease onset was in the first decade in 50% of cases and before the age of 20 years in 70% of cases. Severe disability was rarely observed, only 2 patients walking with a cane. No clear influence of gender over clinical severity was observed. Interestingly, genetic anticipation was observed in all the families. Data on the influence of environmental factors will also be presented. 相似文献