诊断早期蕈样霉菌病组织学标准的效验--欧洲癌症研究和治疗组织皮肤淋巴瘤研究组的调查报告

为了效验组织病理学标准在诊断早期蕈样霉菌病(ＭＦ)病变中的价值,作者回顾分析了18例患者24次皮肤活检切片,取材时患者处于ＭＦＩａ期。这些病人都具备以下条件:①临床明确诊断ＭＦ或在随后的活检中出现了确切无疑的ＭＦ组织学形态。②通过完整的随访(从起病到发生明显的淋巴瘤,或病人死亡),证明在疾病随后进展中出现了ＭＦ经典“ＡｌｉｂｅｒＢａｚｉｎ”型中典型的斑点、班块、肿瘤期的发展过程。③病人处于Ｉａ期(即局限性斑块、丘疹或湿疹性斑点出现,覆盖面积小于身体表面10%)至少5年。选择13例苔藓样、海绵状及牛皮癣样与ＭＦ相似的病…     

Spontaneous periodic hypothermia with lipoma of the corpus callosum

A patient with spontaneous periodic hypothermia who had both a lipoma and agenesis of the corpus callosum is described. Spontaneous periodic hypothermia associated with corpus callosum abnormalities is a distinct entity and although the mechanism underlying the hypothermic episodes is unexplained, the term “diencephalic autonomic epilepsy” does not seem appropriate.     

Simon nitinol inferior vena cava filter: initial clinical experience. Work in progress

The Simon nitinol filter for percutaneous interruption of the vena cava to prevent pulmonary embolism is currently undergoing a multicenter clinical trial. Preliminary clinical results are reported as work in progress. The results in 44 patients at two centers are analyzed in detail, and major events are reported from 103 patients in 17 centers in the United States during a 10-month period. The filter was successfully inserted via the femoral or jugular route in all patients through a 9-F catheter. The placement procedure was easy and without significant complications. Follow-up studies included plain radiography, ultrasonography, magnetic resonance (MR) imaging, and clinical evaluation. No filter migration or perforation occurred among the 103 patients. Symptomatic occlusions occurred in 7%-9%, comparable to other series, and some asymptomatic occlusions were detected with MR imaging only. The implications of occlusion of the filter are discussed.     

Medical Negligence - Going Dutch

Forensic DNA profiling combines the dynamic science of molecular genetics with the complexities of the forensic context. As such, throughout its twenty-year history, this field has seen continuous change, remarkable growth and an associated level of public, legal and scientific scrutiny previously unknown in the forensic sciences. Almost ubiquitously, applications of forensic DNA profiling have focused on resolving the identity of the donor of a particular sample of biological evidence. This is typically achieved by targeting non-coding microsatellite (or STR) loci. Recently however, novel techniques have emerged that target different DNA polymorphisms and allow scientists to address additional questions about the evidence, beyond the identification of source. In this short review we introduce an example of this next generation of forensic DNA techniques, single nucleotide polymorphisms (or SNPs). We summarise their molecular and technological basis and the manner in which they may be applied to further advance the capabilities of forensic investigators.     

Increased rotational mobility and extractability of band 3 from protein 4.2-deficient erythrocyte membranes: evidence of a role for protein 4.2 in strengthening the band 3-cytoskeleton linkage

Band 3 (anion-exchange protein 1-[AE1]) is the major integral membrane protein of human erythrocytes and links the membrane to the underlying cytoskeleton via high-affinity binding to ankyrin. It is unclear whether other cytoskeletal proteins participate in strengthening the ankyrin-band 3 linkage, but a putative role for protein 4.2 (P4.2) has been proposed based on the increased osmotic fragility and spherocytic morphology of P4.2-deficient red blood cells (RBCs). The present study was designed to investigate the hypothesis that P4.2 has a direct role in strengthening the band 3-cytoskeleton linkage in human RBCs, by measuring independent features of this interaction in normal and P4.2- deficient RBCs. The features examined were the rotational mobility of band 3 assayed by time-resolved phosphorescence emission anisotropy (TPA), and the extractability of band 3 by octyl-beta-glucoside, the latter being a nonionic detergent that selectively extracts only band 3 that is not anchored to the cytoskeleton. We find that the amplitude of the most rapidly rotating population of band 3 (correlation time, approximately 30 to 60 microseconds) is increased 81% and 67% in P4.2- deficient ghosts (P4.2NIPPON and band 3MONTEFIORE, respectively) compared with control ghosts. The amplitude of the intermediate speed rotating population of band 3 (correlation time, approximately 200 to 500 microseconds) is increased 23% and 8% in P4.2-deficient ghosts (P4.2NIPPON and band 3MONTEFIORE, respectively) compared with control ghosts, at the expense of the slowly rotating component (correlation time, approximately 2,000 to 3,000 microseconds, amplitude decreased 43% and 39% in P4.2NIPPON and band 3MONTEFIORE, respectively) and immobile component (immobile on this experimental time scale; amplitude decreased 26% and 10% in P4.2NIPPON and band 3MONTEFIORE, respectively) of band 3. These results demonstrate that P4.2 deficiency partially removes band 3 rotational constraints, ie, it increases band 3 rotational mobility. The nonionic detergent octyl-beta-glucoside, which does not disturb band 3-cytoskeleton associations, ie, it extracts only band 3 that is not attached to the cytoskeleton, extracted 30% and 61% more band 3 from P4.2NIPPON and band 3MONTEFIORE ghost membranes, respectively, compared with control ghosts. The octyl-beta-glucoside ghost extracts from both P4.2-deficient phenotypes were enriched in band 3 oligomeric species (tetramers, higher-order oligomers, and aggregates) compared with controls. Since band 3 oligomers selectively associate with the cytoskeleton, these results are consistent with a weakened band 3-cytoskeleton linkage in P4.2-deficient RBC membranes. P4.2 deficiency does not affect band 3 anion transport activity, since uptake of radiolabeled sulfate was similar for control and P4.2- deficient RBCs. Thus, we propose that P4.2 directly participates in strengthening the band 3-cytoskeleton linkage.