沈阳地区76例非何杰金淋巴瘤的分类及其免疫表型病理分析

目的：探讨我国沈阳地区非何杰金淋巴瘤（NHL）的病理特点及免疫组化表型分析。方法：对76例NHL按照WHO新分类进行病理观察和10种抗体免疫表型测定。结果：B系占78．9％,其中弥漫性大B细胞淋巴瘤（DLBL）最多;其次为MALT型（＋／－单核细胞样B细胞）及淋巴浆细胞性淋巴瘤（LPL）。T系占21．1％,以外周T非特殊型最多。CD79a对B－NHL染色阳性率达100％,未见与T细胞的交叉反应。CD3对T－NHL染色阳性率为88％,与B细胞仅有5％的交叉反应。结论：我国沈阳地区NHL以DLBL和外周T非特殊型为最多见,结内单核B和淋巴浆细胞性比例较高。CD79a和CD3分别是具有高度敏感性和特异性的B、T细胞标志抗体。     

The extracellular matrix can regulate vascular cell migration, proliferation, and survival: relationships to vascular disease

The extracellular matrix (ECM) of the normal artery wall is a collection of fibrous proteins and associated glycoproteins embedded in a hydrated ground substance of glycosaminoglycans and proteoglycans. These distinct molecules are organized into a highly ordered network that are closely associated with the vascular cells that produce them. In addition to providing the architectural framework for the artery wall that imparts mechanical support and viscoelasticity, the ECM can regulate the behaviour of vascular cells, including their ability to migrate, proliferate and survive injury. The composition of the ECM is different within intimal lesions of atherosclerosis, which are composed of monocytes and lymphocytes from the circulation and smooth muscle cells (SMC) that migrate from the media to the intima (Ross 1993, 1999), and these differences may contribute to the altered phenotype of vascular cells within lesions. This review will briefly outline the ECM changes observed in atherosclerosis and restenosis and the potential relationship of these changes to altered vascular cell functions.     

神经系统副肿瘤综合征临床诊断进展

神经系统副肿瘤综合征（paraneoplastic neurological syndrome,PNS）是肿瘤通过远隔效应累及神经系统的一组罕见的免疫介导性疾病,其临床症状多样,通常伴有特异性的神经抗体。2021年,国际专家组修订了2004年PNS诊断标准,提出了新的临床评分系统,突出强调了“表型-抗体-肿瘤”的关联性。本研究回顾近年文献,围绕新标准,对PNS的临床诊断进展作一综述。     

睡眠呼吸暂停综合征患者T细胞,NK细胞功能的研究

目的：检测睡眠呼吸暂停综合征（ＳＡＳ）患者外周血Ｔ淋巴细胞、ＮＫ细胞的变化,探讨ＳＡＳ对细胞免疫功能的影响。方法：应用ＡＰＡＡＰ桥联酶标法测定外周血淋巴细胞表型。结果：ＳＡＳ患者组ＣＤ３＾＋细胞与正常对照组差异无显著性（Ｐ〉０．０５）,ＣＤ４＾＋、ＣＤ４＾＋／ＣＤ８＾＋、ＣＤ１６＾＋、ＣＤ２５＾＋细胞ＳＡＳ组均低于正常对照组,而ＣＤ８＾＋细胞高于正常对照组（Ｐ〈０．０５,Ｐ〈０．０１）。结论：ＳＡ     

PSP57不是增生和癌变前列腺组织PSP94 mRNA可变剪切特有的产物

目的：比较人正常,增生和癌变前列腺组织中ＰＳＰ９４和ＰＳＰ５７ｍＲＮＡ的表达情况。方法：提取事故死亡的正常成年人前列腺组织及手术得到的增生和前列腺癌组织总ＲＮＡ,进行ＲＴ－ＰＣＲ,其产物分别以ＰＳＰ９４和ＰＳＰ５７共有的外显子及ＰＳＰ９４外显子Ⅲ作探针进行Ｓｏｕｔｈｅｒｎ ｂｌｏｔｔｉｎｇ分析;ＲＴ－ＰＣＲ产物经克隆后进行序列测定。结果：三种前列腺组织中均有ＰＳＰ９４和ＰＳＰ５７ｍＲＮＡ的表达;人     

1p36 deletion syndrome: Review and mapping with further characterization of the phenotype,a new cohort of 86 patients

Chromosome 1p36 deletion syndrome (1p36DS) is one of the most common terminal deletion syndromes (incidence between 1/5000 and 1/10,000 live births in the American population), due to a heterozygous deletion of part of the short arm of chromosome 1. The 1p36DS is characterized by typical craniofacial features, developmental delay/intellectual disability, hypotonia, epilepsy, cardiomyopathy/congenital heart defect, brain abnormalities, hearing loss, eyes/vision problem, and short stature. The aim of our study was to (1) evaluate the incidence of the 1p36DS in the French population compared to 22q11.2 deletion syndrome and trisomy 21; (2) review the postnatal phenotype related to microarray data, compared to previously publish prenatal data. Thanks to a collaboration with the ACLF (Association des Cytogénéticiens de Langue Française), we have collected data of 86 patients constituting, to the best of our knowledge, the second-largest cohort of 1p36DS patients in the literature. We estimated an average of at least 10 cases per year in France. 1p36DS seems to be much less frequent than 22q11.2 deletion syndrome and trisomy 21. Patients presented mainly dysmorphism, microcephaly, developmental delay/intellectual disability, hypotonia, epilepsy, brain malformations, behavioral disorders, cardiomyopathy, or cardiovascular malformations and, pre and/or postnatal growth retardation. Cardiac abnormalities, brain malformations, and epilepsy were more frequent in distal deletions, whereas microcephaly was more common in proximal deletions. Mapping and genotype–phenotype correlation allowed us to identify four critical regions responsible for intellectual disability. This study highlights some phenotypic variability, according to the deletion position, and helps to refine the phenotype of 1p36DS, allowing improved management and follow-up of patients.     

解剖型钢板治疗肱骨远端粉碎性骨折

目的：探讨解剖Ｙ型钢板的临床应用价值。方法：报告３６例解剖Ｙ型钢板内固定肱骨远端粉碎性骨折,进行临床分析讨论,其中列,女８例,平均年龄３６．８岁。３６例中均行肘后标准切口尽骨鹰嘴截骨暴整个肱骨远端,Ｙ型钢板、螺钉内固定。结果：本组随访１～５年,其中优良率８６．２％。结论：解剖Ｙ型钢板治疗治疗肱骨远端粉碎骨折,效果确切,术后有利早期活动关节,减少并发症。     

46,XY gonadal dysgenesis associated with congenital nephrotic syndrome and sepsis

The occurrence of nephrosis in the first 3 months of life is rare and is termed ’congenital nephrotic syndrome.’ The congenital nephrotic syndrome is a group of heterogeneous diseases with a clinical course that differs markedly from the childhood nephrotic syndrome. The coexistence of a congenital nephrotic syndrome and gonadal dysgenesis in a 46,XY karyotype with normal female external genitalia is extremely rare. Frequent severe infections are often seen in the Finnish type, but sepsis leading to death is rare in the neonatal onset of gonadal dysgenesis. This report describes an unusual case of complete XY gonadal dysgenesis in a 46,XY female neonate with the congenital nephrotic syndrome and overwhelming sepsis. Received: 4 January 1999 / Revised: 24 May 1999 / Accepted: 25 May 1999