寻常型天疱疮、大疱性类天疱疮及有关大疱性皮肤病的免疫学

器官特异性自身免疫性疾病,包括寻常型天疱疮和大疱性类天疱疮等.这些疾病的特点,是在患者的皮肤损害处发生有高度特异性的自身免疫性反应.下表是收集这类大疱性皮肤病的组织学、超微结构及免疫荧光的检查资料.一、寻常型天疱疮寻常型天疱疮是一种损害皮肤与粘膜的疾病,以多处水疱和糜烂为特征性表现.虽     

肿瘤标记物联合检测对肿瘤高危人群筛查的临床意义

目的 研究应用肿瘤标记物联合检测对恶性肿瘤高危人群筛查的临床价值.方法 选择血清肿瘤标记物组合对3 766例恶性肿瘤高危人员进行检测,对肿瘤标记(CA125、CEA、Cyfra21-1、CA19-9、AFP、NSE、CA72-4、SCC、CA153)升高人员进一步行颈胸部CT、腹盆部CT或内窥镜检查,女性进行妇科检查,未明确诊断者进行长期随访(大于6个月).结果 经过随访,共发现肿瘤患者69例,其中发现肿瘤标志物升高6个月内诊断为恶性肿瘤20例(62.5％),6个月至1年诊断5例(15.6％),1年至2年诊断4例(12.5％),2年至3年诊断2例(9.4％).COX分析显示肿瘤标志物升高的程度(RR=2.308,95％ CI:2.517 ～2.475,P＜0.001)、肿瘤标志物逐渐升高(RR=7.727,95％ CI:3.008 ～ 19.836,P＜0.05)和有相关临床症状(RR=7.879,95％ CI:2.357 ～ 26.384,P＜0.05)是筛查肿瘤标志物升高人员患肿瘤的危险因素.结论 血清肿瘤标记物联合检测对肿瘤筛查人群应答率高,危险因素调查是肿瘤筛查的有效手段.     

12例大疱性类天疱疮患者的护理体会

大疱性类天疱疮是一种获得性自身免疫性大疱性皮肤病,其特征为表皮下形成大疱,基膜带有免疫球蛋白和补体沉积.针对该病慢性、复发性、用药时间长的特点,除了积极治疗外,更需要护理的密切配合[1].     

Dsg1、Dsg3及抗BP-180抗体水平与天疱疮及大疱性类天疱疮患者病情活动度的相关性研究

目的 分析研究桥粒芯糖蛋白1(Dsg1)、Dsg3及抗BP-180抗体水平与天疱疮及大疱性类天疱疮(BP)患者病情活动度的相关性.方法 选择2016年1月至2020年1月医院住院治疗的天疱疮患者62例(天疱疮组)和BP患者58例(BP组),另选取医院同一时间段内收治的非天疱疮及BP患者50例,作为对照组,ELISA法检测Dsg1、Dsg3及抗BP-180抗体水平,比较天疱疮及BP患者初发以及治疗后不同阶段自身免疫性大疱性皮肤病严重程度评分(ABSIS),并采用Pearson双变量检验分析天疱疮及BP患者Dsg1、Dsg3及抗BP-180抗体水平与ABSIS评分的相关性.结果 天疱疮患者和对照组Dsg1、Dsg3抗体阳性率以及BP患者和对照组抗BP-180抗体阳性率比较,差异均有统计学意义(P<0.05);治疗后,天疱疮患者Dsg1、Dsg3抗体水平、ABSIS评分以及BP患者抗BP-180抗体水平、ABSIS评分均显著低于初发时(P<0.05);Pearson双变量检验结果显示,初发以及治疗后不同阶段天疱疮患者Dsg1、Dsg3抗体水平与ABSIS评分均呈正相关(P<0.05),初发以及治疗后不同阶段PB患者抗BP-180抗体水平与ABSIS评分呈正相关(P<0.05).结论 天疱疮及BP患者Dsg1、Dsg3及抗BP-180抗体水平与ABSIS评分存在相关性,Dsg1、Dsg3及抗BP-180抗体水平可在一定程度上反映患者病情活动度,从而有助于指导临床制定合理治疗方案.     

原发性卵巢类癌6例并临床病理分析

目的探讨卵巢原发性类癌的临床特点及病理特征。方法总结6例原发性卵巢类癌的临床资料和病理学特点,并复习相关文献。结果 6例患者发病年龄28~54岁,多以发现盆腔肿块就诊,B超示囊实性占位而入院治疗。查肿瘤指标CEA、CA199、CA125未见明显升高。肿瘤发生于卵巢左、右侧各3例。组织学类型:梁状型3例,甲状腺肿型2例,岛状型1例。卵巢类癌常合并成熟性囊性畸胎瘤。结论卵巢原发性类癌为罕见的恶性肿瘤,具有低度恶性,预后良好。肿瘤转移虽少见但可晚期复发,故需长期随访。肿瘤体积较小时,需充分取材以避免漏诊。诊断时需与卵巢颗粒细胞瘤、Sertoli细胞瘤、恶性卵巢甲状腺肿、转移性类癌、卵巢甲状腺肿、Brenner瘤等鉴别。     

隐性营养不良性大疱性表皮松解症皮肤的免疫荧光研究

目的：为了确定P200蛋白质抗原的性质。方法：收集了12例抗P200类天疱疮血清，对6例隐性营养不良性大疱性表皮松解症(RDEB)皮肤切片进行了间接免疫荧光研究。结果：发现所有12例抗P200类天疱疮血清与5例RDEB皮肤基底膜带(BMZ)反应，而获得性大疱表皮松解症(EBA)血清对这些皮肤为阴性。另外，在1例RDEB，EBA血清既与BMZ反应又与Ⅶ型胶原沉积部位的胞浆反应，而抗P200类天疱疮血清无此反应。结论：这些结果提示200kD抗原不是Ⅶ型胶原成份，而是一种特异的自身抗原。     

霍奇金淋巴瘤伴显著肉芽肿反应2例

例 1 女性,47 岁,2017 年4 月因发现左侧颈部肿块入院,术中冷冻切片及常规病理诊断为"淋巴结慢性肉芽肿性炎,考虑为结核".抗结核治疗2年余,疗效欠佳.颈部淋巴结渐增大、增多.再次入院查体:左侧颈部见数个肿大淋巴结,最大径0. 5 ～6 cm,质硬,无压痛,活动差,见陈旧性皮肤疤痕,无红肿及窦道.辅助检查:血常...     

产前超声诊断单脐动脉的临床价值

目的探讨单脐动脉的产前超声诊断及其临床价值。方法对产前超声检查中发现的40例单脐动脉胎儿进一步进行全面筛查,并行脐血染色体检查,对妊娠结局进行随访。结果 40例单脐动脉中,单纯性单脐动脉28例(70%),合并其他畸形12例(30%),其中心血管系统畸形3例,中枢神经系统畸形2例,泌尿系统畸形2例,多发性畸形2例,消化系统畸形、桡骨发育不良及膈疝各1例;接受脐血染色体检查7例,染色体核型异常3例;单纯性单脐动脉预后良好。结论产前超声可以明确诊断单脐动脉。单脐动脉合并其他畸形的发生率高于正常,合并畸形时染色体异常发生率增高。当超声检查发现单脐动脉时,应进一步作系统筛查及染色体检查,并对单脐动脉胎儿生长发育指标进行监测评估,均具有重要的临床意义。     

孕中期母血清标记物筛查唐氏综合征及其它先天畸形的研究

目的进行唐氏综合征及其它先天畸形的产前筛查、诊断,以降低出生缺陷.方法对2812例孕14～20w的孕妇采用酶联免疫方法检测孕妇血清AFP和β-HCG浓度,通过"优生胜算"软件计算危险系数,对唐氏高危孕妇取羊水作染色体和基因诊断;对神经管缺损高危孕妇行B超检查.结果在2812例孕妇中筛查出唐氏综合征、神经管缺、及18、13三体高危315例,占筛查总数的11.2%;有121人愿意进一步确诊,发现2例异常妊娠; 27例18、13三体高危孕妇,虽未作进一步确诊,但有2例已胎死宫内,证明也为异常妊娠;在神经管缺损高危的79例中,全部作了B超检查,发现1例神经管缺损患儿.结论母血清标记物进行唐氏综合征及其它先天畸形的产前筛查并结合其它方法进行诊断,对降低出生缺陷具有积极意义.     

二例免疫缺陷病(CVID)的报告

<正> 我们从二例免疫球蛋白定量检测中发现异常,经进一步追踪和补充一些免疫学检查,最终协助临床确诊为原发性普通变异型免疫缺陷病(简称CVID)。现报道如下。 例1:女,19岁,未婚,上海市人。因咳嗽、胸痛、伴高热数日而收治入院。主诉:自幼常患感冒、肺炎、支气管炎等呼吸道感染病症。也曾患天疱疮和副鼻窦炎病,10岁时染上乙脑,愈后记忆力减退,体质变差。     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.     

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: Substrate beriberi

About 1900, modern food selection and processing caused widespread $\text{epidemics}$ of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a $\text{panh}$ypovitaminosis B, i.e., a mixture of $\text{substrate}$ beriberi and $\text{substrate}$ pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse $\text{endemic}$ disease. This would consitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modermized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by our diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w3-EFA, is safe for $\text{primates}$. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.     

'Very sore nights and days': the child's experience of illness in early modern England, c.1580-1720

Sick children were ubiquitous in early modern England, and yet they have received very little attention from historians. Taking the elusive perspective of the child, this article explores the physical, emotional, and spiritual experience of illness in England between approximately 1580 and 1720. What was it like being ill and suffering pain? How did the young respond emotionally to the anticipation of death? It is argued that children’s experiences were characterised by profound ambivalence: illness could be terrifying and distressing, but also a source of emotional and spiritual fulfilment and joy. This interpretation challenges the common assumption amongst medical historians that the experiences of early modern patients were utterly miserable. It also sheds light on children’s emotional feelings for their parents, a subject often overlooked in the historiography of childhood. The primary sources used in this article include diaries, autobiographies, letters, the biographies of pious children, printed possession cases, doctors’ casebooks, and theological treatises concerning the afterlife.     

Guidelines for the Validation and Use of Immunoassays for Determination of Introduced Proteins in Biotechnology Enhanced Crops and Derived Food Ingredients

Recent advancements in agricultural biotechnology have created a need for analytical techniques to determine introduced proteins in crops enhanced through modern biotechnology techniques. These proteins are expressed in plant tissues and may be present in food ingredients. Immunoassays are ideally suited for protein detection and may be used as both quantitative and threshold methods. Microplate ELISA and lateral flow devices are two of the most commonly used immunoassay formats for agricultural biotechnology applications. This paper provides general background information and a discussion of criteria for the validation and application of immunochemical methods to the analysis of proteins introduced into plants and food ingredients using biotechnology methods. It is the result of a collaborative effort of members of the Analytical Environmental Immunochemical Consortium. This collaborative effort represents the combined expertise of several organizations to reach consensus on establishing guidelines for the validation and use of immunoassays. Further, the paper offers developers and users a consistent approach to adopting the technology as well as aid in producing accurate and meaningful results.     

Ultracryotomy: development and application (with examples from the yeast cytology) (author's transl)

The preparation steps usually necessary for obtaining ultrathin frozen sections of biological material (chemical prefixation, enclosing, cryoprotective treatment, freezing, sectioning, and post-staining the sections for transmission electron microscopy) are submitted to a critical analysis. The application of cryo-ultramicrotomy, in particularly for cytochemical purposes, is reviewed. Fundamental considerations of chemical prefixation and poststaining are supported by examples from yeast cytology. Furthermore, the efficiency of the cryo-ultramicrotomy (electron optical resolution of ultrastructural details) is demonstrated on yeast cells and protoplasts.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

The case for allele‐specific recognition by the TCR

There is a sharp difference in how one views TCR structure–function–behaviour dependent on whether its recognition of major histocompatibility complex‐encoded restriction elements (R) is germline selected or somatically generated. The generally accepted or Standard model is built on the assumption that recognition of R is by the V regions of the αβ TCR, which is not driven by allele specificity, whereas the competing model posits that recognition of R is allele‐specific. The establishing of allele‐specific recognition of R by the TCR would rule out the Standard model and clear the road to a consideration of a competing construct, the Tritope model. Here, the case for allele‐specific recognition (germline selected) is detailed making it obvious that the Standard model is untenable.     

The universal muscular chamber. A basic unit of the genitourinary, cardiovascular, gastro-intestinal, skeletal, cutaneous, respiratory and other systems, endocameral hypertension; and spasm, the key to their idiopathic diseases and arthropathies

Starting with the integument, we see many organs are contractile sacs or multiples thereof, which tubes or bags constitute the major part of the entire body. Recognition of this basic unit and its characteristics sheds new light, individually and collectively, on many disorders previously considered unrelated. Muscular tears and perforations develop in the walls of these chambers, being no way peculiar to those organs, wherein, hydrochloric acid occurs. So, it is not necessary to explain the absence of excessive acid from patients who exhibit holes in the gastric, uterine, aortic, duodenal, rectal, pulmonary, retina, and other walls. Muscle, not acid is the great common factor relating idiopathic disorders in the gastrointestinal tract to each other and to similar diseases in other systems. When the units are linked together, the lesions tend to appear as arthropathies, i.e. at the joints. Rephrasing common-place observations, frees us from conventional, conceptual cul-de-sacs. An observation is only as good as its interpretation, so all possibilities must be considered, otherwise, we will remain blinded by our misconceptions.