乳头汗腺瘤样腺瘤1例及文献复习

目的 探讨乳头汗腺瘤样腺瘤的临床病理和免疫表型特点及诊断与治疗。方法 通过HE及免疫组化染色观察1例乳头汗腺瘤样腺瘤,并结合文献讨论。结果 肿瘤发生于乳头,由上皮细胞形成的不规则巢、小条索或小管在致密的纤维间质及平滑肌之间呈浸润性生长,其中可见“蝌蚪状”细胞巢及角化囊肿,病变边界不清。免疫表型：CK阳性,p63、SMA部分阳性,ER、PR及GCDFP-15均阴性。结论 乳头汗腺瘤样腺瘤是一种呈局部浸润性生长的非常罕见的乳头部良性肿瘤,局部切除后可复发,但不转移。组织学上主要应与乳头管腺瘤、导管内乳头状瘤伴鳞化及管状癌鉴别。适当的治疗应该包括肿瘤及其周边充分的正常组织完整切除,且标本切缘阴性。     

甲状腺伴胸腺样分化的梭形细胞肿瘤临床病理分析

目的 探讨甲状腺伴胸腺样分化的梭形细胞肿瘤(spindle cell tumor with thymus-like differentiation,SETTLE)临床病理学特征及其鉴别诊断.方法 报道1例罕见的甲状腺SETTLE,并结合文献对其临床特征、组织学形态、免疫表型及鉴别诊断等进行分析.结果 镜下肿瘤组织主要为梭形细胞成分与腺样成分互相融合的双相分化表现.免疫表型:CK、EMA和vimentin均阳性.结论 甲状腺SETTLE的诊断依靠形态学及免疫表型,需与甲状腺未分化癌、滑膜肉瘤、甲状腺伴胸腺样分化的癌、异位错构瘤型胸腺瘤等进行鉴别.手术切除预后较好.     

子宫颈鳞状移行细胞癌临床病理分析及组织分化观察

目的：探讨子宫颈鳞状一移行细胞癌的临床病理、免疫表型、诊断和鉴别诊断。方法：按2003年WHO宫颈肿瘤分类标准观察5例宫颈鳞状移行细胞癌患者的临床病理特点,应用免疫组织化学方法对宫颈活组织和手术标本进行组织形态和免疫表型的检测。结果：患者年龄中位数为45岁,临床主要表现为阴道异常出血,肉眼子宫颈肥大、呈颗粒状、息肉样或菜花样新生物（4／5）。显微镜下未见明显挖空细胞,呈移行或鳞状细胞样分化,部分似有腺细胞样分化。组织形态学：肿瘤由纤维血管轴心的乳头状结构构成,乳头由多层异型鳞状及移行细胞样上皮覆盖。2例非浸润性,2例乳头间质浸润,1例子宫颈壁浸润;免疫组化显示P16和细胞角蛋（cytokeratin,CK）19和CK5／6均强阳性,CK7阳性强弱不等,1例CK20上皮表层灶状阳性,余为阴性;4例高危型HPV检测阳性。结论：鳞状移行细胞癌是兼有鳞状-移行细胞形态和一定腺样分化的非腺性乳头状鳞状细胞癌,它与高危型HPV感染相关,具有向乳头间质和宫颈壁浸润、复发及转移的特点。鳞状移行细胞癌需与宫颈其他乳头状肿瘤鉴别。     

膀胱非尿路上皮起源癌的诊断与鉴别诊断

发生在膀胱非尿路上皮起源的癌(以下简称非尿路上皮癌, non-urothelial carcinomas, N-UC)相对少见, 可具有鳞状、腺样或神经内分泌癌(neuroendocrine carcinoma, NEC)的分化特征。伴有鳞状分化的膀胱癌主要包括传统型鳞状细胞癌、疣状癌、伴广泛鳞状分化的尿路上皮癌及基底细胞样鳞状细胞癌。伴有腺样分化特征的膀胱癌主要包括原发性膀胱腺癌、脐尿管腺癌、苗勒管型肿瘤和肝样腺癌。膀胱神经内分泌肿瘤, 主要包括小细胞NEC、大细胞NEC、分化好的神经内分泌肿瘤(类癌)和副神经节瘤。在诊断膀胱原发性N-UC之前, 首先要排除其他部位的癌直接蔓延或转移到膀胱的可能性, 因此重点了解患者的临床病史十分有必要。该文重点阐述了这三组肿瘤中不同类型之间的形态学特征、免疫表型、诊断与鉴别诊断及临床预后因素等, 避免一些实际诊断工作中的陷阱问题。     

气管腺样囊性癌3例报道并文献复习

目的 探讨气管腺样囊性癌的病理临床特征,以提高对肿瘤的认识.方法 对3例气管腺样囊性癌进行临床及组织学观察,并用免疫组化方法研究肿瘤的免疫表型.结果 气管腺样囊性癌主要发生于气管支气管,呈浸润性生长,临床症状无特征;组织学上呈筛状、管状和实性生长方式;肿瘤导管上皮表达EMA和CK8,基底细胞表达p63和SMA,肿瘤细胞还表达CD117、S-100和NSE;肿瘤发展缓慢,长期预后不佳.结论 气管腺样囊性癌是一种少见肿瘤,组织学形态、免疫组化染色及特染有助于气管腺样囊性癌的诊断及鉴别诊断.     

支气管上皮-肌上皮癌临床病理观察

目的探讨支气管上皮-肌上皮癌(epithelial-myoepithelial carcinoma,EMC)的临床病理学特点、诊断及鉴别诊断。方法采用HE、免疫组化SP法对4例支气管EMC的标本进行组织学观察及免疫组化标记,并分析病理组织学形态和免疫表型。结果 EMC由内层腺上皮及外层肌上皮构成,呈双套管结构。腺上皮成分CK、EMA均呈阳性,肌上皮成分Calponin、p63、CK、CD117、S-100、GFAP均呈阳性。结论原发于支气管的EMC临床罕见,多数位于大支气管内,诊断依靠组织学形态及免疫表型,应与黏液表皮样癌、腺样囊性癌、多形性腺瘤转移性透明细胞癌鉴别。EMC治疗以手术切除为主,但也可复发或转移。     

原发性子宫内膜鳞状细胞癌伴子宫内膜不典型增生1例

原发性子宫内膜鳞状细胞癌(primary endometrial squamous cell carcinomas, PESCC)是一种罕见的子宫内膜癌。本文报道1例55岁患者, 刮宫标本及经腹腔镜筋膜外全子宫切除标本组织学形态均显示不同分化程度的鳞状上皮呈浸润性生长, 并见短梭形细胞, 胞质丰富、透亮。刮宫标本中未见正常子宫内膜腺体及异常腺性结构;全子宫切除标本中短梭形与鳞状上皮穿插生长, 侵犯浅肌层, 周围子宫内膜可见慢性子宫内膜炎, 局灶符合子宫内膜不典型增生;免疫表型:明确的鳞状上皮成分及短梭形细胞广谱细胞角蛋白、细胞角蛋白(CK)5/6、p63、β-catenin、CD10均阳性, CK7、CDX2阴性。患者随访8个月, 身体状况良好。PESCC需要严格掌握诊断标准, 除外子宫颈来源的鳞状细胞癌及子宫内膜样癌伴广泛鳞化。该文描述PESCC组织学特点、刮宫及全子宫标本诊断难点、阐述其诊断要点, 并复习和总结相关文献。     

甲状腺样滤泡性肾细胞癌的临床病理特点

甲状腺样滤泡性肾细胞癌(thyroid-like follicular renal cell carcinoma,TLFRCC)是一种非常少见的肾原发性恶性上皮性肿瘤,确诊主要依靠病理组织学及免疫组织化学,外科手术切除是最好的治疗方法,预后好.通过对郑州人民医院2014年收治的1例TLFRCC患者的临床资料、大体特征、组织病理学及免疫表型等进行分析,并结合文献报道的28例,进一步探讨TLFRCC的临床病理特点、免疫表型、诊断与鉴别诊断及遗传学特点.     

牙源性鳞状细胞瘤临床病理观察

目的 探讨牙源性鳞状细胞瘤的临床病理特征、诊断及鉴别诊断.方法 光镜观察及免疫组化法对1例牙源性鳞状细胞瘤进行临床病理分析,并复习文献.结果 肿瘤位于右下颌骨,病灶为多房囊性,表面骨质菲薄,内容物为灰白色凝胶样,骨质破坏.光镜下可见肿瘤组织由形态大小不等、分化良好的鳞状上皮岛和纤维间质构成,具有局部浸润性.免疫组化:Ki-67和p53均阴性.结论 牙源性鳞状细胞瘤是一种罕见的良性上皮性肿瘤,组织学形态特点是其诊断的主要依据,需与成釉细胞瘤、高分化鳞状细胞癌、牙源性钙化上皮瘤或成釉细胞纤维瘤相鉴别.     

伴脑膜上皮样漩涡和骨化生去分化脂肪肉瘤的临床病理分析

目的探讨伴脑膜上皮样漩涡和骨化生去分化脂肪肉瘤的临床特点、组织学特征、免疫表型、遗传学特点、诊断及鉴别诊断。方法回顾性分析1例伴脑膜上皮样漩涡和骨化生去分化脂肪肉瘤的临床资料、组织学特征、免疫表型和遗传学特点,并复习相关文献。结果伴脑膜上皮样漩涡和骨化生去分化脂肪肉瘤界限清楚,由硬化性脂肪肉瘤、脑膜上皮样漩涡状、骨化生和脂肪瘤样脂肪肉瘤等成分构成,脑膜上皮样漩涡状成分由上皮样细胞或梭形细胞呈漩涡状排列构成,与其它成分界限清楚。免疫表型:肿瘤细胞vimentin、MDM2、CDK4、p16、CD10和CD56均呈弥漫强阳性;CK、EMA、CD34、Caldesmon、PGP9.5和PR均呈阴性;S-100、Ki-67和p53在不同成分中表达不一致。遗传学检测显示MDM2扩增。结论伴脑膜上皮样漩涡和骨化生去分化脂肪肉瘤属于罕见的去分化脂肪肉瘤,脑膜上皮样漩涡状成分免疫表型和遗传学特点与其它脂肪肉瘤成分大致相同,提示漩涡状结构为去分化脂肪肉瘤的去分化成分,结合组织学特征、免疫表型和遗传学特点,可与其它具有漩涡状结构的肿瘤鉴别。     

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.     

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.     

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.     

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.     

Der Einfluß von Nahrungsmitteln und Rauchen auf die klinisch-chemische Diagnostik von Phäochromozytom,Neuroblastom und Karzinoid-Syndrom

Zusammenfassung Der Einfluß von verschiedenen Nahrungsmitteln auf Methoden zur Bestimmung von Adrenalin (AD), Noradrenalin (NA), Vanillinmandelsäure (VMS), Metanephrinen (MN), Homovanillinsäure (HVS) und 5-Hydroxyindolessigsäure (5-HIE) im 24 h-Harn zur Diagnose des Phäochromozytoms bzw. Karzinoid-Syndroms wurde untersucht. Die in die Untersuchung einbezogenen Nahrungsmittel waren: Tee, Kaffee, Mandeln, Ananas, Käse, Walnüsse, Vanillepudding, Bananen, Tomaten und Milchschokolade. Außerdem wurde der Einfluß des Zigarettenrauchens auf die Bestimmung von AD, NA, VMS und MN untersucht.Walnüsse führten zu einer starken Erhöhung der 5-HIE-Ausscheidung. Bananen erhöhten die Ausscheidung von AD, NA, VMS, MN und 5-HIE. Kaffee und Ananas bewirkten eine geringe Zunahme der MN-Werte. Rauchen von 20–30 Zigaretten/Tag beeinflußte keine der vier Variablen.Wenn die beschriebenen Methoden benutzt werden, sollte lediglich auf den Verzehr von Bananen und Walnüssen vor und während der Harnsammelperioden verzichtet werden, da die oberen Normgrenzen im Harn überschritten werden könnten. Ein Verzicht auf Kaffee und Ananas in normalen Mengen ist nicht erforderlich. Es besteht kein Anlaß, weiterhin die bisherigen umfangreichen Restriktionen der übrigen Nahrungsmittel beizubehalten.