膀胱假肉瘤性肌纤维母细胞增生临床病理分析及文献复习

目的 探讨膀胱假肉瘤性肌纤维母细胞增生的临床病理特征.方法 3例膀胱肌纤维母细胞增生光镜和免疫组化检查.结果 3例均为男性,年龄分别45、46、55岁,主要临床症状为无痛性血尿和排尿困难,无外伤史,随访3～6个月无复发.组织学检查:病变主要由松散排列的梭形细胞区,可见节细胞样细胞和致密条束状梭形细胞区,黏液样间质内弥漫分布急慢性炎细胞.分裂象3～5/10 HPF,无不典型核分裂.病变主要发生在膀胱壁浅层,病变累及固有肌,出血明显.免疫表型:3例梭形细胞Vim和SMA弥漫阳性,MSA2/3例+>70%、Des+50%、CK+>70%、EMA+30%、ALK-1蛋白+70%、Ki-67+5%～20%、CD68、S-100蛋白、CD34均-.结论 膀胱假肉瘤性肌纤维母细胞增生为一种良性非肿瘤性病变,复发率4.5%,要避免误诊为恶性.     

泌尿生殖道炎性假肉瘤

Roth于1980年首次描述了泌尿生殖道炎性假肉瘤(inflammatory pseudosarcoma ,IPS).该瘤少见,属于纤维母细胞/肌纤维母细胞性病变[1],与术后梭形细胞结节主要区别在于前者近期无手术病史[2].该病变曾命名为炎性假瘤、假肉瘤、结节性筋膜炎、假肉瘤样纤维母细胞增生、炎性肌纤维母细胞性假瘤、不典型肌纤维母细胞瘤、肌纤维母细胞瘤、炎性假肉瘤、肌纤维母细胞假瘤及硬化性炎性假瘤[3].国内文献报道较少,多以膀胱炎性假瘤命名[4～6].     

卵巢黏液性肿瘤伴肉瘤样附壁结节3例临床病理分析

目的 探讨卵巢黏液性囊性肿瘤伴肉瘤样附壁结节的临床表现、病理形态及免疫组化特点.方法 观察3例卵巢黏液性肿瘤伴肉瘤样附壁结节的临床资料、组织学形态、免疫组化特点,并对相关文献进行复习.结果 3例肉瘤样附壁结节主要由破骨样巨细胞、卵圆形的单核细胞以及多少不等的梭形细胞构成.可见核分裂象和病理性核分裂,部分区域细胞显示多形性,类似恶性纤维组织细胞瘤,但结节体积均较小且境界清楚,缺乏血管浸润.单核细胞和梭形细胞vimentin(+),desmin、CK(-);多核巨细胞CD68、vimentin(+),CK、desmin(-).结论 肉瘤样附壁结节是一种良性病变,卵巢黏液性肿瘤伴肉瘤样结节的生物学行为取决于黏液性肿瘤本身的性质.熟悉肉瘤样附壁结节的组织学特征和免疫表型可避免将其误诊为恶性肿瘤.     

膀胱癌肉瘤3例临床病理分析

目的探讨膀胱癌肉瘤的临床病理特征和组织发生。方法回顾性分析3例膀胱癌肉瘤患者的临床资料,进行病理组织学及免疫组化观察并进行文献复习。结果3例患者中男2例,女1例。发病年龄50～75岁,均以肉眼血尿入院,其中1例有尿痛。膀胱镜检查见膀胱顶部或侧壁可见菜花样或息肉状肿物,浸润性生长。2例行膀胱部分切除术,1例行经尿道膀胱肿瘤电切术。病理检查瘤组织均由明确的癌和肉瘤成分构成。分别构成于腺癌和梭形细胞肉瘤、移行上皮癌和横纹肌肉瘤、移行上皮癌和软骨肉瘤。结论膀胱癌肉瘤是一种少见的高度恶性的膀胱肿瘤,预后较差。诊断主要靠病理组织学检查及免疫组化染色。     

膀胱假肉瘤性肌纤维母细胞增生

炎性假瘤、假肉瘤样纤维黏液性肿瘤（PSFMT）和手术后梭形细胞结节（PSCN）是膀胱少见的病变。尽管这些病变病理机制不明,但其组织形态学比较相似。PSMFT和PSCN是否应该被认为是一种同一的疾病,以及它们是作为一种肿瘤性疾病还是反应性增生还存在争议。作者研究了42例膀胱梭形细胞病变,其中PSMFT33例,PSCN9例,分析了它们的病因病理发生、组织形态学、生物学行为以及与儿童的炎性肌纤维母细胞肿瘤的关系。     

泌尿生殖道炎假肉瘤

Ｒｏｔｈ于 1 980年首次描述了泌尿生殖道炎性假肉瘤 (ｉｎ ｆｌａｍｍａｔｏｒｙｐｓｅｕｄｏｓａｒｃｏｍａ ,ＩＰＳ)。该瘤少见 ,属于纤维母细胞 /肌纤维母细胞性病变〔1〕,与术后梭形细胞结节主要区别在于前者近期无手术病史〔2〕。该病变曾命名为炎性假瘤、假肉瘤、结节性筋膜炎、假肉瘤样纤维母细胞增生、炎性肌纤维母细胞性假瘤、不典型肌纤维母细胞瘤、肌纤维母细胞瘤、炎性假肉瘤、肌纤维母细胞假瘤及硬化性炎性假瘤〔3〕。国内文献报道较少 ,多以膀胱炎性假瘤命名〔4～ 6〕。就诊原因 :泌尿生殖道ＩＰＳ的患者就诊原因各不相同。…     

痛风结节伴发纤维肉瘤变1例并文献复习

目的探讨痛风结节伴发纤维肉瘤变的临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例痛风结节伴发纤维肉瘤变患者的临床表现、治疗、病理学特征并复习相关文献。结果患者男性,57岁,因左手中指深红色隆起包块,伴少量血性液体渗出,疼痛及周围皮肤轻度红肿入院。X线示左手中指近节指骨头体部呈虫蚀状骨破坏区,骨小梁结构不清,近中指间关节间隙稍狭窄,周围软组织肿胀,左侧腕关节诸骨呈多发小囊片状密度影,关节间隙模糊。镜下见瘤细胞呈实性、弥漫片状分布,浸润性生长,瘤细胞梭形,胞质红,核质比升高,核梭形,核染色质增多,核仁可见,部分区域核多形性。瘤细胞中可见痛风结节病灶。免疫表型:瘤细胞中vimentin和CD163阳性,SMA、SYT、S-100、CD34、desmin、MyoD 1、EMA、HMB-45及CKpan均阴性,Ki-67增殖指数约20%。结论痛风结节并发纤维肉瘤变较罕见。确诊主要根据其组织病理学和免疫表型特征,需与其他梭形细胞肿瘤鉴别。因该病罕见,故怀疑其恶变或其他病变时应及早进行病理学检查。     

腹腔内淋巴结外滤泡树突细胞肉瘤的临床病理学分析

目的探讨腹腔内结外滤泡树突细胞肉瘤的临床病理学特征、免疫表型及与EB病毒感染的相关性。方法分析4例腹腔内结外滤泡树突细胞肉瘤的临床和形态特点,采用CD21、CD23、CD35、波形蛋白、S-100蛋白、上皮细胞膜抗原（EMA）、CD68、CD34、CD117、HLA-DR、CD1a、结蛋白和d-平滑肌肌动蛋白等抗体进行免疫组织化学标记（EnVision法）,采用原位杂交检测EBER。结果患者发病年龄28-63岁（平均42岁）,男性3例,女性1例。临床上表现为腹部不适、腹痛和腹部肿块。影像学检查显示,1例患者同时含有2个病灶,分别位于胃黏膜下和肝左叶,另3例表现为腹腔内肿块,术后显示分别位于阑尾、空肠系膜和大网膜。2例在术前被诊断为胃肠道间质瘤。大体上,肿瘤呈结节状,平均直径为10．8cm,3例肿瘤内可见明显的坏死灶。镜下4例非肝脏性肿瘤均由胖梭形、卵圆形至上皮样细胞组成,可见散在的多核瘤细胞。瘤细胞主要呈席纹状、旋涡状或条束状排列,部分区域呈片状分布。肿瘤内可见散在的淋巴细胞,常围绕血管分布,并与瘤细胞形成双相细胞性形态。2例肿瘤显示明显的异型性,核分裂象易见（包括病理性）。3例肿瘤内可见凝固性坏死。1例肝脏肿瘤由大量的慢性炎性细胞和散在的异形大细胞组成,呈炎性假瘤样。免疫组织化学标记显示,瘤细胞均弥漫强阳性表达CD21、CD23和波形蛋白,部分表达CD35、S-100蛋白、CD68、HLA-DR和EMA,不表达CD34和CD117。2例EBER病毒检测均为阴性。结论发生于腹腔内的结外滤泡树突细胞肉瘤非常少见,熟悉其特征性的病理形态和免疫表型有助于与腹腔内其他类型梭形细胞肿瘤（特别是胃肠道间质瘤）的鉴别诊断。除经典型外,发生于肝脏者可呈炎性假瘤样形态,易被误诊。腹腔内非肝脏性滤泡树突细胞肉瘤与EB病毒感染的关系并不密切。     

肢端黏液炎性纤维母细胞性肉瘤

目的 探讨肢端黏液炎性纤维母细胞性肉瘤的临床病理学特征、免疫学表型及其鉴别诊断。方法 对1例发生于足背和右小腿远端的肢端黏液炎性纤维母细胞性肉瘤进行光镜观察和免疫组化标记。结果 患者因足背皮下“结节性筋膜炎”局部切除术后复发就诊。体检发现足背至右小腿远端前外侧皮下多发性结节,直径1～4cm,影像学检查提示肿瘤累及深部骨膜。镜下肿瘤由比例不等的黏液样区、透明变性区及炎症性区域混合组成。黏液样区域主要由交织条柬状排列的梭形瘤细胞组成,核显示轻至中度异型性,核分裂象罕见,间质疏松、黏液样,局部区域可见黏液湖形成。其内可见单空泡印戒样或多空泡状假脂肪母细胞,形态类似黏液纤维肉瘤。透明样区域由散在的胖梭形至卵圆形的瘤细胞和透明样间质混和组成。炎症性区域由成簇的淋巴细胞组成,与黏液样区域和透明变性区相混杂。病变内可见体积较大类似节细胞或R-S细胞的畸形细胞。免疫组化标记显示瘤细胞弥漫表达Vim,个别细胞表达p53,而CD68、actin、Des、CD34、CD30和S-100蛋白等标记均为阴性,多数淋巴细胞表达CD45RO。结论 肢端黏液炎性纤维母细胞性肉瘤是一种罕见的低度恶性软组织肉瘤,瘤细胞由变异的纤维母细胞衍化而来,熟悉其形态学特征对避免误诊为其它良性或恶性病变具有重要意义。该瘤常在局部呈侵袭性生长,具有较高的复发率,临床上应予以完整切除。     

假肌源性血管内皮瘤3例临床病理观察并文献复习

目的探讨假肌源性血管内皮瘤(pseudomyogenic hemangioendothelioma,PHE)的临床病理特征、诊断及鉴别诊断。方法回顾性分析3例PHE的临床病理特征、免疫表型、诊断及鉴别诊断、预后等,并复习相关文献。结果 3例患者,年龄38～70岁,中位年龄56岁,肿块平均直径4 cm,边界不清,浸润性生长。镜下见肿瘤细胞结节状生长,瘤细胞上皮样,卵圆形或梭形,大而肥胖,胞质嗜酸性,核仁小,核分裂罕见。免疫表型:肿瘤细胞表达CD31、Fli-1、CK(AE1/AE3),不表达S-100、desmin、SMA。结论 PHE是一种少见的软组织肿瘤,需与上皮样肉瘤、上皮样血管内皮瘤、上皮样血管肉瘤等肿瘤鉴别。     

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.     

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.     

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.     

What will studies of Fulani individuals naturally exposed to malaria teach us about protective immunity to malaria?

There are an estimated over 200 million yearly cases of malaria worldwide. Despite concerted international effort to combat the disease, it still causes approximately half a million deaths every year, the majority of which are young children with Plasmodium falciparum infection in sub-Saharan Africa. Successes are largely attributed to malaria prevention strategies, such as insecticide-treated mosquito nets and indoor spraying, as well as improved access to existing treatments. One important hurdle to new approaches for the treatment and prevention of malaria is our limited understanding of the biology of Plasmodium infection and its complex interaction with the immune system of its human host. Therefore, the elimination of malaria in Africa not only relies on existing tools to reduce malaria burden, but also requires fundamental research to develop innovative approaches. Here, we summarize our discoveries from investigations of ethnic groups of West Africa who have different susceptibility to malaria.     

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.     

'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.     

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.