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].     

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。…     

结节性筋膜炎的病理特点和亚型

结节性筋膜炎由Ｋｎｏｗａｌｅｒ等〔１〕于１９５５年首次提出并命名，又称为假肉瘤性筋膜炎。本病既非炎症性疾病，亦非真性肿瘤，而是一种以纤维母细胞和肌纤维母细胞增生为主的软组织瘤样病变。由于其核分裂象多见，富于细胞，增生的纤维母细胞生长活跃，“上窜下跳”...     

尿路膀胱软组织肿瘤（Ⅱ）：恶性肿瘤

大多数原发膀胱癌为移形细胞（尿路上皮）癌，鳞状细胞癌、原发腺癌或小细胞癌均较少碰到，其它膀胱癌均非常少见。在上一期杂志中，作者提出了“尿路膀胱的软组织肿瘤第1部分：肌纤维母细胞增生、良性肿瘤和具有不确定恶性潜能的肿瘤”，此两部分综述的第Ⅱ部分描述了不同类型的膀胱恶性间质病变，包括：平滑肌肉瘤、横纹肌肉瘤、血管肉瘤、恶性纤维组织细胞瘤（未分化肉瘤）、原始神经外胚叶肿瘤、恶性外周神经鞘瘤、血管外皮细胞瘤和腺泡状软组织肉瘤，并描述了这些病变的临床表现、形态学特点和免疫组化特征。     

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。     

肢端黏液样炎性纤维母细胞肉瘤2例及文献复习

目的探讨肢端黏液样炎性纤维母细胞肉瘤的临床病理学特征及鉴别诊断。方法对2例发生在下肢末端的黏液样炎性纤维母细胞肉瘤进行光镜观察和免疫组化标记，并复习文献。结果2例发生在下肢末端的病程较长的渐进性肿块，术后局部复发。镜检：病变呈多结节状，边界不清；黏液样基质中见数量不等的各类炎细胞浸润，散在或灶性分布梭形、奇异形和多空泡状脂肪母细胞样3种形态的瘤细胞。免疫表型：肿瘤细胞Vim弥漫阳性，CD68和CD34灶性阳性，CK、SMA、HHF-35、S-100蛋白、CD45、CD45R0、CD15、CD30均阴性。结论此病病程较长，术后易局部复发，是一种低度恶性的肿瘤。鉴于病变黏液样基质及各类炎细胞浸润的背景较为突出，而特征性的瘤细胞稀疏，应注意与炎症性病变或具有相似组织形态的良性或恶性肿瘤鉴别。     

乳腺纤维母细胞/肌纤维母细胞性肿瘤临床病理分析

目的 探讨乳腺纤维母细胞/肌纤维母细胞性肿瘤(breast fibroblastic/ myofibroblastic tumors BF/MFT)的临床病理、免疫表型特点及相关鉴别诊断.方法 应用HE染色、免疫组化标记对7例BF/MFT进行形态学观察并进行文献复习.结果 7例患者均为女性,2例为孤立性纤维性肿瘤;2例为肌纤维母细胞瘤;1例为纤维瘤病;1例为炎性肌纤维母细胞瘤;1例为低度恶性肌纤维母细胞肉瘤.结论 BF/MFT非常少见,不同的类型肿瘤生物学行为不同,它们的鉴别诊断主要是组织学及免疫组化.     

炎症性肌纤维母细胞瘤及低度恶性肌纤维母细胞肉瘤

肌纤维母细胞是一种同时具有纤维母细胞及平滑肌细胞超微结构特点的高度分化细胞,常见于损伤修复和肿瘤的间质反应等多种病理状态。以往对于肌纤维母细胞能否构成真性肿瘤一直存有争议。直到近年炎症性肌纤维母细胞瘤（inflammatorymy ofibroblastic tumor,IMT）被确定是一种具有复发潜能的真性肿瘤,细胞遗传学的研究结果亦支持这一观点。WHO新分类已把IMT及低度恶性肌纤维母细胞肉瘤（low grade myofibrobla sticoma,LGMS）归人中间性（偶有转移性）的纤维母细胞／肌纤维母细胞肿瘤中。[第一段]     

牙龈低度恶性肌纤维母细胞肉瘤

目的:探讨牙龈低度恶性肌纤维母细胞肉瘤的临床病理特征.方法:对2例发生于14岁和16岁病人的低度恶性肌纤维母细胞肉瘤的临床病理特点进行研究,其中包括肿瘤的生长方式、细胞密度、核异型性和核分裂象.对石蜡包埋组织进行免疫组织化学检测和电镜观察.结果:大体观察,肿瘤大小为2.5 ～3.0 cm,呈结节状生长,边界不清,切面实...     

结节性筋膜炎50例临床病理特征

目的 探讨结节性筋膜炎(nodular fasciitis,NF)的临床病理特征及鉴别诊断.方法 重新复查原诊断NF的病例50例,应用光镜和免疫组化技术进行研究,同时复习临床资料并文献复习.结果 50例原诊断NF的病例中,43例仍维持原诊断,7例为其他软组织肿瘤.43例NF中,男26例,女17例,年龄18～56岁,平均32.5岁.病变主要侵犯全身皮下组织,上肢最多见(20例46.5%),其次为躯干(9例20.9%)、头颈部(8例18.6%),下肢少见(6例13.9%).临床表现为病程短、生长迅速的孤立性结节,体积小,术后切除无复发、无转移.病理组织学主要表现为增生肥胖的梭形纤维母细胞/肌纤维母细胞呈束状、C形或不典型车辐状排列,细胞无多形性,核分裂象易见;间质疏松,黏液样,含有丰富的血管及红细胞外渗,以及不规则裂隙、微囊等.另7例分别为黏液纤维肉瘤、黏液型脂肪肉瘤、低度恶性纤维黏液样肉瘤、富于细胞纤维组织细胞瘤、栅栏状肌纤维母细胞瘤、真皮纤维瘤、韧带样纤维瘤,误诊率为14%.免疫表型:11例vimentin、SMA、MSA阳性,而CK、desmin、CD34、β-catenin阴性.结论 NF是一种以纤维母细胞/肌纤维母细胞增生性、良性反应性软组织假肉瘤性病变,病理诊断应紧密结合临床和组织学形态的特征,注意防止过度诊断或诊断不足.     

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.     

'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.