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。…     

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。     

卵巢黏液性肿瘤中附壁结节3例临床病理分析

目的观察卵巢黏液性肿瘤中附壁结节的临床表现、组织病理学和免疫表型特征，强调诊断标准并探讨病因机制。方法分析3例卵巢黏液性肿瘤中附壁结节的临床资料、组织学形态及其免疫学表型，并复习相关文献。结果3例卵巢黏液性肿瘤中附壁结节的病例1为良性的肉瘤样附壁结节，以两种细胞成分为主：其一为纤维组织细胞样单核细胞，呈卵圆形或梭形，部分细胞形态较一致、温和，部分细胞出现高度的核异型性，核分裂多见并可见病理性核分裂象；另一种细胞为散在分布的破骨样巨细胞，常围绕于小的出血腔隙周围。病变中见出血坏死区，并伴明显的炎症反应背景；病例2为恶性的肉瘤性附壁结节，其组织学形态与肉瘤样附壁结节极其相似，但体积大，并出现血管浸润。该2例中的单核细胞表达vimentin，灶性表达AE1／AE3，多核巨细胞表达CD68为主。病例3为恶性的化生性癌性附壁结节，其中的瘤细胞为化生性或低分化癌成分，以表达上皮性标记如AE1／AE3和CK7为主，同时也表达vimentin。结论卵巢黏液性肿瘤中附壁结节为罕见的伴发病变或肿瘤，具有广泛的病理形态图像谱，不同类型的卵巢黏液性肿瘤中附壁结节有完全不同的预后及治疗原则，正确的诊断具有重要的临床意义。     

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。     

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。     

低度恶性肌纤维母细胞性肉瘤

目的 探讨低度恶性肌纤维母细胞性肉瘤的临床病理学特征、免疫学表型和超做结构特点。方法 对1例发生于左上颌窦的低度恶性肌纤维母细胞性肉瘤进行临床资料复习、光镜观察、免疫组化标记和电镜检测。结果 患者因“左上颌窦囊肿”2次行切除活检，病理诊断分别为“纤维组织增生”和“鳞状细胞癌Ⅰ级”。4个月后因左上颌骨隐痛行左上颌骨部分切除术，术后病理检查未发现肿瘤组织。5个月后左上后磨牙区肿块复发，再行左上颌、颊、颈联合根治术，诊断为“侵袭性纤维瘤病”。9个月后左侧颧部出现包块，术后病理为“左上颌纤维肉瘤，部分伴平滑肌分化”复片显示，第1次活检标本中的“纤维组织增生”实际上是梭形细胞肿瘤组织，而第2次活检中的“鳞状细胞癌1级”实为鳞状上皮假上皮瘤样增生。第3次术后标本中肿瘤组织不明显，而第4次术后标本则由成束的梭形细胞组成，弥漫浸润至邻近的软组织内，类似侵袭性纤维瘤病，但部分区域内可见鱼骨样排列结构，类似低度恶性纤维肉瘤。第5次术后标本中瘤细胞显示轻～中度的异型性，可见核分裂象(2个／10HPF)，并弥漫浸润横纹肌组织，在部分区域内，瘤细胞穿插在肌束之间形成类似增生性肌炎中的棋盘样结构，另一些区域则在形态上类似经典的纤维肉瘤。免疫表型：瘤细胞表达Vim、α—SMA和Ki—67，不表达MSA、Des、h—caldesmon和S—100蛋白。电镜下瘤细胞胞质内可见到平行肌丝。结论 低度恶性肌纤维母细胞性肉瘤是一种少见的软组织肉瘤，组织学形态、免疫表型及超微结构均显示瘤细胞具肌纤维母细胞性分化。     

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.     

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.     

The universal muscular chain reaction,muscle spasm,Torsions, ruptures and extravasations. Chameleons of pathology and some manifestations of simple muscular disorders

Most bodily functions require the coordinated actions of complementary and supplementary paired muscle groups. Where this essential muscular cooperation is lacking, hollow organs may burst and others become literally screwed up, giving rise to many similar spastic diseases such as Torticollis, Twisted ovarian cyst, Torsion of the Testis, Volvulus of the intestines, Varicose Veins, Megacolon, Aortamegaly, Scoliosis, Erb's Palsy, Peyronie's Disease, Main-en-Griffe, Undescended Foot (Pes Cavus), Talipes, Strabismus. Spasm is “panenepidemic” and unclassified examples of Torsion Dystonia and Dyskinesia really are as common as debt and taxes.     

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.     

Hypo- und Hypermagnesämie aus kardiologischer Sicht

Zusammenfassung Eine Reihe pathologischer Zustände bedingen Magnesiummangel. Zustände mit Hypermagnesämie sind ebenfalls bekannt, doch wesentlich seltener. Für den Kardiologen beachtenswert ist, daß unter Therapie mit bestimmten Diuretica bei Herzinsuffizienz, bei Herzinfarkt, Kardiomyopathie, Digitalisintoxikation und bestimmten Herzrhythmusstörungen Hypomagnesämie beobachtet wurde. Leider kann in der klinischen Routine nur ein extracelluläres Magnesiumdefizit durch Serumbestimmungen gemessen werden; über Magnesiummangel einzelner Organe kann nichts ausgesagt werden. Hinweise für Magnesiummangel geben aber neben der Messung des Serumspiegels Anamnese, klinischer Befund, bestimmte EKG-Veränderungen wie auch evtl. Hypokalämie, ein Zustand, bei dem sich oft — besonders bei Aldosteronismus — parallele Veränderungen zeigten.Tierexperimente deuten darauf hin, daß infarktähnliche Läsionen unter Magnesiummangel entstehen, doch ob Herzinfarkt beim Menschen durch Magnesiummangel ausgelöst werden kann, ist noch ungeklärt. In Leichenherzen zeigte sich im Infarktgebiet neben Calciumakkumulation signifikanter Magnesiumverlust, wobei unklar blieb, ob sich Ursache oder Folge des Infarktes widerspiegelten. Falls ein ursächlicher Zusammenhang besteht, ist er im Myokardstoffwechsel selbst zu suchen, wie bei der Alkoholkardiomyopathie, wo myokardialer Magnesiummangel zumindest als pathogenetischer Teilfaktor anerkannt wird. Andererseits versucht man aber auch Beziehungen zwischen Atherosklerose, Blutgerinnung und Hypomagnesämie herzustellen, in der Meinung, daß Magnesiummangel auch über den coronaren Pathomechanismus des Herzinfarktes wirken könnte. Sicher scheint, daß gewisse EKG-Veränderungen und Herzrhythmusstörungen durch einen irritierten Magnesiumhaushalt bedingt sein können, da sie bei Gabe bzw. Entzug von Magnesium verschwinden. Daß Magnesiummangel die Glykosidtoleranz verringert, wird tierexperimentell bestätigt. Unter Hypomagnesämie bewirkt Acetylstrophanthidin eher und länger Rhythmusstörungen als ohne, außerdem lassen diese sich durch Magnesiumgaben eliminieren. Da in gewissen Fällen spontane und digitalisinduzierte Herzrythmusstörungen durch Magnesiuminjektionen beseitigt wurden, scheint Magnesium als Therapeuticum angebracht. Einsatz verschiedener Magnesiumsalze bei Angina pectoris, degenerativen Herzerkrankungen und Herzinsuffizienz ohne geprüften und offensichtlich gestörten Magnesiumhaushalt ist fragwürdig, weil keine eindeutigen klinischen Erfolgsbeweise vorliegen. Immerhin mag es aber larvierte, durch Serumbestimmungen nicht erfaßbare Mangelzustände geben. Allgemein erscheint es aus kardiologischer Sicht ratsam, den Magnesiumhaushalt zu überwachen und in entsprechenden Fällen auszugleichen, um möglichen Myokardläsionen oder fatalen Herzrhythmusstörungen entgegenzuwirken.     

Environmental risk factors and their role in the management of atopic dermatitis

Introduction: The etiology of atopic dermatitis (AD) is multifactorial with interaction between genetics, immune and environmental factors.

Areas covered: We review the role of prenatal exposures, irritants and pruritogens, pathogens, climate factors, including temperature, humidity, ultraviolet radiation, outdoor and indoor air pollutants, tobacco smoke exposure, water hardness, urban vs. rural living, diet, breastfeeding, probiotics and prebiotics on AD.

Expert commentary: The increased global prevalence of AD cannot be attributed to genetics alone, suggesting that evolving environmental exposures may trigger and/or flare disease in predisposed individuals. There is a complex interplay between different environmental factors, including individual use of personal care products and exposure to climate, pollution, food and other exogenous factors. Understanding these complex risk factors is crucial to developing targeted interventions to prevent the disease in millions. Moreover, patients require counseling on optimal regimens for minimization of exposure to irritants and pruritogens and other harmful exposures.     

'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.