难道不值得高兴吗？

有人觉得，快乐与丰厚的物质有关，即财富越多，快乐越多，财富越少，快乐越少；也有人认为，快乐与所处的环境有关，即环境越好，快乐越多，环境越差，快乐越少。而事实上，快乐与物质无关，与环境无关，只与我们的心灵相关。     

神经症患者家庭教养与MMPI测定结果探析

本文对５０名焦虑症患者进行ＥＭＢＵ，ＭＭＰＩ测定，结果表明患者在Ｐｔ，Ｐａ，Ｓｃ量表上显著增高，父亲因子２与Ｐｔ，因子４与Ｈｙ，因子５与Ｐｔ，Ｐａ，母亲因子３与Ｐｔ等之间的相关，达到显著性水平。提示伙虑患者的人格特征与父母的教养方式具有一定的关系，否认，惩罚等教育方式不利于孩子心理健康。     

人类与医学遗传学群体与家系资料分析计算机系统的功能与菜单结…

本文介绍我们用Ｃ语言编制的人类与医学遗传学群体与家系资料分析计算机系统的菜单结构与各子系统的功能，该系统可用于基因频率与群体分化分析，相关与遗传度分析，疾病关联分析，遗传质性分析，ＡＧＦＡＰ遗传方式分析，尚可用于通径分析，数量性状的混合分布分析；分离分析与连锁分析等。对人类与医学遗传学及遗传流行病学研究都很有实用价值。     

《解剖与临床》2011年征稿征订启事

《解剖与临床》杂志（CN11—5357／R，ISSN1671—7163）由中华人民共和国卫生部主管，中国医师协会、蚌埠医学院主办，蚌埠医学院第一附属医院承办。它是临床医学专家与解剖学专家共同创办的跨学科学术期刊，是解剖与临床之间相互交流的学术园地。它以解决临床问题为目的，以广大临床与解剖工作者为服务对象，以临床与解剖相结合为特色，以提高与普及并重为办刊方针，面向临床，服务于临床。     

电刺激与神经兴奋性有关因素的探讨

电刺激与神经兴奋性有关因素的探讨张歆斌，孙雅娟，杜莉熟练与灵活地运用电刺激参数，了解患者的生理与病理因素，对于提高工作质量与效率，减轻患者痛苦是重要的。现就电刺激与神经兴奋性的有关影响因素，结合我们的工作实践，谈谈体会，以供同道参考。１电流参数１．１...     

《解剖与临床》2011年征稿征订启事

《解剖与临床》杂志（CN11—5357／R，ISSN1671—7163）由中华人民共和国卫生部主管，中国医师协会、蚌埠医学院主办，蚌埠医学院第一附属医院承办。它是临床医学专家与解剖学专家共同创办的跨学科学术期刊，是解剖与临床之间相互交流的学术园地。它以解决临床问题为目的，以广大临床与解剖工作者为服务对象，以临床与解剖相结合为特色，以提高与普及并重为办刊方针，面向临床，服务于临床。     

老年前期与老年期WMS测验结果分析

采用修订的韦氏记忆量表（ＷＭＳ）测试１０３名老年前期与老年期人群组，量表分与记忆商（ＭＱ）统计结果，各职业性组间比较，顺数，倒数，记图，联想，理解５项与ＭＱ科技组明显高于行政组，其中记图，联想与ＭＱ亦高于工人组，差异显著，文化组间比较，顺数，倒数累加，联想，触摸５项小学组明显低于其它文化组，理解与ＭＱ大学，高中高于小学，初中文化组，差异显著，信度较大再生，各文化组间均有显著差异，而较容易的再认记忆     

《解剖与临床》2011年征稿征订启事

《解剖与临床》杂志（CN11—5357／R，ISSN1671—7163）由中华人民共和国卫生部主管，中国医师协会、蚌埠医学院主办，蚌埠医学院第一附属医院承办。它是临床医学专家与解剖学专家共同创办的跨学科学术期刊，是解剖与临床之间相互交流的学术园地。它以解决临床问题为目的，以广大临床与解剖工作者为服务对象，以临床与解剖相结合为特色，以提高与普及并重为办刊方针，面向临床，服务于临床。     

《解剖与临床》杂志稿约

《解剖与临床》杂志是由解剖学专家与临床医学专家共同创办的跨学科学术期刊，是解剖与临床两大学科的共同学术园地。她以广大解剖与临床工作者为主要读者对象，以促进解剖与临床两大学科之间的信息传播和学术交流为宗旨，坚持基础与临床、理论与实践、普及与提高相结合的办刊方针，面向临床，服务于临床。     

《解剖与临床》2011年征稿征订启事

《解剖与临床》杂志（CN11—5357／R，ISSN1671—7163）由中华人民共和国卫生部主管，中国医师协会、蚌埠医学院主办，蚌埠医学院第一附属医院承办。它是临床医学专家与解剖学专家共同创办的跨学科学术期刊，是解剖与临床之间相互交流的学术园地。它以解决临床问题为目的，以广大临床与解剖工作者为服务对象，以临床与解剖相结合为特色，以提高与普及并重为办刊方针，面向临床，服务于临床。     

Occurrence of tubuloreticular structures and intracisternal paracrystalline inclusions in endothelial cells of tissue from different epidemiological types of Kaposi's sarcoma

Biopsied tissue specimens from 40 cases of classic, atypical classic, endemic, and AIDS-associated Kaposi's sarcoma (KS) were investigated by electron microscopy. To search for ultrastructural differences between non-AIDS-associated KS and AIDS-associated KS, the occurrence of the following 2 ultrastructural abnormalities of the rough-surfaced endoplasmic reticulum in KS cells was evaluated semi-quantitatively: tubuloreticular structures (TRS) and intracisternal paracrystalline inclusions (IPI). These peculiar structures were found in 23 of the 40 KS cases. Two types of TRS could be distinguished: loose TRS (LTRS) and compact ones (CTRS). LTRS were observed in endothelial cells of tissue from all the different epidemiological types of KS. CTRS were confined to AIDS-associated KS. IPI were present in endothelial tumor cells of only 3 non-AIDS-associated KS cases. The study shows that in cells of KS tissue only CTRS, but not LTRS, are an ultrastructural marker for AIDS-associated KS.     

Unidentified Bacterial Microorganisms Entrapped Within Blood Capillary Spaces of Tissue From Different Epidemiological Types of Kaposi's Sarcoma

Tissue specimens of different epidemiological types of Kaposís sarcoma (KS) from various geographical regions were investigated by transmission electron microscopy. Freshly fixed KS biopsies originated from 9 German patients: 3 classic KS cases, 5 AIDS-associated KS cases, and 1 atypical classic KS case. Additionally, KS autopsy material from the brain of a German AIDS patient was examined. Further biopsies came from 29 Ugandan patients: 16 endemic KS cases and 13 AIDS-associated KS cases. While investigating the ultrastructure, we discovered relatively small-sized bacterial microorganisms within blood capillary spaces of tumor tissue from 5 KS cases of different epidemiological type. The microorganisms often occurred in clusters. They were of coccoid-bacillary form and limited by a wrinkled multilayered cell wall. Many of them were encapsulated. They were not observed outside of the capillary lumen. The bacterial structures were often seen attached to capillary endothelial cells, which sometimes showed blistering into the capillary lumen. The observed bacterial microorganisms obviously represented agents of a bloodstream infection and must have been entrapped and accumulated within capillary spaces of KS tissue. The bacteria, which had an almost identical morphology in all 5 KS cases, could not be identified. If they are of pathogenic significance, it remains unknown.     

The inflammatory component of Kaposi sarcoma

Kaposi sarcoma (KS) is intimately linked to several aspects of the host immune system. KS development is linked to immunodeficiency in several clinical-epidemiological settings. The development of KS at local inflammatory sites has also been documented. Inflammatory cells are almost always present within KS lesions. Depending upon the inflammatory milieu, KS lesions may progress or regress. Not surprisingly, iatrogenic manipulation of host immunity with drugs may provoke KS growth and/or flare. Given the close association between KS and the immune system, the etiologic agent Kaposi Sarcoma Herpesvirus has developed a variety of mechanisms to evade the host immune system, all of which have cleverly evolved to promote oncogenesis and viral persistence.     

Human herpesvirus-8: detection of novel herpesvirus-like DNA sequences in Kaposi's sarcoma and other lesions

Kaposi's sarcoma (KS) is a malignancy suspected of having an infectious etiology. Unique viral DNA sequences were recognized in KS lesions, using a novel technique that identifies small differences between two complex genomes. The virus had homology with the herpesvirus family, especially Epstein Barr virus (EBV), yet it was distinct from the known herpesviridae, and was appropriately named human herpesvirus 8 (HHV-8) or Kaposi's sarcoma-associated herpesvirus (KSHV). HHV-8 DNA sequences were present in AIDS-associated KS, classic KS, African endemic KS, Mediterranean KS, iatrogenic KS, and KS in homosexual men without HIV infection. HHV-8 DNA sequences were also present in peripheral blood mononuclear cells (PBMC) of KS+ patients; body-cavity-based lymphomas in HIV positive patients without KS; and in tissue from a number of malignant and non-malignant lesions in patients without HIV infection. The role of HHV-8 in KS and other malignancies is not known. Viruses are notoriously trophic for lesional tissue. Therefore, in order to determine the role of HHV-8 in KS pathogenesis, HHV-8 needs to be isolated and shown to induce immortalization in a suitable system. Regardless of its role in KS, another human herpesvirus has been discovered, and the extent of its pathogenicity needs to be uncovered.Abbreviations KS Kaposi's sarcoma - HHV-8 human herpesvirus-8 - KSHV Kaposi's sarcoma-associated herpesvirus - EBV Epstein-Barr virus RDA representational difference analysis     

Unidentified bacterial microorganisms entrapped within blood capillary spaces of tissue from different epidemiological types of Kaposi's sarcoma.

Tissue specimens of different epidemiological types of Kaposis sarcoma (KS) from various geographical regions were investigated by transmission electron microscopy. Freshly fixed KS biopsies originated from 9 German patients: 3 classic KS cases, 5 AIDS-associated KS cases, and 1 atypical classic KS case. Additionally, KS autopsy material from the brain of a German AIDS patient was examined. Further biopsies came from 29 Ugandan patients: 16 endemic KS cases and 13 AIDS-associated KS cases. While investigating the ultrastructure, we discovered relatively small-sized bacterial microorganisms within blood capillary spaces of tumor tissue from 5 KS cases of different epidemiological type. The microorganisms often occurred in clusters. They were of coccoid-bacillary form and limited by a wrinkled multilayered cell wall. Many of them were encapsulated. They were not observed outside of the capillary lumen. The bacterial structures were often seen attached to capillary endothelial cells, which sometimes showed blistering into the capillary lumen. The observed bacterial microorganisms obviously represented agents of a bloodstream infection and must have been entrapped and accumulated within capillary spaces of KS tissue. The bacteria, which had an almost identical morphology in all 5 KS cases, could not be identified. If they are of pathogenic significance, it remains unknown.     

Congenital hyperinsulinism as the presenting feature of Kabuki syndrome: clinical and molecular characterization of 10 affected individuals

PurposeDescribe the clinical and molecular findings of patients with Kabuki syndrome (KS) who present with hypoglycemia due to congenital hyperinsulinism (HI), and assess the incidence of KS in patients with HI.MethodsWe documented the clinical features and molecular diagnoses of 10 infants with persistent HI and KS via a combination of sequencing and copy-number profiling methodologies. Subsequently, we retrospectively evaluated 100 infants with HI lacking a genetic diagnosis, for causative variants in KS genes.ResultsMolecular diagnoses of KS were established by identification of pathogenic variants in KMT2D (n = 5) and KDM6A (n = 5). Among the 100 infants with HI of unknown genetic etiology, a KS diagnosis was uncovered in one patient.ConclusionsThe incidence of HI among patients with KS may be higher than previously reported, and KS may account for as much as 1% of patients diagnosed with HI. As the recognition of dysmorphic features associated with KS is challenging in the neonatal period, we propose KS should be considered in the differential diagnosis of HI. Since HI in patients with KS is well managed medically, a timely recognition of hyperinsulinemic episodes will improve outcomes, and prevent aggravation of the preexisting mild to moderate intellectual disability in KS.     

Insights into the genotype–phenotype relationship of ocular manifestations in Kabuki syndrome

We aim to assess if genotype–phenotype correlations are present within ocular manifestations of Kabuki syndrome (KS) among a large multicenter cohort. We conducted a retrospective, medical record review including clinical history and comprehensive ophthalmological examinations of a total of 47 individuals with molecularly confirmed KS and ocular manifestations at Boston Children's Hospital and Cincinnati Children's Hospital Medical Center. We assessed information regarding ocular structural, functional, and adnexal elements as well as pertinent associated phenotypic features associated with KS. For both type 1 KS (KS1) and type 2 KS (KS2), we observed more severe eye pathology in nonsense variants towards the C-terminus of each gene, KMT2D and KDM6A, respectively. Furthermore, frameshift variants appeared to be not associated with structural ocular elements. Between both types of KS, ocular structural elements were more frequently identified in KS1 compared with KS2, which only involved the optic disc in our cohort. These results reinforce the need for a comprehensive ophthalmologic exam upon diagnosis of KS and regular follow-up exams. The specific genotype may allow risk stratification of the severity of the ophthalmologic manifestation. However, additional studies involving larger cohorts are needed to replicate our observations and conduct powered analyses to more formally risk-stratify based on genotype, highlighting the importance of multicenter collaborations in rare disease research.     

Immunihistochemical detection of Bcl-2 in AIDS-associated and classical Kaposi's sarcoma.

Kaposi''s Sarcoma (KS) is an angioproliferative disease that is characterized by proliferation of spindle-shaped cells predominantly of vascular endothelial cell origin, neoangiogenesis, inflammatory cell infiltration, and edema. Although the lesions of classical KS and AIDS-associated KS (AIDS-KS) share common histological features, AIDS-KS occurs at a markedly higher frequency with a more aggressive clinical course. Immunohistochemical analyses of 26 evolutionarily staged AIDS-KS lesions derived from HIV-infected patients demonstrate significant cytoplasmic levels of Bcl-2, a protooncogene known to prolong cellular viability and to antagonize apoptosis. Bcl-2 expression increases as the pathological stage of KS advances. Immunohistochemical analyses of classical KS lesions demonstrate prevalent expression of Bcl-2 as well, indicating that upregulation of Bcl-2 may be important in the pathogenesis of both classical and AIDS-associated KS. Coexpression of Bcl-2 and factor VIII-related antigen in spindle-shaped cells present within KS lesions suggests that Bcl-2 is upregulated within the vascular endothelial spindle-shaped cells of KS. The consequences of upregulated Bcl-2 expression within KS lesions may be prolonged spindle cell viability which, when coupled with dysregulated cellular proliferation due in part to synergistic activities of inflammatory and angiogenic cytokines and HIV-1 Tat protein, may result in the maintenance, growth, and progression of KS.     

Kallistatin抗炎作用的研究进展

Kallistatin(KS)是一种激肽释放酶结合蛋白(kallikrein-binding protein)，广泛分布于多种组织及体液中。KS不但具有抑制组织型激肽释放酶的作用，还具有抗炎、抗血管生成、抗肿瘤等多种生物学作用。KS生物学功能与其分子结构中不同的结构域有关。而KS抗炎作用被认为主要与肝素结合结构域有关。KS通过其肝素结合结构域，能竞争性抑制肿瘤坏死因子(tumor necrosis factor α，TNF-α)与它的受体的结合，从而起到影响TNF-α-NF-κB信号通路，发挥抗炎作用。KS也能与Kruppel样因子4(Kruppel-like Factor 4，KLF4)结合，激活内皮型一氧化氮合酶(endothelial nitric oxide synthase，eNOS)，抑制TNF-α活化核因子-κB(nuclear factor-κB，NF-κB)，发挥抗炎作用。但这与肝素结合结构域的机制不同。不同的机制可能为抗炎治疗提供新的思路，本文将就Kallistatin抗炎作用的相关研究进展进行阐述。