Current Development Status and Consideration for Rare Hemorrhagic Disease Drugs / 罕见病研究

Hemophilia is the only rare hereditary hemorrhagic disorder included in the First Rare Diseases catalogue. However, rare bleeding diseases identified in the clinic are far more common than hemophilia. Most other rare hemorrhagic disorders have less effective treatment than hemophilia. Hemophilia has a history of successful drug development in rare hemorrhagic diseases, and the cycle between clinical research and drug development has been gradually realized. Drug research and pharmaceutical companies can refer to the drug research and development process in the field of hemophilia, learn from the experience of hemophilia drug research and develop treatments. The industry can increase drug development by strengthening basic research, focusing on the value of natural history research, the application of quantitative pharmacological tools and improving the efficiency of drug development to meet the urgent unmet medical needs of patients with rare hemorrhagic diseases.

Thromboelastography:clinical applications and new development / 国际检验医学杂志

Under normal physiological conditions, hemostatic process is a dynamic balance process dependent on many factors.It involves interaction between primary hemostasis (vasoconstriction, platelet clot formation), secondary hemostasis (thrombin generation, fibrin polymerization) and fibrinolysis, and any abnormalities of the three may cause hemorrhagic or thrombotic diseases.Conventional coagulation tests are useful for a limited degree since they only reflect a portion of the coagulation cascade.Thromboelastography (TEG) provides a more complete picture of coagulation status, taking into account more factors involved in the clotting process, including platelet activity and clotting factors, which has been widely used in clinic.In this article, we will briefly discuss clinical applications and new development of TEG.

Detection of bluetongue virus in Brazilian cervids in São Paulo state / Detecção de vírus da língua azul em cervídeos brasileiros no estado de São Paulo

Viral hemorrhagic diseases in cervids occur worldwide and include epizootic hemorrhagic disease (EHD), bluetongue (BT), and adenoviral hemorrhagic disease (AHD). Since gross lesions in all three hemorrhagic diseases are identical (hemorrhagic enteropathy, pulmonary edema, systemic petechial and suffusion hemorrhages), it is necessary to use accurate techniques for a definitive etiologic diagnosis. Archival material (paraffin blocks) at the Department of Veterinary Pathology of FCAV - Unesp was reviewed for lesions of hemorrhagic disease and 42 captive and free-living Brazilian deer were selected to include in this study. Paraffin-embedded tissues were evaluated using immunohistochemistry and tested negative for adenovirus. Using real time RT-PCR, EHD virus was not detected in paraffin-embedded tissues in any of the cases evaluated. The same technique was used for detection of BT virus and seven positive animals (16,66%) were confirmed after agarose 4% gel electrophoresis and gene sequencing. The main macroscopic changes observed in the positive animals were hemorrhagic intestinal contents, reddish mucous membrane of the gastrointestinal tract, ulcers on tongue and petechiae in various organs. Microscopic changes observed were lymphocytic inflammatory infiltrate in liver, kidney and lungs, hemorrhage, and congestion in various organs. All positive cases were from captive animals, three females (two young and one adult), and four young males. This study demonstrates that the bluetongue virus is involved in hemorrhagic disease outbreaks of deer in Brazil.(AU)

Doenças hemorrágicas virais em cervídeos ocorrem no mundo todo e incluem a doença epizoótica hemorrágica (DEH), língua azul (LA), e doença hemorrágica por adenovírus (DHA). Uma vez que as lesões nas três doenças hemorrágicas são idênticas (enteropatia hemorrágica, edema pulmonar, petéquias sistêmicas e sufusões hemorrágicas), é necessário utilizar técnicas precisas para um diagnóstico etiológico definitivo. Material de arquivo (blocos de parafina) do Departamento de Patologia Veterinária da FCAV - Unesp foi revisado para lesões de doenças hemorrágicas e 42 cervídeos brasileiros de cativeiro e de vida livre foram selecionados e incluídos neste estudo. Tecidos embebidos em parafina foram avaliados usando imunohistoquímica e foram negativos para adenovírus. Usando o RT-PCR em tempo real, o vírus da DEH não foi detectado nos tecidos de nenhum dos casos avaliados. A mesma técnica foi utilizada para detecção do vírus da LA e sete animais positivos (16,66%) foram confirmados após eletroforese em gel de agarose a 4% e sequenciamento genético. As principais alterações macroscópicas observadas nos animais positivos foram conteúdo intestinal hemorrágico, mucosa do trato gastrointestinal avermelhada, úlceras na língua e petéquias em vários órgãos. As alterações microscópicas observadas foram infiltrado inflamatório linfocítico em fígado, rins e pulmões, e hemorragia e congestão em vários órgãos. Todos os casos positivos foram de animais de cativeiro, três fêmeas (dois jovens e um adulto), e quatro jovens do sexo masculino. Este estudo demonstra que o vírus da lingual azul está envolvido nos surtos de doença hemorrágica em veados no Brasil.(AU)