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Acta Medica Indonesiana 2004
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Postgraduate Medical Journal Aug 1973In the late summer of 1967 an epidemic in thirty-one patients in Germany and Yugoslavia of a disease transmitted from African green monkeys occurred; seven patients...
In the late summer of 1967 an epidemic in thirty-one patients in Germany and Yugoslavia of a disease transmitted from African green monkeys occurred; seven patients died. The incubation period was from 4 to 7 days. The main clinical features were headache, high fever, diarrhoea, a very characteristic rash, severe bleeding tendency and involvement of the central nervous system. Nearly all organs were involved and showed severe cell necroses. The aetiological agent was identified as an RNS-virus and was named Marburg virus. It was detected in the blood, urine, throat-washing and seminal fluid.
Topics: Animals; Blood Cell Count; Brain; Central Nervous System Diseases; Complement Fixation Tests; Diarrhea; Female; Haplorhini; Headache; Hemorrhagic Disorders; Humans; Liver; Liver Diseases; Lymph Nodes; Male; Monkey Diseases; Muscular Diseases; RNA Viruses; Skin Diseases; Virus Diseases; Zoonoses
PubMed: 4207635
DOI: 10.1136/pgmj.49.574.542 -
Anaesthesia Jan 1979The statement that amniotic fluid embolism is the most dangerous and untreatable condition in obstetrics appears to be true. It must be suspected in any patient who... (Review)
Review
The statement that amniotic fluid embolism is the most dangerous and untreatable condition in obstetrics appears to be true. It must be suspected in any patient who collapses or bleeds excessively during labour or the immediate post-partum period. Attempts should be made to secure a definitive diagnosis in life by examination of blood obtained from the right side of the heart and the sputum for elements of amniotic fluid. Lung scanning is a useful aid to diagnosis. The principal factors that have been implicated in the clinical syndrome of amniotic fluid embolism are anaphylaxis, vascular obstruction by particulate matter, vascular spasm due to prostaglandins and possibly some other vasoactive substances, and the possibility that all the changes could be explained by disseminated intravascular coagulation as a primary event. Further work is required to elucidate the relative contributions of these various factors. Due to the suddeness of the catastrophe and the very high mortality, haemodynamic data in humans is virtually non-existent. With improved methods of resuscitation it is to be hoped that the mortality rate will be reduced and that such data will become available. In this way it might become possible to apply the results of animal research and indicate the most effective method of treatment.
Topics: Adolescent; Adult; Anaphylaxis; Animals; Diagnosis, Differential; Embolism, Amniotic Fluid; Female; Hemorrhagic Disorders; Humans; Maternal Age; Middle Aged; Obstetric Labor Complications; Parity; Pregnancy; Pulmonary Circulation; Vascular Diseases
PubMed: 371460
DOI: 10.1111/j.1365-2044.1979.tb04862.x -
Journal of Thrombosis and Haemostasis :... Jul 2010Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by severe and recurrent nosebleeds, mucocutaneous telangiectases,... (Review)
Review
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by severe and recurrent nosebleeds, mucocutaneous telangiectases, and, in some cases, life-threatening visceral arteriovenous malformations of various types, including pulmonary, hepatic, cerebral, and spinal. Gastrointestinal telangiectases are frequent and may cause severe bleeding. HHT type 1 results from mutations in ENG on chromosome 9 (coding for endoglin), and HHT type 2 results from mutations in ACVRL1 on chromosome 12 (coding for activin receptor-like kinase 1). Mutations of either of these two genes account for most clinical cases. In addition, mutations in MADH4 (encoding SMAD4), which cause a juvenile polyposis/HHT overlap syndrome, have been described, and recently, an HHT3 locus on chromosome 5 (5q31.3-5q32) has been reported. The mutated genes in HHT encode proteins that modulate transforming growth factor-beta superfamily signaling in vascular endothelial cells. Management of patients has changed considerably in the last 20 years, in terms of both treatment and the prevention of complications. The goal of this review was to describe the underlying molecular and cellular physiopathology, explore clinical and genetic diagnostic strategies for HHT, and present clinical management recommendations in order to treat symptomatic disease and to screen for vascular malformations.
Topics: Animals; Arteriovenous Malformations; Epistaxis; Humans; Signal Transduction; Telangiectasia, Hereditary Hemorrhagic
PubMed: 20345718
DOI: 10.1111/j.1538-7836.2010.03860.x -
PloS One 2016Canine herpesvirus is a widespread alphaherpesvirus that causes a fatal haemorrhagic disease of neonatal puppies. We have used high-throughput methods to determine the...
Canine herpesvirus is a widespread alphaherpesvirus that causes a fatal haemorrhagic disease of neonatal puppies. We have used high-throughput methods to determine the genome sequences of three viral strains (0194, V777 and V1154) isolated in the United Kingdom between 1985 and 2000. The sequences are very closely related to each other. The canine herpesvirus genome is estimated to be 125 kbp in size and consists of a unique long sequence (97.5 kbp) and a unique short sequence (7.7 kbp) that are each flanked by terminal and internal inverted repeats (38 bp and 10.0 kbp, respectively). The overall nucleotide composition is 31.6% G+C, which is the lowest among the completely sequenced alphaherpesviruses. The genome contains 76 open reading frames predicted to encode functional proteins, all of which have counterparts in other alphaherpesviruses. The availability of the sequences will facilitate future research on the diagnosis and treatment of canine herpesvirus-associated disease.
Topics: Alphaherpesvirinae; Amino Acid Sequence; Animals; Base Sequence; Chromosome Mapping; Dog Diseases; Dogs; Genes, Viral; Genome, Viral; Hemorrhagic Disorders; Madin Darby Canine Kidney Cells; Open Reading Frames; Sequence Analysis, DNA; Viral Proteins
PubMed: 27213534
DOI: 10.1371/journal.pone.0156015 -
American Journal of Hematology Sep 2008Variation in bleeding in the perioperative period is a complex and multifactorial event associated with immediate and delayed consequences for the patient and health... (Review)
Review
Variation in bleeding in the perioperative period is a complex and multifactorial event associated with immediate and delayed consequences for the patient and health care resources. Little is known about the complex genetic influences on perioperative bleeding. With the discovery of multiple variations in the human genome and ever-growing databases of well-phenotyped surgical patients, better identification of patients at risk of bleeding is becoming a reality. In this review, polymorphisms in the platelet receptor genes, plasminogen activator inhibitor, and angiotensin genes among others will be discussed. We will explore the nature, effects, and implications of the genetics that influence perioperative bleeding above and beyond surgical bleeding, particularly in cardiac surgery.
Topics: Angiotensins; Blood Coagulation Factors; Blood Coagulation Tests; Blood Loss, Surgical; Blood Proteins; Cardiac Surgical Procedures; Genetic Predisposition to Disease; Genetic Variation; Genotype; Hemorrhagic Disorders; Hemostasis; Humans; Plasminogen Inactivators; Platelet Activation; Polymorphism, Genetic; Postoperative Hemorrhage; Risk Factors
PubMed: 18508320
DOI: 10.1002/ajh.21205 -
Blood Jan 2019
Topics: Blood Coagulation Disorders; Hemorrhagic Disorders; Humans
PubMed: 30559265
DOI: 10.1182/blood-2018-12-877779 -
Clinical and Applied... Jul 2001This review has summarized the more important diseases that may be accompanied by or lead to a disorder of hemostasis or thrombosis via alterations of the vasculature.... (Review)
Review
This review has summarized the more important diseases that may be accompanied by or lead to a disorder of hemostasis or thrombosis via alterations of the vasculature. It is to be stressed that the vascular component of hemostasis is often overlooked by clinicians caring for patients with disorders of hemostasis and thrombosis. It should be appreciated that the vasculature is intricately related to the coagulation protein system and to platelets when involved in thrombohemorrhagic diatheses. Although many vascular disorders may lead to hemorrhage or thrombosis, it must be appreciated that often it is impossible to discern between a primary vascular defect/damage and a defect that has been induced by platelet activation/dysfunction or procoagulant abnormalities.
Topics: Amyloidosis; Autoimmune Diseases; Behcet Syndrome; Collagen Diseases; Cushing Syndrome; Diabetes Complications; Ehlers-Danlos Syndrome; Genetic Predisposition to Disease; Hemangioma, Cavernous; Hemorrhagic Disorders; Hemostatic Disorders; Homocystinuria; Humans; Hyperhomocysteinemia; Infections; Marfan Syndrome; Osteogenesis Imperfecta; Paraproteinemias; Platelet Function Tests; Pseudoxanthoma Elasticum; Telangiectasia, Hereditary Hemorrhagic; Thrombophilia; Vasculitis
PubMed: 11441978
DOI: 10.1177/107602960100700302 -
Hong Kong Medical Journal = Xianggang... Feb 2015To review the current evidence for the use of viscoelastic haemostatic assays in different surgical settings including trauma, cardiac surgery, liver transplantation, as... (Review)
Review
OBJECTIVES
To review the current evidence for the use of viscoelastic haemostatic assays in different surgical settings including trauma, cardiac surgery, liver transplantation, as well as the monitoring of antiplatelet agents and anticoagulants prior to surgery.
DATA SOURCES
PubMed database.
STUDY SELECTION
Key words for the literature search were "thromboelastography" or "ROTEM" in combination with "trauma", "antiplatelet", "cardiac surgery", "liver transplantation" or "anticoagulants".
DATA EXTRACTION
Original and major review articles related to the use of viscoelastic haemostatic assays.
DATA SYNTHESIS
Haemostatic function is a critical factor determining patient outcomes in emergency or elective surgery. The increasing use of antiplatelet agents and anticoagulants has potentially increased the risks of haemorrhages and the need for transfusion. Conventional coagulation tests have limitations in detecting haemostatic dysfunctions in subgroups of patients and are largely ineffective in diagnosing hyperfibrinolysis. The viscoelastic haemostatic assays are potentially useful point-of-care tools that provide information on clot formation, clot strength, and fibrinolysis, as well as to guide goal-directed transfusion and antifibrinolytic therapy. They may also be used to monitor antiplatelet and anticoagulant therapy. However, standardisation of techniques and reference ranges is required before these tests can be widely used in different clinical settings.
CONCLUSIONS
Viscoelastic haemostatic assays, as compared with conventional coagulation tests, are better for detecting coagulopathy and are the only tests that can provide rapid diagnosis of hyperfibrinolysis. Goal-directed administration of blood products based on the results of viscoelastic haemostatic assays was associated with reduction in allogeneic blood product transfusions in trauma, cardiac surgery, and liver transplantation cases. However, there is currently no evidence to support the routine use of viscoelastic haemostatic assays for monitoring platelet function prior to surgery.
Topics: Anticoagulants; Elective Surgical Procedures; Hemorrhagic Disorders; Hemostasis, Surgical; Humans; Platelet Aggregation Inhibitors; Preoperative Care; Surgical Procedures, Operative; Thrombelastography; Viscoelastic Substances
PubMed: 25082123
DOI: 10.12809/hkmj134147 -
American Journal of Hematology Jan 2022Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is...
Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is associated with a BD in adolescents. Moreover, the threshold of ferritin associated with fatigue in adolescents with HMB is unclear. In this multicenter study, we enrolled adolescents with HMB without BD. Participants underwent BD and anemia work-up in Young Women's Hematology Clinics and completed the Peds QL™ fatigue scale. BDs were defined as von Willebrand Disease, platelet function defect, clotting factor deficiencies, and hypermobility syndrome. Two hundred and fifty consecutive adolescents were enrolled, of whom 196 met eligibility criteria. Overall, 43% (95% confidence interval: 36%-50%) were diagnosed with BD. A total of 61% (n = 119) had serum ferritin levels < 15 ng/mL, 23.5% (n = 46) had iron deficiency only, and 37% (n = 73) had iron deficiency anemia. Low ferritin or ferritin dichotomized as < 15 or ≥ 15 ng/mL was not associated with BD on univariable analysis (p = .24) or when accounting for age, race, ethnicity, body mass index, and hemoglobin (p = .35). A total of 85% had total fatigue score below the population mean of 80.5, and 52% (n = 102) were > 2 SD (or < 54) below the mean, the cut-off associated with severe fatigue. A ferritin threshold of < 6 ng/mL had a specificity of 79.8% but a sensitivity of 36% for severe fatigue. In conclusion, iron deficiency without anemia is not a predictor of BD in adolescents with HMB in a specialty setting. Severe fatigue, especially sleep fatigue, is prevalent in adolescents with BD. Ferritin of < 6 ng/mL has ~80% specificity for severe fatigue in adolescents with HMB.
Topics: Adolescent; Adult; Fatigue; Female; Ferritins; Hemorrhagic Disorders; Humans; Iron Deficiencies; Male; Menorrhagia; Young Adult; von Willebrand Diseases
PubMed: 34710246
DOI: 10.1002/ajh.26389