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Archives of Pathology & Laboratory... Apr 2002Dysfibrinogenemia is a coagulation disorder caused by a variety of structural abnormalities in the fibrinogen molecule that result in abnormal fibrinogen function. It... (Review)
Review
Dysfibrinogenemia is a coagulation disorder caused by a variety of structural abnormalities in the fibrinogen molecule that result in abnormal fibrinogen function. It can be inherited or acquired. The inherited form is associated with increased risk of bleeding, thrombosis, or both in the same patient or family. Traditionally, dysfibrinogenemia is diagnosed by abnormal tests of fibrin clot formation; the thrombin time and reptilase time are the screening tests, and the fibrinogen clotting activity-antigen ratio is the confirmatory test. The inherited form is diagnosed by demonstrating similar laboratory test abnormalities in family members, and if necessary by analysis of the fibrinogen protein or fibrinogen genes in the patient. The acquired form is diagnosed by demonstrating abnormal liver function tests and by ruling out dysfibrinogenemia in family members. This article reviews the laboratory testing of dysfibrinogenemia and presents an algorithm for sequential test selection that can be used for diagnosis.
Topics: Algorithms; Blood Coagulation Disorders, Inherited; Fibrinogen; Humans; Mutation; Thrombin Time; Thrombophilia
PubMed: 11900586
DOI: 10.5858/2002-126-0499-LDOD -
Journal of Thrombosis and Haemostasis :... May 2016Severe burn injury is associated with systemic coagulopathy. The changes in coagulation described in patients with severe burns resemble those found patients with sepsis... (Review)
Review
Severe burn injury is associated with systemic coagulopathy. The changes in coagulation described in patients with severe burns resemble those found patients with sepsis or major trauma. Coagulopathy in patients with severe burns is characterized by procoagulant changes, and impaired fibrinolytic and natural anticoagulation systems. Both the timing of onset and the severity of hemostatic derangements are related to the severity of the burn. The exact pathophysiology and time course of coagulopathy are uncertain, but, at least in part, result from hemodilution and hypothermia. As the occurrence of coagulopathy in patients with severe burns is associated with increased comorbidity and mortality, coagulopathy could be seen as a potential therapeutic target. Clear guidelines for the treatment of coagulopathy in patients with severe burns are lacking, but supportive measures and targeted treatments have been proposed. Supportive measures are aimed at avoiding preventable triggers such as tissue hypoperfusion caused by shock, or hemodilution and hypothermia following the usually aggressive fluid resuscitation in these patients. Suggested targeted treatments that could benefit patients with severe burns include systemic treatment with anticoagulants, but sufficient randomized controlled trial evidence is lacking.
Topics: Anticoagulants; Blood Coagulation; Blood Coagulation Disorders; Burns; Coagulants; Fibrinolysis; Hemodilution; Hemostasis; Humans; Recombinant Proteins; Smoke Inhalation Injury
PubMed: 26854881
DOI: 10.1111/jth.13283 -
Journal of Thrombosis and Haemostasis :... Dec 2007Plasminogen (plg) deficiency has been classified as (i) hypoplasminogenemia or 'true' type I plg deficiency, and (ii) dysplasminogenemia, also called type II plg... (Review)
Review
Plasminogen (plg) deficiency has been classified as (i) hypoplasminogenemia or 'true' type I plg deficiency, and (ii) dysplasminogenemia, also called type II plg deficiency. Both forms, severe hypoplasminogenemia and dysplasminogenemia, are not causally linked to venous thrombosis. Dysplasminogenemia does not lead to a specific clinical manifestation and probably represents only a polymorphic variation in the general population, mainly in Asian countries. Severe hypoplasminogenemia is associated with compromised extracellular fibrin clearance during wound healing, leading to pseudomembraneous (ligneous) lesions on affected mucous membranes (eye, middle ear, mouth, pharynx, duodenum, upper and lower respiratory tract and female genital tract). Ligneous conjunctivitis is by far the most common clinical manifestation. More than 12% of patients with severe hypoplasminogenemia exhibit congenital occlusive hydrocephalus. In milder cases of ligneous conjunctivitis, topical application of plg-containing eye drops, fresh frozen plasma, heparin, corticosteroids or certain immunosuppressive agents (such as azathioprine) may be more or less effective. Oral treatment with sex hormones was successful in two female patients with ligneous conjunctivitis. In severe cases with possibly life-threatening multi-organ involvement, true therapeutic options are not available at present. The plg-knockout mouse is a useful tool to study the many different properties of plg in a variety of settings, such as wound healing, tissue repair and tissue remodeling, virulence and invasiveness of certain bacteria in the human host, tumor growth and dissemination, as well as arteriosclerosis.
Topics: Amino Acid Sequence; Animals; Blood Coagulation Disorders; Conjunctivitis; Disease Models, Animal; Fibrinolysin; Fibrinolysis; Genetic Predisposition to Disease; Heterozygote; Homozygote; Humans; Mice; Mice, Knockout; Molecular Sequence Data; Mutation; Phenotype; Plasminogen; Protein Conformation; Risk Assessment; Risk Factors; Venous Thrombosis
PubMed: 17900274
DOI: 10.1111/j.1538-7836.2007.02776.x -
Chinese Journal of Traumatology =... Jun 2017Trauma-induced coagulopathy (TIC) is a clinical syndrome caused by imbalance between clotting, anti-coagulation and fibrinolysis resulting from multiple pathological... (Review)
Review
Trauma-induced coagulopathy (TIC) is a clinical syndrome caused by imbalance between clotting, anti-coagulation and fibrinolysis resulting from multiple pathological factors such as hemorrhage and tissue injury in the early stage of trauma, and is closely related to the outcome of trauma patients. It is proved in growing evidence that the endogenous coagulation disturbance in trauma itself is the activating factor of TIC, rather than dilution or other acquired coagulopathy. Therefore, a thorough understanding of the molecular mechanisms in the pathogenesis and progression is crucial for effective prevention and treatment in patients with TIC. This review focuses on transitions in the concept of TIC and mechanical progress.
Topics: Blood Coagulation Disorders; Endothelial Cells; Humans; NLR Family, Pyrin Domain-Containing 3 Protein; Wounds and Injuries
PubMed: 28554592
DOI: 10.1016/j.cjtee.2017.03.002 -
Journal of Thrombosis and Haemostasis :... Jun 2015Trauma-induced coagulopathy (TIC) includes heterogeneous coagulopathic syndromes with different underlying causes, and treatment is challenged by limited diagnostic... (Review)
Review
Trauma-induced coagulopathy (TIC) includes heterogeneous coagulopathic syndromes with different underlying causes, and treatment is challenged by limited diagnostic tests to discriminate between these entities in the acute setting. We provide an overview of progress in understanding the mechanisms of TIC and the context for several of the hypotheses that will be tested in 'TACTIC'. Although connected to ongoing clinical trials in trauma, TACTIC itself has no intent to conduct clinical trials. We do anticipate that 'early translation' of promising results will occur. Functions anticipated at this early translational level include: (i) basic science groundwork for future therapeutic candidates; (ii) development of acute coagulopathy scoring systems; (iii) coagulation factor composition-based computational analysis; (iv) characterization of novel analytes including tissue factor, polyphosphates, histones, meizothrombin and α-thrombin-antithrombin complexes, factor XIa, platelet and endothelial markers of activation, signatures of protein C activation and fibrinolysis markers; and (v) assessment of viscoelastic tests and new point-of-care methods.
Topics: Animals; Blood Coagulation; Blood Coagulation Disorders; Cooperative Behavior; Humans; Interinstitutional Relations; Translational Research, Biomedical; Wounds and Injuries
PubMed: 26149052
DOI: 10.1111/jth.12981 -
Journal of Thrombosis and Thrombolysis Nov 2020The new outbreak of Coronavirus Disease 2019 (COVID-19) has emerged as a serious global public health concern. A more in-depth study of blood coagulation abnormality is...
The new outbreak of Coronavirus Disease 2019 (COVID-19) has emerged as a serious global public health concern. A more in-depth study of blood coagulation abnormality is needed. We retrospectively analyzed 147 consecutive patients with COVID-19 who were admitted to three ICUs in Wuhan from February 9th, 2020 to March 20th, 2020. The baseline coagulation and other characteristics were studied. Our results showed that the prolonged PT, FDP, DD were positively correlated with the levels of neutrophils, ferritin, LDH, total bilirubin, multi-inflammation cytokines, and negatively correlated with the lymphocytes level (p < 0.01). The level of ATIII was significantly negatively correlated with the levels of neutrophils, ferritin, LDH, total bilirubin, IL2R, IL6 and IL8 (p < 0.05). The patients in the ARDS group had a more prominent abnormality in PT, FDP, DD and ATIII, while the patients in the AKI group had more prolonged PT, more severe FDP and DD level, more inferior ATIII and Fib level than those in the non-AKI group (p < 0.01). The value of PT, DD and FDP were positively correlated with the classical APACHE II, SOFA and qSOFA scores, while the ATIII was negatively correlated with them (p < 0.001). The high levels of PT, FDP and DD were correlated with in-hospital mortality (p < 0.001). In conclusion, blood coagulation disorder was prominent in ICU patients with COVID-19 and was correlated with multi-inflammation factors. The abnormality of blood coagulation parameters could be an adverse prognostic indicator for ICU patients with COVID-19.
Topics: Aged; Antithrombin III; Betacoronavirus; Biomarkers; Blood Coagulation; Blood Coagulation Disorders; COVID-19; Coronavirus Infections; Female; Fibrin Fibrinogen Degradation Products; Hospital Mortality; Host-Pathogen Interactions; Humans; Inflammation; Inflammation Mediators; Intensive Care Units; Male; Middle Aged; Pandemics; Pneumonia, Viral; Prognosis; Retrospective Studies; Risk Factors; SARS-CoV-2; Time Factors
PubMed: 32761495
DOI: 10.1007/s11239-020-02174-9 -
International Journal of Molecular... Jan 2022Vitamin K dependent coagulation factor deficiency type 1 (VKCFD1) is a rare hereditary bleeding disorder caused by mutations in γ-Glutamyl carboxylase () gene. The GGCX... (Review)
Review
Vitamin K dependent coagulation factor deficiency type 1 (VKCFD1) is a rare hereditary bleeding disorder caused by mutations in γ-Glutamyl carboxylase () gene. The GGCX enzyme catalyzes the γ-carboxylation of 15 different vitamin K dependent (VKD) proteins, which have function in blood coagulation, calcification, and cell signaling. Therefore, in addition to bleedings, some VKCFD1 patients develop diverse non-hemorrhagic phenotypes such as skin hyper-laxity, skeletal dysmorphologies, and/or cardiac defects. Recent studies showed that mutations differentially effect γ-carboxylation of VKD proteins, where clotting factors are sufficiently γ-carboxylated, but not certain non-hemostatic VKD proteins. This could be one reason for the development of diverse phenotypes. The major manifestation of non-hemorrhagic phenotypes in VKCFD1 patients are mineralization defects. Therefore, the mechanism of regulation of calcification by specific VKD proteins as matrix Gla protein (MGP) and Gla-rich protein (GRP) in physiological and pathological conditions is of high interest. This will also help to understand the patho-mechanism of VKCFD1 phenotypes and to deduce new treatment strategies. In the present review article, we have summarized the recent findings on the function of GRP and MGP and how these proteins influence the development of non-hemorrhagic phenotypes in VKCFD1 patients.
Topics: Alleles; Animals; Biomarkers; Blood Coagulation; Blood Coagulation Disorders, Inherited; Calcification, Physiologic; Calcium-Binding Proteins; Carrier Proteins; Disease Models, Animal; Extracellular Matrix Proteins; Genetic Association Studies; Genetic Predisposition to Disease; Genotype; Humans; Mutation; Phenotype; Protein Binding; Matrix Gla Protein
PubMed: 35054981
DOI: 10.3390/ijms23020798 -
Anaesthesiology Intensive Therapy 2016Sepsis is a life-threatening condition characterized by a systemic response to microbial infection. Despite considerable progress in intensive care medicine, the... (Review)
Review
Sepsis is a life-threatening condition characterized by a systemic response to microbial infection. Despite considerable progress in intensive care medicine, the incidence of sepsis and the number of sepsis-related deaths are increasing world-wide. There is a complex relationship between the coagulation, immune and inflammatory systems in sepsis. Activation of the coagulation cascade in sepsis is a result of a pathogen invasion and is a part of a immuno-inflammatory host response. In sepsis, the close cooperation of the immune and coagulation systems through cross signalling results in immunothrombosis. According to a recently described new theory, immunothrombosis is a immune response in which the local activation of coagulation facilitates the recognition and destruction of pathogens. Small amounts of clot formation are beneficial for the host because of bacteria trapping and prevention of the systemic spread of infection. Sepsis is a dynamic syndrome and in all patients with sepsis coagulation changes may progress from a normal profile to hypercoagulability and hypofibrinolysis, hyperfibrinolysis, and ultimately hypocoagulability.
Topics: Blood Coagulation; Blood Coagulation Disorders; Blood Platelets; Humans; Sepsis; Thrombosis
PubMed: 27824218
DOI: 10.5603/AIT.a2016.0051 -
Sovremennye Tekhnologii V Meditsine 2021was to study the clinical course of COVID-19 in the presence of diabetes mellitus (DM) and elucidate possible mechanisms of their mutual aggravation.
UNLABELLED
was to study the clinical course of COVID-19 in the presence of diabetes mellitus (DM) and elucidate possible mechanisms of their mutual aggravation.
MATERIALS AND METHODS
The study included 64 patients with COVID-19; of them, 32 were with DM (main group) and 32 were DM-free (control group). The groups were formed according to the "case-control" principle. During hospitalization, the dynamics of clinical, glycemic, and coagulation parameters, markers of systemic inflammation, as well as kidney and liver functions were monitored and compared.
RESULTS
Among patients with DM, the course of viral pneumonia was more severe, as evidenced by a 2.2-fold higher number of people with extensive (>50%) lung damage (p=0.05), an increased risk of death according to the CURB-65 algorithm (1.3-fold, p=0.043), and a longer duration of insufficient blood oxygen saturation (p=0.0004). With the combination of COVID-19 and DM, hyperglycemia is persistent, without pronounced variability (MAGE - 1.5±0.6 mmol/L), the levels of C-reactive protein (p=0.028), creatinine (p=0.035), and fibrinogen (p=0.013) are higher, manifestations of hypercoagulability persist longer, including slower normalization of antithrombin III (p=0.012), fibrinogen (p=0.037), and D-dimer (p=0.035).
CONCLUSION
The course of COVID-19 in patients with DM is associated with a high severity and extension of pneumonia, persistent decrease in oxygen supply, high hyperglycemia, accelerated renal dysfunction, systemic inflammation, and hypercoagulability.
Topics: Blood Coagulation Disorders; COVID-19; Diabetes Mellitus; Humans; Inflammation; SARS-CoV-2
PubMed: 34796000
DOI: 10.17691/stm2020.12.5.01 -
Annals of Hepatology 2013The clotting process is a dynamic array of multiple processes which can be described in four phases: platelet plug initiation and formation, clotting process propagation... (Review)
Review
The clotting process is a dynamic array of multiple processes which can be described in four phases: platelet plug initiation and formation, clotting process propagation by the coagulation cascade, clotting termination by antithrombotic mechanisms and clot removal by fibrinolysis. The liver plays a central role in each of these phases of clotting process, as it synthesizes the majority of coagulation factors and proteins involved in fibrinolysis as well as thrombopoeitin, which is responsible for platelet production from megakaryocytes. Many pathological processes associated with cirrhosis, such as portal hypertension and endothelial dysfunction, as well as co-morbid conditions, may also alter the coagulation process. Consequently, patients with liver disease have a disturbed balance of procoagulant and anti-coagulant factors which deviates from the normal coagulation cascade. This situation poses an additional problem in the diagnostic and therapeutic approach to this group of patients, since traditional coagulation test may not be reliable for assessing bleeding or thrombotic risk and traditional transfusional strategies may not be applicable in cirrhotic patients. In this article, we review the pathophysiological bases of coagulation abnormalities, in cirrhotic patients, the diagnostic therapeutic strategies to be followed and its impact on the clinical outcome in the cirrhotic patient.
Topics: Blood Coagulation; Blood Coagulation Disorders; Blood Coagulation Factor Inhibitors; Blood Coagulation Factors; Blood Coagulation Tests; Blood Platelets; Fibrinolysis; Hemorrhage; Humans; Liver; Liver Cirrhosis; Prognosis; Risk Factors; Thrombosis
PubMed: 24018489
DOI: No ID Found