FibrinCoagulationDysfibrinogenemiaAfibrinogenemiaThrombinAbnormalDeficiencyFetalHypofibrinogenemiaDeficienciesCryoprecipitateHemostaticMutationsPlatelet aggregationAcuteDiagnosisSevereBleedingCardiacPatientsHemolyticDevelopmentalAmyloidosisIntrinsic2018ProteinOccursInfantsClotPresentConcentrateConcentrationNewbornBloodPlateletsDisease
Fibrin10
- In normal conditions, fibrinogen is converted to fibrin when it is cleaved by the enzyme thrombin in the blood. (wikipedia.org)
- Since there is no fibrinogen present during afibrinogenemia, fibrin can not be formed to aid in this process. (wikipedia.org)
- Thrombin time is a screening coagulation test designed to assess fibrin formation from fibrinogen in plasma. (medscape.com)
- A normal thrombin time excludes abnormalities in the fibrin formation process of the coagulation cascade. (medscape.com)
- A prolonged thrombin time indicates a fibrinogen abnormality, impairment of fibrin formation, and/or a thrombin inhibitory effect. (medscape.com)
- Interestingly, mice carrying alanine mutations in fibrinogen residues γ390-396 (Fibγ 390-396A mice) 8 exhibit decreased binding of FXIII to fibrinogen, delayed FXIII activation, and delayed fibrin crosslinking, and phenocopied FXIII-deficient mice, producing smaller venous thrombi with reduced RBC content. (zedira.com)
- 5 These data demonstrate the importance of FXIII localization on fibrinogen for normal FXIII activation and fibrin crosslinking, and indicate that disrupting FXIII-fibrinogen interactions reduces RBC retention in clots and clot size. (zedira.com)
- Using recombinant fibrinogen variants that lack γ- or α-chain crosslinking residues and FXIIIa inhibitor concentrations that preferentially block formation of α-chain-rich high-molecular weight crosslinked species but not γ-γ dimers, we showed that the effect of FXIIIa on RBC retention is mediated by its ability to crosslink fibrin α-chains. (zedira.com)
- Fibrin glue is composed of a main glue, containing fibrinogen dissolved in a phosphate buffer solution, and a thrombin catalyst dissolved in a calcium chloride solution. (curvexpo.com)
- Fibrin degradation products (FDPs) can be falsely elevated, and fibrinogen can be falsely low. (vdocuments.net)
Coagulation12
- Congenital afibrinogenemia is a rare, genetically inherited blood fibrinogen disorder in which the blood does not clot normally due to the lack of fibrinogen, a blood protein necessary for coagulation. (wikipedia.org)
- Cryoprecipitate is less pure, contains several coagulation factors, and a higher volume is required than for fibrinogen concentrate. (octapharma.com)
- Normal in all other disorders of coagulation except von Willebrand's disease deficiency and some cases of very low plasma fibrinogen. (pediaindia.net)
- Most of the congenital coagulation protein disorders reported in domestic animals are deficiencies or abnormalities of a single factor. (msdvetmanual.com)
- Overview of Vascular Bleeding Disorders Bleeding may result from abnormalities in Platelets Coagulation factors Blood vessels Vascular bleeding disorders result from defects in blood vessels, typically causing cutaneous or mucosal. (merckmanuals.com)
- When they present with the sudden onset of painful knee swelling after a local trauma, they may suggest a traumatic arthropathy or a congenital coagulation disorder. (biomedcentral.com)
- Not only is it the site of production of many of the coagulation factors, including the vitamin K-dependent factors, fibrinogen and factor VIII (endothelial cells in the liver, not hepatocytes) and inhibitors (protein C, protein S, antithrombin), but it is also responsible for clearance and degradation of factors, factor-inhibitor complexes and D-dimer/FDPs. (eclinpath.com)
- Although coagulation abnormalities are common in naturally occurring liver disease (93% of dogs in one study and 82% of cats in another study had at least one abnormal coagulation test), clinical signs of hemorrhage are not commonly seen, except in severe fulminant liver disease (which initiates DIC or induces synthetic failure). (eclinpath.com)
- Although there is no doubt that coagulation abnormalities (mostly prolonged PT and APTT) are seen in liver disease, the clinical relevance of these defects is far from certain. (eclinpath.com)
- Severe liver disease resulting in synthetic liver failure can produce coagulation abnormalities from decreased production of coagulation factors. (eclinpath.com)
- Bassline coagulation testing for prothrombin (PT) and partial prothrombin (PTT) times, fibrinogen was within normal limits for age and gestational age. (childrensmercy.org)
- However, neither scoring systems nor routine coagulation tests detect patients with acquired or congenital hypercoagulable states. (biomedcentral.com)
Dysfibrinogenemia3
- Cryoprecipitate is also used for fibrinogen replacement in patients with congenital or acquired abnormalities in fibrinogen, such as dysfibrinogenemia or hypofibrinogenemia. (saurashtranews.com)
- Low fibrinogen levels are detected by measuring fibrinogen, but dysfibrinogenemia, where fibrinogen is present but functionally defective, can be best detected by the thrombin time. (saurashtranews.com)
- Abnormal fibrinogen molecules (dysfibrinogenemia) is a feature of some liver diseases, such as hepatomas and acute and chronic liver disease, in human patients. (eclinpath.com)
Afibrinogenemia5
- citation needed] When a problem of fibrinogen is suspected, the following tests can be ordered: PT PTT Fibrinogen level in blood (total and clottable) Reptilase time Thrombin time Blood fibrinogen levels of less than 0.1 g/L and prolonged bleeding test times are indicators of an individual having afibrinogenemia. (wikipedia.org)
- A suspicion of congenital afibrinogenemia may appear on a platelet aggregation function test. (wikipedia.org)
- A case from 2015 was seen in which congenital afibrinogenemia was resolved in a patient after receiving a liver transplant. (wikipedia.org)
- Congenital afibrinogenemia (Factor I deficiency) has been reported in a family of Saanen dairy goats but not in dogs or cats. (msdvetmanual.com)
- Afibrinogenemia is characterized by the complete absence of immunoreactive fibrinogen. (nih.gov)
Thrombin1
- Elevated fibrinogen in an acute phase reaction prolongs the reptilase time but typically not the thrombin time. (medscape.com)
Abnormal1
- This article reviews the diagnostic approach to hematologic laboratory abnormalities and abnormal bleeding in adults, as well as basic preventive care and hemostatic management of adults with bleeding disorders. (medscape.com)
Deficiency7
- Today, scientific progress allows us to produce high-purity human fibrinogen concentrate to treat acquired fibrinogen deficiency (AFD). (octapharma.com)
- The 2011 launch of the clinical trial to investigate the use of our high-purity human fibrinogen concentrate in congenital fibrinogen deficiency was the start of a clinical development programme in a very rare disease. (octapharma.com)
- But it also became an early step in one of the most audacious clinical programmes which Octapharma has conducted, targeting acquired fibrinogen deficiency (AFD). (octapharma.com)
- This approval extends the original 2017 marketing authorisation for Octapharma's high-purity human fibrinogen concentrate for use in congenital fibrinogen deficiency. (octapharma.com)
- 1 ] [ 2 ] A deficiency in this protease can be congenital or the result of an acquired auto antibody to ADAMTS-13 and can lead to extensive platelet adhesion and clumping and possibly secondary end-organ damage. (thieme-connect.de)
- 5 These observations were consistent with the "excessive red cell fallout" reported many years ago in a study of a family with congenital FXIII deficiency 7 , and suggested a previously-unrecognized role for FXIII(a) in mediating clot composition. (zedira.com)
- We conclude that Fgl2 deficiency is not associated with obvious structural cardiac defects but is associated with a high incidence of neonatal death as well as contractile dysfunction and rhythm abnormalities during embryonic and postnatal development in mice. (mcmaster.ca)
Fetal2
- Cesarean deliveries may be indicated for a number of either maternal or fetal reasons, most commonly including fetal intolerance to labor, arrest of labor, a history of prior uterine surgery, fetal malpresentation, and placental abnormalities. (lecturio.com)
- It is recommended to establish the causes of elevated homocysteine levels, also for the prevention of some fetal congenital abnormalities. (treegene.kz)
Hypofibrinogenemia2
- Hypofibrinogenemia is characterized by reduced amounts of immunoreactive fibrinogen. (nih.gov)
- In these situations, there will be other clinical and laboratory evidence of severe liver failure, e.g. hypoalbuminemia, hypocholesterolemia, low urea nitrogen (note that not all of these abnormalities may be present in individual patients), in conjunction with a long PT, APTT, and ACT due to hypofibrinogenemia (that is often very severe). (eclinpath.com)
Deficiencies3
- However, unlike in congenital fibrinogen deficiencies, AFD arises due to consumption of fibrinogen after excessive blood loss. (octapharma.com)
- After Octapharma's high-purity human fibrinogen concentrate obtained regulatory approval in the USA, Canada and the EU in 2017 for the treatment of very rare congenital fibrinogen deficiencies (with further approval obtained in Switzerland in 2018 for both acquired and congenital fibrinogen deficiencies), clinical initiatives continued with FORMA-05, the first new clinical study in AFD. (octapharma.com)
- Recurrent miscarriage may be a feature of some congenital factor (eg, factor XIII) and fibrinogen deficiencies. (medscape.com)
Cryoprecipitate6
- The most common treatment is fibrinogen replacement therapy, including cryoprecipitate, blood plasma, and fibrinogen concentration transfusions to help with bleeding episodes or prior to surgery. (wikipedia.org)
- With no other alternatives, AFD is treated with cryoprecipitate in many countries around the world and thus there is a high medical need for a fibrinogen concentrate approved in this indication. (octapharma.com)
- The ambitious goal of this programme was to show that our high-purity human fibrinogen concentrate is at least as effective as cryoprecipitate for AFD - and is a more practical and reliable alternative to it. (octapharma.com)
- The rationale for the FORMA-05 study was to investigate whether a fibrinogen concentrate is an effective alternative to cryoprecipitate in a model indication of AFD. (octapharma.com)
- In many parts of the world, patients are still treated with cryoprecipitate, as a fibrinogen concentrate is not licensed for AFD. (octapharma.com)
- The FORMA-05 clinical study was a prospective, randomised, single-blind, controlled, non-inferiority study which compared the efficacy of Octapharma's high-purity human fibrinogen concentrate to that of cryoprecipitate during cytoreductive surgery to treat pseudomyxoma peritonei, a rare cancer that usually starts in the appendix. (octapharma.com)
Hemostatic4
- However, they may bleed when exposed to trauma or if they have a second associated hemostatic abnormality. (nih.gov)
- In cases of asymptomatic hematologic laboratory abnormalities, the particular abnormality should narrow down the potentially affected hemostatic component(s), ideally streamlining subsequent investigation. (medscape.com)
- While severe bleeding disorders and most congenital bleeding disorders initially present during childhood, conditions associated with heavy bleeding only after a hemostatic challenge (such as surgery or trauma) often present during adulthood. (medscape.com)
- The alpha granules contain hemostatic proteins such as fibrinogen, vWf, and growth factors (eg, platelet-derived growth factor and transforming growth factors). (medscape.com)
Mutations1
- Chediak-Higashi syndrome (CHS) is a rare, autosomal recessive congenital immunodeficiency caused by mutations in CHS1 , a gene encoding a putative lysosomal trafficking protein. (biomedcentral.com)
Platelet aggregation1
- Grant CU/381/2012 - "Abnormalities of platelet aggregation: genetic variability of glycopropein 6 gene', Juraj Sokol, MD - 2012. (uniba.sk)
Acute2
- Levels of plasma total adrenomedullin are related with two acute phase inflammatory reactants (fibrinogen and sialic acid) but not with markers of endothelial dysfunction in Type 1 diabetes Adrenomedullin and vascular risk factors in Type 1 DM. (phoenixpeptide.com)
- Histopathology and Doppler assessments suggested that death was due to acute congestive cardiac failure without evidence of valvular or other obvious cardiac structural abnormalities. (mcmaster.ca)
Diagnosis1
- Diagnosis requires demonstrating characteristic laboratory test abnormalities, including direct antiglobulin test-negative hemolytic anemia. (merckmanuals.com)
Severe2
- May be useful to monitor treatment of active hemorrhage in patients with prolonged BT due to uremia, Von Willebrand's disease, congenital platelet function abnormalities or severe anemia. (pediaindia.net)
- It exists in both congenital and acquired forms and is associated with the absence or severe depletion of von Willebrand factor cleaving protease known as a disintegrin and metalloproteinase with thrombospondin-like repeats (ADAMTS-13). (thieme-connect.de)
Bleeding5
- The lack of fibrinogen expresses itself with excessive and, at times, uncontrollable bleeding. (wikipedia.org)
- If you ever needed proof that Octapharma is a company with the courage to take a calculated risk and invest in science to develop new medicines that could save the lives of patients with bleeding abnormalities, then this AFD project is it," says Sigurd Knaub, Senior Vice-President of Clinical Research & Development Haematology. (octapharma.com)
- Abnormalities in the fibrinolytic system may be associated with thrombosis or bleeding. (arupconsult.com)
- Acquired clotting or bleeding abnormalities should be considered before the less common congenital fibrinolytic abnormalities. (arupconsult.com)
- Adults with bleeding disorders may present to their family physician with minor bleeding symptoms or hematologic laboratory abnormalities discovered during evaluation for surgery or another purpose. (medscape.com)
Cardiac3
- In 2017, Octapharma started discussing another study using Octapharma's high-purity human fibrinogen concentrate in cardiac surgery with a group of Canadian physicians led by Dr Keyvan Karkouti in Toronto. (octapharma.com)
- We hypothesized that cardiac dysfunction was responsible for the high perinatal lethality that we previously reported in fibrinogen-like protein 2 ( Fgl2) knockout (KO) mice. (mcmaster.ca)
- Preoperative ROTEM analysis using extrinsic rotational thromboelastometry (EXTEM), intrinsic rotational thromboelastometry (INTEM), and fibrinogen rotational thromboelastometry (FIBTEM) activators was performed on 313 patients undergoing major non-cardiac surgery. (biomedcentral.com)
Patients4
- Routine laboratory tests do not detect patients with acquired or congenital hypercoagulability who may be at increased risk of perioperative thromboembolism. (biomedcentral.com)
- RCM may manifest as a solitary abnormality, although restrictive filling patterns of the left ventricle can also be seen in patients with dilated or hypertrophic cardiomyopathy. (medscape.com)
- Six patients only exhibited symptoms of cauda equina compression with sensory abnormalities in the perineum/saddle area, bladder and bowel dysfunction, or a burning sensation around the anus. (curvexpo.com)
- For these symptomatic sacral arachnoid cysts, patients presenting with mild pain or sensory abnormalities should initially undergo medical treatments with anti-inflammatory drugs, neurotrophic drugs, and physical therapy. (curvexpo.com)
Hemolytic3
- Congenital complement disorders may also increase the risk of hemolytic-uremic sydrome (HUS) following infection. (merckmanuals.com)
- Hemolysis can either be caused by abnormalities in RBCs ( hemoglobin , the RBC membrane, or intracellular enzymes), which is called intrinsic hemolytic anemia , or by external causes (immune-mediated or mechanical damage), which is called extrinsic hemolytic anemia . (amboss.com)
- Congenital hemolytic anemias are present at birth and may or may not be inherited. (innvista.com)
Developmental1
- CONCLUSION: Understanding the prevalence of developmental delays and healthcare needs of children with congenital Zika virus exposure can inform health systems and planning to ensure services are available for affected families. (cdc.gov)
Amyloidosis1
- A group of rare renal diseases, characterized by amyloid fibril deposition of apolipoprotein A-I or A-II (AApoAI or AApoAII amyloidosis), lysozyme (ALys amyloidosis) or fibrinogen A-alpha chain (AFib amyloidosis) in one or several organs. (nih.gov)
Intrinsic1
- Hereditary forms are the result of intrinsic (cellular) abnormalities, typically of the erythrocyte's plasma membrane or cytoplasmic contents (enzymes of hemoglobin). (innvista.com)
20181
- We evaluated all infants having congenital heart surgery at Loma Linda University Children's Hospital between 1 July, 2013 and 31 December, 2018. (cambridge.org)
Protein3
- citation needed] A missense or nonsense mutation to the genes that code for the fibrinogen protein are affected. (wikipedia.org)
- citation needed] Individuals with the disorder have a mutation to their fibrinogen gene that prevents the formation of the protein. (wikipedia.org)
- For instance, some dogs with chronic hepatitis and portosystemic shunts have low AT and protein C (indeed, Protein C appears to be consistently low in dogs with acquired or congenital portosystemic shunts compared to dogs with microvascular dysplasia) ( Toulza et al 2006 ). (eclinpath.com)
Occurs1
- Reduced or dysfunctional fibrinogen occurs in various congenital fibrinogen-related disorders. (octapharma.com)
Infants1
- 91.8% of infants had brain abnormalities or microcephaly, 23.0% had eye abnormalities, and 14.8% had both. (cdc.gov)
Clot1
- As such, ROTEM can provide a more detailed evaluation of clot formation and breakdown in the individual patient and can identify specific cascade abnormalities. (biomedcentral.com)
Present1
- 1] The heart is grossly normal, although histologic abnormalities are often present, depending on the etiology of the restrictive cardiomyopathy. (medscape.com)
Concentrate4
- Although some thrombotic complications have been reported following replacement therapy, transfusions of fibrinogen concentrate are widely considered to be the most beneficial. (wikipedia.org)
- Fibrinogen concentrate is pure, contains a known quantity of fibrinogen, is virally inactivated, and is transfused in small amounts. (wikipedia.org)
- Eight years on, and after the detailed design, planning and conduct of clinical trials by a dedicated Octapharma team, our high-purity human fibrinogen concentrate has now been approved for use in AFD in 15 countries of the EU. (octapharma.com)
- Members of the dedicated Octapharma human fibrinogen concentrate team. (octapharma.com)
Concentration1
- Decreased concentration of fibrinogen in the blood. (nih.gov)
Newborn3
- The congenital neuromuscular subtype presents in the newborn period with profound hypotonia, respiratory distress, and dilated cardiomyopathy. (beds.ac.uk)
- SUMMARY An early term infant with uncomplicated perinatal history was found to have a large thrombus in the aortic arch after he failed regular newborn critical congenital heart defect (CCHD) screen. (childrensmercy.org)
- The infant presented to our unit after he failed the standard congenital heart newborn screen. (childrensmercy.org)
Blood2
- Fibrinogen, also known as factor I, is a naturally occurring blood plasma glycoprotein. (octapharma.com)
- Lack of detectable fibrinogen in the blood circulation. (nih.gov)
Platelets1
- On resting platelets, GP IIb/IIIa is unable to bind fibrinogen or vWf. (medscape.com)
Disease1
- This includes milder phenotypes of congenital disorders such as hemophilia and von Willebrand disease (VWD). (medscape.com)