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ATC Code B06
B06A Other hematological agents B06AA Enzymes :B06AA02 Fibrinolysin and desoxyribonuclease :B06AA03 Hyaluronidase :B06AA04 Chymotrypsin :B06AA07 Trypsin :B06AA10 Desoxyribonuclease :B06AA55 Streptokinase, combinations B06AB Heme products :B06AB01 Hemin B06AC Drugs used in hereditary angioedema :B06AC01 C1-inhibitor, plasma derived :B06AC02 Icatibant :B06AC03 Ecallantide :B06AC04 Conestat alfa :B06AC05 Lanadelumab :B06AC06 Berotralstat B06AX Other hematological agents :B06AX01 Crizanlizumab :B06AX02 Betibeglogene autotemcel :B06AX03 Voxelotor Voxelotor, sold under the brand name Oxbryta, is a medication used for the treatment of sickle cell disease. Developed by Global Blood Therapeutics, voxelotor is the first hemoglobin oxygen-affinity modulator. Voxelotor has been shown to have di ... References {{Other hematological agents B06 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hematological
Hematology ( always spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, bone marrow, platelets, blood vessels, spleen, and the mechanism of coagulation. Such diseases might include hemophilia, blood clots (thrombus), other bleeding disorders, and blood cancers such as leukemia, multiple myeloma, and lymphoma. The laboratory analysis of blood is frequently performed by a medical technologist or medical laboratory scientist. Specialization Physicians specialized in hematology are known as hematologists or haematologists. Their routine work mainly includes the care and treatment of patients with hematological diseases, although some may also work at the hematology laboratory viewing blood films and bone marrow slides under the mic ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
C1-inhibitor
C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation but also as the major regulator of the contact system. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/ L. The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also inhibits proteases of the fibrinolytic, clotting, and kinin pathways. Note that C1-inhibitor is the most important physiological inhibitor of plasma kallikrein, fXIa, and fXIIa. Proteomics ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Betibeglogene Autotemcel
Betibeglogene autotemcel, sold under the brand name Zynteglo, is a medication for the treatment for beta thalassemia. It was developed by Bluebird Bio and was given breakthrough therapy designation by the U.S. Food and Drug Administration in February 2015. The most common adverse reactions include reduced platelet and other blood cell levels, as well as mucositis, febrile neutropenia, vomiting, pyrexia (fever), alopecia (hair loss), epistaxis (nosebleed), abdominal pain, musculoskeletal pain, cough, headache, diarrhea, rash, constipation, nausea, decreased appetite, pigmentation disorder and pruritus (itch). It was approved for medical use in the European Union in May 2019, Text was copied from this source which is © European Medicines Agency. Reproduction is authorized provided the source is acknowledged. and in the United States in August 2022. Medical uses Betibeglogene autotemcel is indicated for the treatment of people twelve years and older with transfusion-dependent b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Crizanlizumab
Crizanlizumab, sold under the brand name Adakveo & Ryverna both by Novartis, is a monoclonal antibody medication that binds to P-selectin. It is a drug used to reduce the frequency of vaso-occlusive crisis in people aged 16 years and older who have sickle cell anemia. Vaso-occlusive crisis is a common and painful complication of sickle cell disease that occurs when blood circulation is obstructed by sickled red blood cells (red cells are usually round and flexible, but sometimes many red cells in a person with sickle cell anemia will become rigid and crescent-shaped due to polymerization of hemoglobin). The result of the Phase II SUSTAIN clinical trial was published in December 2016, and in November 2019, crizanlizumab-tmca was approved in the United States. The U.S. Food and Drug Administration (FDA) considers it to be a first-in-class medication. Pathophysiology P-selectin molecules are present on the surface of activated platelets and vascular endothelial cells and have been ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Berotralstat
Berotralstat, sold under the brand name Orladeyo, is a medication used to prevent attacks of hereditary angioedema (HAE) in people aged twelve years and older. The most common side effects include abdominal pain, vomiting, diarrhea, back pain, and heartburn. Berotralstat was approved for medical use in the United States in December 2020, and in the European Union in April 2021. History Berotralstat was approved based on evidence from one clinical trial (Trial 1 /NCT03485911) of 120 participants with hereditary angioedema Hereditary angioedema (HAE) is a disorder that results in recurrent attacks of severe swelling. The swelling most commonly affects the arms, legs, face, intestinal tract, and airway. If the intestinal tract is affected, abdominal pain and vomitin .... The trial was conducted at 40 sites in the United States, the European Union, and Canada. Trial investigators evaluated participants 12 years and older with hereditary angioedema for eight weeks to determine ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lanadelumab
Lanadelumab, sold under the brand name Takhzyro, is a human monoclonal antibody (class IgG1 kappa) that targets plasma kallikrein (pKal) in order to promote prevention of angioedema in people with hereditary angioedema. Lanadelumab, was approved in the United States as the first monoclonal antibody indicated for prophylactic treatment to prevent hereditary angioedema (HAE) attacks. Lanadelumab is the first treatment for hereditary angioedema (HEA) prevention made by using cells within a lab, not human plasma. Common side effects include pain associated with injection site reactions, injection site bruising, upper respiratory infection, headache, rash, myalgia, dizziness, and diarrhea. The US Food and Drug Administration (FDA) approved the use of lanadelumab in August 2018, for people that are 12 years and older and have either type I or type II hereditary angioedema (HEA). Medical uses In the United States, lanadelumab is indicated for the prophylaxis of hereditary angio ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Conestat Alfa
C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation but also as the major regulator of the contact system. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/ L. The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also inhibits proteases of the fibrinolytic, clotting, and kinin pathways. Note that C1-inhibitor is the most important physiological inhibitor of plasma kallikrein, fXIa, and fXIIa. Proteomics ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ecallantide
Ecallantide (trade name Kalbitor) is a drug used for the treatment of hereditary angioedema (HAE) and in the prevention of blood loss in cardiothoracic surgery. It is an inhibitor of the protein kallikrein and a 60-amino acid polypeptide which was developed from a Kunitz domain through phage display to mimic antibodies inhibiting kallikrein. Medical uses Angioedema On November 27, 2009, ecallantide was approved by the FDA for the treatment of acute attacks of hereditary angioedema for persons over 16 years of age. A single dose requires three separate injections, which are given under the skin. Ecallantide does not appear to be efficacious for the treatment of angioedema due to ACE inhibitors. Adverse effects The most common adverse effects are headache, nausea, fatigue and diarrhea. Less common, but observed in more than 5% of patients in clinical trials, are respiratory tract infections, fever, vomiting, itching and upper abdominal pain. Up to 4% of patients showed anaphylax ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Icatibant
Icatibant, sold under the brand name Firazyr, is a medication for the symptomatic treatment of acute attacks of hereditary angioedema (HAE) in adults with C1-esterase-inhibitor deficiency. It is not effective in angioedema caused by medication from the ACE inhibitor class. It is a peptidomimetic consisting of ten amino acids, which is a selective and specific antagonist of bradykinin B2 receptors. Mechanism of action Bradykinin is a peptide-based hormone that is formed locally in tissues, very often in response to a trauma. It increases vessel permeability, dilates blood vessels and causes smooth muscle cells to contract. Bradykinin plays an important role as the mediator of pain. Surplus bradykinin Bradykinin (BK) (Greek brady-, slow; -kinin, kīn(eîn) to move) is a peptide that promotes inflammation. It causes arterioles to dilate (enlarge) via the release of prostacyclin, nitric oxide, and endothelium-derived hyperpolarizing factor and ... is responsible for the typi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Blood Plasma
Blood plasma is a light amber-colored liquid component of blood in which blood cells are absent, but contains proteins and other constituents of whole blood in suspension. It makes up about 55% of the body's total blood volume. It is the intravascular part of extracellular fluid (all body fluid outside cells). It is mostly water (up to 95% by volume), and contains important dissolved proteins (6–8%; e.g., serum albumins, globulins, and fibrinogen), glucose, clotting factors, electrolytes (, , , , , etc.), hormones, carbon dioxide (plasma being the main medium for excretory product transportation), and oxygen. It plays a vital role in an intravascular osmotic effect that keeps electrolyte concentration balanced and protects the body from infection and other blood-related disorders. Blood plasma is separated from the blood by spinning a vessel of fresh blood containing an anticoagulant in a centrifuge until the blood cells fall to the bottom of the tube. The blood plasma is t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hereditary Angioedema
Hereditary angioedema (HAE) is a disorder that results in recurrent attacks of severe swelling. The swelling most commonly affects the arms, legs, face, intestinal tract, and airway. If the intestinal tract is affected, abdominal pain and vomiting may occur. Swelling of the airway can result in its obstruction and trouble breathing. Without preventive treatment, attacks typically occur every two weeks and last for a few days. There are three main types of HAE. Types I and II are caused by a mutation in the '' SERPING1'' gene, which makes the C1 inhibitor protein, while type III is often due to a mutation in the '' F12'' (factor XII) gene. The result is increased levels of bradykinin, which promotes swelling. The condition may be inherited in an autosomal dominant manner or occur as a new mutation. Triggers for an attack may include minor trauma or stress, but attacks often occur without any obvious preceding event. Diagnosis of types I and II is based on measurement of C4 and C ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. Almost all metabolic processes in the cell need enzyme catalysis in order to occur at rates fast enough to sustain life. Metabolic pathways depend upon enzymes to catalyze individual steps. The study of enzymes is called ''enzymology'' and the field of pseudoenzyme analysis recognizes that during evolution, some enzymes have lost the ability to carry out biological catalysis, which is often reflected in their amino acid sequences and unusual 'pseudocatalytic' properties. Enzymes are known to catalyze more than 5,000 biochemical reaction types. Other biocatalysts are catalytic RNA molecules, called ribozymes. Enzymes' specificity comes from their unique three-dimensional structures. Like all catalysts, enzymes increase the reaction ra ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
