Group of hemorrhagic disorders in which the VON WILLEBRAND FACTOR is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.
A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in VON WILLEBRAND DISEASES is due to the deficiency of this factor.
A subtype of von Willebrand disease that results from qualitative deficiencies of VON WILLEBRAND FACTOR. The subtype is divided into several variants with each variant having a distinctive pattern of PLATELET-interaction.
A subtype of von Willebrand disease that results from a partial deficiency of VON WILLEBRAND FACTOR.
A subtype of von Willebrand disease that results from a total or near total deficiency of VON WILLEBRAND FACTOR.
An antibiotic mixture of two components, A and B, obtained from Nocardia lurida (or the same substance produced by any other means). It is no longer used clinically because of its toxicity. It causes platelet agglutination and blood coagulation and is used to assay those functions in vitro.
Blood-coagulation factor VIII. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation and this action is markedly enhanced by small amounts of thrombin.
A synthetic analog of the pituitary hormone, ARGININE VASOPRESSIN. Its action is mediated by the VASOPRESSIN receptor V2. It has prolonged antidiuretic activity, but little pressor effects. It also modulates levels of circulating FACTOR VIII and VON WILLEBRAND FACTOR.
Duration of blood flow after skin puncture. This test is used as a measure of capillary and platelet function.
Platelet membrane glycoprotein complex essential for normal platelet adhesion and clot formation at sites of vascular injury. It is composed of three polypeptides, GPIb alpha, GPIb beta, and GPIX. Glycoprotein Ib functions as a receptor for von Willebrand factor and for thrombin. Congenital deficiency of the GPIb-IX complex results in Bernard-Soulier syndrome. The platelet glycoprotein GPV associates with GPIb-IX and is also absent in Bernard-Soulier syndrome.
An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.
Venoms from snakes of the subfamily Crotalinae or pit vipers, found mostly in the Americas. They include the rattlesnake, cottonmouth, fer-de-lance, bushmaster, and American copperhead. Their venoms contain nontoxic proteins, cardio-, hemo-, cyto-, and neurotoxins, and many enzymes, especially phospholipases A. Many of the toxins have been characterized.
Surface glycoproteins on platelets which have a key role in hemostasis and thrombosis such as platelet adhesion and aggregation. Many of these are receptors.
Agents acting to arrest the flow of blood. Absorbable hemostatics arrest bleeding either by the formation of an artificial clot or by providing a mechanical matrix that facilitates clotting when applied directly to the bleeding surface. These agents function more at the capillary level and are not effective at stemming arterial or venous bleeding under any significant intravascular pressure.
Rod-shaped storage granules for VON WILLEBRAND FACTOR specific to endothelial cells.
The process whereby PLATELETS adhere to something other than platelets, e.g., COLLAGEN; BASEMENT MEMBRANE; MICROFIBRILS; or other "foreign" surfaces.
The attachment of PLATELETS to one another. This clumping together can be induced by a number of agents (e.g., THROMBIN; COLLAGEN) and is part of the mechanism leading to the formation of a THROMBUS.
Laboratory tests for evaluating the individual's clotting mechanism.
The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
A hereditary motor and sensory neuropathy transmitted most often as an autosomal dominant trait and characterized by progressive distal wasting and loss of reflexes in the muscles of the legs (and occasionally involving the arms). Onset is usually in the second to fourth decade of life. This condition has been divided into two subtypes, hereditary motor and sensory neuropathy (HMSN) types I and II. HMSN I is associated with abnormal nerve conduction velocities and nerve hypertrophy, features not seen in HMSN II. (Adams et al., Principles of Neurology, 6th ed, p1343)
The process which spontaneously arrests the flow of BLOOD from vessels carrying blood under pressure. It is accomplished by contraction of the vessels, adhesion and aggregation of formed blood elements (eg. ERYTHROCYTE AGGREGATION), and the process of BLOOD COAGULATION.
Spontaneous or near spontaneous bleeding caused by a defect in clotting mechanisms (BLOOD COAGULATION DISORDERS) or another abnormality causing a structural flaw in the blood vessels (HEMOSTATIC DISORDERS).
The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.
A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair.
Bleeding or escape of blood from a vessel.
An autosomal recessively inherited glycogen storage disease caused by GLUCAN 1,4-ALPHA-GLUCOSIDASE deficiency. Large amounts of GLYCOGEN accumulate in the LYSOSOMES of skeletal muscle (MUSCLE, SKELETAL); HEART; LIVER; SPINAL CORD; and BRAIN. Three forms have been described: infantile, childhood, and adult. The infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (CARDIOMYOPATHY, HYPERTROPHIC). The childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. The adult form consists of a slowly progressive proximal myopathy. (From Muscle Nerve 1995;3:S61-9; Menkes, Textbook of Child Neurology, 5th ed, pp73-4)
A family of membrane-anchored glycoproteins that contain a disintegrin and metalloprotease domain. They are responsible for the proteolytic cleavage of many transmembrane proteins and the release of their extracellular domain.
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.
The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Disorders caused by abnormalities in platelet count or function.
Agents capable of exerting a harmful effect on the body.
The process of generating thrombocytes (BLOOD PLATELETS) from the pluripotent HEMATOPOIETIC STEM CELLS in the BONE MARROW via the MEGAKARYOCYTES. The humoral factor with thrombopoiesis-stimulating activity is designated THROMBOPOIETIN.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.
Proteins prepared by recombinant DNA technology.
Endogenous substances, usually proteins, that are involved in the blood coagulation process.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
An individual having different alleles at one or more loci regarding a specific character.

Gain-of-function GPIb ELISA assay for VWF activity in the Zimmerman Program for the Molecular and Clinical Biology of VWD. (1/5)

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Pathologic shear triggers shedding of vascular receptors: a novel mechanism for down-regulation of platelet glycoprotein VI in stenosed coronary vessels. (2/5)

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The genetics of Canadian type 3 von Willebrand disease: further evidence for co-dominant inheritance of mutant alleles. (3/5)

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The molecular characterization of von Willebrand disease: good in parts. (4/5)

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Terminal platelet production is regulated by von Willebrand factor. (5/5)

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Von Willebrand disease (vWD) is a genetic bleeding disorder caused by deficiency or dysfunction of the von Willebrand factor (VWF), a protein involved in blood clotting. The VWF plays a crucial role in the formation of a stable platelet plug during the process of hemostasis, which helps to stop bleeding.

There are three main types of vWD:

1. Type 1: This is the most common form, characterized by a partial quantitative deficiency of functional VWF. Bleeding symptoms are usually mild.
2. Type 2: In this type, there is a qualitative defect in the VWF protein leading to various subtypes (2A, 2B, 2M, and 2N) with different bleeding patterns. Symptoms can range from mild to severe.
3. Type 3: This is the most severe form of vWD, characterized by a near or complete absence of functional VWF and Factor VIII. Affected individuals have a high risk of spontaneous and severe bleeding episodes.

The clinical manifestations of vWD include easy bruising, prolonged nosebleeds (epistaxis), heavy menstrual periods in women, and excessive bleeding after dental procedures, surgeries, or trauma. The diagnosis is made based on laboratory tests that assess VWF antigen levels, VWF activity, and Factor VIII coagulant activity. Treatment options include desmopressin (DDAVP) to stimulate the release of VWF from endothelial cells, recombinant VWF, or plasma-derived VWF concentrates, and antifibrinolytic agents like tranexamic acid to reduce bleeding.

Von Willebrand factor (vWF) is a large multimeric glycoprotein that plays a crucial role in hemostasis, the process which leads to the cessation of bleeding and the formation of a blood clot. It was named after Erik Adolf von Willebrand, a Finnish physician who first described the disorder associated with its deficiency, known as von Willebrand disease (vWD).

The primary functions of vWF include:

1. Platelet adhesion and aggregation: vWF mediates the initial attachment of platelets to damaged blood vessel walls by binding to exposed collagen fibers and then interacting with glycoprotein Ib (GPIb) receptors on the surface of platelets, facilitating platelet adhesion. Subsequently, vWF also promotes platelet-platelet interactions (aggregation) through its interaction with platelet glycoprotein IIb/IIIa (GPIIb/IIIa) receptors under high shear stress conditions found in areas of turbulent blood flow, such as arterioles and the capillary bed.

2. Transport and stabilization of coagulation factor VIII: vWF serves as a carrier protein for coagulation factor VIII (FVIII), protecting it from proteolytic degradation and maintaining its stability in circulation. This interaction between vWF and FVIII is essential for the proper functioning of the coagulation cascade, particularly in the context of vWD, where impaired FVIII function can lead to bleeding disorders.

3. Wound healing: vWF contributes to wound healing by promoting platelet adhesion and aggregation at the site of injury, which facilitates the formation of a provisional fibrin-based clot that serves as a scaffold for tissue repair and regeneration.

In summary, von Willebrand factor is a vital hemostatic protein involved in platelet adhesion, aggregation, coagulation factor VIII stabilization, and wound healing. Deficiencies or dysfunctions in vWF can lead to bleeding disorders such as von Willebrand disease.

Von Willebrand disease (VWD) is a genetic bleeding disorder caused by deficiency or dysfunction of the von Willebrand factor (VWF), a protein involved in blood clotting. There are several types of VWD, and type 2 is further divided into four subtypes (2A, 2B, 2M, and 2N) based on the specific defects in the VWF protein.

Type 2 von Willebrand disease is characterized by qualitative abnormalities in the VWF protein, which affect its ability to function properly. The four subtypes of type 2 VWD are defined as follows:

* Type 2A: This subtype is caused by a decrease in the amount of high molecular weight multimers (HMWM) of VWF, which are essential for effective platelet adhesion and clot formation. The reduction in HMWM leads to a prolonged bleeding time and increased susceptibility to bleeding.
* Type 2B: This subtype is characterized by an increased affinity of VWF for platelets, leading to the formation of large platelet aggregates and a decrease in the amount of circulating VWF. This results in a shortened bleeding time but increased bleeding severity due to the loss of HMWM.
* Type 2M: This subtype is caused by defects in the VWF protein that affect its ability to bind to platelets, leading to a decrease in platelet adhesion and clot formation. The HMWM are present but do not function properly, resulting in a prolonged bleeding time.
* Type 2N: This subtype is characterized by a decreased affinity of VWF for factor VIII, which is necessary for the normal coagulation cascade. This results in a decrease in the half-life of factor VIII and an increased risk of bleeding, particularly during surgery or trauma.

In summary, type 2 von Willebrand disease is a genetic bleeding disorder caused by qualitative abnormalities in the VWF protein, leading to defects in platelet adhesion and clot formation. The four subtypes of type 2 VWD are defined based on specific defects in the VWF protein that affect its ability to bind to platelets, factor VIII, or both.

Von Willebrand disease (VWD) is a genetic bleeding disorder caused by deficiency or abnormality of the von Willebrand factor (VWF), a protein involved in blood clotting. Type 1 VWD is the most common form and is characterized by a partial decrease in the amount of functional VWF in the blood, which can lead to prolonged bleeding times after injury or surgery. The symptoms are usually mild to moderate and may include easy bruising, nosebleeds, heavy menstrual periods, and excessive bleeding following dental work or childbirth. Type 1 VWD is inherited in an autosomal dominant manner, meaning that a person has a 50% chance of inheriting the disorder if one of their parents has it.

Von Willebrand disease (VWD) is a genetic bleeding disorder caused by deficiency or abnormality of the von Willebrand factor (VWF), a protein involved in blood clotting. Type 3 is the most severe form of VWD, characterized by extremely low levels or complete absence of VWF and Factor VIII, another clotting factor. This results in a significant impairment of the primary hemostasis, leading to spontaneous and severe bleeding episodes, including mucocutaneous bleeding (nosebleeds, gum bleeding, skin bruising), gastrointestinal bleeding, joint bleeds, and menorrhagia in women. Type 3 VWD is inherited in an autosomal recessive manner, meaning that an individual must inherit two copies of the abnormal gene, one from each parent, to have the disease.

Ristocetin is not a medical condition but a type of antibiotic used to treat infections caused by certain Gram-positive bacteria that are resistant to other antibiotics. Ristocetin is an glycopeptide antibiotic, which works by binding to the bacterial cell wall and inhibiting its synthesis, leading to bacterial death. It is not commonly used due to its potential to cause blood disorders, such as thrombocytopenia (low platelet count) and platelet aggregation.

In medical literature, ristocetin is also known for its use in the laboratory setting as a reagent for the platelet function test, called the ristocetin-induced platelet aggregation (RIPA) assay. This test is used to evaluate the ability of platelets to aggregate and form clots in response to ristocetin, which can help diagnose certain bleeding disorders such as Bernard-Soulier syndrome and von Willebrand disease.

Factor VIII is a protein in the blood that is essential for normal blood clotting. It is also known as antihemophilic factor (AHF). Deficiency or dysfunction of this protein results in hemophilia A, a genetic disorder characterized by prolonged bleeding and easy bruising. Factor VIII works together with other proteins to help form a clot and stop bleeding at the site of an injury. It acts as a cofactor for another clotting factor, IX, in the so-called intrinsic pathway of blood coagulation. Intravenous infusions of Factor VIII concentrate are used to treat and prevent bleeding episodes in people with hemophilia A.

Desmopressin, also known as 1-deamino-8-D-arginine vasopressin (dDAVP), is a synthetic analogue of the natural hormone arginine vasopressin. It is commonly used in medical practice for the treatment of diabetes insipidus, a condition characterized by excessive thirst and urination due to lack of antidiuretic hormone (ADH).

Desmopressin works by binding to V2 receptors in the kidney, which leads to increased water reabsorption and reduced urine production. It also has some effect on V1 receptors, leading to vasoconstriction and increased blood pressure. However, its primary use is for its antidiuretic effects.

In addition to its use in diabetes insipidus, desmopressin may also be used to treat bleeding disorders such as hemophilia and von Willebrand disease, as it can help to promote platelet aggregation and reduce bleeding times. It is available in various forms, including nasal sprays, injectable solutions, and oral tablets or dissolvable films.

Bleeding time is a medical test that measures the time it takes for a small blood vessel to stop bleeding after being cut. It's used to evaluate platelet function and the effectiveness of blood clotting. The most common method used to measure bleeding time is the Ivy method, which involves making a standardized incision on the forearm and measuring the time it takes for the bleeding to stop. A normal bleeding time ranges from 2 to 9 minutes, but this can vary depending on the specific method used. Prolonged bleeding time may indicate an impairment in platelet function or clotting factor deficiency.

The platelet glycoprotein GPIb-IX complex is a crucial receptor on the surface of platelets that plays a vital role in hemostasis and thrombosis. It is a heterotetrameric transmembrane protein complex composed of two disulfide-linked glycoprotein subunits, GPIbα, GPIbβ, GPV (Glycoprotein V), and GPIX (Glycoprotein IX).

The GPIb-IX complex is responsible for the initial interaction between platelets and von Willebrand factor (vWF) in the circulation. When blood vessels are damaged, exposed collagen recruits vWF to the site of injury, where it binds to the GPIbα subunit of the GPIb-IX complex, leading to platelet adhesion and activation. This interaction is critical for primary hemostasis, which helps prevent excessive blood loss from injured vessels.

Genetic mutations or deficiencies in the genes encoding these glycoproteins can lead to bleeding disorders such as Bernard-Soulier syndrome, a rare autosomal recessive disorder characterized by thrombocytopenia and large platelets with impaired vWF binding and platelet adhesion.

Glycogen Storage Disease Type I (GSD I) is a rare inherited metabolic disorder caused by deficiency of the enzyme glucose-6-phosphatase, which is necessary for the liver to release glucose into the bloodstream. This leads to an accumulation of glycogen in the liver and abnormally low levels of glucose in the blood (hypoglycemia).

There are two main subtypes of GSD I: Type Ia and Type Ib. In Type Ia, there is a deficiency of both glucose-6-phosphatase enzyme activity in the liver, kidney, and intestine, leading to hepatomegaly (enlarged liver), hypoglycemia, lactic acidosis, hyperlipidemia, and growth retardation. Type Ib is characterized by a deficiency of glucose-6-phosphatase enzyme activity only in the neutrophils, leading to recurrent bacterial infections.

GSD I requires lifelong management with frequent feedings, high-carbohydrate diet, and avoidance of fasting to prevent hypoglycemia. In some cases, treatment with continuous cornstarch infusions or liver transplantation may be necessary.

Blood platelets, also known as thrombocytes, are small, colorless cell fragments in our blood that play an essential role in normal blood clotting. They are formed in the bone marrow from large cells called megakaryocytes and circulate in the blood in an inactive state until they are needed to help stop bleeding. When a blood vessel is damaged, platelets become activated and change shape, releasing chemicals that attract more platelets to the site of injury. These activated platelets then stick together to form a plug, or clot, that seals the wound and prevents further blood loss. In addition to their role in clotting, platelets also help to promote healing by releasing growth factors that stimulate the growth of new tissue.

Crotalid venoms are the toxic secretions produced by the members of the Crotalinae subfamily, also known as pit vipers. This group includes rattlesnakes, cottonmouths (or water moccasins), and copperheads, which are native to the Americas, as well as Old World vipers found in Asia and Europe, such as gaboon vipers and saw-scaled vipers.

Crotalid venoms are complex mixtures of various bioactive molecules, including enzymes, proteins, peptides, and other low molecular weight components. They typically contain a variety of pharmacologically active components, such as hemotoxic and neurotoxic agents, which can cause extensive local tissue damage, coagulopathy, cardiovascular dysfunction, and neuromuscular disorders in the victim.

The composition of crotalid venoms can vary significantly between different species and even among individual specimens within the same species. This variability is influenced by factors such as geographic location, age, sex, diet, and environmental conditions. As a result, the clinical manifestations of crotalid envenomation can be highly variable, ranging from mild local reactions to severe systemic effects that may require intensive medical treatment and supportive care.

Crotalid venoms have been the subject of extensive research in recent years due to their potential therapeutic applications. For example, certain components of crotalid venoms have shown promise as drugs for treating various medical conditions, such as cardiovascular diseases, pain, and inflammation. However, further studies are needed to fully understand the mechanisms of action of these venom components and to develop safe and effective therapies based on them.

Platelet membrane glycoproteins are specialized proteins found on the surface of platelets, which are small blood cells responsible for clotting. These glycoproteins play crucial roles in various processes related to hemostasis and thrombosis, including platelet adhesion, activation, and aggregation.

There are several key platelet membrane glycoproteins, such as:

1. Glycoprotein (GP) Ia/IIa (also known as integrin α2β1): This glycoprotein mediates the binding of platelets to collagen fibers in the extracellular matrix, facilitating platelet adhesion and activation.
2. GP IIb/IIIa (also known as integrin αIIbβ3): This is the most abundant glycoprotein on the platelet surface and functions as a receptor for fibrinogen, von Willebrand factor, and other adhesive proteins. Upon activation, GP IIb/IIIa undergoes conformational changes that enable it to bind these ligands, leading to platelet aggregation and clot formation.
3. GPIb-IX-V: This glycoprotein complex is involved in the initial tethering and adhesion of platelets to von Willebrand factor (vWF) in damaged blood vessels. It consists of four subunits: GPIbα, GPIbβ, GPIX, and GPV.
4. GPVI: This glycoprotein is essential for platelet activation upon contact with collagen. It associates with the Fc receptor γ-chain (FcRγ) to form a signaling complex that triggers intracellular signaling pathways, leading to platelet activation and aggregation.

Abnormalities in these platelet membrane glycoproteins can lead to bleeding disorders or thrombotic conditions. For example, mutations in GPIIb/IIIa can result in Glanzmann's thrombasthenia, a severe bleeding disorder characterized by impaired platelet aggregation. On the other hand, increased expression or activation of these glycoproteins may contribute to the development of arterial thrombosis and cardiovascular diseases.

Hemostatics are substances or agents that promote bleeding cessation or prevent the spread of bleeding. They can act in various ways, such as by stimulating the body's natural clotting mechanisms, constricting blood vessels to reduce blood flow, or forming a physical barrier to block the bleeding site.

Hemostatics are often used in medical settings to manage wounds, injuries, and surgical procedures. They can be applied directly to the wound as a powder, paste, or gauze, or they can be administered systemically through intravenous injection. Examples of hemostatic agents include fibrin sealants, collagen-based products, thrombin, and oxidized regenerated cellulose.

It's important to note that while hemostatics can be effective in controlling bleeding, they should be used with caution and only under the guidance of a healthcare professional. Inappropriate use or overuse of hemostatic agents can lead to complications such as excessive clotting, thrombosis, or tissue damage.

Weibel-Palade bodies are rod-shaped, membrane-bound organelles found in the cytoplasm of endothelial cells, which line the interior surface of blood vessels. They were first described by Edwin Weibel and George Palade in 1964. These organelles are unique to endothelial cells and serve as storage sites for von Willebrand factor (vWF) and other proteins involved in hemostasis, inflammation, and vasomotor functions.

The main components of Weibel-Palade bodies include:

1. Von Willebrand factor (vWF): A multimeric glycoprotein that plays a crucial role in platelet adhesion and aggregation at the site of vascular injury, as well as mediating the transport of coagulation factors VIII and V.
2. P-selectin: A cell adhesion molecule that facilitates leukocyte rolling and recruitment to sites of inflammation.
3. Endothelial nitric oxide synthase (eNOS): An enzyme responsible for the production of nitric oxide, a potent vasodilator that regulates vascular tone and blood flow.
4. Angiopoietin-2: A growth factor involved in angiogenesis and vascular remodeling.
5. Tissue plasminogen activator (tPA): A serine protease that plays a role in fibrinolysis, the process of breaking down blood clots.

Upon stimulation by various agonists such as thrombin, histamine, or vascular endothelial growth factor (VEGF), Weibel-Palade bodies undergo exocytosis, releasing their contents into the extracellular space. This process contributes to hemostatic responses, inflammatory reactions, and modulation of vascular tone.

Platelet adhesiveness refers to the ability of platelets, which are small blood cells that help your body form clots to prevent excessive bleeding, to stick to other cells or surfaces. This process is crucial in hemostasis, the process of stopping bleeding after injury to a blood vessel.

When the endothelium (the lining of blood vessels) is damaged, subendothelial structures are exposed, which can trigger platelet adhesion. Platelets then change shape and release chemical signals that cause other platelets to clump together, forming a platelet plug. This plug helps to seal the damaged vessel and prevent further bleeding.

Platelet adhesiveness is influenced by several factors, including the presence of von Willebrand factor (vWF), a protein in the blood that helps platelets bind to damaged vessels, and the expression of glycoprotein receptors on the surface of platelets. Abnormalities in platelet adhesiveness can lead to bleeding disorders or thrombotic conditions.

Platelet aggregation is the clumping together of platelets (thrombocytes) in the blood, which is an essential step in the process of hemostasis (the stopping of bleeding) after injury to a blood vessel. When the inner lining of a blood vessel is damaged, exposure of subendothelial collagen and tissue factor triggers platelet activation. Activated platelets change shape, become sticky, and release the contents of their granules, which include ADP (adenosine diphosphate).

ADP then acts as a chemical mediator to attract and bind additional platelets to the site of injury, leading to platelet aggregation. This forms a plug that seals the damaged vessel and prevents further blood loss. Platelet aggregation is also a crucial component in the formation of blood clots (thrombosis) within blood vessels, which can have pathological consequences such as heart attacks and strokes if they obstruct blood flow to vital organs.

Blood coagulation tests, also known as coagulation studies or clotting tests, are a series of medical tests used to evaluate the blood's ability to clot. These tests measure the functioning of various clotting factors and regulatory proteins involved in the coagulation cascade, which is a complex process that leads to the formation of a blood clot to prevent excessive bleeding.

The most commonly performed coagulation tests include:

1. Prothrombin Time (PT): Measures the time it takes for a sample of plasma to clot after the addition of calcium and tissue factor, which activates the extrinsic pathway of coagulation. The PT is reported in seconds and can be converted to an International Normalized Ratio (INR) to monitor anticoagulant therapy.
2. Activated Partial Thromboplastin Time (aPTT): Measures the time it takes for a sample of plasma to clot after the addition of calcium, phospholipid, and a contact activator, which activates the intrinsic pathway of coagulation. The aPTT is reported in seconds and is used to monitor heparin therapy.
3. Thrombin Time (TT): Measures the time it takes for a sample of plasma to clot after the addition of thrombin, which directly converts fibrinogen to fibrin. The TT is reported in seconds and can be used to detect the presence of fibrin degradation products or abnormalities in fibrinogen function.
4. Fibrinogen Level: Measures the amount of fibrinogen, a protein involved in clot formation, present in the blood. The level is reported in grams per liter (g/L) and can be used to assess bleeding risk or the effectiveness of fibrinogen replacement therapy.
5. D-dimer Level: Measures the amount of D-dimer, a protein fragment produced during the breakdown of a blood clot, present in the blood. The level is reported in micrograms per milliliter (µg/mL) and can be used to diagnose or exclude venous thromboembolism (VTE), such as deep vein thrombosis (DVT) or pulmonary embolism (PE).

These tests are important for the diagnosis, management, and monitoring of various bleeding and clotting disorders. They can help identify the underlying cause of abnormal bleeding or clotting, guide appropriate treatment decisions, and monitor the effectiveness of therapy. It is essential to interpret these test results in conjunction with a patient's clinical presentation and medical history.

Hemophilia A is a genetic bleeding disorder caused by a deficiency in clotting factor VIII. This results in impaired blood clotting and prolonged bleeding, particularly after injuries or surgeries. Symptoms can range from mild to severe, with the most severe form resulting in spontaneous bleeding into joints and muscles, leading to pain, swelling, and potential joint damage over time. Hemophilia A primarily affects males, as it is an X-linked recessive disorder, and is usually inherited from a carrier mother. However, about one third of cases result from a spontaneous mutation in the gene for factor VIII. Treatment typically involves replacement therapy with infusions of factor VIII concentrates to prevent or control bleeding episodes.

Charcot-Marie-Tooth disease (CMT) is a group of inherited disorders that cause nerve damage, primarily affecting the peripheral nerves. These are the nerves that transmit signals between the brain and spinal cord to the rest of the body. CMT affects both motor and sensory nerves, leading to muscle weakness and atrophy, as well as numbness or tingling in the hands and feet.

The disease is named after the three physicians who first described it: Jean-Martin Charcot, Pierre Marie, and Howard Henry Tooth. CMT is characterized by its progressive nature, meaning symptoms typically worsen over time, although the rate of progression can vary significantly among individuals.

There are several types of CMT, classified based on their genetic causes and patterns of inheritance. The two most common forms are CMT1 and CMT2:

1. CMT1: This form is caused by mutations in the genes responsible for the myelin sheath, which insulates peripheral nerves and allows for efficient signal transmission. As a result, demyelination occurs, slowing down nerve impulses and causing muscle weakness, particularly in the lower limbs. Symptoms usually begin in childhood or adolescence and include foot drop, high arches, and hammertoes.
2. CMT2: This form is caused by mutations in the genes responsible for the axons, the nerve fibers that transmit signals within peripheral nerves. As a result, axonal degeneration occurs, leading to muscle weakness and atrophy. Symptoms usually begin in early adulthood and progress more slowly than CMT1. They primarily affect the lower limbs but can also involve the hands and arms.

Diagnosis of CMT typically involves a combination of clinical evaluation, family history, nerve conduction studies, and genetic testing. While there is no cure for CMT, treatment focuses on managing symptoms and maintaining mobility and function through physical therapy, bracing, orthopedic surgery, and pain management.

Hemostasis is the physiological process that occurs to stop bleeding (bleeding control) when a blood vessel is damaged. This involves the interaction of platelets, vasoconstriction, and blood clotting factors leading to the formation of a clot. The ultimate goal of hemostasis is to maintain the integrity of the vascular system while preventing excessive blood loss.

Hemorrhagic disorders are medical conditions characterized by abnormal bleeding due to impaired blood clotting. This can result from deficiencies in coagulation factors, platelet dysfunction, or the use of medications that interfere with normal clotting processes. Examples include hemophilia, von Willebrand disease, and disseminated intravascular coagulation (DIC). Treatment often involves replacing the missing clotting factor or administering medications to help control bleeding.

I must clarify that the term "pedigree" is not typically used in medical definitions. Instead, it is often employed in genetics and breeding, where it refers to the recorded ancestry of an individual or a family, tracing the inheritance of specific traits or diseases. In human genetics, a pedigree can help illustrate the pattern of genetic inheritance in families over multiple generations. However, it is not a medical term with a specific clinical definition.

A point mutation is a type of genetic mutation where a single nucleotide base (A, T, C, or G) in DNA is altered, deleted, or substituted with another nucleotide. Point mutations can have various effects on the organism, depending on the location of the mutation and whether it affects the function of any genes. Some point mutations may not have any noticeable effect, while others might lead to changes in the amino acids that make up proteins, potentially causing diseases or altering traits. Point mutations can occur spontaneously due to errors during DNA replication or be inherited from parents.

Hemorrhage is defined in the medical context as an excessive loss of blood from the circulatory system, which can occur due to various reasons such as injury, surgery, or underlying health conditions that affect blood clotting or the integrity of blood vessels. The bleeding may be internal, external, visible, or concealed, and it can vary in severity from minor to life-threatening, depending on the location and extent of the bleeding. Hemorrhage is a serious medical emergency that requires immediate attention and treatment to prevent further blood loss, organ damage, and potential death.

Glycogen Storage Disease Type II, also known as Pompe Disease, is a genetic disorder caused by a deficiency of the enzyme acid alpha-glucosidase (GAA). This enzyme is responsible for breaking down glycogen, a complex sugar that serves as energy storage, within lysosomes. When GAA is deficient, glycogen accumulates in various tissues, particularly in muscle cells, leading to their dysfunction and damage.

The severity of Pompe Disease can vary significantly, depending on the amount of functional enzyme activity remaining. The classic infantile-onset form presents within the first few months of life with severe muscle weakness, hypotonia, feeding difficulties, and respiratory insufficiency. This form is often fatal by 1-2 years of age if left untreated.

A later-onset form, which can present in childhood, adolescence, or adulthood, has a more variable clinical course. Affected individuals may experience progressive muscle weakness, respiratory insufficiency, and cardiomyopathy, although the severity and rate of progression are generally less pronounced than in the infantile-onset form.

Enzyme replacement therapy with recombinant human GAA is available for the treatment of Pompe Disease and has been shown to improve survival and motor function in affected individuals.

ADAM (A Disintegrin And Metalloprotease) proteins are a family of type I transmembrane proteins that contain several distinct domains, including a prodomain, a metalloprotease domain, a disintegrin-like domain, a cysteine-rich domain, a transmembrane domain, and a cytoplasmic tail. These proteins are involved in various biological processes such as cell adhesion, migration, proteolysis, and signal transduction.

ADAM proteins have been found to play important roles in many physiological and pathological conditions, including fertilization, neurodevelopment, inflammation, and cancer metastasis. For example, ADAM12 is involved in the fusion of myoblasts during muscle development, while ADAM17 (also known as TACE) plays a crucial role in the shedding of membrane-bound proteins such as tumor necrosis factor-alpha and epidermal growth factor receptor ligands.

Abnormalities in ADAM protein function have been implicated in various diseases, including cancer, Alzheimer's disease, and arthritis. Therefore, understanding the structure and function of these proteins has important implications for the development of novel therapeutic strategies.

A missense mutation is a type of point mutation in which a single nucleotide change results in the substitution of a different amino acid in the protein that is encoded by the affected gene. This occurs when the altered codon (a sequence of three nucleotides that corresponds to a specific amino acid) specifies a different amino acid than the original one. The function and/or stability of the resulting protein may be affected, depending on the type and location of the missense mutation. Missense mutations can have various effects, ranging from benign to severe, depending on the importance of the changed amino acid for the protein's structure or function.

Glycogen Storage Disease Type III, also known as Cori or Forbes disease, is a rare inherited metabolic disorder caused by deficiency of the debranching enzyme amylo-1,6-glucosidase, which is responsible for breaking down glycogen in the liver and muscles. This results in an abnormal accumulation of glycogen in these organs leading to its associated symptoms.

There are two main types: Type IIIa affects both the liver and muscles, while Type IIIb affects only the liver. Symptoms can include hepatomegaly (enlarged liver), hypoglycemia (low blood sugar), hyperlipidemia (high levels of fats in the blood), and growth retardation. In Type IIIa, muscle weakness and cardiac problems may also occur.

The diagnosis is usually made through biochemical tests and genetic analysis. Treatment often involves dietary management with frequent meals to prevent hypoglycemia, and in some cases, enzyme replacement therapy. However, there is no cure for this condition and life expectancy can be reduced depending on the severity of the symptoms.

Hematologic pregnancy complications refer to disorders related to the blood and blood-forming tissues that occur during pregnancy. These complications can have serious consequences for both the mother and the fetus if not properly managed. Some common hematologic pregnancy complications include:

1. Anemia: A condition characterized by a decrease in the number of red blood cells or hemoglobin in the blood, which can lead to fatigue, weakness, and shortness of breath. Iron-deficiency anemia is the most common type of anemia during pregnancy.
2. Thrombocytopenia: A condition characterized by a decrease in the number of platelets (cells that help blood clot) in the blood. Mild thrombocytopenia is relatively common during pregnancy, but severe thrombocytopenia can increase the risk of bleeding during delivery.
3. Gestational thrombotic thrombocytopenic purpura (GTTP): A rare but serious disorder that can cause blood clots to form in small blood vessels throughout the body, leading to a decrease in the number of platelets and red blood cells. GTTP can cause serious complications such as stroke, kidney failure, and even death if not promptly diagnosed and treated.
4. Disseminated intravascular coagulation (DIC): A condition characterized by abnormal clotting and bleeding throughout the body. DIC can be triggered by various conditions such as severe infections, pregnancy complications, or cancer.
5. Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome: A serious complication of pregnancy that can cause damage to the liver and lead to bleeding. HELLP syndrome is often associated with preeclampsia, a condition characterized by high blood pressure and damage to organs such as the liver and kidneys.

It's important for pregnant women to receive regular prenatal care to monitor for these and other potential complications, and to seek prompt medical attention if any concerning symptoms arise.

A mutation is a permanent change in the DNA sequence of an organism's genome. Mutations can occur spontaneously or be caused by environmental factors such as exposure to radiation, chemicals, or viruses. They may have various effects on the organism, ranging from benign to harmful, depending on where they occur and whether they alter the function of essential proteins. In some cases, mutations can increase an individual's susceptibility to certain diseases or disorders, while in others, they may confer a survival advantage. Mutations are the driving force behind evolution, as they introduce new genetic variability into populations, which can then be acted upon by natural selection.

Blood platelet disorders are conditions that affect the number and/or function of platelets, which are small blood cells that help your body form clots to stop bleeding. Normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. A lower-than-normal platelet count is called thrombocytopenia, while a higher-than-normal platelet count is called thrombocytosis.

There are several types of platelet disorders, including:

1. Immune thrombocytopenia (ITP): A condition in which the immune system mistakenly attacks and destroys platelets, leading to a low platelet count. ITP can be acute (lasting less than six months) or chronic (lasting longer than six months).
2. Thrombotic thrombocytopenic purpura (TTP): A rare but serious condition that causes blood clots to form in small blood vessels throughout the body, leading to a low platelet count, anemia, and other symptoms.
3. Hemolytic uremic syndrome (HUS): A condition that is often caused by a bacterial infection, which can lead to the formation of blood clots in the small blood vessels of the kidneys, resulting in kidney damage and a low platelet count.
4. Hereditary platelet disorders: Some people inherit genetic mutations that can affect the number or function of their platelets, leading to bleeding disorders such as von Willebrand disease or Bernard-Soulier syndrome.
5. Medication-induced thrombocytopenia: Certain medications can cause a decrease in platelet count as a side effect.
6. Platelet dysfunction disorders: Some conditions can affect the ability of platelets to function properly, leading to bleeding disorders such as von Willebrand disease or storage pool deficiency.

Symptoms of platelet disorders may include easy bruising, prolonged bleeding from cuts or injuries, nosebleeds, blood in urine or stools, and in severe cases, internal bleeding. Treatment for platelet disorders depends on the underlying cause and may include medications, surgery, or other therapies.

"Noxae" is a term derived from Latin, which means "causes of damage or injury." In medical contexts, it is used to refer to harmful agents or factors that can cause harm, damage, or disease in an organism or a biological system. These harmful agents can include physical, chemical, or biological factors such as radiation, toxins, infectious microorganisms, and mechanical injuries.

Thrombopoiesis is the process of formation and development of thrombocytes or platelets, which are small, colorless cell fragments in our blood that play an essential role in clotting. Thrombopoiesis occurs inside the bone marrow, where stem cells differentiate into megakaryoblasts, then progressively develop into promegakaryocytes and megakaryocytes. These megakaryocytes subsequently undergo a process called cytoplasmic fragmentation to produce platelets.

The regulation of thrombopoiesis is primarily controlled by the hormone thrombopoietin (TPO), which is produced mainly in the liver and binds to the thrombopoietin receptor (c-Mpl) on megakaryocytes and their precursors. This binding stimulates the proliferation, differentiation, and maturation of megakaryocytes, leading to an increase in platelet production.

Abnormalities in thrombopoiesis can result in conditions such as thrombocytopenia (low platelet count) or thrombocytosis (high platelet count), which may be associated with bleeding disorders or increased risk of thrombosis, respectively.

A phenotype is the physical or biochemical expression of an organism's genes, or the observable traits and characteristics resulting from the interaction of its genetic constitution (genotype) with environmental factors. These characteristics can include appearance, development, behavior, and resistance to disease, among others. Phenotypes can vary widely, even among individuals with identical genotypes, due to differences in environmental influences, gene expression, and genetic interactions.

Glycogen Storage Disease Type IV (GSD IV), also known as Andersen's disease, is a rare inherited metabolic disorder that affects the body's ability to break down glycogen, a complex carbohydrate that serves as a source of energy for the body.

In GSD IV, there is a deficiency in the enzyme called glycogen branching enzyme (GBE), which is responsible for adding branches to the glycogen molecule during its synthesis. This results in an abnormal form of glycogen that accumulates in various organs and tissues, particularly in the liver, heart, and muscles.

The accumulation of this abnormal glycogen can lead to progressive damage and failure of these organs, resulting in a variety of symptoms such as muscle weakness, hypotonia, hepatomegaly (enlarged liver), cardiomyopathy (heart muscle disease), and developmental delay. The severity of the disease can vary widely, with some individuals experiencing milder symptoms while others may have a more severe and rapidly progressing form of the disorder.

Currently, there is no cure for GSD IV, and treatment is focused on managing the symptoms and slowing down the progression of the disease. This may include providing nutritional support, addressing specific organ dysfunction, and preventing complications.

Recombinant proteins are artificially created proteins produced through the use of recombinant DNA technology. This process involves combining DNA molecules from different sources to create a new set of genes that encode for a specific protein. The resulting recombinant protein can then be expressed, purified, and used for various applications in research, medicine, and industry.

Recombinant proteins are widely used in biomedical research to study protein function, structure, and interactions. They are also used in the development of diagnostic tests, vaccines, and therapeutic drugs. For example, recombinant insulin is a common treatment for diabetes, while recombinant human growth hormone is used to treat growth disorders.

The production of recombinant proteins typically involves the use of host cells, such as bacteria, yeast, or mammalian cells, which are engineered to express the desired protein. The host cells are transformed with a plasmid vector containing the gene of interest, along with regulatory elements that control its expression. Once the host cells are cultured and the protein is expressed, it can be purified using various chromatography techniques.

Overall, recombinant proteins have revolutionized many areas of biology and medicine, enabling researchers to study and manipulate proteins in ways that were previously impossible.

Blood coagulation factors, also known as clotting factors, are a group of proteins that play a crucial role in the blood coagulation process. They are essential for maintaining hemostasis, which is the body's ability to stop bleeding after injury.

There are 13 known blood coagulation factors, and they are designated by Roman numerals I through XIII. These factors are produced in the liver and are normally present in an inactive form in the blood. When there is an injury to a blood vessel, the coagulation process is initiated, leading to the activation of these factors in a specific order.

The coagulation cascade involves two pathways: the intrinsic and extrinsic pathways. The intrinsic pathway is activated when there is damage to the blood vessel itself, while the extrinsic pathway is activated by tissue factor released from damaged tissues. Both pathways converge at the common pathway, leading to the formation of a fibrin clot.

Blood coagulation factors work together in a complex series of reactions that involve activation, binding, and proteolysis. When one factor is activated, it activates the next factor in the cascade, and so on. This process continues until a stable fibrin clot is formed.

Deficiencies or abnormalities in blood coagulation factors can lead to bleeding disorders such as hemophilia or thrombosis. Hemophilia is a genetic disorder that affects one or more of the coagulation factors, leading to excessive bleeding and difficulty forming clots. Thrombosis, on the other hand, occurs when there is an abnormal formation of blood clots in the blood vessels, which can lead to serious complications such as stroke or pulmonary embolism.

Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.

A heterozygote is an individual who has inherited two different alleles (versions) of a particular gene, one from each parent. This means that the individual's genotype for that gene contains both a dominant and a recessive allele. The dominant allele will be expressed phenotypically (outwardly visible), while the recessive allele may or may not have any effect on the individual's observable traits, depending on the specific gene and its function. Heterozygotes are often represented as 'Aa', where 'A' is the dominant allele and 'a' is the recessive allele.

Differentiating between type 2B and platelet-type VWD, as well as between type 2N VWD and hemophilia A. Von Willebrand factor ... "Canine von Willebrand Disease - Breed Summaries". ahdc.vet.cornell.edu. 2019-02-08. "Canine von Willebrand Disease". vetgen.com ... GeneReviews/NCBI/NIH/UW entry on von Willebrand Disease NHLBI von Willebrand Disease Expert Panel (January 2008). The Diagnosis ... Sadler JE (April 1994). "A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of ...
Usefulness in type 2A, 2M, or 2N von Willebrand disease is variable. Generally not recommended in 2B and type 3 von Willebrand ... von Willebrand disease, and high blood urea levels. In hemophilia A and von Willebrand disease, it should only be used for mild ... is usually the first line treatment for mild to moderate type 1 von Willebrand disease. It is not recommended in severe disease ... mild or moderate type 1 von Willebrand disease and symptomatic carriers of haemophilia A". Haemophilia. 7 (3): 258-266. doi: ...
This results in Von Willebrand disease type IIA (acquired). Von Willebrand factor is synthesized in the walls of the blood ... They also proposed a possible mechanism for the acquired von Willebrand's disease, noting that von Willebrand factor is most ... "Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter ... and acquired von Willebrand's disease type IIA, which is corrected by surgical replacement of the aortic valve. They also noted ...
LJohnson GS; Turrentine MA; Kraus, KH (1988). "Canine von Willebrand's disease". Veterinary Clinics of North America. 18 (1): ... However the AKC Standard also reads "The question of coat and general type of balance takes precedence over any scoring table ... The breed is subject to a number of hereditary diseases. These include, but are not limited to: Exercise-induced collapse Hip ... Dogs from both Chesapeake Bay shores were recognized as one of three types of Chesapeake Bay Ducking Dog in 1877.[citation ...
Bohemian Terrier' or 'Czech Terrier') is a small terrier type dog originating in Czechoslovakia. The Cesky Terrier was created ... and Type 3 von Willebrand disease, all of which have been documented issues in the breed. Dogs portal List of dog breeds ... Color The Cesky Terrier has two varieties of color: In mature dogs, 3 years or older: 1. Any shade of gray from charcoal to ... Retrieved 3 December 2012. "The Kennel Club". Retrieved 2016-11-26. "In the UK". Cesky Terrier Club (UK). Retrieved 3 December ...
A number of human diseases arise from mutations in VWA domains. The domain is named after the von Willebrand factor (VWF) type ... Von Willebrand factor C and EGF domain-containing protein (VWCE) Von Willebrand factor (VWF) Chordin (CHRD) Chordin-like 1 ( ... Von Willebrand factor, type C (VWFC or VWC)is a protein domain is found in various blood plasma proteins: complement factors B ... "The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform ...
This phenomenon is characterised by a form of von Willebrand disease (type 2a). ADAMTS5 ENSG00000281244 GRCh38: Ensembl release ... ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)-also known as von Willebrand ... Fujimura Y, Matsumoto M, Yagi H, Yoshioka A, Matsui T, Titani K (January 2002). "Von Willebrand factor-cleaving protease and ... Kremer Hovinga JA, Studt JD, Lämmle B (2005). "The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of ...
... and von Willebrand disease. Being a hunt-driven dog, the Jack Russell will usually pursue most creatures that it encounters. ... The type aimed for were not as big as the show Fox Terrier and were usually less than 7 kg (15 lb). Arthur Blake Heinemann ... Some are prone to dislocation of the kneecaps, inherited eye diseases, deafness and Legg Perthes-a disease of the hip joints of ... There are two types, posterior luxation (where the lens slips to the back of the eye) and anterior luxation (where the lens ...
This is useful in the treatment of hemophilia A as well as Von Willebrand disease. In the kidney, AVPR2's primary property is ... Its activity is mediated by the Gs type of G proteins, which stimulate adenylate cyclase. AVPR2 is expressed in the kidney ... Stimulation causes the release of von Willebrand factor and factor VIII from the endothelial cells. Because von Willebrand ... Robben JH, Knoers NV, Deen PM (Aug 2006). "Cell biological aspects of the vasopressin type-2 receptor and aquaporin 2 water ...
A number of human diseases arise from mutations in vWA domains. Secondary structure prediction from 75 aligned vWA sequences ... The von Willebrand factor type A (vWA) domain is a protein domain named after its occurrence in von Willebrand factor (vWF), a ... Perkins SJ, Edwards YJ (1995). "The protein fold of the von Willebrand factor type A domain is predicted to be similar to the ... Bork, P; Rohde, K (1991). "More von Willebrand factor type a domains? Sequence similarities with malaria thrombospondin-related ...
Moake JL (2004). "von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura". Seminars in Hematology. 41 (1): 4 ... This list of autoimmune diseases is categorized by organ and tissue type to help locate diseases that may be similar. Overview ... "Celiac Disease". Archived from the original on 2009-07-20. Meize-Grochowski R (2005). "Celiac disease: a multisystem autoimmune ... These conditions are included here because: The disease was listed in the prior version of this table The disease is included ...
"Use of the collagen-binding assay for von Willebrand factor in the analysis of type 2M von Willebrand disease: a comparison ... ristocetin cofactor assay but has the added benefit in that it helps in the diagnosis of type 2B/pseudo von Willebrand disease ... It measures platelet aggregation with the help of von Willebrand factor (vWF) and exogenous antibiotic ristocetin added in a ... the antibiotic ristocetin causes von Willebrand factor to bind the platelet receptor glycoprotein Ib (GpIb), so when ristocetin ...
Stuttering Triose Phosphate Isomerase deficiency tyrosinemia Von Willebrand Disease G-banding ideograms of human chromosome 12 ... achondrogenesis type 2 collagenopathy, types II and XI cornea plana 2 episodic ataxia hereditary hemorrhagic telangiectasia ... Gilbert F, Kauff N (2000). "Disease genes and chromosomes: disease maps of the human genome.Chromosome 12". Genet Test. 4 (3): ... cblB type MON2: encoding protein Protein MON2 homolog MUCL1: encoding protein Mucin-like protein 1 MYO1A: myosin IA NANOG: NK-2 ...
Nonetheless, there are two exceptions in this falling tendency: coagulation factor VIII and von Willebrand factor, a platelet ... Glycogen Storage Disease Type II (Pompe Disease). Seattle (WA): University of Washington, Seattle. PMID 20301438. " ... Liver disease, or hepatic disease, is any of many diseases of the liver. If long-lasting it is termed chronic liver disease. ... Non-alcoholic fatty liver disease is a spectrum of disease associated with obesity and metabolic syndrome. Hereditary diseases ...
"Platelet glycoprotein Ibalpha forms catch bonds with human WT vWF but not with type 2B von Willebrand disease vWF". Journal of ... Other type of "dynamic bonds" have been defined in additional to the original types of catch bonds, slip bonds and ideal bonds ... The above bonds involve bimolecular interactions, which arguably represents the simplest types. A new type of catch bonds ... "The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet ...
Active Pcsk6 has been reported to process substrates such as transforming growth factor β, pro-albumin, von Willebrand factor, ... Cardiovascular disease: Pcsk6 is increasing interest as indicator and factor of cardiovascular disease. Pcsk6 KO mice was shown ... Proprotein convertase subtilisin/kexin type 6 is an protease that in humans is encoded by the PCSK6 gene which is located in ... Some of its substrates are - transforming growth factor beta related proteins, pro-albumin, von Willebrand factor, and corin. ...
Various types of hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy. Rare ... May 16, 2009 Von Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009 McCain J (June 2005). "The future of ... Different drugs can be prescribed depending on the type of disease. Vitamins (K, P and C) are essential in case of obstruction ... Several types of coagulopathy are distinguished, ranging from mild to lethal. Coagulopathy can be caused by thinning of the ...
O blood group is associated with reduced levels of von Willebrand factor - because of increased clearance - and factor VIII, ... Inflammatory bowel disease (ulcerative colitis and Crohn's disease) predispose to thrombosis, particularly when the disease is ... The minor ("type 2") thrombophilias are much more common. Factor V Leiden is present in 5% of the population of Northern ... Platelet aggregation may be increased, and there are higher levels of coagulation proteins such as von Willebrand factor, ...
It is considered the shortest and least complex member of the family, consisting of only one Von Willebrand Factor A domain, ... The types of cartilage that Matrilin-3 has been observed in are cartilaginous tissue, which includes articular and epiphyseal ... Not only does it maintain cartilage structure but also its function and disease development. Matrilin-3 is mainly expressed in ... Each member of the Matrilin family consist of one or two Von Willebrand Factor A (vWFA) domains, several epidermal growth ...
Type 1 von Willebrand Disease, Type 2A von Willebrand Disease, Type 2B von Willebrand Disease, Type 2M von Willebrand Disease, ... Although von Willebrand did not identify the definite cause, he distinguished von Willebrand disease (vWD) from hemophilia and ... Type 2N von Willebrand Disease, Type 3 von Willebrand Disease Overview of all the structural information available in the PDB ... "Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand ...
It is not recommended for use in dogs with bleeding disorders (such as Von Willebrand's disease), as safety has not been ... These include: The type of drug; Wide use; Duration of use. Long-term use can result in a higher risk for adverse reactions. It ... McKellar QA, Bogan JA, von Fellenberg RL, Ludwig B, Cawley GD (July 1991). "Pharmacokinetic, biochemical and tolerance studies ... Carprofen should not be given at the same time with other types of medications, such as other NSAIDs (aspirin, etodolac, ...
... does not identify those at risk It is distinct from common bleeding disorders in other breeds such von Willebrand's disease, ... "Greyhound Type - The Archtypical Sighthound". Greyhound Club Of America. Retrieved 2021-10-07. Gunnar von Boehn. "Shepparton ( ... Gunnar von Boehn. "Singleton (NSW) Track Records". Greyhound-data.com. Retrieved 2011-05-31. Gunnar von Boehn. "Capalaba (QLD) ... Greyhounds are defined as a tall, muscular, smooth-coated, "S-shaped" type of sighthound with a long tail and tough feet. ...
March 2005). "The prevalence of the cysteine1584 variant of von Willebrand factor is increased in type 1 von Willebrand disease ... which means that type A and B parents can have an AB child. A couple with type A and type B can also have a type O child if ... circulating von Willebrand factor is modified by ABO blood group genotype and is a major determinant of plasma von Willebrand ... "The effect of ABO blood group on the diagnosis of von Willebrand disease". Blood. 69 (6): 1691-1695. doi:10.1182/blood.V69.6. ...
van Genderen PJ, Leenknegt H, Michiels JJ, Budde U (September 1996). "Acquired von Willebrand disease in myeloproliferative ... This type of thrombosis arises by mechanisms different from those of a normal clot: namely, extending the fibrin of venous ... An elevated CT with EPI and collagen can indicate intrinsic defects such as von Willebrand disease, uremia, or circulating ... "Acquired von Willebrand's disease in association with essential thrombocythemia: regression following treatment". Acta ...
This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A ... Bushby KM, Collins J, Hicks D (2014). "Collagen Type VI Myopathies". Progress in Heritable Soft Connective Tissue Diseases. ... "Missense mutation in a von Willebrand factor type A domain of the alpha 3(VI) collagen gene (COL6A3) in a family with Bethlem ... similarity to von Willebrand factor, fibronectin, actin, salivary proteins and aprotinin type protease inhibitors". The EMBO ...
Commonly, Pembrokes can suffer from monorchidism, Von Willebrand's disease, hip dysplasia, degenerative myelopathy (DM), and ... Both groups have worked hard to ensure the appearance and type of breed are standardized through careful selective breeding. ... Retrieved 3 July 2016. Ho, Eric (2017-11-17). "How I Became The Leader Of The New York City Corgi Meet Ups". Medium. Retrieved ... On April 3 2019, the film The Queen's Corgi was released. Dogs portal List of dog breeds Wheeler, Jill C. (2010). Welsh Corgis ...
... some of which are the cause of Bernard-Soulier syndromes and platelet-type von Willebrand disease. GP1BA has been shown to ... "Mutation in the gene encoding the alpha chain of platelet glycoprotein Ib in platelet-type von Willebrand disease". Proceedings ... Titani K, Takio K, Handa M, Ruggeri ZM (August 1987). "Amino acid sequence of the von Willebrand factor-binding domain of ... The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent ...
Von Willebrand's disease) and idiopathic thrombocytopenic purpura. Food and supplements that may increase antiplatelet drugs' ... Examples of these types of surgeries include some cancer surgery and possibly some orthopedic surgery (non-urgent/emergent ... Glycoprotein IIb/IIIa receptor antagonists block a receptor on the platelet for fibrinogen and von Willebrand factor. 3 classes ... Peripheral Vascular Disease/Peripheral Arterial Disease and apical/ventricular/mural thrombus. Treatment of established ...
"Von Willebrand's Disease (vWD): A Type of Hemophilia in Dogs". Drs. Foster & Smith, Inc. Retrieved 10 May 2009. "Genetic ... German Shepherds have a higher-than-normal incidence of Von Willebrand disease, a common inherited bleeding disorder, and ... The FCI accepted the long-haired type in 2010, listing it as the variety b, while the short-haired type is listed as the ... After purchasing the dog he changed his name to Horand von Grafrath and von Stephanitz founded the Verein für Deutsche ...
Other OPG ligands include syndecan-1, glycosaminoglycans, von Willebrand factor, and factor VIII-von Willebrand factor complex ... Naot D, Wilson LC, Allgrove J, Adviento E, Piec I, Musson DS, Cundy T, Calder AD (2019). "Juvenile Paget's disease with ... Multiple myeloma is a type of cancer involving malignant plasma cells, called myeloma cells, within the bone marrow. Multiple ... 49) Osteoporosis is a bone-related disease caused by increased rates of bone resorption compared to bone formation. A higher ...
Centers for Disease Control and Prevention (CDC). (2021, April 1). What is von Willebrand Disease (VWD)?Centers for Disease ... Â VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), one of several types of proteins in the blood ... What else should I know about VONVENDI and von Willebrand Disease?. Your body can form inhibitors to von Willebrand factor or ... About von Willebrand disease (VWD). VWD is the most common inherited bleeding disorder, affecting up to one percent of the U.S ...
Type 1 von Willebrand Disease. Type 1 VWD is the most common type. About 85% of the people with VWD have this type. In Type 1 ... Type 2 von Willebrand Disease. Type 2 VWD is the next most common type. About 15% of people with VWD have Type 2. They usually ... Types of von Willebrand Disease. Language. : English. Español. Change language to Español ... have more severe bleeding problems than people with Type 1. In Type 2 VWD, there is enough von Willebrand factor but it does ...
Dominant type I von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor. Blood ... Autosomal dominant type 1 von Willebrand disease due to G3639T mutation (C1130F) in exon 26 of von Willebrand factor gene: ... Autosomal recessive disease, C2362F, Desmopressin response, Multimeric patterns, von Willebrand disease type 3 ... Autosomal Recessive von Willebrand Disease Type 1 or 2 due to Homozygous or Compound Heterozygous Mutations in the von ...
... von Willebrand disease (VWD) is in fact a family of bleeding disorders caused by an abnormality of the von Willebrand factor ( ... VWF). von Willebrand disease is the most common hereditary bleeding disorder. ... laboratory results are similar to those of certain patients with type 2A von Willebrand disease. Type 2M von Willebrand disease ... It is a variant of the disease with primarily qualitative defects of von Willebrand factor. Type 2 von Willebrand disease can ...
Von Willebrand Disease (VWD) is a rare blood disorder that affects how the body forms blood clots. It can cause a range of ... There are three types of VWD: 1) Type 1, which is the most common and mildest form of the disease; 2) Type 2, which is a ... If you or someone you know is living with von Willebrand Disease, then you know how important it is to stay informed and up-to- ... Von Willebrand Disease (VWD) is an inherited blood-clotting disorder that affects both sexes. It causes people to have ...
Easy bruising and excessive bleeding can be signs of Von Willebrand disease, a genetic disorder that affects bloods ability to ... What Are the Types of Von Willebrand Disease?. There are various forms of VWD:. *In Type 1, the level of Von Willebrand factor ... Von Willebrand multimers test, which helps to classify the type of Von Willebrand disease ... What Is Von Willebrand Disease?. Von Willebrand disease, or VWD, is a genetic (inherited) bleeding disorder that prevents blood ...
Managing patients with von Willebrand disease type 1, 2 and 3 with desmopressin and von Willebrand factor-factor VIII ... Guidelines and recommendations for the acute and prophylactic treatment of bleeding in von Willebrand disease (VWD) patients ... type 1, type 2 or type 3). The VW/FVIII concentrates should be assessed using the parameters FVIII:coagulant activity (C), VWF: ... As the bleeding tendency is moderate in VWD type 2 and severe in type 3 and because the FVIII:C levels are subnormal in type 2 ...
Differentiating between type 2B and platelet-type VWD, as well as between type 2N VWD and hemophilia A. Von Willebrand factor ... "Canine von Willebrand Disease - Breed Summaries". ahdc.vet.cornell.edu. 2019-02-08. "Canine von Willebrand Disease". vetgen.com ... GeneReviews/NCBI/NIH/UW entry on von Willebrand Disease NHLBI von Willebrand Disease Expert Panel (January 2008). The Diagnosis ... Sadler JE (April 1994). "A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of ...
... particularly in types 1 and 3 von Willebrand disease (VWD) is becoming more widely practised. The sequence of the entire VWF ... Examination of the entire von Willebrand factor (VWF) gene for mutations, ... Examination of the entire von Willebrand factor (VWF) gene for mutations, particularly in types 1 and 3 von Willebrand disease ... On behalf of the ISTH SSC Subcommittee on von Willebrand factor Thromb Haemost. 2001 May;85(5):929-31. ...
von Willebrand Diseases (von Willebrands Disease) 10/01/1998 - "Studies of multimerin in patients with von Willebrand disease ... Type 3 von Willebrand Disease 10/01/1998 - "In type 3 VWD platelets, alpha-granular electron-lucent zones lacking VWF- ... von Willebrand factor, and fibrinogen, and an epinephrine aggregation defect.". ... Related Diseases. 1. Thrombosis (Thrombus) 05/01/2010 - "Mice with deleted multimerin 1 and alpha-synuclein genes have impaired ...
Annette BOWYER, this presentation will discuss the diagnosis of von Willebrand disease in the laboratory and the use of the ... A reduction or dysfunction of vWF causes the Haemorrhagic disorder von Willebrand disease (vWD). VWD is the commonest bleeding ... The contribution of multimer analysis in the diagnosis of von Willebrand disease. ... The contribution of multimer analysis in the diagnosis of von Willebrand disease ...
Read Volume 26 Issue 3 of GE - Portuguese Journal of Gastroenterology. ... Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare ... Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare ... Vitamin D Deficiency in a Portuguese Cohort of Patients with Inflammatory Bowel Disease: Prevalence and Relation to Disease ...
Von Willebrand Disease Market Size, Share, Trends, By Type (Type 1 Von Willebrand disease, Type 2 Von Willebrand disease, Type ... 3 Von Willebrand disease), By Route of Administration (Oral, Parenteral, Others), By Product (Injection, Tablets or Capsules, ... Von Willebrand Disease Market Size, Share, Trends, By Type (Type 1 Von Willebrand disease, Type 2 Von Willebrand disease, Type ... Von Willebrand Disease Market. ... 3 Von Willebrand disease), By Route of Administration (Oral, ...
The vWD market research report offers an overview of vWD, including epidemiology, symptoms, diagnosis, and disease management. ... The von Willebrand Disease (vWD) market size in the three major markets (3MM) (US, Germany, and UK) was $892 million in 2022. ... There are three types of hereditary vWD-type 1, type 2, and type 3-of which type 2 contains various subtypes (2A, 2B, 2M, and ... 2 Disease Overview. 2.1 Overview of von Willebrand Disease. 2.2 Classification of von Willebrand Disease. 3 Epidemiology. 3.1 ...
Von Willebrand disease occurs in individuals with low levels of, or a malfunction in, the von Willebrand factor: a protein in ... Von Willebrand disease (VWD) The CDC recognizes this disease as the most common of the disorders found in up to 1 percent of ... For instance, when you get a cut, it is the von Willebrand factor which forms clots at the site of the injury to regulate ... When the von Willebrand Factor isnt working properly, minor injuries can lead to heavy and often dangerous bleeding. ...
Market, By Disease Type:. *Hemophilia A *Hemophilia B *Von Willebrand Disease *Others Market, By Distribution Channel:. * ... 6.2.2. By Disease Type (Hemophilia A, Hemophilia B, Von Willebrand Disease, Others) *6.2.3. By Distribution Channel (Hospital ... 8.3.5.2.1 By Drug Type *8.3.5.2.2 By Disease Type *8.3.5.2.3 By Distribution Channel *8.3.5.2.4 By End User 10. Middle East and ... 8.3.5.2.1. By Drug Type *8.3.5.2.2. By Disease Type *8.3.5.2.3. By Distribution Channel *8.3.5.2.4. By End User 9. Asia-Pacific ...
People who have type 1 or type 2 VWD may not have major bleeding problems. Thus, they may not be diagnosed unless they have ... On the other hand, type 3 VWD can cause major bleeding problems during infancy and childhood. So, children who have type 3 VWD ... Early diagnosis of von Willebrand disease (VWD) is important to make sure that youre treated and can live a normal, active ... Diagnosing Von Willebrand Disease. Early diagnosis of von Willebrand disease (VWD) is important to make sure that youre ...
... vWD type 1 b) vWD type 2A c) vWD type 2N d) vWD type 3 Correct Answer - D Ans. is d i.e., vWD type 3 ConditionDefect vWD type ... Rarest type of Von Willebrand disease :. a) vWD type 1. b) vWD type 2A. c) vWD type 2N. d) vWD type 3. Correct Answer - D. Ans ... type 1. Mild to moderate quantitative. deficiency of vWF (ie, about 20-. 25% of normal levels).. vWD. type 2A. The most common ... type 2B. Loss of only large multimers as. mutant vWF spontaneously binds. to Gplb in the absence of. subendothelial contact. ...
Type 1 VWD. 69 (15.5%). 7. (0-14). 10.3. (5 - 17). 4 (3-8). 8.5 (3.25 - 15). [48%]. 293 (258-300). [87.5%]. 299.5 (262-300). [ ... Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » VWF and von Willebrand Factor Disorders - ... The diagnosis of von Willebrand disease (VWD) in low- and medium-income countries (LMIC) is challenging due to cost and lack of ... Sensitivity of ISTH Bleeding Assessment Tool, Bleeding Time and PFA-200 in the Diagnosis of von Willebrand Disease. T. Geevar1 ...
Von Willebrand disease is a bleeding disorder that slows the blood clotting process, causing prolonged bleeding after an injury ... Von Willebrand disease can have different inheritance patterns.. Most cases of type 1 and type 2 von Willebrand disease are ... Variants in the VWF gene that reduce the amount of von Willebrand factor cause type 1 von Willebrand disease. In type 1C, the ... Von Willebrand disease is divided into three types. Type 1 has one subtype (1C), and type 2 is divided into four subtypes (2A, ...
Platelet Factor 4 Binds to Released Von Willebrand Factor Strands from Injured Endothelium and Forms a Prothrombotic HIT ... Clonal Hematopoiesis of Indeterminate Potential (CHIP) in Patients with Coronary Artery Disease and in Centenarians. Further ... type 3 von Willebrand disease.. This award will be presented during the invited speaker session of the Special Symposium on the ... Novel Mechanism of Post-Transcriptional Regulation of PD-L1 Expression By 3-UTR Binding Proteins ...
... that advance medical and scientific knowledge of CSL Behring products and the diseases they have been designed to treat. ... Von Willebrand Disease. *Clinical studies that generate evidence of efficacy or safety in selected von Willebrand Disease (vWD ... type 3, type 2, menorrhagia, pregnancy, or prophylaxis for angiodysplasia, epistaxis and joint bleeds ... Role of von Willebrand Factor (vWF) in reducing inhibitor development. *Clinical studies that increase knowledge of immune ...
Type 3: virtually complete absence of VWF; severe symptoms. Investigations of suspected Von Willebrand disease. History and ... Principles of care for the diagnosis and treatment of von Willebrand disease. Haematologica 98.5 (2013): 667-674. ... Laboratory diagnosis of congenital von Willebrand disease.Seminars in thrombosis and hemostasis. Vol. 28. No. 02. Copyright© ... von Willebrand factor collagen binding (VWF:CB). VWF level and FVIII level are the tests which correlate with severity of ...
Identification of mutations in the canine von Willebrand factor gene associated with type III von-Willebrand-disease Thrombosis ... Von Willebrand?s disease (vWD) is a blood disease caused by a deficiency of von Willebrand Factor (vWF), an adhesive ... Von Willebrand?s disease (vWD) is a blood disease caused by a deficiency of von Willebrand Factor (vWF), an adhesive ... The inheritance of the severe forms (Types II and III) is autosomal recessive. The milder form (Type I) may be autosomal ...
Disease. The most common form of canine hereditary bleeding disorders is the von Willebrand disease (vWD). The condition is ... Von Willebrand disease (vWD) is a bleeding disorder caused by a quantitative or qualitative... more ... VWD is divided into three major categories: type 1, type 2 and type 3 as well as diverse subtypes. Type 3 of vWD displays the ... von Willebrand disease type I (vWD1) * von-Willebrand disease type II (vWD 2) ...
Patients with Type 1 or 2 Willebrand disease (VWD), depending on their baseline von Willebrand factor (VWF) ristocetin cofactor ... People with bleeding disorders are not at greater risk of contracting COVID-19 or developing a severe form of the disease, so ... The U.K. Medicines and Healthcare Products Regulatory Agency and the U.S. Centers for Disease Control and Prevention have ... Patients with Type 3 VWD should be given a VWF-containing injection.. ...
Von Willebrand Disease - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical ... Symptoms and Signs of Von Willebrand Disease Bleeding manifestations in the most common type I von Willebrand disease (VWD) ... Diagnosis of Von Willebrand Disease *. Total plasma von Willebrand factor (VWF) antigen concentration ... Von Willebrand disease (VWD) is a hereditary quantitative deficiency or functional abnormality of von Willebrand factor (VWF), ...
Spotlight Von Willebrand Disease. Cody Kester: Patient, Advocate, High Gear Activist. Cody Kester has Type 3/Severe Von ... Von Willebrand Disease Conference ever to be held in the United States. This was early 2017; before VWD Connect Foundation was ... Von Willebrand Disease Conference ever to be held in the United States. This was early 2017; before VWD Connect Foundation was ... Cody Kester has Type 3/Severe Von Willebrand Disease. He is four months into his job as a volunteer fireman for hometown Alma, ...
What types of von Willebrand disease (vWD) does desmopressin/DDAVP work in?. By William Aird ... What types of von Willebrand disease (vWD) does desmopressin/DDAVP work in? ... Type 3 vWD because there is no von Willebrand factor (vWF) to release. ... Type 2 vWD because the drug releases and raises a functionally defective vWF. ...
Types of Hemophilia. In: The Hemophilia, von Willebrand Disease & Platelet Disorders Handbook [Internet]. Hemophilia of Georgia ... Centers for Disease Control and Prevention. Facts About von Willebrand Disease. http://www.cdc.gov/ncbddd/vwd/facts.html ( ... The physiotherapy management of haemophilia is very similar to the management of Von Willebrand Disease. ... Types of Haemophilia[edit , edit source]. Haemophilia is usually an inherited disease, but in rare cases, it can be acquired. ...

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