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VEXAS Syndrome
VEXAS syndrome is an adult-onset autoinflammatory disease primarily affecting males, caused by a somatic mutation of the '' UBA1'' gene in hematopoietic progenitor cells. The name VEXAS is an acronym deriving from the core features of disease: : V: Vacuoles are often identified in the bone marrow stem cells of patients presenting with VEXAS. : E: The E1 ubiquitin conjugating enzyme encoded by the '' UBA1'' gene is mutated in patients. : X: The mutated '' UBA1'' gene is recessive and located on the X-chromosome and thus the disease is almost exclusively found in individuals with a single X chromosome and thus said to be X-linked. : A: Patients with VEXAS present with a wide array of Autoinflammatory conditions : S: The mutations which cause VEXAS are Somatic: they are acquired throughout life, not inherited, and are not passed on to offspring. Signs and symptoms The disease arises in late adulthood (typically after the age of 50) and causes both autoinflammatory and hematol ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Autoinflammatory Disease
Periodic fever syndromes are a set of disorders characterized by recurrent episodes of systemic and organ-specific inflammation. Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, people with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells. Instead, the autoinflammatory diseases are characterized by errors in the innate immune system. The syndromes are diverse, but tend to cause episodes of fever, joint pains, skin rashes, abdominal pains and may lead to chronic complications such as amyloidosis. Most autoinflammatory diseases are genetic and present during childhood. The most common genetic autoinflammatory syndrome is familial Mediterranean fever, which causes short episodes of fever, abdominal pain, serositis, lasting less than 72 hours. It is caused by mutations in the MEFV gene, which codes for the protein pyrin. Pyrin is a protein ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Blood (journal)
''Blood'' is a peer-reviewed medical journal published by the American Society of Hematology. It was established by William Dameshek in 1946. The journal changed from semimonthly (24 times annually) to weekly publication at the start of 2009. It covers clinical and basic research in all areas of hematology, including disorders of leukocytes, both benign and malignant, erythrocytes, platelets, hemostatic mechanisms, vascular biology, immunology, and hematologic oncology. Abstracting and indexing ''Blood'' is indexed and abstracted by: According to the ''Journal Citation Reports'', the journal has a 2023 impact factor The impact factor (IF) or journal impact factor (JIF) of an academic journal is a type of journal ranking. Journals with higher impact factor values are considered more prestigious or important within their field. The Impact Factor of a journa ... of 21.1, and has a Journal Impact Factor rank of 2/97 in the Hematology category. References External l ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
David B
David Robert Jones (8 January 194710 January 2016), known as David Bowie ( ), was an English singer, songwriter and actor. Regarded as one of the most influential musicians of the 20th century, Bowie was acclaimed by critics and musicians, particularly for his innovative work during the 1970s. His career was marked by reinvention and visual presentation, and his music and stagecraft have had a great impact on popular music. Bowie studied art, music and design before embarking on a professional career as a musician in 1963. He released a string of unsuccessful singles with local bands and David Bowie (1967 album), a self-titled solo album (1967) before achieving his first top-five entry on the UK singles chart with "Space Oddity" (1969). After a period of experimentation, he re-emerged in 1972 during the glam rock era with the alter ego Ziggy Stardust (character), Ziggy Stardust. The success of the single "Starman (song), Starman" and its album ''The Rise and Fall of Ziggy Star ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Adverse Effects
An adverse effect is an undesired harmful effect resulting from a medication or other intervention, such as surgery. An adverse effect may be termed a "side effect", when judged to be secondary to a main or therapeutic effect. The term complication is similar to adverse effect, but the latter is typically used in pharmacological contexts, or when the negative effect is expected or common. If the negative effect results from an unsuitable or incorrect dosage or procedure, this is called a medical error and not an adverse effect. Adverse effects are sometimes referred to as "iatrogenic" because they are generated by a physician/treatment. Some adverse effects occur only when starting, increasing or discontinuing a treatment. Using a drug or other medical intervention which is contraindicated may increase the risk of adverse effects. Adverse effects may cause complications of a disease or procedure and negatively affect its prognosis. They may also lead to non-compliance with a t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involved in a wide range of physiological processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Some common naturally occurring steroid hormones are cortisol (), corticosterone (), cortisone () and aldosterone () (cortisone and aldosterone are isomers). The main corticosteroids produced by the adrenal cortex are cortisol and aldosterone. The etymology of the '' cortico-'' part of the name refers to the adrenal cortex, which makes these steroid hormones. Thus a corticosteroid is a "cortex steroid". Classes * Glucocorticoids such as cortisol affect carbohydrate, fat, and protein metabolism, and have anti ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Mortality Rate
Mortality rate, or death rate, is a measure of the number of deaths (in general, or due to a specific cause) in a particular Statistical population, population, scaled to the size of that population, per unit of time. Mortality rate is typically expressed in units of deaths per 1,000 individuals per year; thus, a mortality rate of 9.5 (out of 1,000) in a population of 1,000 would mean 9.5 deaths per year in that entire population, or 0.95% out of the total. It is distinct from "morbidity", which is either the prevalence or Incidence (epidemiology), incidence of a disease, and also from the incidence rate (the number of newly appearing cases of the disease per unit of time). An important specific mortality rate measure is the crude death rate, which looks at mortality from all causes in a given time interval for a given population. , for instance, the Central Intelligence Agency, CIA estimates that the crude death rate globally will be 7.7 deaths per 1,000 people in a population p ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Erythropoeisis
Erythropoiesis (from Greek ''erythro'', meaning ''red'' and ''poiesis'', meaning ''to make'') is the process which produces red blood cells (erythrocytes), which is the development from erythropoietic stem cell to mature red blood cell. It is stimulated by decreased O2 in circulation, which is detected by the kidneys, which then secrete the hormone erythropoietin.Sherwood, L, Klansman, H, Yancey, P: ''Animal Physiology'', Brooks/Cole, Cengage Learning, 2005. This hormone stimulates proliferation and differentiation of red cell precursors, which activates increased erythropoiesis in the hemopoietic tissues, ultimately producing red blood cells (erythrocytes). In postnatal birds and mammals (including humans), this usually occurs within the red bone marrow. In the early fetus, erythropoiesis takes place in the mesodermal cells of the yolk sac. By the third or fourth month, erythropoiesis moves to the liver. After seven months, erythropoiesis occurs in the bone marrow. Increased lev ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Myeloid Tissue
Myeloid tissue, in the bone marrow sense of the word '' myeloid'' ('' myelo-'' + ''-oid''), is tissue of bone marrow, of bone marrow cell lineage, or resembling bone marrow, and myelogenous tissue (''myelo-'' + '' -genous'') is any tissue of, or arising from, bone marrow; in these senses the terms are usually used synonymously, as for example with chronic myeloid/myelogenous leukemia. In hematopoiesis, myeloid cells, or myelogenous cells are blood cells that arise from a progenitor cell for granulocytes, monocytes, erythrocytes, or platelets (the common myeloid progenitor, that is, CMP or CFU-GEMM), or in a narrower sense also often used, specifically from the lineage of the myeloblast (the myelocytes, monocytes, and their daughter types). Thus, although all blood cells, even lymphocytes, are normally born in the bone marrow in adults, myeloid cells in the narrowest sense of the term can be distinguished from lymphoid cells, that is, lymphocytes, which come from common ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Vacuole
A vacuole () is a membrane-bound organelle which is present in Plant cell, plant and Fungus, fungal Cell (biology), cells and some protist, animal, and bacterial cells. Vacuoles are essentially enclosed compartments which are filled with water containing inorganic and organic molecules including enzymes in Solutes, solution, though in certain cases they may contain solids which have been engulfed. Vacuoles are formed by the fusion of multiple membrane Vesicle (biology), vesicles and are effectively just larger forms of these. The organelle has no basic shape or size; its structure varies according to the requirements of the cell. Discovery Antonie van Leeuwenhoek described the plant vacuole in 1676. Contractile vacuoles ("stars") were first observed by Spallanzani (1776) in protozoa, although mistaken for respiratory organs. Félix Dujardin, Dujardin (1841) named these "stars" as ''vacuoles''. In 1842, Matthias Jakob Schleiden, Schleiden applied the term for plant cells, to dist ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Bone Marrow Examination
Bone marrow examination refers to the pathologic analysis of samples of bone marrow obtained by bone marrow biopsy (often called trephine biopsy) and bone marrow aspiration. Bone marrow examination is used in the diagnosis of a number of conditions, including leukemia, multiple myeloma, lymphoma, anemia, and pancytopenia. The bone marrow produces the cellular elements of the blood, including platelets, red blood cells and white blood cells. While much information can be gleaned by testing the blood itself (drawn from a vein by phlebotomy), it is sometimes necessary to examine the source of the blood cells in the bone marrow to obtain more information on hematopoiesis; this is the role of bone marrow aspiration and biopsy. Components of the procedure Bone marrow samples can be obtained by aspiration and trephine biopsy. Sometimes, a bone marrow examination will include both an aspirate and a biopsy. The aspirate yields semi-liquid bone marrow, which can be examined by a pa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Myelodysplastic Syndrome
A myelodysplastic syndrome (MDS) is one of a group of cancers in which blood cells in the bone marrow do not mature, and as a result, do not develop into healthy blood cells. Early on, no symptoms typically are seen. Later, symptoms may include fatigue, shortness of breath, bleeding disorders, anemia, or frequent infections. Some types may develop into acute myeloid leukemia. Risk factors include previous chemotherapy or radiation therapy, exposure to certain chemicals such as tobacco smoke, pesticides, and benzene, and exposure to heavy metals such as Mercury (element), mercury or lead. Problems with blood cell formation result in some combination of low anemia, red blood cell, thrombocytopenia, platelet, and white blood cell counts. Some types of MDS cause an increase in the production of immature blood cells (called blast cell, blasts), in the bone marrow or peripheral blood, blood. The different types of MDS are identified based on the specific characteristics of the chang ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hematologic Malignancies
Tumors of the hematopoietic and lymphoid tissues (American English) or tumours of the haematopoietic and lymphoid tissues (British English) are tumors that affect the blood, bone marrow, lymph, and lymphatic system. Because these tissues are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making aplastic anemia, aplasia, myeloproliferative neoplasm, myeloproliferation and lymphoproliferative disorders, lymphoproliferation (and thus the leukemias, myelomas, and the lymphomas) closely related and often overlapping problems. While uncommon in solid tumors, chromosomal translocations are a common cause of these diseases. This commonly leads to a different approach in diagnosis and treatment of hematological malignancies. Hematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. In some centers "hematology/o ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
