Muckle–Wells Syndrome
   HOME
*





Muckle–Wells Syndrome
Muckle–Wells syndrome (MWS) is a rare autosomal dominant disease which causes sensorineural deafness and recurrent hives, and can lead to amyloidosis. Individuals with MWS often have episodic fever, chills, and joint pain. As a result, MWS is considered a type of periodic fever syndrome. MWS is caused by a defect in the CIAS1 gene which creates the protein cryopyrin. MWS is closely related to two other syndromes, familial cold urticaria and neonatal onset multisystem inflammatory disease—in fact, all three are related to mutations in the same gene and subsumed under the term cryopyrin-associated periodic syndromes (CAPS). Sign and symptoms * Sensorineural deafness * Recurrent urticaria (hives) * Fevers * Chills * Arthralgia (painful joints) Causes MWS occurs when a mutation in the ''CIAS1'' gene, encoding for NLRP3, leads to increased activity of the protein cryopyrin. This protein is partly responsible for the body's response to damage or infection. During these states, a ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


picture info

Clinical Immunology
Immunology is a branch of medicineImmunology for Medical Students, Roderick Nairn, Matthew Helbert, Mosby, 2007 and biology that covers the medical study of immune systems in humans, animals, plants and sapient species. In such we can see there is a difference of human immunology and comparative immunology in veterinary medicine and animal biosciences. Immunology measures, uses charts and differentiate in context in medicine the studies of immunity on cell and molecular level, and the immune system as part of the physiological level as its functioning is of major importance. In the different states of both health, occurring symptoms and diseases; the functioning of the immune system and immunological responses such as autoimmune diseases, allergic hypersensitivities, or in some cases malfunctioning of immune system as for example in immunological disorders or in immune deficiency, and the specific transplant rejection) Immunology has applications in numerous disciplines of me ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


picture info

Macrophage
Macrophages (abbreviated as M φ, MΦ or MP) ( el, large eaters, from Greek ''μακρός'' (') = large, ''φαγεῖν'' (') = to eat) are a type of white blood cell of the immune system that engulfs and digests pathogens, such as cancer cells, microbes, cellular debris, and foreign substances, which do not have proteins that are specific to healthy body cells on their surface. The process is called phagocytosis, which acts to defend the host against infection and injury. These large phagocytes are found in essentially all tissues, where they patrol for potential pathogens by amoeboid movement. They take various forms (with various names) throughout the body (e.g., histiocytes, Kupffer cells, alveolar macrophages, microglia, and others), but all are part of the mononuclear phagocyte system. Besides phagocytosis, they play a critical role in nonspecific defense (innate immunity) and also help initiate specific defense mechanisms (adaptive immunity) by recruiting other immune ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


picture info

House (TV Series)
''House'' (also called ''House, M.D.'') is an American medical drama television series that originally ran on the Fox network for eight seasons, from November 16, 2004, to May 21, 2012. The series' main character is Dr. Gregory House (Hugh Laurie), an unconventional, misanthropic medical genius who, despite his dependence on pain medication, leads a team of diagnosticians at the fictional Princeton–Plainsboro Teaching Hospital (PPTH) in New Jersey. The series' premise originated with Paul Attanasio, while David Shore, who is credited as creator, was primarily responsible for the conception of the title character. The series' executive producers included Shore, Attanasio, Attanasio's business partner Katie Jacobs, and film director Bryan Singer. It was filmed largely in a neighborhood and business district in Los Angeles County's Westside called Century City. The show received high critical acclaim, and was consistently one of the highest rated series in the United States. ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


Brian Goldman
Howard Brian Goldman (born 1956) is a Canadians, Canadian emergency medicine, emergency physician, author, public speaker, and radio personality. Education and career He completed his undergraduate medical education at the University of Toronto in 1980. In July 1980, he began residency (medicine), postgraduate medical education in family medicine. On 15 January 1982, Goldman obtained his Independent Practice Certificate from the College of Physicians and Surgeons of Ontario. He received his certification in family medicine from the College of Family Physicians of Canada (CFPC) in July 1984, followed by a certificate of advanced competency in emergency medicine from the CFPC in November 1985. He practices in the Schwartz Reisman Emergency Centre at Mount Sinai Hospital (Toronto), Mount Sinai Hospital in Toronto, Ontario, Canada. Goldman has been a regular medical columnist on CBC Radio One since 1999. Goldman was also awarded fellowship in the CFPC, thereby entitled to display ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


White Coat, Black Art
''White Coat, Black Art'' is a Canadian radio documentary series on CBC Radio One, hosted by physician Brian Goldman that examines the business and culture of medicine from an insider's perspective. Its name is a reference to the white coats that doctors wear, and to the ways that practicing medicine is still in some ways a "black art" rather than a science: mysterious, intuitive, and difficult to master. The series began as a summer series in 2007 and became a regular program on the network's schedule. The Canadian Medical Association awarded the program the 2011 Media Award for Health Reporting (Excellence in Radio Reporting / In-Depth).CMA Announces Recipients of 2011 Media Awards for Health Reporting
at the

picture info

CBC Radio One
CBC Radio One is the English-language news and information radio network of the publicly owned Canadian Broadcasting Corporation. It is commercial-free and offers local and national programming. It is available on AM and FM to 98 percent of Canadians and overseas over the Internet, and through mobile apps. CBC Radio One is simulcast across Canada on Bell Satellite TV satellite channels 956 and 969, and Shaw Direct satellite channel 870. A modified version of Radio One, with local content replaced by additional airings of national programming, is available on Sirius XM channel 169. It is downlinked to subscribers via SiriusXM Canada and its U.S.-based counterpart, Sirius XM Satellite Radio. In 2010, Radio One reached 4.3 million listeners each week. It was the largest radio network in Canada. History CBC Radio began in 1936, and is the oldest branch of the corporation. In 1949, the facilities and staff of the Broadcasting Corporation of Newfoundland were transferred to CB ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


picture info

Protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, responding to stimuli, providing structure to cells and organisms, and transporting molecules from one location to another. Proteins differ from one another primarily in their sequence of amino acids, which is dictated by the nucleotide sequence of their genes, and which usually results in protein folding into a specific 3D structure that determines its activity. A linear chain of amino acid residues is called a polypeptide. A protein contains at least one long polypeptide. Short polypeptides, containing less than 20–30 residues, are rarely considered to be proteins and are commonly called peptides. The individual amino acid residues are bonded together by peptide bonds and adjacent amino acid residues. The sequence of amino acid residue ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


Monoclonal Antibody
A monoclonal antibody (mAb, more rarely called moAb) is an antibody produced from a cell Lineage made by cloning a unique white blood cell. All subsequent antibodies derived this way trace back to a unique parent cell. Monoclonal antibodies can have monovalent affinity, binding only to the same epitope (the part of an antigen that is recognized by the antibody). In contrast, polyclonal antibodies bind to multiple epitopes and are usually made by several different antibody-secreting plasma cell lineages. Bispecific monoclonal antibodies can also be engineered, by increasing the therapeutic targets of one monoclonal antibody to two epitopes. It is possible to produce monoclonal antibodies that specifically bind to virtually any suitable substance; they can then serve to detect or purify it. This capability has become an investigative tool in biochemistry, molecular biology, and medicine. Monoclonal antibodies are being used on a clinical level for both the diagnosis and therapy ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  




Canakinumab
Canakinumab (International Nonproprietary Name, INN), sold under the brand name Ilaris, is a medication for the treatment of systemic juvenile idiopathic arthritis (SJIA) and active Still's disease, including adult-onset Still's disease (AOSD). It is a human monoclonal antibody targeted at IL1B, interleukin-1 beta. It has no cross-reactivity with other members of the interleukin-1 family, including interleukin-1 alpha. Common side effects include infections (colds and upper respiratory tract infections), abdominal pain and injection-site reactions. Medical uses Canakinumab was approved for the treatment of cryopyrin-associated periodic syndromes (CAPS) by the U.S. Food and Drug Administration (FDA) in June 2009 and by the European Medicines Agency (EMA) in October 2009. Text was copied from this source which is © European Medicines Agency. Reproduction is authorized provided the source is acknowledged. CAPS is a spectrum of autoinflammatory syndromes including Familial Cold ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


Rilonacept
Rilonacept, sold under the brand name Arcalyst, is a medication used to treat cryopyrin-associated periodic syndromes, including familial cold autoinflammatory syndrome, and Muckle–Wells syndrome; deficiency of interleukin-1 receptor antagonist; and recurrent pericarditis. Rilonacept is an interleukin 1 inhibitor. Rilonacept is a dimeric fusion protein consisting of the ligand-binding domains of the extracellular portions of the human interleukin-1 receptor component (IL-1R1) and IL-1 receptor accessory protein (IL-1RAcP) linked in-line to the fragment-crystallizable portion (Fc region) of human IgG1 that binds and neutralizes IL-1. Rilonacept was given an orphan drug designation by the U.S. Food and Drug Administration (FDA) and is used for the treatment of cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome, Muckle–Wells syndrome. Rilonacept is the first drug approved by the FDA to treat recurrent pericarditis. Rilonacept was ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


Interleukin 1
The Interleukin-1 family (IL-1 family) is a group of 11 cytokines that plays a central role in the regulation of immune and inflammatory responses to infections or sterile insults. Discovery Discovery of these cytokines began with studies on the pathogenesis of fever. The studies were performed by Eli Menkin and Paul Beeson in 1943–1948 on the fever-producing properties of proteins released from rabbit peritoneal exudate cells. These studies were followed by contributions of several investigators, who were primarily interested in the link between fever and infection/inflammation. The basis for the term "interleukin" was to streamline the growing number of biological properties attributed to soluble factors from macrophages and lymphocytes. IL-1 was the name given to the macrophage product, whereas IL-2 was used to define the lymphocyte product. At the time of the assignment of these names, there was no amino acid sequence analysis known and the terms were used to define bio ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]  


Anakinra
Anakinra, sold under the brand name Kineret, is a biopharmaceutical medication used to treat rheumatoid arthritis, cryopyrin-associated periodic syndromes, familial Mediterranean fever, and Still's disease. It is a recombinant and slightly modified version of the human interleukin 1 receptor antagonist protein. It is marketed by Swedish Orphan Biovitrum. Anakinra is administered by subcutaneous injection. Medical uses It is used as a second line treatment to manage symptoms of rheumatoid arthritis after treatment with a disease-modifying antirheumatic drug (DMARD) has failed. It can be used in combination with some DMARDs. It is used to people with a cryopyrin-associated periodic syndrome, including neonatal-onset multisystem inflammatory disease. It is used to treat Schnitzler's syndrome (off label in the US). Its response rate is such that it has been suggested that "Treatment failures should lead to reconsider the diagnosis." Off label, it is used to treat systemic juven ...
[...More Info...]      
[...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]