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Complement deficiency information


Complement deficiency
Complement pathway (normal)
SpecialtyHematology Edit this on Wikidata
SymptomsRecurring infection, rheumatic disease[1]
CausesCan be inherited or acquired[2]
Diagnostic methodCH50 measurement, Plasma level[3]
TreatmentImmunosuppressive therapy[2]

Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins.[4] Because of redundancies in the immune system, many complement disorders are never diagnosed. Some studies estimate that less than 10% are identified.[5] Hypocomplementemia may be used more generally to refer to decreased complement levels,[6] while secondary complement disorder means decreased complement levels that are not directly due to a genetic cause but secondary to another medical condition.[7]

  1. ^ Cite error: The named reference osk was invoked but never defined (see the help page).
  2. ^ a b Cite error: The named reference pat was invoked but never defined (see the help page).
  3. ^ Cite error: The named reference scape was invoked but never defined (see the help page).
  4. ^ Winkelstein, Jerry A. (2004). "The Complement System". In Gorbach, Sherwood L.; Bartlett, John G.; Blacklow, Neil R. (eds.). Infectious Diseases. Lippincott Williams & Wilkins. pp. 8–13. ISBN 978-0-7817-3371-7.
  5. ^ Sjöholm, A.G.; Jönsson, G.; Braconier, J.H.; Sturfelt, G.; Truedsson, L. (2006). "Complement deficiency and disease: An update". Molecular Immunology. 43 (1–2): 78–85. doi:10.1016/j.molimm.2005.06.025. PMID 16026838. – via ScienceDirect (Subscription may be required or content may be available in libraries.)
  6. ^ Moreland, Larry W., ed. (2004). Rheumatology and Immunology Therapy: A to Z Essentials. Berlin: Springer. p. 425. ISBN 9783540206255. Archived from the original on 12 January 2023. Retrieved 30 August 2016.
  7. ^ Complement-Related Disorders at eMedicine

and 28 Related for: Complement deficiency information

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Complement deficiency

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Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. Because of redundancies in...

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Complement system

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The complement system, also known as complement cascade, is a part of the humoral, innate immune system and enhances (complements) the ability of antibodies...

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Complement 3 deficiency

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Complement 3 deficiency is a genetic condition affecting complement component 3 (C3). People can suffer from either primary or secondary C3 deficiency...

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Complement component 3

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of the complement system. Its activation is required for both classical and alternative complement activation pathways. People with C3 deficiency are susceptible...

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Angioedema

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bradykinin formation is caused by continuous activation of the complement system due to a deficiency in one of its prime inhibitors, C1-esterase (aka: C1-inhibitor...

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Terminal complement pathway deficiency

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Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC). It involves deficiencies of C5, C6...

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Immunodeficiency

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Asplenia, where there is no function of the spleen Complement deficiency is where the function of the complement system is deficient In reality, immunodeficiency...

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Hereditary angioedema

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cases with C1-inhibitor deficiency. C1-inhibitor deficiency is caused by mutations of the SERPING1 gene, the gene encoding complement C1-inhibitor. More than...

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Complement component 2

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the complement system, acting as a multi-domain serine protease. Deficiency of C2 has been associated with certain autoimmune diseases. The Complement system...

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Complement 2 deficiency

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Complement 2 deficiency is a type of complement deficiency caused by any one of several different alterations in the structure of complement component...

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Complement 4 deficiency

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Complement 4 deficiency is a genetic condition affecting complement component 4. It can present with lupus-like symptoms. Parija (2009). Textbook of Microbiology...

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List of primary immunodeficiencies

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CARD11 deficiency MST1 deficiency TCRα deficiency LCK deficiency IL-21 deficiency IL-21R deficiency UNC119 deficiency NIK deficiency OX40 deficiency IKBKB...

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Classical complement pathway

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cells, killing them. Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema. C1-inhibitor...

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Complement component 5

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the complement membrane attack complex. Deficiency is thought to cause Leiner's disease. Complement component 5 is the fifth component of complement, which...

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Complement component 1q

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the C1 complex (C1qr2s2), the first component of the serum complement system. Deficiency of C1q has been associated with lupus erythematosus and glomerulonephritis...

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Complement receptor

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Schwartz RA, Thomas I. "Complement Receptor Deficiency: eMedicine Dermatology". Medscape. Retrieved 7 December 2010. Complement+receptors at the U.S. National...

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Properdin deficiency

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Properdin deficiency is a rare X-linked disease in which properdin, an important complement factor responsible for the stabilization of the alternative...

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Lupus erythematosus

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but this is rare. When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The influence...

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CD55 deficiency

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CD55 deficiency, also called DAF deficiency or CHAPLE syndrome, is a rare genetic disorder of the immune system. CHAPLE stands for "CD55 deficiency with...

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Lupus

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and C4 by immune complex-induced inflammation or to congenitally complement deficiency, which may predispose to SLE. Renal disorder: More than 0.5 g per...

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Acquired C1 esterase inhibitor deficiency

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of the complement cascade, such that complement proteins remain in check and do not lead to unnecessary activity. When there is a deficiency of C1-INH...

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List of syndromes

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Coffin–Siris syndrome Cogan syndrome Cohen syndrome Compartment syndrome Complement deficiency Complete androgen insensitivity syndrome Complex regional pain syndrome...

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Total complement activity

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C3/C4 values but a decreased CH50, that can indicate a terminal complement pathway deficiency while if one has low C3 and CH50 values that can indicate an...

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Complement component 4

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Complement component 4 (C4), in humans, is a protein involved in the intricate complement system, originating from the human leukocyte antigen (HLA) system...

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MBL deficiency

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MBL deficiency or mannose-binding lectin deficiency is an illness that has an impact on immunity. Low levels of mannose-binding lectin, an immune system...

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Lectin pathway

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pathway is a type of cascade reaction in the complement system, similar in structure to the classical complement pathway, in that, after activation, it proceeds...

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Homocystinuria

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inherited disorder of the metabolism of the amino acid methionine due to a deficiency of cystathionine beta synthase or methionine synthase. It is an inherited...

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Meningitis

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such anatomical abnormalities, 36% are due to immune deficiencies (such as complement deficiency, which predisposes especially to recurrent meningococcal...

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