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Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins.^[1]

The disorders can be divided into two categories:

Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema and hemolytic-uremic syndrome.^{[citation needed]}

Disorders of the proteins that act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections.^{[citation needed]}

Because there are redundancies in the immune system, many complement disorders are never diagnosed. A recent study estimated that less than 10% are identified.^[2]

Hypocomplementemia

Hypocomplementemia may be used more generally to refer to decreased complement levels^[3] while secondary complement disorder is to low complement levels that are not directly due to a genetic cause but secondary to another medical condition.^[4]

The total hemolytic complement complement CH₅₀ level in the blood will be low or undetectable with complement deficiencies.^{[citation needed]}

Individual complement levels can be used to distinguish conditions:

Systemic lupus erythematosus is associated with low C3 and C4
Membranoproliferative glomerulonephritis causes low C3, but normal C4.
Deficiencies of the terminal complement components are inherited in an autosomal recessive manner and cause increased susceptibility to infections by Neisseria.^[5]
Properdin deficiency is an X-linked disorder that also causes susceptibility to neisserial infections.
C1-inhibitor deficiency or hereditary angioedema will have low C4 with normal C1 and C3 levels.^[6]

Vaccinations for encapsulated organisms is crucial for preventing infections in complement deficiencies.^{[citation needed]}

Epidemiology

C2 deficiency is prevalent in 120,000 people in Western countries.^[7] It occurs in about 1 in 10,000 persons.^[8]

References

^ Winkelstein, Jerry A. (2004). "The Complement System". In Gorbach, Sherwood L.; Bartlett, John G.; Blacklow, Neil R. Infectious Diseases. Lippincott Williams & Wilkins. pp. 8–13. ISBN 978-0-7817-3371-7.
^ 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.
^ "hypocomplementemia" at Dorland's Medical Dictionary
^ Complement-Related Disorders at eMedicine
^ Fauci, Anthony; Braunwald, Eugene; Kasper, Dennis; Hauser, Stephen; Longo, Dan; Jameson, J.; Loscalzo, Joseph, eds. (2008). Harrison's Principles of Internal Medicine (17th ed.). ISBN 978-0-07-146633-2. ^{[page needed]}
^ Zuraw, Bruce L. (2008). "Hereditary Angioedema". New England Journal of Medicine 359 (10): 1027–36. doi:10.1056/NEJMcp0803977. PMID 18768946.
^ http://www.patient.co.uk/doctor/complement-deficiencies^{[full citation needed]}
^ http://www.omim.org/entry/217000^{[full citation needed]}

UpToDate Contents

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1. 補体系の後天性疾患 acquired disorders of the complement system
2. 補体系の概要および臨床的評価 overview and clinical assessment of the complement system
3. 糸球体疾患の鑑別診断および評価 differential diagnosis and evaluation of glomerular disease
4. 成人におけるブドウ球菌随伴糸球体腎炎 staphylococcus associated glomerulonephritis in adults
5. リウマチ性血管炎の臨床症状および診断 clinical manifestations and diagnosis of rheumatoid vasculitis

English Journal

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy.

Caltik A, Demircin G, Bülbül M, Erdogan O, Akyüz SG, Arda N.SourcePediatric Nephrology, Dr Sami Ulus Children Hospital, Ankara, Turkey, acaltik@hotmail.com.
Rheumatology international.Rheumatol Int.2013 Jan;33(1):219-22. doi: 10.1007/s00296-010-1540-0. Epub 2010 Jun 8.
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old b
PMID 20532511

C3 glomerulopathy masquerading as acute postinfectious glomerulonephritis.

Sandhu G, Bansal A, Ranade A, Jones J, Cortell S, Markowitz GS.SourceDivision of Nephrology, Department of Medicine, St. Luke's-Roosevelt Hospital Center, Columbia University College of Physicians & Surgeons, New York, NY, USA. gsandhu@chpnet.org
American journal of kidney diseases : the official journal of the National Kidney Foundation.Am J Kidney Dis.2012 Dec;60(6):1039-43. doi: 10.1053/j.ajkd.2012.04.032. Epub 2012 Jul 6.
We report the case of a 63-year-old man who presented with acute kidney injury, active urine sediment, nephrotic syndrome, and hypocomplementemia after a recent report of a sore throat. Kidney biopsy showed diffuse proliferative and exudative glomerulonephritis with C3-dominant staining by immunoflu
PMID 22770945

Japanese Journal

臨床研究・症例報告一過性に低補体血症を呈した急性蕁麻疹の1例

清益功浩,児玉暖,竹下佳弘 [他]
小児科臨床 67(10), 1695-1698, 2014-10
NAID 40020199128

High Level of Rheumatoid Factor is Associated with Hepatitis B Viremia in Patients with Chronic Hepatitis B

ARAI Jun,ITO Takayoshi,MIYASHITA Miyuki,SHIMODUMA Yuu,UCHIKOSHI Manabu,MORIKAWA Kenichi,EGUCHI Junichi,HAYASHI Eiichi,NOZAWA Hisako,YOSHIDA Hitoshi
The Showa University Journal of Medical Sciences 26(1), 75-83, 2014
… LPD markers, such as cryoglobulinemia, high levels of rheumatoid factor (RF), hypocomplementemia, and B cell clonality, were measured and analyzed along with viral factors. …
NAID 130004691847

ネフローゼ症候群を呈し，非典型的な経過をたどった溶連菌感染後急性糸球体腎炎の1 例

橋本淳也,浅野貴子,加藤環,釜江智佳子,若松太,尾田高志,大橋隆治,長田道夫,野々山恵章
Nihon Shoni Jinzobyo Gakkai Zasshi 26(2), 278-284, 2014
ネフローゼ症候群，遷延する蛋白尿，反復する肉眼的血尿発作など，溶連菌感染後急性糸球体腎炎（PSAGN）としては非典型的な経過をたどった症例を経験した。症例は11 歳男児。一過性の肉眼的血尿発作の3 か月後に溶連菌感染に伴う肉眼的血尿・蛋白尿を認めた。低補体血症はなく，慢性腎炎の急性増悪と考えたが，感染から約3週間後に，肉眼的血尿・ネフローゼ症候群・低補体血症を伴う急性糸球体腎炎を発症した。初回腎生 …
NAID 130004495422

Related Pictures

★リンクテーブル★

リンク元

「低補体血症」

Complement deficiency
Classification and external resources
Specialty	hematology
ICD-10	D84.1
ICD-9-CM	279.8
OMIM	217000 120820, 120900, 610102
DiseasesDB	1847 1869 , 1873 , 7384 , 34381
eMedicine	med/419 ped/447

　　[★]

英: hypocomplementemia
関: 補体

原因

コレステロール塞栓症、悪性関節リウマチ、ループス腎炎、溶連菌感染後急性糸球体腎炎、膜性増殖性糸球体腎炎、クリオグロブリン血症、C肝炎ウイルス関連腎症

低補体血症＋腎炎

YN.E-53

[GorbachBartlett2004-1] Winkelstein, Jerry A. (2004). "The Complement System". In Gorbach, Sherwood L.; Bartlett, John G.; Blacklow, Neil R. Infectious Diseases. Lippincott Williams & Wilkins. pp. 8–13. ISBN 978-0-7817-3371-7.

[2] 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.

[3] "hypocomplementemia" at Dorland's Medical Dictionary

[4] Complement-Related Disorders at eMedicine

[Harrison.27s_Principles_of_Internal_Medicine.2C_17th_Edition-5] Fauci, Anthony; Braunwald, Eugene; Kasper, Dennis; Hauser, Stephen; Longo, Dan; Jameson, J.; Loscalzo, Joseph, eds. (2008). Harrison's Principles of Internal Medicine (17th ed.). ISBN 978-0-07-146633-2. ^{[page needed]}

[6] Zuraw, Bruce L. (2008). "Hereditary Angioedema". New England Journal of Medicine 359 (10): 1027–36. doi:10.1056/NEJMcp0803977. PMID 18768946.

[7] ttp://www.patient.co.uk/doctor/complement-deficiencies^{[full citation needed]}

[8] ttp://www.omim.org/entry/217000^{[full citation needed]}

匿名

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案内

案内

hypocomplementemia