補体不全症
WordNet
- make complete or perfect; supply what is wanting or form the complement to; "I need some pepper to complement the sweet touch in the soup"
- a complete number or quantity; "a full complement"
- one of a series of enzymes in the blood serum that are part of the immune response
- something added to complete or embellish or make perfect; "a fine wine is a perfect complement to the dinner"; "wild rice was served as an accompaniment to the main dish" (同)accompaniment
- number needed to make up a whole force; "a full complement of workers" (同)full complement
- a word or phrase used to complete a grammatical construction
- either of two parts that mutually complete each other
PrepTutorEJDIC
- (あるものを完全にするため)(…を)補う物(事)《+『of』+『名』》 / 補語(文法で文の成分の一つ) / (必要な)全数,全量;(船の)定員 / …を補足する,補う
- 〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/05/08 14:06:35」(JST)
[Wiki en表示]
Complement deficiency |
Classification and external resources |
ICD-10 |
D84.1 |
ICD-9 |
279.8 |
OMIM |
217000 120820, 120900, 610102 |
DiseasesDB |
1847 1869, 1873, 7384, 34381 |
eMedicine |
med/419 ped/447 |
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.
- 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.
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 [edit]
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]
These levels can be used to distinguish conditions:
- Systemic lupus erythematosus causes low C3 and C4
- Membranoproliferative glomerulonephritis causes low C3, but normal C4
See also [edit]
- Paroxysmal nocturnal hemoglobinuria
References [edit]
- ^ Sherwood L. Gorbach; John G. Bartlett; Neil R. Blacklow (2004). Infectious diseases. Lippincott Williams & Wilkins. pp. 11–. ISBN 978-0-7817-3371-7. Retrieved 30 May 2010.
- ^ Sjöholm AG, Jönsson G, Braconier JH, Sturfelt G, Truedsson L (2006). "Complement deficiency and disease: an update". Mol. Immunol. 43 (1–2): 78–85. doi:10.1016/j.molimm.2005.06.025. PMID 16026838.
- ^ "hypocomplementemia" at Dorland's Medical Dictionary
- ^ http://emedicine.medscape.com/article/136368-overview
Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4)
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Primary |
Antibody/humoral (B)
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Hypogammaglobulinemia
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- X-linked agammaglobulinemia
- Transient hypogammaglobulinemia of infancy
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Dysgammaglobulinemia
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- IgA deficiency
- IgG deficiency
- IgM deficiency
- Hyper IgM syndrome (2
- 3
- 4
- 5)
- Wiskott-Aldrich syndrome
- Hyper-IgE syndrome
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Other
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- Common variable immunodeficiency
- ICF syndrome
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T cell deficiency (T)
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- thymic hypoplasia: hypoparathyroid (Di George's syndrome)
- euparathyroid (Nezelof syndrome
- Ataxia telangiectasia)
peripheral: Purine nucleoside phosphorylase deficiency
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Severe combined (B+T)
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- x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
- Omenn syndrome
- ZAP70 deficiency
- Bare lymphocyte syndrome
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Acquired |
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Leukopenia:
Lymphocytopenia |
- Idiopathic CD4+ lymphocytopenia
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Complement deficiency |
- C1-inhibitor (Angioedema/Hereditary angioedema)
- Complement 2 deficiency/Complement 4 deficiency
- MBL deficiency
- Properdin deficiency
- Complement 3 deficiency
- Terminal complement pathway deficiency
- Paroxysmal nocturnal hemoglobinuria
- Complement receptor deficiency
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cell/phys/auag/auab/comp, igrc
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UpToDate Contents
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- 1. 補体系の遺伝性疾患 inherited 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. 補体系の後天性疾患 acquired disorders of the complement system
- 4. 感染症を繰り返す成人に対するアプローチ approach to the adult with recurrent infections
- 5. 髄膜炎菌感染の疫学 epidemiology of neisseria meningitidis infection
English Journal
- Genetic risk factors of systemic lupus erythematosus in the Malaysian population: a minireview.
- Chai HC, Phipps ME, Chua KH.SourceJeffrey Cheah School of Medicine and Health Sciences, Monash University, Sunway Campus, 46150 Selangor Darul Ehsan, Malaysia.
- Clinical & developmental immunology.Clin Dev Immunol.2012;2012:963730. Epub 2011 Sep 20.
- SLE is an autoimmune disease that is not uncommon in Malaysia. In contrast to Malays and Indians, the Chinese seem to be most affected. SLE is characterized by deficiency of body's immune response that leads to production of autoantibodies and failure of immune complex clearance. This minireview att
- PMID 21941582
Japanese Journal
- Membranoproliferative pattern of glomerular injury associated with complement component 9 deficiency due to Arg95Stop mutation
- MIURA Takayoshi,GOTO Shin,IGUCHI Seitaro,SHIMADA Hisaki,UENO Mitsuhiro,NISHI Shin-ichi,NARITA Ichiei
- Clinical and experimental nephrology 15(1), 86-91, 2011-02-01
- NAID 10029004557
- Procarboxypeptidase R Deficiency Causes Increased Lethality in Concanavalin A-Induced Hepatitis in Female Mice
- Asai Suzuka,Kimbara Noriaki,Tada Toyohiro,Imai Masaki,Campbell William,Okada Hidechika,Okada Noriko
- Biological and Pharmaceutical Bulletin 33(7), 1256-1259, 2010
- Carboxypeptidase R (CPR), also known as thrombin-activatable fibrinolysis inhibitor (TAFI), is an enzyme generated by proteolytic cleavage of its zymogen (proCPR). CPR removes the C-terminal arginine …
- NAID 130000300299
Related Links
- Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. The disorders can be divided into two categories: Disorders of the proteins that act to inhibit the complement ...
- 15 Apr 2013 ... Complement Deficiencies. The complement system is part of the innate immune system.
Related Pictures
★リンクテーブル★
[★]
- 英
- complement deficiency
- 関
- 補体
[★]
- 不足、欠乏、欠失、欠如、欠損、不十分。栄養不足、栄養素欠乏、欠乏症。(遺伝子)(染色体内の)遺伝子欠失
- 欠けているもの、不足している物。不足分。不完全なもの、欠点のあるもの
- 関
- absence, agenesis, dearth, defect, defective, deficient, deficit, delete, deletion, deletional, depletion, deprivation, deprive, lack, miss, missing, morphological defect, paucity, scarce, scarcity, starve
[★]
- 補って完全にする物、補完物。(免疫)補体
- (必要な)全数、善良