WordNet
- the 8th letter of the Roman alphabet (同)h
PrepTutorEJDIC
- hydrogenの化学記号
- 鉛筆の硬度 / 《俗》heroin
- hemoglobin
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/05/03 23:09:42」(JST)
[Wiki en表示]
Hemoglobin C |
Classification and external resources |
ICD-10 |
D58.2 |
ICD-9 |
282.7 |
DiseasesDB |
29693 |
MedlinePlus |
000572 |
MeSH |
D006445 |
Hemoglobin C (abbreviated as Hb C or HbC) is an abnormal hemoglobin in which substitution of a glutamic acid residue with a lysine residue at the 6th position of the β-globin chain has occurred (E6K substitution).[1]
Contents
- 1 Clinical significance
- 2 Presentation
- 3 Combinations with other conditions
- 4 Epidemiology
- 5 References
- 6 External links
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Clinical significance
This mutated form reduces the normal plasticity of host erythrocytes causing a hemoglobinopathy. In those who are heterozygous for the mutation, about 28–44% of total hemoglobin (Hb) is HbC, and no anemia develops.
In homozygotes, nearly all Hb is in the HbC form, resulting in mild hemolytic anemia.[2]
Presentation
Target cells, microspherocytes and HbC crystals are found in a blood smear from a homozygous patient.
Combinations with other conditions
Individuals with sickle cell–hemoglobin C (HbSC), have the gene for HbS inherited from one parent and the gene for HbC is inherited from the other parent: they are "heterozygous". Since HbC does not polymerize as readily as HbS, there is less sickling (fewer sickle cells). The peripheral smear demonstrates mostly target cells and only a few sickle cells. There are fewer acute vaso-occlusive events. However, persons with hemoglobin SC disease (HbSC) have more significant retinopathy, ischemic necrosis of bone, and priapism than those with pure SS disease. [2]
Epidemiology
Hemoglobin C gene is found in 2-3% of US Blacks while 8% of US Blacks have hemoglobin S (Sickle) gene. Thus Hemoglobin SC disease is significantly more common than Hemoglobin CC disease. Hemoglobin C is found predominantly in Yorubas (A profile of sickle cell disease in Nigeria By O.O Akinyanju, Department of Medicine University of Lagos). It is also found in areas of West Africa, such as Ghana, where Yorubas once lived (MAN Volume 56 March 1956 pages 34-36)[3]
References
- (2007) Olubunmi A.O. The Rise and Fall of The Yoruba Race 10,000BC-1960AD ISBN 978-2457-38-8
- ^ Hemoglobin C (HBB, GLU6LYS)
- ^ a b Nagel RL, Fabry ME, Steinberg MH (September 2003). "The paradox of hemoglobin SC disease". Blood Rev. 17 (3): 167–78. doi:10.1016/S0268-960X(03)00003-1. PMID 12818227. Retrieved 2009-02-24.
- ^ Fairhurst RM, Fujioka H, Hayton K, Collins KF, Wellems TE (April 2003). "Aberrant development of Plasmodium falciparum in hemoglobin CC red cells: implications for the malaria protective effect of the homozygous state". Blood 101 (8): 3309–15. doi:10.1182/blood-2002-10-3105. PMID 12480691.
External links
- Hemoglobin C at the US National Library of Medicine Medical Subject Headings (MeSH)
- Hemoglobin C Disease at the US National Library of Medicine Medical Subject Headings (MeSH)
Proteins: hemeproteins
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Globins |
Hemoglobin
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Subunits
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Alpha locus on 16: α (HBA1, HBA2) · ζ (HBZ) · θ (HBQ1) · μ (HBM)
Beta locus on 11: β (HBB) · δ (HBD) · γ (HBG1, HBG2) · ε (HBE1)
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Tetramers
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stages of development: HbA (α2β2) · HbA2 (α2δ2) · HbF/Fetal (α2γ2) · HbE Gower 2 (α2ε2) · HbE Gower 1 (ζ2ε2)
pathology: HbH (β 4) · Barts (γ 4) · HbS (α 2β S2) · HbC (α 2β C2) · HbE (α 2β E2)
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Compounds
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Carboxyhemoglobin · Carbaminohemoglobin · Oxyhemoglobin/Deoxyhemoglobin · Sulfhemoglobin
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Other human
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Glycated hemoglobin · Methemoglobin
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Nonhuman
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Chlorocruorin · Erythrocruorin
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Other
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human: Myoglobin (Metmyoglobin) · Neuroglobin · Cytoglobin
plant: Leghemoglobin
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Other |
Cytochrome (Cytochrome b, Cytochrome P450) · Hemocyanin · Methemalbumin
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see also disorders of globin and globulin proteins
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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Pathology: hematology, hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287)
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Red
blood cells |
↑ |
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↓ |
Anemia |
Nutritional |
- Micro-: Iron deficiency anemia
- Macro-: Megaloblastic anemia
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Hemolytic
(mostly Normo-) |
Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski-Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
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Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
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Aplastic
(mostly Normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
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Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
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Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
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Coagulation/
coagulopathy |
↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- acquired:Thrombocytosis
- DIC
- Congenital afibrinogenemia
- Purpura fulminans
- autoimmune
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↓ |
Hypo-
coagulability |
Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- Heparin-induced thrombocytopenia
- May-Hegglin anomaly
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Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
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Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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UpToDate Contents
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English Journal
- ABO blood group, hepatitis B viral infection and risk of pancreatic cancer.
- Wang DS, Chen DL, Ren C, Wang ZQ, Qiu MZ, Luo HY, Zhang DS, Wang FH, Li YH, Xu RH.SourceState Key Laboratory of Oncology in South China, Sun Yat-sen University Cancer Center, Guangzhou, China; Department of Medical Oncology, Sun Yat-sen University Cancer Center, Guangzhou, China.
- International journal of cancer. Journal international du cancer.Int J Cancer.2012 Jul 15;131(2):461-8. doi: 10.1002/ijc.26376. Epub 2011 Nov 30.
- Little is known about the role of association between ABO blood type and risk of pancreatic cancer develops through effects on hepatitis B viral (HBV) infection. Our study aimed to determine whether joint ABO blood type and HBV infection could increase the risk for pancreatic cancer. A total of 645
- PMID 21858814
Japanese Journal
- A Hospital-Based Analysis in the Department of Clinical Chemistry for the Patients with HBV Reactivation after Anti-Cancer or Immunosuppressive Therapy
- MATSUZAKI Tomoe,OHKUBO Kumiko,YUKI Makiko [他]
- 臨床病理 : 日本臨床検査医学会誌 63(3), 297-304, 2015-03
- NAID 40020422190
- 人体通信技術 : 人とクルマのコミュニケーション (特集 次世代のクルマのHMI技術) -- (クルマ領域以外の最新HMI)
Related Links
- HBC。札幌市中央区。ニュース、テレビ・ラジオ番組表、イベント、プレゼント、ショッピング情報。
- 北海道・札幌のテレビ・ラジオ局。北海道の最新情報を毎日発信。グルメ、観光スポットなど、北海道のおすすめ情報や、動画ニュース、ライブカメラ、さっぽろ雪まつり、観光情報なども提供。
Related Pictures
★リンクテーブル★
[★]
ヘモグロビンC、HbC
[★]
- 同
- COHb, HbCO
- 関
- Carboxyhemoglobin
[★]
[★]
ヘモグロビン hemoglobin