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9 Cards in this Set
- Front
- Back
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gneral structure of HB
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tetramer of globin polypeptide chains( pair of alfa -like and pair of non alfa like
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adult hb structure
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hem A -- a2b2
hem A2-- a2 b2 |
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fetal hb structure
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hm F- a2 y2
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symptoms of hemoglobinopaties
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variable from mild anemia to chronic cyanosis, const fatigue and intrauterine death
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ds
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with high oxygen affinity hemoglobins- cyanosis with normal PO2
with methhemoglobin level> 30%- BROWN CHOCOLATE SERUM |
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pathology
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mostly inherited
rarely acquired--- toxin , neoplasms |
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classifications
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1 structural hemoglobinopaties- mutated amino acids sequences . as in
a) abnormal polymerization-( Hb S) b)reduced solubility c) altered oxygen affinity - 2 types-- 1- increased oxygen affinity-- hb zurich 2... decrease oxygen affinity.. hb kansee d) talasemia f) herediatery persistence of hm F e)acquered hemoglobinopathy--- methhemoglobinemia |
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description
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oxydation of Hb from ferrous( Fe ++) to ferric ( Fe+++) stated wich doesnot transfer oxygen
may be due to 1. globin mutation leading to methhemoglobin formation ( M Hb) 2 toxic ' oxydation"; to meth hemoglobinemia by foreign sunstances ( acetanilid, phenacetin, nitrites, anilin and many others) |
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ts steps
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M Hb- no tx
reductase deficience- oral methylene blue ( 1 mgt/ kg IV in emergency situation or orally in milder forms or vit C |
