Tus qauv qub txeeg qub teg ntawm daim ntawv enzyme deficiency hauv lub cev yog autosomal recessive Hb M yog los ntawm tus qauv autosomal dominant. Tsis muaj kev koom tes ntawm kev sib deev thiab qhov zaus ntawm congenital methemoglobinemia methemoglobinemia Methemoglobinemia tshwm sim thaum cov qe ntshav liab (RBCs) muaj methemoglobin nyob rau theem siab dua 1% Qhov no tuaj yeem yog los ntawm congenital ua, nce synthesis, los yog txo qhov kev tshem tawm. Kev nce qib kuj tseem tuaj yeem tshwm sim los ntawm kev cuam tshuam rau cov co toxins uas cuam tshuam rau cov tshuaj tiv thaiv redox, nce qib methemoglobin. https://emedicine.medscape.com › article › 204178-overview
Methemoglobinemia: Xyaum Qhov Tseem Ceeb, Keeb Kwm …
Puas yog methemoglobinemia yog qhov teeb meem tseem ceeb lossis rov ua haujlwm?
Congenital/Genetic/Hereditary Methemoglobinemia
Nws yog ib qho autosomal dominant mob Hauv cov tib neeg no, hemoglobin ruaj khov dua hauv nws daim ntawv oxidized thiab tiv taus kom txo tau. Muaj plaub hom NADH cytochrome b5 reductase deficiencies, tag nrho cov uas yog autosomal recessive disorders: 1.
Yuav ua li cas methemoglobinemia tuaj yeem tshwm sim?
Methemoglobinemia tuaj yeem yog vim qee yam tshuaj, tshuaj, lossis zaub mov lossis nws tuaj yeem tau txais los ntawm tus neeg niam txiv Cov tshuaj muaj xws li benzocaine, nitrates, lossis dapsone. Cov txheej txheem hauv qab no suav nrog qee cov hlau hauv hemoglobin hloov pauv los ntawm ferrous [Fe2+] rau ferric [Fe 3+] form.
YYam hloov pauv yam twg yog methemoglobin?
Hemoglobin uas muaj ferric hlau hu ua methemoglobin. HBB noob hloov uas ua rau methemoglobinemia, beta-globin hom hloov pauv cov qauv ntawm beta-globin thiab txhawb cov heme hlau hloov ntawm ferrous mus rau ferric. Cov hlau ferric tsis tuaj yeem khi cov pa oxygen thiab ua rau cyanosis thiab xim xim av.
Ntau npaum li cas methemoglobin?
Cov lus xaus thiab qhov cuam tshuam: Tag nrho qhov muaj feem ntau ntawm methemoglobinemia yog qis ntawm 0.035%; txawm li cas los xij, muaj kev pheej hmoo ntau ntxiv hauv cov neeg mob pw hauv tsev kho mob thiab nrog tshuaj loog benzocaine.
