B6反応性貧血

書誌事項

タイトル別名
  • Vitamin B6 Responsive Anemia
  • B6 ハンノウセイ ヒンケツ

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抄録

The etiology and pathogenesis of pyridoxine-responsive anemia (PRA) were briefly discussed on the basis of literature survey and our own observations. It is apparent that, in the majority of patients, the conditions are not due to the dietary deficiency, but due to some more basic metabolic abnormality in hemoglobin formation, which could make the corresponding enzyme system dependent upon vitamin B6. The enzyme defect found in PRA may not be uniform, but may be variable. Horrigan and Harris classified PRA into two groups, i.e. classic form and non-classic form. In the former, the hemopoiesis is strictly dependent upon the administration of vitamin B6, and other hematinics (Fe, Vit. B12, folic acid) are without effect. In the latter, however, some responses may follow the administration of other hematinics especially folic acld. We have reported two cases of non-classic form of PRA, responding to both vit. B6 and folic acid. Investigations on the folate metabolism in these two cases revealed that the primary metabolic defects could be deficiencies of formiminotransferase and N5-methyl-tetrahydrofolate transferase respectively, leading to the impaired nucleic acid synthesis in each case.

収録刊行物

  • 臨床血液

    臨床血液 11 (3), 358-360, 1970

    一般社団法人 日本血液学会

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