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Erworbene vitamin-B₆-responsive, primäre sideroblastische Anämie, ein Enzymdefekt der Hämsynsthese

Meier, P. J. and Giger, U. and Brändli, O. and Fehr, J.. (1981) Erworbene vitamin-B₆-responsive, primäre sideroblastische Anämie, ein Enzymdefekt der Hämsynsthese. Schweizerische medizinische Wochenschrift, Jg. 111 , Nr. 41. pp. 1533-1535.

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Official URL: http://edoc.unibas.ch/dok/A5261817

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Abstract

The activity of delta-aminolevulinic acid synthetase (ALAS), the rate-limiting enzyme in heme synthesis, has been found to be markedly reduced (13% of controls) in erythroblasts of a patient with acquired, primary sideroblastic anemia. Administration of vitamin B6 (pyridoxin, 200-600 mg/d) resulted in complete reconstitution of erythroblastic ALAS-activity with concomitant disappearance of all hematologic abnormalities. The findings show that the therapeutic efficacy of pyridoxin in primary sideroblastic anemia is due to its effect on defective ALAS. More generally, the data support the view that almost all features of primary sideroblastic anemia can be ascribed to a disturbance of heme synthesis in erythroblasts.
Faculties and Departments:11 Rektorat und Verwaltung > Vizerektorat Forschung
UniBasel Contributors:Meier-Abt, Peter J.
Item Type:Article, refereed
Article Subtype:Book Review
Bibsysno:Link to catalogue
Publisher:Benno Schwabe
ISSN:0036-7672
Note:Note: Summary in english -- Note: Standartisierter englischer Titel: Acquired, vitamin B6-responsive, primary sideroblastic anemia, an enzyme deficiency in heme synthesis -- Publication type according to Uni Basel Research Database: Journal item
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Last Modified:22 Mar 2012 14:24
Deposited On:22 Mar 2012 13:38

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