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‘Gd(-) Hôtel Dieu’: A new G-6PD variant with chronic hemolysis in a Negro patient from Senegal

✍ Scribed by Axel Kahn; Christian Dao; Dominique Cottreau; Georges Bilski-Pasquier

Publisher: Springer
Year: 1977
Tongue: English
Weight: 236 KB
Volume: 39
Category: Article
ISSN: 0340-6717
DOI: 10.1007/bf00295431

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✦ Synopsis

A G-6PD deficiency was detected in a Negro patient from Senegal suffering from congenital nonspherocytic hemolytic anemia. The main characteristics of this variant were: profound defect of G-6PD activity in the red cells, decreased immunologic specific activity, fast electrophoretic mobility, decreased Km-G-6P and normal Km-NADP+, normal inhibition by ATP and NADPH, slightly increased utilization of the substrate analogues, slightly biphasic pH curve, high heat lability, subnormal activation energy. The characteristics of this variant being unique, it was called 'G-6PD Hôtel Dieu.'

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✍ J. L. Viallard; D. Cottreau; A. Kahn; B. Dastugue 📂 Article 📅 1979 🏛 Springer 🌐 English ⚖ 132 KB

A low rate value of G6PD was found in red blood cells from a Cambodian boy. Enzyme mapping was performed according to the WHO standard methods. G6PD presented all the characteristics of the A(-) variant encountered in the Negroes and behaved distinct from fast migrating enzymes described in China. N