A Second Family with HB Minneapolis-Laos or α₂β₂118(GH1)Phetyr

K. Kleman Children's Hospital Oakland Research Institute, 747 52nd Street, Oakland, CA, 94609, USA

B. Lubin Children's Hospital Oakland Research Institute, 747 52nd Street, Oakland, CA, 94609, USA

A. Kutlar Department of Cell and Molecular Biology Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, GA, 30912-1100, USA

J. B. Wilson Department of Cell and Molecular Biology Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, GA, 30912-1100, USA

B. B. Webber Department of Cell and Molecular Biology Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, GA, 30912-1100, USA

T. H.J. Huisman Department of Cell and Molecular Biology Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, GA, 30912-1100, USA

Abstract

Recently, a one-year-old /Cambodian child was admitted to the Children's Hospital Medical Center because of pneumonia and severe anemia. Hematological analyses gave these results: Hb 3.6 g/dl, MCV 50 fl, RBC 2.3 X 10¹²/1, free erythrocyte ptotoparphysin 351 μ/100 ml RBC (normal: <100), ferritin 25 ng/ml, iron saturation 10%. The child recovered after transfusion and treatment for pneumonia and for severe iron deficiency. Hemoglobin (Hb) analysis of a blood sample collected prior to transfusion, by cellulose acetate electrophoresis at alkaline pH (1), showed tow components, one Quantitation of these Hbs was by cation exchange high performance liquid chromatography (HPLC) (2); the Hb E-like component constituted 26% of the hemoglobin.

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A Second Family with HB Minneapolis-Laos or α₂β₂118(GH1)Phetyr