Prevalence of sickle cell disease, hemoglobin S, and hemoglobin C among Haitian newborns

Hemoglobin S gelation and sickle cell disease.

By William A. Eaton and James Hofrichter T HE FUNDAMENTAL cause ofsickle cell disease is the decreased deformability of the sickled red cell produced by gelation of hemoglobin S. Partial inhibition of gelation should therefore reduce clinical severity, while complete inhibition should result in a “cure.” These basic ideas have stimulated an enormous effort to understand the gelation process in ...

Intraerythrocytic Hemoglobin Crystals in Sickle Cell-hemoglobin C Disease.

Sickle cell-hemoglobin C disease. Quantitative determination of iron kinetics and hemoglobin synthesis.

Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB.

Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2-121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9...

Hemoglobin S Antilles: a variant with lower solubility than hemoglobin S and producing sickle cell disease in heterozygotes.

We have found a sickling variant, Hb S Antilles, alpha 2 beta 2(6 Glu----Val, 23 Val----Ile), that has the same electrophoretic mobility as Hb S but a distinct isoelectric focus and produces sickling in the carriers of the Hb A/S Antilles trait. The carriers' erythrocytes tend to sickle at O2 partial pressures similar to those that induce sickling in Hb S/C disease. Pure deoxy-Hb S Antilles is ...