Sickle-cell anemia fetal hemoglobin

M. A., Moore, J. W., Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia, Blood 69 (1987), p. 109-116... 

Dover GJ, Brusilow S, Charache S (1994) Induction of fetal hemoglobin production in subjects with sickle cell anemia by oral sodium phenylbutyrate. Blood 84 339-343 El Kharroubi A, Martin MA (1996) cis-acting sequences located downstream of the human immunodeficiency virus type 1 promoter affect its chromatin structure and transcriptional activity. Mol Cell Biol 16 2958-2966... 

Individuals with sickle cell anemia have a functional gene for the gamma chain. If the production of fetal hemoglobin could be turned back on the affected individuals could function normally except during pregnancy. 

H59. Huisman, T. H. J., Schroeder, W. A., Bouver, N., Miller, A., Shelton, J. R., Shelton, J. B., and Apell, G., Chemical heterogeneity of fetal hemoglobin in subjects with sickle cell anemia, homozygous Hb-C disease, SC-disease, and various combination of hemoglobin variants. Clin. Chim. Acta 38, 5-16 (1972). 

Resar LMS, Segal JB, Fitzpatric LK, et al. Induction of fetal hemoglobin synthesis in children with sickle cell anemia on low-dose oral sodium phenylbutyrate therapy. J Pediatr Hematol Oncol 2002 24 737— 741. 

Other approaches to drug therapy for sickle-cell anemia are to develop competitive inhibitors of sickling, to switch on the fetal Hb genes in the adult, and to develop ways of enlarging the erythrocytes to reduce the concentration of hemoglobin. 

Two observations have led to a possible treatment of these diseases. First, physicians found some sickle cell anemia patients who suffered only mild symptoms because they continued to make high levels of fetal hemoglobin. Second was the observation that some babies born to diabetic mothers contin-... 

In addition. Will Sichel was treated with hydroxyurea therapy, which increases the production of red blood cells containing fetal hemoglobin. ITbF molecules cannot participate in sickling. Will Sichel s acute symptoms gradually subsided. Had his severe pain persisted, partial exchange blood transfnsions would have been considered because no other effective therapy is cnrrently available. Patients with sickle cell anemia periodically experience sickle cell crises, and Will s physician nrged him to seek medical help whenever symptoms reappeared. 

Itano, H. A.. The identification of fetal hemoglobin ill sickle-cell anemia by electrophoretic, spectrophoto-metric, and solubility studies unpublished investigation. 

Goodman, M., and Campbell, D. H. 1953. DifFetences in antigenic specificity of human normal adult, fetal, and sickle cell anemia hemoglobin. Blood 8, 422. 

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