Sickle cell disease and fetal hemoglobin

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Induction of fetal hemoglobin in sickle cell disease.

THIS ISSUE OF BLOOD features a report by Atweh et al1 on the induction of fetal hemoglobin (Hb F) by pulse butyrate. This study is a solid addition to the recent and gratifying momentum in the development of effective therapy for sickle cell disease. Underlying this report is a series of novel and convincing in vivo studies extending from model systems in the chicken,2 sheep,3 and baboons4-6 to...

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Fetal hemoglobin in sickle cell anemia

Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high level...

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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 ...

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Fetal hemoglobin and hemolysis markers in sickle cell anemia☆

It has been known for many years that high levels of fetal hemoglobin (Hb F) have an important clinical benefit in patients with sickle cell anemia (SCA). This knowledge was initially based on the observation that populations of Indian and Arabian SCA patients with high Hb F levels have a milder clinical form of the disease.1 Later on, epidemiological studies demonstrated that patients with Hb ...

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Fetal hemoglobin during infancy and in sickle cell adults.

BACKGROUND Fetal hemoglobin has been implicated in the modulation of sickle cell crisis though it is functional during infancy. OBJECTIVE The purpose of this study was to determine the waning time of fetal hemoglobin (HbF) and its persistence in later life. METHOD Ninety infants aged 0-12 months, admitted at hospital, were tested for their HbF levels. Adult patients numbering 690 were also ...

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ژورنال

عنوان ژورنال: Saudi Journal of Medicine and Medical Sciences

سال: 2018

ISSN: 1658-631X

DOI: 10.4103/sjmms.sjmms_128_18