Background and Objectives. To explore the role of cis-regulatory sequences within the β globin gene cluster at chromosome 11 on human γ globin gene expression related to Hb E allele, we analyze baseline hematological data and Hb F values together with β globin haplotypes in homozygous Hb E. Patients and Methods. 80 individuals with molecularly confirmed homozygous Hb E were analyzed for the β g...

We evaluated four methods of acute cutaneous injury: physical abrasion, hot and cold branding, and chemical scalds, to predispose channel catfish (Ictalurus punctatus) to columnaris disease, caused by the bacterium Flavobacterium columnare. Only physical abrasion (PA) and hot branding (HB) produced lasting alterations of epithelial architecture. Immersion challenge of PA or HB fish 10 cfu/ml vi...

Fetal hemoglobin was studied in 50 unselected patients with polycythemia vera with chemical determinations of Hb F and measurements of F-cell levels using fluorescent anti-Hb F antibodies. Although in the majority of the patients Hb F production did not differ from that in normal controls, in 20% of the patients F-cell values were above the normal range. There was no correlation between F-cell ...

Sickle cell disease (SCD) is a monogenetic disorder caused by single amino acid change in the beta globin gene. This mutation facilitates the production of unstable sickle hemoglobin (Hb S) which upon deoxygenation forms higher order aggregates which cause erythrocyte rigidity. These rigid cells block the microvasculature resulting in vaso-occlusion, tissue ischemia, organ damage, pain and deat...

Wit/i the technical assistance of Anne Council F ETAL HEMOGLOBIN ( Hb F) is the major respiratory pigment formed during intrauterine life. In the adult, however, only trace concentrations normally are present. In man there are at least two distinct conditions in which genetic factors cause elevated levels of Hb F in adult life. They are the hereditary persistence of fetal hemoglobin syndromes a...

Maximal fetal hemoglobin (Hb F) elevations in the baboon subsequent to phenyl hydrazine-induced hemolytic anemia, bleeding, bleeding plus hydroxyurea (HU), or cytosine arabinoside were two to three times lower than those achieved with bleeding plus 5-azacytidine (azaC). Because, in the baboon, maximal elevations in F cell numbers occurred with bleeding alone, changes in the levels of Hb F in he...

High performance liquid chromatography (HPLC) demonstrated advantages over conventional procedures employed in newborn and adult hemoglobinopathy screening programs for the identification of Hb variants has promoted the need to reassess our knowledge of hemoglobin reference ranges as it relates to HPLC quantitation. In this study, the HPLC hemoglobin reference ranges derived from 200 normal Afr...

A modification of a high pressure liquid chromatographic (HPLC) procedure is described that enables the complete separation and quantitation of the A gamma T, A gamma I, and G gamma chains in human fetal hemoglobin. The method, which is fast and accurate, requires 5 to 2000 micrograms Hb F. The purity of the Hb F is not essential and admixture of up to 70% adult Hb does not interfere with the d...

Accurate and precise hemoglobin separation and the quantitation of Hb A2 and Hb F are essential for the diagnosis of the thalassemias and hemoglobinopathies. Presented in this study is the validation of the the Hb A2 assay of the HbGold analyzer, a fully automated liquid chromatography system for hemoglobin separation and quantitation. Variability of Hb A2 quantitation was quite low; the CV's o...