By PHAEDON FESSAS AND GEORGE STAMATOYANNOPOULOS H EREDITARY PERSISTENCE of fetal hemoglobin was described in a Greek family in 1961 and included the combination of this anomaly with aand with -thalassemia.1’2 Since then, further cases or families have been observed in Greece, two of which have been reported in whole or in part in Greek medical literature.3’4 This material now permits an adequat...

Since KORBER1) discovered in 1865 that human fetal hemoglobin is more resistant to alkali than the adult one, numerous works indicating differences in properties of the fetal and the adult pigment have been published . Biochemically, a considerable dissimilarity is found in their amino acid compositions and primary structures, i. e. amino acid sequences2). Physiologically , it has long been kno...

A persistent elevation of the fetal hemoglobin in the low-density, reticulocyte-rich cell frac(Hgb F) level (5%-15%) was observed in a tion. In contrast, the highest Hgb F levels were 22-yr-old white male with paroxysmal nocfound in the high-density, reticulocyte-poor turnal hemoglobinuria (PNH). Acid treatment fraction. Further evidence for the segregation of the peripheral smear (Betke-Kleiha...

Increased levels of fetal hemoglobin (HbF) can ameliorate the severity of the β-hemoglobin disorders, sickle cell disease (SCD) and β-thalassemia, which are major sources of morbidity and mortality worldwide. As a result, there has been a longstanding interest in developing therapeutic approaches for inducing HbF. For more than 3 decades, the majority of HbF inducers developed were based on emp...

Pulse oximetry has become a standard of care in anesthetic, pediatric and neonatal practice (l,2). It measures the per cent saturation of hemoglobin based upon the Beer Lambart's spectrophotometric principle of differential absorption of red and infrared light waves (660 nm and 940 nm, respectively) by the oxyhemoglobin and reduced hemoglobin(3). Pulse oximetry has been recently adopted for con...

INTRODUCTION The hemoglobin S (HbS) mutation is accompanied by other mutations in the region of chromosome 11 known as "beta globin cluster". The pattern of combination of these polymorphisms giving rise to the haplotypes that co-inherit the HbS mutation, are called haplotypes bs, and are of great epidemiological and clinical significance. OBJECTIVE The frequencies of major haplotypes associa...

Studies were carried out during the neonatal period in three infants with D1 trisomy syndrome to measure the proportion of fetal and adult hemoglobin being synthesized. These values were compared on the one hand to those previously reported from samples obtained from cord blood of normal newborn infants ranging from 25 to 43 wk gestation, and on the other hand to those values determined in crit...

In a previous report, it was suggested that intrauterine crowding impaired fetal erythropoiesis and that fetal erythropoiesis was accelerated in Meishan pigs during early pregnancy. Because these conclusions were based on limited numbers of observations, the present experiment was undertaken to provide a more extensive investigation of these phenomena. Intact white crossbred gilts, unilaterally...

Studies were carried out during the neonatal period in three infants with D1 trisomy syndrome to measure the proportion of fetal and adult hemoglobin being synthesized. These values were compared on the one hand to those previously reported from samples obtained from cord blood of normal newborn infants ranging from 25 to 43 wk gestation, and on the other hand to those values determined in crit...

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...