"FAST" HEMOGLOBIN COMPONENT FOUND IN UMBILICAL-CORD BLOOD OF THAI BABIES

PEDIATRICS ◽  
1959 ◽  
Vol 24 (1) ◽  
pp. 43-49
Author(s):  
Soodsarkorn Tuchinda ◽  
Chitra Vareenil ◽  
Partraporn Bhanchit ◽  
Virginia Minnich
Keyword(s):  
Cord Blood ◽  
Saline Solution ◽  
Fetal Hemoglobin ◽  
Paper Electrophoresis ◽  
Fast Component ◽  
Thai Population ◽  
Hemoglobin E ◽  
Normal Limits ◽  
Abnormal Hemoglobin ◽  
Hemoglobin A2

Four hundred and fifteen specimens of cord blood collected from the Thai population were examined; 22 contained an electrophoretically "fast"-moving component of hemoglobin, an incidence of 5.2%. No other abnormal hemoglobin was found in the specimens of cord blood. Eleven of the babies with abnormal hemoglobin were examined; hematologic findings were within normal limits. Five were followed for 2½ to 8 months; the abnormal component disappeared within 76 to 101 days. One of these children subsequently developed hemoglobin E. Another baby, 2 days of age when first seen, had the "fast" component of hemoglobin in the blood. She was the only child in the group who had marked hepatomegaly, splenomegaly, reticulocytosis, and nucleated erythrocytes in the peripheral blood. These abnormalities subsequently disappeared, but at 112 days a small amount of the "fast" component of hemoglobin persisted and hemoglobin E had appeared. The blood of 412 mothers revealed an incident of 10% hemoglobin E, the only abnormal hemoglobin detected by paper electrophoresis. The concentration of hemoglobin A2 was determined on the blood drawn from nine parents of babies with abnormal "fast" hemoglobin; the value was normal in all instances. Microcytosis and increased resistance of the erythrocytes in hypotonic saline solution, however, was found frequently among the parents of affected babies. The abnormal "fast" component of hemoglobin was identical with "Barts" hemoglobin. Its characteristics suggest that it may be an abnormal fetal hemoglobin.

scholarly journals A New Variant of Human Fetal Hemoglobin: Hb FRoma

Blood ◽  
1963 ◽  
Vol 22 (5) ◽  
pp. 545-553 ◽  
Author(s):  
E. SILVESTRONI ◽  
I. BIANCO
Keyword(s):  
Cord Blood ◽  
Electrophoretic Mobility ◽  
Fetal Hemoglobin ◽  
Alkaline Ph ◽  
Healthy Newborn ◽  
New Variant ◽  
Newborn Girl ◽  
Abnormal Hemoglobin ◽  
New Type ◽  
Hb Bart's

Abstract A new type of abnormal fetal hemoglobin, identified in Rome from the cord blood hemolysate of a healthy newborn girl, is described. This abnormal hemoglobin, which the authors provisionally name Hb FRoma, has an electrophoretic mobility at alkaline pH identical to that of Hb Bart’s and a spectrum in the U. V. of the fetal type. It is, however, composed of normal alpha chains and altered gamma chains. Hb FRoma, which was present at birth in the portion of 17 per cent, disappeared completely during the 5th month of life. No abnormal hemoglobins were identified in the parents.

scholarly journals Glycerol-3-phosphate dehydrogenase activity in the red cells of patients with thalassemia

Blood ◽  
1980 ◽  
Vol 55 (4) ◽  
pp. 564-569
Author(s):  
P Fessas ◽  
NP Anagnou ◽  
D Loukopoulos
Keyword(s):  
Cord Blood ◽  
Fetal Hemoglobin ◽  
Thalassemia Major ◽  
Red Cells ◽  
Beta Thalassemia ◽  
Gamma Chain ◽  
Cord Blood Cells ◽  
Chain Synthesis ◽  
Glycerol 3 Phosphate Dehydrogenase ◽  
Normal Adults

L-alpha-Glycerol-3-phosphate dehydrogenase (EC 1.1.1.8) has been reported to be absent in the erythrocytes of normal adults, but can be found in those of cord blood and of thalassemia major. The aid of this study was to investigate whether there is any relation between GDH and gamma-chain synthesis. Erythrocyte GDH activity was determined on 118 different blood samples. It was undetectable in normal adult erythrocytes and definitely high in cord blood cells (23.6 UI/10(11) RBC). Considerable GDH activity was also noted in patients with thalassemia major (11.0 IU10(11) RBC) as well as in cases with pronounced reticulocytosis (11.4 IU/10(11) RBC). Red cells from beta- thalassemia heterozygotes exhibited moderate but distinct GDH activity (5.2 IU/10(11) RBC). After fractionation into young and old erythrocyte populations, clearly higher GDH activity was found in the younger cells; however, there was no significant correlation with the reticulocyte count. Presence of reticulocytes alone appears insufficient to explain the values obtained in cord blood and the thalassemias, especially heterozygous. Furthermore, no direct correlation between GDH and fetal hemoglobin (HbF) was obtained in cord and thalassemic erythrocytes.

An Unusual Type of Hemoglobinopathy Resembling Sickle Cell-Thalassemia Disease in a Jamaican Family

Blood ◽  
1958 ◽  
Vol 13 (6) ◽  
pp. 559-568 ◽  
Author(s):  
L. N. WENT ◽  
J. E. MACIVER
Keyword(s):  
Protective Effect ◽  
Cord Blood ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Three Generations ◽  
The Family ◽  
Follow Up Studies ◽  
Unusual Type ◽  
Mode Of Inheritance

Abstract Three generations of a Jamaican family of African extraction are described, in several members of which an abnormal gene is carried. This gene produces high levels of fetal hemoglobin unassociated with the usual stigmata of thalassemia. It is found in all three generations of the family associated with hemoglobin A only and is also found in at least two members of the family interacting with hemoglobin S. In the latter combination little or no disability results. The mode of inheritance of this abnormal gene is discussed, and reasons are put forward for a possible protective effect of high fetal hemoglobin levels due to inhibition of sickling. The findings in the cord blood of the youngest child, including an unusually high percentage of sickling, are discussed, together with follow-up studies to the age of 25 weeks.

scholarly journals An Elution Procedure for Visualization of Adult Hemoglobins in Human Blood Smears

Blood ◽  
1974 ◽  
Vol 43 (2) ◽  
pp. 239-242 ◽  
Author(s):  
David Kabat
Keyword(s):  
Cord Blood ◽  
Human Blood ◽  
Phosphate Buffer ◽  
Newborn Infants ◽  
Potassium Phosphate ◽  
Fetal Hemoglobin ◽  
Potassium Phosphate Buffer ◽  
Blood Smears

Abstract A procedure is described for visualization of normal and mutant adult hemoglobins in human blood smears. After extraction of blood smears with a concentrated potassium phosphate buffer (2.76 M, pH 7.2), erythrocytes that had adult hemoglobins stained bright red with erythrosin, whereas cells that had only fetal hemoglobin appeared as clear ghosts. Analyses of cord blood from newborn infants indicate that, although most erythrocytes contain only Hb F and a few contain only Hb A, many contain both hemoglobins A and F.

scholarly journals The Interaction of Hemoglobin E With β Thalassemia: A Study of Hemoglobin Synthesis in a Family of Mixed Burmese and Iranian Origin

Blood ◽  
1973 ◽  
Vol 42 (5) ◽  
pp. 783-791 ◽  
Author(s):  
Robert Feldman ◽  
Ronald F. Rieder
Keyword(s):  
Bone Marrow ◽  
Specific Activity ◽  
Family Members ◽  
Hemoglobin F ◽  
Hemoglobin E ◽  
Hemoglobin Synthesis ◽  
Abnormal Hemoglobin ◽  
Thalassemia Trait ◽  
Mixed Origin

Abstract A 5-yr-old girl with hemoglobin E-β thalassemia was discovered in a family of mixed origin. The father is Iranian (β-thalassemia trait) and the mother is Burmese (hemoglobin-E trait). Hemoglobin synthesis was studied in vitro in the blood of the proposita and family members. In the subjects with hemoglobin E trait the ratio of the quantity of hemoglobin A to hemoglobin E was 3:1. However. the βA/βE synthesis ratio in reticulocytes was in the range of 1.5-2.18, and the specific activity of βE was 31%-49% greater than βA, suggesting instability of hemoglobin E with preferential destruction of abnormal hemoglobin. The blood of the proposita exhibited only hemoglobin F and hemoglobin E and reticulocytes and bone marrow showed no βA synthesis. This Iranian β-thalassemia gene is therefore of the β° type. The βE/α synthesis ratio (approximately 0.74) in blood of the proposita was similar to the βA/α ratio in mildly affected relatives with thalassemia trait. These results suggest that the severity of the hemoglobin E-β thalassemia syndrome is attributable to both instability and defective synthesis of hemoglobin E in association with absent βA synthesis due to a β° thalassemia gene.

scholarly journals Paper Electrophoresis of Abnormal Hemoglobins and Its Clinical Applications

Blood ◽  
1954 ◽  
Vol 9 (9) ◽  
pp. 897-910 ◽  
Author(s):  
ARNO G. MOTULSKY ◽  
MILTON H. PAUL ◽  
E. L. DURRUM
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Paper Electrophoresis ◽  
Clinical Applications ◽  
Hemoglobin C ◽  
Hemoglobin Type ◽  
Cell Life ◽  
Practical Tool ◽  
Adult Hemoglobin

Abstract 1. Paper electrophoresis of abnormal hemoglobins is a simple and convenient technic for the study of the hereditary hemoglobinopathies. 2. A semiquantitative paper electrophoretic technic is described, which allows rather accurate quantitation of the various hemoglobin components by inspection alone. 3. For exact results, the more elaborate technics of elution or photoelectric scanning may be employed. The accuracy of these quantitative technics is illustrated by artificial mixture experiments. 4. The clinical applications of the method in the study of sickle cell disease and hemoglobin C abnormalities are discussed. Apart from the more common hemoglobin abnormalities (such as sickle cell trait, sickle cell anemia, C trait, sickle cell-hemoglobin C disease), a patient with 100 per cent hemoglobin C (homozygous hemoglobin C disease) and a Negro patient with sickle cell-thalassemia disease were discovered. Normal adult hemoglobin (hemoglobin A) was found in all other hereditary and acquired anemias studied. Slightly increased amounts of fetal hemoglobin were detected in cases of hereditary nonspherocytic hemolytic disease and aregenerative anemia. 5. This technic may be used for red cell life span determinations by serially following the disappearance of a certain hemoglobin type transfused into a patient with a different hemoglobin variety. Further applications of the technic are suggested. 6. The combination of the technics of paper electrophoresis and alkali denaturation offer an adequate, simple, and practical tool for diagnosis and investigation of hereditary hemoglobinopathies. 7. Identical apparatus and buffer may be used for serum protein electrophoresis.

THE ROLE OF COMPLETE BLOOD COUNT IN THE DIAGNOSIS OF HEMOGLOBIN E IN PSEUDO HIGH LEVEL OF HEMOGLOBIN A2

2015 ◽  
Vol 6 ◽  
pp. 1 ◽  
Author(s):  
Alida Harahap ◽  
Dewi Megawati ◽  
Ita M. Nainggolan ◽  
Maria Swastika ◽  
Iswari Setianingsih ◽  
...  
Keyword(s):  
Blood Count ◽  
Complete Blood Count ◽  
Hemoglobin E ◽  
High Level ◽  
Hemoglobin A2

scholarly journals α-Globin as a molecular target in the treatment of β-thalassemia

Blood ◽  
2015 ◽  
Vol 125 (24) ◽  
pp. 3694-3701 ◽  
Author(s):  
Sachith Mettananda ◽  
Richard J. Gibbons ◽  
Douglas R. Higgs
Keyword(s):  
Globin Gene ◽  
Molecular Genetic ◽  
Allogeneic Bone Marrow Transplantation ◽  
Fetal Hemoglobin ◽  
Developed Countries ◽  
Allogeneic Bone ◽  
Genetic Studies ◽  
Hemoglobin E ◽  
Premature Deaths ◽  
Globin Gene Expression

Abstract The thalassemias, together with sickle cell anemia and its variants, are the world’s most common form of inherited anemia, and in economically undeveloped countries, they still account for tens of thousands of premature deaths every year. In developed countries, treatment of thalassemia is also still far from ideal, requiring lifelong transfusion or allogeneic bone marrow transplantation. Clinical and molecular genetic studies over the course of the last 50 years have demonstrated how coinheritance of modifier genes, which alter the balance of α-like and β-like globin gene expression, may transform severe, transfusion-dependent thalassemia into relatively mild forms of anemia. Most attention has been paid to pathways that increase γ-globin expression, and hence the production of fetal hemoglobin. Here we review the evidence that reduction of α-globin expression may provide an equally plausible approach to ameliorating clinically severe forms of β-thalassemia, and in particular, the very common subgroup of patients with hemoglobin E β-thalassemia that makes up approximately half of all patients born each year with severe β-thalassemia.

Quantitative analysis of movements of potassium in rabbit auricles

1962 ◽  
Vol 156 (962) ◽  
pp. 57-82 ◽  
Keyword(s):  
Steady State ◽  
Quantitative Analysis ◽  
Saline Solution ◽  
Physiological Saline ◽  
Fast Component ◽  
Model Systems ◽  
Ionic Content ◽  
Small Loss ◽  
Main Fraction ◽  
Physiological Saline Solution

When rabbit auricles are isolated in physiological saline solution at 29 or 37 °C, the influx or efflux of 42 K follows a course which can be described by the sum of two exponential terms. The presence of an initial fast component is more evident in beating than in spontaneously quiescent auricles, but the fast component is not due to beating because it occurs also in auricles in which beating is stopped by carbachol (2 x 10 -6 M). A small loss of potassium occurs in untreated auricles and a larger one under the influence of certain drugs, such as ouabain (10 -5 M) or dinitrophenol (10 -4 M). Several model systems are considered, especially those with three compartments all or partially communicating with each other, both with regard to exchange of tracer and to net changes in the total ionic content of the compartments. The simplest model which describes the observations had 79 ± 2 (S. E.) % of the tissue potassium free to exchange with the medium and the rest exchanging more slowly with the main fraction. In a steady state at 29 °C, in quiescent left auricles 1⋅07 % of the main fraction exchanged with the medium and 0⋅37 % with the slow fraction per minute. The corresponding rates for beating right auricles were 2⋅25 and 0⋅20 % per minute. In the presence of ouabain (10 -5 M) uptake from the medium to the main fraction was reduced to about 40% of the normal rate, with corresponding net loss of potassium. Dinitrophenol reduced or stopped uptake to the slow fraction and also accelerated loss from the main fraction to the medium.

scholarly journals Quality of Red Blood Cells Isolated from Umbilical Cord Blood Stored at Room Temperature

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Mariia Zhurova ◽  
John Akabutu ◽  
Jason Acker
Keyword(s):  
Stem Cells ◽  
Cord Blood ◽  
Red Blood Cells ◽  
Future Development ◽  
Blood Cells ◽  
Room Temperature ◽  
Fetal Hemoglobin ◽  
Blood Characteristics ◽  
Rbc Transfusion

Red blood cells (RBCs) from cord blood contain fetal hemoglobin that is predominant in newborns and, therefore, may be more appropriate for neonatal transfusions than currently transfused adult RBCs. Post-collection, cord blood can be stored at room temperature for several days before it is processed for stem cells isolation, with little known about how these conditions affect currently discarded RBCs. The present study examined the effect of the duration cord blood spent at room temperature and other cord blood characteristics on cord RBC quality. RBCs were tested immediately after their isolation from cord blood using a broad panel of quality assays. No significant decrease in cord RBC quality was observed during the first 65 hours of storage at room temperature. The ratio of cord blood to anticoagulant was associated with RBC quality and needs to be optimized in future. This knowledge will assist in future development of cord RBC transfusion product.

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