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PubMed1975

Hemoglobin S-C disease presenting as acute pneumonitis with pulmonary angiographic findings in two patients.

Acute illness characterized by fever, cough, chest pain and pulmonary infiltrates on chest film is characteristic of patients witb sickle cell anemia and with sickle-C disease. The underlying hemoglobinopathy is usually recognized in the former, but because patients with sickle-C disease as a rule have less severe anemia and fewer, less severe crises their abnormal hemoglobin may not be detected until adolescence or adulthood. Acute pulmonary illness in such patients may therefore present a diagnostic dilemma for the unwary. Two cases are presented to highlight this point. Pulmonary angiographic findings in patients with sickle cell states might add information about the pathogenesis of this disorder since vascular occlusion appears to play a major role. Pulmonary angiograms in the two patients we describe documented the presence of localized abnormalities of perfusion. In one, the presence of filling defects in medium-sized arteries suggests intravascular thromboembolism.

Acute Disease↗

PubMed1978

Hemoglobin concentration in white, black, and Oriental children: is there a need for separate criteria in screening for anemia?

The concentration of hemoglobin in blacks was found to be 0.5 to 1.0 g/dl lower than that of income-matched whites in several large surveys. This difference could be a racial characteristic of blacks, or it might be due to a higher frequency of genetic traits such as thalassemia minor and hemoglobinopathies, or to environmental factors such as iron deficiency. To help in making this distinction, we analyzed the data from multiphasic examinations (1973 to 1975) on 1718 white, 741 black, and 315 Oriental healthy, nonindigent children between 5 and 14 years of age. In the entire population, the median hemoglobin concentration averaged 0.5 g/dl lower in blacks than in whites of both sexes (t test, P less than 0.001). The differences still averaged 0.5 g/dl (P less than 0.001) after exclusion of all those with abnormal hemoglobin by electrophoresis (Hgb S and C) and those whose mean corpuscular volume was more than 5% below the normal mean for age (to exclude iron deficiency or thalassemia minor). The data strengthen the impression that blacks normally have a concentration of hemoglobin averaging about 0.5 g/dl less than in whites. If this is the case, about 10% of normal blacks will be mistakenly designated anemic, if the same norms are applied.

Adolescent↗

PubMed1999

Capillary zone electrophoresis: an additional technique for the identification of hemoglobin variants.

Two capillary zone electrophoresis kits (Hb A2 and Hb A1c) were tested for confirmation and identification of hemoglobin variants. The capillary zone electrophoresis experiments were performed at pH 4.7 (Hb A1c kit) and 8.7 (Hb A2 kit) in a 24 cm uncoated fused silica capillary tube (25 microm I.D.). Normal hemoglobins and common hemoglobin variants, including Hbs S, D-Punjab, C, E, O-Arab, and G-Philadelphia, were successfully separated by both methods within a few minutes. Both systems provided completely different elution profiles of normal and abnormal hemoglobin fractions tested and were complementary. The inter-assay coefficient of variations of the migration times of hemoglobin variants were less than 1.0 and 1.3% by the Hb A2 and Hb A1c, respectively. This permits a higher resolution of some hemoglobin variants in low concentrations, like Hb S in newborns, compared with conventional electrophoresis methods. The present capillary zone electrophoresis methods are sensitive, rapid, not labor intensive, and highly selective for the separation of hemoglobin variants. Combination of both methods with some conventional methods, such as isoelectrofocusing, allows identification of Hbs C, E, O-Arab, S, and D-Punjab, as well as their quantification. We have demonstrated that the conventional electrophoresis methods (electrophoresis at pH 6.5 in citrate agar gel and electrophoresis at pH 8.6 on cellulose acetate) can be advantageously replaced by the present capillary zone electrophoresis methods in a clinical laboratory practice for the detection and quantification of hemoglobin variants.

Electrophoresis, Capillary↗

PubMed2004

Hemoglobinopathies, G6PD deficiency, and hereditary elliptocytosis in Bahrain.

The native population of Bahrain has a high prevalence of hemoglobinopathies and G6PD deficiency, probably as a result of past malarial endemism. We used the Biorad-Variant hemoglobin testing system for primary screening of hemoglobinopathies in 20,000 individuals. Hemoglobin abnormalities were detected in 7,206 (36.3%) cases.

Adult↗

PubMed1980

Determination of glycosylated hemoglobins in neonatal blood by isoelectric focusing.

Isoelectric focusing (IEF) with suitable "spacers" can be used to distinguish glycosylated hemoglobins from HbA and HbF in adults and infants, respectively. Using this technique we have shown that there is a good correlation between glycosylated hemoglobins measured by IEF and column chromatography (r = 0.778, P less than 0.001). There was no significant difference in the rate of glycosylation between adult and cord blood hemolysates following incubation with glucose in vitro: In both instances there was an increase from the normal levels of about 5% to 26% through 31% after 18 hours of incubation. The levels of glycosylated hemoglobins in infants of varying gestation, neonates, and normal adults was remarkably constant, varying from 4.7 +/- 1.1% to 6.1 +/- 1.6%. These results show that IEF can be useful in the quantitation of glycosylated hemoglobins, particularly in the presence of hemoglobins (eg, HbF) where standard chromatographic techniques cannot be used. IEF has the added advantage of using small quantities of blood, and allows a number of samples to be tested at the same time, thus facilitating the identification of abnormal hemoglobins.

Adult↗

PubMed1993

[A case of chorea-acanthocytosis associated with low glycohemoglobin A1c].

We reported the first case of chorea-acanthocytosis associated with low glycohemoglobin A1c. Fasting blood sugar, daily profile of blood sugar, serum fructosamine and 75 g glucose tolerance test were all within normal limits. While abnormal hemoglobins were not detected, red blood cell half-life was sharply reduced to 13.4 days. These findings suggest that the low glycohemoglobin A1c in this case is highly ascribable to the reduction of red blood cell half-life rather than to continuous hypoglycemia. However, we can not rule out that the abnormalities of red blood cell membrane, suggested factors in this disease, may be related to the low glycohemoglobin A1c value. Further investigations on chorea-acanthocytosis cases with normal red blood cell half-life are necessary in order to elucidate whether an abnormal glycohemoglobin A1c value is a specific indication regarding this disease.

Acanthocytes↗

PubMed2003

Simulating hemoglobin history.

In one of the truly classic works in anthropological genetics, Frank Livingstone established the interrelationships between agriculture, mosquito ecology, malaria, and, consequently, the frequencies of sickle cell hemoglobin in West Africa. A major inference from Livingstone's study was the recency of malaria as a selective agent in human populations, only becoming significant after the adoption of agriculture in the last few thousand years. Clines of the abnormal hemoglobin alleles might therefore represent continuing waves of advance of adaptive alleles. In order to model the complex interaction of several hemoglobin alleles, selection, and gene flow spreading adaptive mutants, Livingstone turned to computer simulation. Numerous insights concerning the competitive increase of different alleles (hemoglobins S, C, and E and thalassemia), the rate of allele spread under different migration scenarios, including the potential importance of long-range migration, came out of these studies. These experiments also stimulated others to search for mechanisms that might increase the diffusion rate of hemoglobin variants, including kin-structured migration and epidemic disease selection. Recent molecular studies have substantiated major aspects of Livingstone's work (including the recent origin of falciparum malaria) and posed challenges to some of his assumptions (such as the number of mutations to hemoglobins S and E). But whatever the fate of his specific hypotheses, his emphasis on the interaction of genetics, ecology, and culture stands as a model for the anthropological approach to the understanding of human variation and evolution.

Alleles↗

PubMed1977

Restoration of hemoglobin function in stored EDTA blood. Application in identification of hemoglobin variants with abnormal oxygen affinity.

The incubation of stored blood in a mixture of inosine, pyruvate, glucose, and phosphate restores the O2 affinity of hemoglobin to physiologic levels, as measured by the configuration of the dissociation curve and the P50. This regeneration of normal hemoglobin function not only is consistent for samples anticoagulated with EDTA and stored eight days at 4 C but also is demonstrable for at least 19 days of 4 C storage of EDTA-anticoagulated whole blood. This regeneration procedure is simple to perform and makes it possible to measure reliably O2 affinity in blood samples transmitted by mail.

Blood Preservation↗

PubMed2005

Screening for thalassemia and hemoglobinopathy in a rural area of Thailand: a preliminary study.

The present study aimed to screen thalassemia and hemoglobinopathy in Baan Na-Ngam, Chachoengsao Province, Thailand. Blood samples were obtained from 266 volunteers; 105 males and 161 females aged 7 to 49 years. Blood samples screened for thalassemia combining the OF and modified DCIP precipitation tests. CBC, RBC indices, hemoglobin typing, HbA2 and Hb E were determined. Combined OF and DCIP tests found that in normal subjects, 128 out of 155 were negative for both, 3 were -/+ pattern, 22 were +/- pattern and 2 was positive for both. Interestingly, one sample showed an abnormal hemoglobin pattern, which could not be determined by automated LPLC. Three beta-thalassemia trait subjects were positive for only the OF test. For the Hb E trait, 57 out of 94 were -/+ pattern; 37 were positive for both tests. Moreover, 14 homozygous Hb E subjects were positive for both tests. The prevalence of beta-thalassemia trait was 1.1%, Hb E trait was 35.3% and homozygous Hb E was 5.3%. Since DNA analysis was not performed, alpha-thalassemia1 and alpha-thalassemia2 traits cannot be excluded. In conclusion, a combination of the OF and DCIP tests is suitable for preliminary screening for thalassemia and hemoglobinopathy. However, RBC parameters, hemoglobin typing and PCR analysis will provide more specific diagnosis, especially in alpha-thalassemias.

Adolescent↗

PubMed1992

Genetic variants of alpha 1-antitrypsin and hemoglobin typed by isoelectric focusing in preselected narrow pH gradients with PhastSystem.

In this method for automatically running and staining isoelectric focusing (IEF) gels, pre-made dehydrated polyacrylamide gels were rehydrated before assays run with the PhastSystem (Pharmacia LKB Biotechnology). The typing of genetic variants of hemoglobin and alpha 1-antitrypsin in narrow pH gradients (pH 6.7-7.7 and 4.2-4.9, respectively) was simple, convenient, and reproducible. The clinically important variants of alpha 1-antitrypsin (ZZ and SZ) were identified from serum or dried blood on filter paper. The fast screening of abnormal hemoglobin samples (HbS) for cases in clinical medicine was easily performed. The total analysis time for the phenotyping with conventional protein staining was approximately 60 min.

Genetic Variation↗

PubMed1990

Evaluation of diabetic control by using hemoglobin A1 and fructosamine.

Evaluation of diabetic control was performed by using fasting plasma glucose, hemoglobin A1 and fructosamine in 139 patients with diabetes mellitus, and 36 normal controls. A linear correlation of fasting plasma glucose with fructosamine and hemoglobin A1 was found. Using fasting plasma glucose alone was found to be inadequate to define good control. HbA1 and fructosamine had an acceptable sensitivity and specificity in assessment of diabetic control, although fructosamine was slightly less sensitive than HbA1. In patients with thalassemia, hemoglobin A1 levels were elevated in 18 of 19 patients. Fructosamine levels also gave misleading results since 6 to 19 patients had an elevated level and one patient had a decreased level. Patients with hypoproteinemia had a decreased fructosamine and hemoglobin A1 level compared to normal control. HbA1 and fructosamine should be cautiously interpreted in patients with thalassemia and hypoproteinemic states. Using these methods in combination with other measure such as home monitoring of blood glucose would be more precise particularly in diabetic patients with hypoproteinemia, abnormal hemoglobin and other hemolytic disorders.

Adolescent↗

PubMed1987

Hemoglobin C acquired via a blood transfusion. Detection by automated blood counter.

Hemoglobin C was acquired by a 52-year-old man via a blood transfusion. The presence of nonlysis of erythrocytes on the Technicon H6000 (Tarrytown, NY) scattergram after blood transfusions and the absence of this process in the admission peripheral blood count confirmed that this abnormal hemoglobin was acquired and not endogenous to the patient. Homozygous hemoglobin C disease was confirmed in one of the donors of blood received by the patient.

Blood Cell Count↗

PubMed1980

The isolation of the gamma subunit of fetal hemoglocin (HbF) and its use in a radioimmunoassay for HbF.

A method is described for the purification, from fetal hemoglobin (HbF), of the fetal specific globin chain (gamma chain) in its native state. In the absence of alpha chain (the globin chain common to all adult human hemoglobins) gamma chain, when used as an immunogen, is able to express its unique antigenicity. Here, a specific, high titer antiserum raised against gamma chain has been used to establish a sensitive radioimmunoassay for HbF. This approach may be applicable to the measurement of other normal and abnormal hemoglobins.

Absorption↗

PubMed1984

Secondary ion mass spectra of tryptic peptides of human hemoglobin chains.

Secondary ion mass spectra of tryptic peptides of human globin alpha-, beta-, gamma and delta-chains were studied. Almost all mass peaks of protonated molecular ions of tryptic peptides were observed and they were very stable and abundant. The present results show the possibilities for quantitative analysis of two gamma-globin species: A gamma and G gamma chains, and for structural analysis of unknown abnormal hemoglobins.

Adult↗

PubMed1991

An unusual cause of transient ischemic attacks: case report.

A 42-year-old black man, a physician, presented with a three week history of intermittent right arm and leg numbness and weakness, lasting about five minutes. This was not associated with headache, visual changes, seizures, aphasia or loss of consciousness. There was no history of head trauma, migraines, or previous attacks. Positive findings on physical examination were confined to a blood pressure of 182/80; evidence of hypertensive retinopathy; normal carotid pulses without bruits; and a Grade II/VI systolic ejection murmur with normal sinus rhythm. Initial hematocrit was 25.7%; white blood cell count 14,000 cu/mm with a normal differential; platelet count 532,000 cu/mm. An electrocardiogram showed left ventricular hypertrophy. Duplex scan demonstrated normal carotid bifurcations bilaterally, and arteriogram revealed no carotid or intracranial pathology. Hemoglobin electrophoresis revealed sickle cell disease of the SS type. He was treated with transfusion therapy and has remained asymptomatic at 40 months. Approximately 20% of children with the SS type sickle cell disease will have cerebrovascular symptoms caused by small intracranial artery occlusion due to sludging of the abnormal hemoglobin. This unusual cause of transient ischemic attacks can occur in older patients of African-American ancestry and must be recognized to enable early and effective therapy with exchange transfusion.

Adult↗

PubMed1977

Hemoglobin SC, SS/GPhiladelphia and SOArab diseases diagnostic importance of an integrative analysis of clinical, hematologic and electrophoretic findings.

Hemoglobin studies are presented on two children in whom electrophoretic analysis of hemolysates at alkaline pH initially suggested the diagnosis of hemoglobin SC disease. In both patients the course of the disease was unusually severe, blood smears contained irreversibly sickled cells, and crystal formation did not occur when red cells were incubated in 3 per cent sodium chloride solution. Each of these findings is inconsistent with the interpretation of the electrophoretic patterns. Family studies demonstrated that one of the patients had sickle cell anemia and was also a carrier of an alpha chain variant; citrate agar electrophoresis at pH 6.2 of the second child's hemolysate revealed that the "C-like" hemoglobin was not hemoglobin C. Tryptic peptide analyses of the nonhemoglobin S fractions showed that the first child has SS/GPhiladelphia disease and that the second child has SOArab disease. These observations emphasize the importance of integrating the clinical and hematologic data with the results of electrophoretic procedures in order to define precisely the hemoglobin abnormality in patients with sickling disorders.

Anemia, Sickle Cell↗

PubMed1985

Methemoglobinemia.

Oxygen transport, the major function of hemoglobin, is dependent upon reduced heme iron. In the red cell, the heme iron is maintained in the reduced form by the methemoglobin reduction system. When the balance between oxidation and reduction of heme iron is perturbed due to the presence of excessive oxidants, decreased reducing capacity or the presence of abnormal hemoglobin, methemoglobinemia ensues. In most cases methemoglobinemia is transitory and of no major clinical consequence. Occasionally, however, it can be life threatening and must be rapidly diagnosed and treated. When methemoglobinemia is of hereditary nature, either due to deficiency of red cell NADH-methemoglobin reductase or due to the presence of M hemoglobin, it is a lifelong problem. Since most of these patients do not have major disabling symptoms, the treatment is aimed at correction of cyanosis.

Ascorbic Acid↗

PubMed1992

Prevalence of hemoglobinopathies in north Jordan.

Blood samples from 1,000 subjects, 2-80 years old, were tested to explore the prevalence of alpha-thalassemia trait, high persistent HbF (HPFH), sickle cell trait and beta-thalassemia minor in northern Jordan. Hematological parameters and hemoglobin electrophoresis were carried out on all samples. Results showed 10 (1%) subjects were sickle cell trait, 35 (3.5%) were heterozygous beta thalassemia, 31 (3.1%) alpha thalassemia trait and 10 (1%) high persistent HbF. The prevalence rates were different from those reported in neighbouring countries and their significance is discussed. No other abnormal hemoglobin types were observed.

Adolescent↗