Evaluation of a commercial kit for microchromatographic quantitation of hemoglobin A2 in the presence of hemoglobin S.

1981 ◽  
Vol 27 (7) ◽  
pp. 1244-1247 ◽  
Author(s):  
R M Baine ◽  
H G Brown
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Accurate Method ◽  
Beta Thalassemia ◽  
Coefficients Of Variation ◽  
Hb S ◽  
Slow Moving ◽  
Commercial Kit ◽  
Hemoglobin A2

Abstract Commercial microcolumns introduced in 1976 by Helena Laboratories ("Hb A2 Quik Column") and by Isolab, Inc. ("Quik-Sep") provide a rapid, simple, accurate method for quantitation of hemoglobin A2 (Hb A2). However, these kits cannot be used for the quantitation of Hb A2 in the presence of slow-moving variants such as Hb S. Recently, Isolab, Inc., produced a new kit ("Quik-Sep Improved Hb A2 Test") for quantitation of both Hb A2 and Hb S. We compared results obtained with the new Isolab kit to results obtained with the original Tris/HCl method for quantitation of Hb A2 and Hb S. Blood was drawn from persons with sickle cell trait (A/S), sickle cell anemia (S/S), sickle cell/beta+ thalassemia (S/beta+ thal) and sickle cell/beta 0 thalassemia (S/beta 0 thal) and percentages of Hb A2 and Hb S were determined by each method. We found no significant differences in Hb A2 percentages by the two methods, and the coefficients of variation were similar. Both methods showed only slight overlap of Hb A2 values from subjects with some form of beta thalassemia and those with A/S or S/S. However, the Tris/HCl method consistently gave values for Hb S that were higher and closer to those expected, suggesting that the Isolab kit does not accurately quantitate Hb S.

scholarly journals Frequency distribution of sickle cell anemia, sickle cell trait and sickle/beta-thalassemia among anemic patients in Saudi Arabia

2015 ◽  
Vol 6 (3) ◽  
pp. 85 ◽  
Author(s):  
Shoeb Qureshi ◽  
Mohieldin Elsayid ◽  
MohammedJahman Al-Shehri ◽  
YasserAbdullah Alkulaibi ◽  
Abdullah Alanazi
Keyword(s):  
Saudi Arabia ◽  
Frequency Distribution ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Beta Thalassemia

Difficulties in the diagnosis of Hb S/Beta thalassemia: Really a mild disease?

2021 ◽  
Author(s):  
Süheyl UCUCU
Keyword(s):  
Sequence Analysis ◽  
Dna Sequence ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dna Sequence Analysis ◽  
Current Status ◽  
Beta Thalassemia ◽  
Carrier Status ◽  
Laboratory Findings ◽  
Hb S

OBJECTIVE: HbS/β cases having clinical, hematologic and electrophoretic similarities cannot be sufficiently distinguished from sickle cell anemia cases, and are misdiagnosed as sickle cell anemia. This study will investigate the congruence between the HPLC thalassemia scanning tests and the laboratory findings in comparison with the DNA sequence analysis results of the patients diagnosed with SCA between 2016 and 2020. This study also aims to indicate the current status to accurately diagnose sickle cell anemia and HbS/β in the light of hematologic, electrophoretic and molecular studies. MATERIAL METHOD: Fourteen patients who were diagnosed with SCA in hospitals at different cities in Turkey and followed by the Thalassemia Diagnosis, Treatment and Research Center, Muğla Sıtkı Koçman University were included in this retrospective study. The socio demographic characteristics, hemogram, hemoglobin variant analysis results and DNA chain analysis results of the patients were taken from the database of the center and then examined. The informed consents were taken from the patients. The patients were administered a survey containing questions about transfusion history and diagnostic awareness. The Beta-Thalassemia mutations were analyzed using DNA sequencer (Dade Behring, Germany) based on the Sanger method. RESULTS: According to the DNA sequence analysis results of these patients diagnosed with SCA in hospitals in different cities of Turkey: Of 14 patients, 8 had Hb S/β0 and Hb S/β+ and one had Hb S carrier, and one had Hb-O, and three had SCA. The patient with HbS carrier status also contains three additional mutations all of which are heterozygous. We discovered that although two of three mutations, which are c.315+16G>C and c.316-185C>T, are previously reported as benign, at least one of the two mentioned mutations, when combined with Hb S, causes transfusion-dependent Hb S/β. CONCLUSION: Briefly, HbSS and HbS/β thalassemia genotypes cannot be definitely characterized by electrophoretic and hematologic data, resulting in misdiagnosis. c.315+16G>C and c.316-185C>T, are previously reported as benign, at least one of the two mentioned mutations, when combined with Hb S, causes transfusion-dependent Hb S/β.  In undeveloped or some developing countries, molecular diagnosis methods and genetic analyses cannot be used. If mutation analyses could be performed, then such differential diagnosis errors would reduce. However, if mutation analysis cannot be performed, other methods such as HPLC, capillary electrophoresis absolutely be sought to have insight into the parental carriage status.

PATHOGENESIS OF HYPOSTHENURIA IN PERSONS WITH SICKLE CELL ANEMIA OR THE SICKLE CELL TRAIT

PEDIATRICS ◽  
1960 ◽  
Vol 26 (2) ◽  
pp. 249-254
Author(s):  
L. Schlitt ◽  
H. G. Keitel
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Renal Damage ◽  
Sickle Cell Trait ◽  
Urinary Concentration ◽  
Cell Disease ◽  
Renal Vessels

Hyposthenuria was investigated in subjects with sickle cell trait and in patients with sickle cell anemia. The following were observed: 1) in subjects with sickle cell trait both normal and reduced maxima of urinary concentration are found, whereas all untreated patients with sickle cell anemia over 6 months of age have hyposthenuria; 2) hyposthenuria becomes increasingly more severe with advancing age in both sickle cell anemia and sickle cell trait; 3) in a 6-month-old patient with sickle cell anemia and hyposthenuria, the maxima of urinary concentration returned to normal after two transfusions of normal erythrocytes. Reasons are presented for favoring the hypothesis that hyposthenuria in sickle cell disease is due to renal damage, possibly from intravascular sickling of erythrocytes in renal vessels or from the presence of "free" circulating S-hemoglobin.

Prevalence of Red Blood Cell Alloimmunization Among Beta-Thalassemia and Sickle Cell Anemia Patients: a Study from Saudi Arabia

2021 ◽  
Vol 67 (10/2021) ◽  
Author(s):  
Raed Felimban ◽  
Ahmed Alsharyufi ◽  
Jasem Aljehani ◽  
Ahmed Sahlool ◽  
Hamead Aljabri ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Beta Thalassemia

scholarly journals The -158 site 5' to the G gamma gene and G gamma expression

Blood ◽  
1985 ◽  
Vol 66 (6) ◽  
pp. 1463-1465
Author(s):  
D Labie ◽  
O Dunda-Belkhodja ◽  
F Rouabhi ◽  
J Pagnier ◽  
A Ragusa ◽  
...  
Keyword(s):  
Linkage Disequilibrium ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Beta Thalassemia ◽  
Surrounding Area ◽  
Area 5 ◽  
Highly Correlated

To test the hypothesis advanced by Gilman and Huisman that the -158 site 5′ to the G gamma gene determines the G gamma expression after the first 4 months of life, we have examined DNA from sickle cell anemia (SS) patients from Africa and beta-thalassemic homozygotes from Algeria. We find that the Xmnl site is strongly linked to the Senegal haplotype among SS patients, to haplotype IX (most probably identical to the Senegal haplotype), and to haplotype III among the Algerian thalassemics. Thalassemics with haplotypes I/I and V/V have no Xmnl site and low G gamma expression. In contrast, beta-thalassemia- associated haplotype II (also characterized by high G gamma expression) fails to exhibit the Xmnl site. We conclude that, although highly correlated, the -158 C----T substitution does not perfectly predict the presence of high G gamma expression. These findings also exclude the possibility that the Xmnl site is solely involved in the determination of high G gamma expression and suggest that either several different site substitutions in the area 5′ to the gamma gene might have the same effect or that, alternatively, the Xmnl site and its surrounding area is not involved in G gamma expression and may be only in linkage disequilibrium with a controlling sequence elsewhere.

scholarly journals Elastic property of sickle cell anemia and sickle cell trait red blood cells

2021 ◽  
Vol 26 (09) ◽  
Author(s):  
Endris Muhammed ◽  
James Cooper ◽  
Daniel Devito ◽  
Robert Mushi ◽  
Maria del Pilar Aguinaga ◽  
...  
Keyword(s):  
Elastic Property ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Blood Cells ◽  
Sickle Cell Trait

scholarly journals Progressive glomerular and tubular damage in sickle cell trait and sickle cell anemia mouse models

2018 ◽  
Vol 197 ◽  
pp. 1-11 ◽  
Author(s):  
Santosh L. Saraf ◽  
Justin R. Sysol ◽  
Alexandru Susma ◽  
Suman Setty ◽  
Xu Zhang ◽  
...  
Keyword(s):  
Mouse Models ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Tubular Damage

Sickle Cell Trait and Sickle Cell Anemia: A Review

1983 ◽  
Vol 148 (9) ◽  
pp. 701-706 ◽  
Author(s):  
Georges C. Benjamin
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait

Improved Method for the Determination of Hemoglobin A2 by Starch-Gel Electrophoresis

1960 ◽  
Vol 6 (3) ◽  
pp. 254-262 ◽  
Author(s):  
C A J. Goldberg ◽  
A C Ross
Keyword(s):  
Sickle Cell ◽  
Gel Electrophoresis ◽  
Sickle Cell Trait ◽  
Starch Gel Electrophoresis ◽  
Improved Method ◽  
Starch Gel ◽  
Thalassemia Trait ◽  
Healthy Persons ◽  
Hemoglobin A2

Abstract It has been shown that variations in the preparation of the starch gel and in photographic interpretation can significantly affect the accuracy of the measurement of hemoglobin A2. An improved method for the determination of hemoglobin A2 by starch-gel electrophoresis has been presented which affords greater accuracy than was previously achieved. Hemoglobin A2 concentrations for healthy persons and patients with sickle cell trait, various nonthalassemic anemias, and thalassemia trait have been presented.

scholarly journals Sickle-Cell Anemia in South Turkey. A Study of Fifteen Cases in Twelve White Families

Blood ◽  
1956 ◽  
Vol 11 (5) ◽  
pp. 460-472 ◽  
Author(s):  
MUZAFFER AKSOY
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Frequent Occurrence ◽  
White Families ◽  
White Community ◽  
High Incidence ◽  
Southern Turkey

Abstract 1. Fifteen cases of sickle-cell anemia in twelve white families in southern Turkey are reported. 2. In all these cases of sickle-cell anemia and their families, there was no suggestion of anything Negroid in their features or coloring. 3. The hereditary trait of these patients has been investigated. In 53 healthy members of twelve families 37 persons had sickle-cell traits. 4. With the exception of case 12, both parents of all cases had sickle-cell trait. 5. The possible explanations of the frequent occurrence of sickle-cell anemia and the fairly high incidence of sickle-cell trait in this white community have also been considered.

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