دورية أكاديمية

Hemoglobin C Disorder in Anemic Patients Referred to the National Center for Thalassemia and Genetic Counseling in Damascus.

التفاصيل البيبلوغرافية
العنوان: Hemoglobin C Disorder in Anemic Patients Referred to the National Center for Thalassemia and Genetic Counseling in Damascus.
المؤلفون: Ahmad, Aya, Fattoum, Karam, Imam, Wael, Mukhalalaty, Mhd Yasser, Murad, Musab, Al Quobaili, Faizah Ali
المصدر: International Journal of Hematology-Oncology & Stem Cell Research; Apr2024, Vol. 18 Issue 2, p183-191, 9p
مصطلحات موضوعية: GENETIC counseling, HEMOGLOBIN polymorphisms, HEMOGLOBINS, THALASSEMIA, HEMOLYTIC anemia, FETAL hemoglobin
مصطلحات جغرافية: DAMASCUS (Syria)
مستخلص: Background: Hemoglobinopathies are common inherited blood disorders in our Mediterranean area. The main structural hemoglobin variants are hemoglobin S and hemoglobin C, due to their prevalence. We conducted this retrospective study to investigate and characterize hemoglobin C patients referred to the National Center for Thalassemia and Genetic Counseling and the management of hemoglobin C disease in Damascus. Materials and Methods: The study included patients referred to the National Center for Thalassemia and Genetic Counseling in Damascus between 2000 and 2022 for hemoglobin C detection. Gender, age, geographical origin, hemoglobin electrophoresis profile, and blood transfusion were considered for hemoglobin C patient classification. Blood transfusion in five consecutive years and linear regression with hemoglobin S and C values were determined. Results: 30 (14 males and 16 females) out of 624 patients between 3 and 46 years old (mean ± SD: 17.3 ± 9.7 years) showed hemoglobin C disease. Only eight patients (one male and seven females) received blood transfusions, and the remaining patients (13 males and 9 females) did not receive any transfusion. Only one patient with 100% hemoglobin C was detected; 19 showed HbSC, and 10 had HbAC. There was a significant correlation between hemoglobin S and geographical origin (P-value=0). Conclusion: A Homozygote hemoglobin C patient has mild hemolytic anemia, whereas the hemoglobin C 100% patient has only a one-time blood transfusion (he was 17 years old) in our study. The inherited combination of hemoglobin C and S is less severe than hemoglobin S alone. There is a significant relationship between hemoglobin S and geographical origin (p-value=0). [ABSTRACT FROM AUTHOR]
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قاعدة البيانات: Complementary Index