ABSTRACT
Phitsanulok is a province situated in the southern part of northern Thailand. Studies of hemoglobinopathies of 2,806 individuals during the period 1988-1990 showed an overall incidence of hemoglobinopathies of 38.89%, with HbE as high as 25% which is the highest incidence of HbE in the North of Thailand. Buddhachinaraj Hospital conducted a maternal screening study on 1,015 pregnant women in 1991 as part of a prevention/control program and found that 22.56% (229 women) had hemoglobinopathies. Of those, 102 (44.54%) individuals (83.33% HbE heterozygotes and 8.82% HbE homozygotes) with their spouses participated in a prenatal diagnosis (PND) counseling program; 100% of the females and 96% of the male were willing to accept PND; 71% of the females and 75.6% of the males had no moral objection in PND.
Subject(s)
Female , Genetic Counseling , Geography , Health Knowledge, Attitudes, Practice , Hemoglobin E , Hemoglobinopathies/epidemiology , Hemoglobinuria/epidemiology , Genetic Carrier Screening , Homozygote , Humans , Incidence , Male , Mass Screening , Morals , Pregnancy , Surveys and Questionnaires , Thailand/epidemiology , beta-Thalassemia/epidemiologyABSTRACT
Thalassemia is a relatively common hemolytic anemia in Southeast Asia. Alpha and beta thalassemia, hemoglobin (Hb) E, and Hb Constant Spring (CS) are prevalent in Thailand. Different gene combinations lead to over 60 thalassemic syndromes. One hundred and forty-nine thalassemia families were retrospectively studied. They were 4 homozygous beta-thalassemia (beta-thal/ beta-thal), 79 beta-thal/Hb E, 22 Hb H disease, 32 Hb with Hb CS, and 6 AE Bart's disease. The first clinical manifestation and hematologic data including hemoglobin electrophoresis were analysed. Most homozygous beta-thalassemia and beta-thal/Hb E presented with anemia (100% vs 81%), hepatomegaly (40% vs 21%), and splenomegaly (20% vs 27%). In Hb H disease and Hb H with Hb CS, the clinical findings were anemia (74% vs 79%), hepatomegaly (9% vs 8%), splenomegaly (9% vs 13%), jaundice (24% vs 13%), and fever (18% vs 25%). The 317 hematologic data and hemoglobin types of the patients, their parents and relative were also analyzed. These findings can be used as reference values for childhood thalassemia and heterozygous states.
Subject(s)
Adolescent , Asia, Southeastern/epidemiology , Child , Child, Preschool , Female , Genotype , Hemoglobin H/analysis , Hemoglobinopathies/epidemiology , Hemoglobins, Abnormal/analysis , Hemoglobinuria/epidemiology , Homozygote , Humans , Infant , Male , Phenotype , Prevalence , Retrospective Studies , Thailand/epidemiology , beta-Thalassemia/epidemiologyABSTRACT
Thalassemia hemoglobinopathies and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are prevalent in Thailand. We studied the prevalence of these disorders from 1,000 cord bloods collected during 14 months period, using EDTA as anticoagulant. Red blood cell G-6-PD quantitative assay was performed in all male subjects. Nine hundred and eighty five specimens were available for hemoglobin (Hb) typing by starch gel electrophoresis. Further evaluation by cellulose acetate electrophoresis and follow up were made in the cases who had Hb E and/or high level of Hb Bart's. It was found that out of 505 males, 61 cases (12.08%) had G-6-PD deficiency. Among 985 cases studied for Hb typing, 61.92% revealed normal Hb type AF while Hb E was present in 18.68% and Hb Bart's designated alpha-thalassemias were present in 25.18% respectively. Of these 985 cases, 18.78% had low Hb Bart's level ie detectable to 8.2% consistent with alpha-thal2, Hb Constant Spring (CS) or alpha-thal1 trait. Ten cases (1.02%) had high levels of Hb Bart's ranging from 16.1-35% without or with Hb CS and E, and further follow-up revealed homozygous Hb CS, Hb A-E-Bart's, Hb H and Hb H with Hb CS disease. The other 53 cases (5.38%) had low level of Hb Bart's with Hb E consistent with alpha-thalassemia trait with Hb E trait. There were 127 cases (12.89%) who had only Hb E trait and 3 cases (0.3%) who had Hb F and E without Hb A initially.