Silicone tissue assays.
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Biomedical subjects
Publications and source records attributed to R B Marion.
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A number of varieties of thalassemia were found to be common in the Vancouver area and in other parts of British Columbia. Of 3117 patients whose blood samples were studied by hemoglobin electrophoresis at the Vancouver General Hospital between Jan 1, 1965 and June 30,1977, 813 had the beta-thalassemia trait, 18 had homozygous beta-thalassemia, 97 had alpha-thalassemia trait, 24 had hemoglobin H disease and 14 had miscellaneous variants. Eight patients had interactions of beta-thalassemia with hemoglobin S,C, D, O arab or Vancouver, and one patient had alpha thalassemia associated with hemoglobin Constant Spring. Twelve other variants were noted. They included hemoglobins B2, E, Q, GHsi Tsou, J Bangkok, British Columbia, KOLN, Lepore, Rampa, Tacoma, St. Claude and an unidentified alpha-chain variant.
Hb Vancouver (alpha2beta2 73 (E17) ASP yieldTYR) was found in combination with betaomicron thalassemia trait in a Chinese male who presented with splenomegaly and thalassemia intermedia (3). The family study has revealed two members with beta thalassemia trait, one heterozygote for Hb E, and two heterozygotes for Hb Vancouver. The Hb Vancouver heterozygotes were clinically normal but their erythrocytes showed reduced osmotic fragility and occasional target cells.
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Admission screening was performed on 684 Chinese-Canadian patients for thalassemia, abnormal hemoglobins and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. Thirty-six healthy Chinese adults were also studied. The incidence of beta-thalassemia minor (hemoglobin A(2) greater than 3.5%) was 3.8%. Presumptive alpha-thalassemia minor (demonstration of occasional red cells containing hemoglobin H inclusion bodies) was found in 6.7%. Two patients had findings consistent with alpha-beta-thalassemia. The incidence of G-6-PD deficiency (abnormal methemoglobin reduction test) in adult males was 4.7%. In a parallel study the incidence of hemoglobin Bart's in 310 Chinese newborns was 6.8%. Two mutant hemoglobins were found - hemoglobin E and hemoglobin J (Bangkok).