Showing papers on "Management of thalassemia published in 1969"

Management of thalassemia: a comparative program.

Abstract: Since 1950, transfusion therapy for homozygous beta thalassemia at Babies Hospital, New York City, has been designed to maintain hemoglobin levels a t 7.0 gm% or higher. Since 1963, attempts have been made to keep the hemoglobin at levels greater than 9.0 gm% for some children previously transfused at the lower values. At Queen Mary Hospital, Hong Kong, transfusion therapy has been utilized more sparingly. This policy has been mandatory rather than deliberate because of the scarcity of available blood due to the reluctance of the native population to act as donors. Consequently, transfusions were given only when hemoglobin values were less than 3.0 or 4.0 gm% or if special clinical considerations existed. The present study was planned to compare the relative merits of transfusion therapy given a t different H b levels. Linear growth and weight, using native standards, were compared for all the groups. In addition morbidity, cardiovascular status and degree of iron overload were also compared for subjects in New York City and for those in Hong Kong. Blood transfusions for patients in New York City consisted of packed red cells, calculated on the basis of 15 cc/kg/body wt, generally not exceeding the amount of packed red cells obtained from 500 cc of whole blood. In Hong Kong blood transfusions were given primarily as whole blood, usually not exceeding a total of 500 cc. Nine children in each city were studied. All of those in Hong Kong were of Chinese extraction. They were presumed to have homozygous beta thalassemia, although H b A2 determinations were not performed on many of the parents. Seven of the New York City subjects were of Italian extraction; the families of the other two originated in Greece. All nine had markedly elevated fetal hemoglobin values and all parents had elevated H b AP. Seven of the Hong Kong subjects were females and two were males. Their ages ranged from 4-8$ years. The shortest period of observation was three years, and the longest seven and a half years. As noted previously, except for occasional special clinical considerations, transfusions were given only if the H b level was less