RESUMEN
The antioxidant status of hemoglobin-E carriers was studied pre- and post-treatment with vitamin E for 3 months. Fourteen hemoglobin-E carriers (age = 21.36 +/- 1.08 years, BMI = 18.32 +/- 1.22 kg/m2) were treated with vitamin E 200 I.U. daily for 3 months. Fasting blood samples were collected and analyzed for erythrocyte superoxide dismutase activity, total antioxidant activity, hemoglobin concentration, hematocrit, MCV, Heinz body formation and osmotic fragility test. The blood parameters before and after vitamin E treatment were compared. The results showed that superoxide dismutase activity in the erythrocytes was significantly decreased, while total antioxidant activity in plasma, and the osmotic fragility of the erythrocytes, was significantly increased after vitamin E supplementation. However, hematocrit, MCV, and Heinz body formation did not change significantly. This demonstrated that vitamin E 200 IU could be used as a lipophilic antioxidant in red blood cells and could help increase the level of antioxidant in hemoglobin-E carriers.
Asunto(s)
Adulto , Antioxidantes/metabolismo , Eritrocitos/enzimología , Femenino , Cuerpos de Heinz , Hematócrito , Hemoglobina E/metabolismo , Humanos , Masculino , Fragilidad Osmótica , Superóxido Dismutasa/sangre , Talasemia/sangre , Vitamina E/administración & dosificaciónRESUMEN
Accurate and precise hemoglobin separation and the quantitation of Hb A2 and Hb F are essential for the diagnosis of the thalassemias and hemoglobinopathies. Presented in this study is the validation of the the Hb A2 assay of the HbGold analyzer, a fully automated liquid chromatography system for hemoglobin separation and quantitation. Variability of Hb A2 quantitation was quite low; the CV's of within-run, between-run and interlaboratory studies were 1.8-3.1%, 3.4-6.0% and 6.8-8.8% respectively. The results of the %Hb A2 quantitated by HbGold analyzer correlated well with those given by the Bio-Rad Variant Hb testing system (r=0.98). The application of the HbGold analyzer for the diagnosis of the thalassemia phenotypes frequently observed in Thailand is considered. In conclusion, the Hb A2 assay of the HbGold analyzer could be used for the quantitation of Hb A2 and Hb F and the presumptive identification of abnormal hemoglobins.
Asunto(s)
Automatización/métodos , Sesgo , Estudios de Casos y Controles , Cromatografía Liquida/instrumentación , Hemoglobina Fetal/metabolismo , Hemoglobina A2/metabolismo , Hemoglobina E/metabolismo , Hemoglobinopatías/sangre , Tamización de Portadores Genéticos/métodos , Homocigoto , Humanos , Immunoblotting , Modelos Lineales , Fenotipo , Reacción en Cadena de la Polimerasa , Sensibilidad y Especificidad , Tailandia/epidemiología , Talasemia/sangreRESUMEN
Prevalence of thalassemias and/or hemoglobinopathies, particularly hemoglobin E, and cholangiocarcinoma were found more prominently in the lower part of the Northeast of Thailand as compared with the upper part of this region or any other area of the country. The aim of this study was to evaluate if there was coincident distribution or some relationship. Hemoglobin typing by the cellulose acetate method was performed in 111 cases of cholangiocarcinoma, mainly diagnosed by ultrasonography, compared with 146 normal controls. It was found that hemoglobin E trait and beta-thalassemia trait were significantly higher in the former group.
Asunto(s)
Adulto , Anciano , Neoplasias de los Conductos Biliares/sangre , Conductos Biliares Intrahepáticos , Estudios de Casos y Controles , Colangiocarcinoma/sangre , Femenino , Hemoglobina A2/metabolismo , Hemoglobina E/metabolismo , Hemoglobinopatías/complicaciones , Hemoglobinas/metabolismo , Humanos , Masculino , Persona de Mediana Edad , Tailandia/epidemiología , Talasemia beta/complicacionesRESUMEN
An 18-year-old woman had a hepatocellular adenoma of 4 cm across in the right lobe of the liver which was severely hemosiderotic because of beta-thalassemia hemoglobin E disease with numerous blood transfusions. The lesion was an accidental postmortem finding. To our knowledge, this is the second example of liver-cell adenoma occurring in a patient with beta-thalassemia hemoglobin E disease with secondary iron overload. It is suggested that this is an association between hepatocellular adenoma and secondary iron overload of liver cells, a similar event to the relation observed in hepatocellular carcinoma and secondary iron overload of hepatocytes.
Asunto(s)
Adenoma de Células Hepáticas/etiología , Adolescente , Transfusión Sanguínea/efectos adversos , Femenino , Hemoglobina E/metabolismo , Humanos , Hierro/metabolismo , Neoplasias Hepáticas/etiología , Talasemia beta/metabolismoRESUMEN
The mean +/- S.D. of LAP score in neutrophil of the normal control, non splenectomised patients and splenectomised patients were 116 +/- 78, 94 +/- 48 and 37 +/- 23 Kaplow units, respectively. The significant reduction in the leukocyte alkaline phosphatase level (P less than 0.05) was found in beta thalassemia/HbE patients. The degree of reduction was greater in the splenectomised patients (P less than 0.05). The decreased LAP levels in PMN was persistent. This was in contrast to the LAP in PMN of other patients where as LAP returned to normal after splenectomy. The relevance to the increased susceptibility to infection and low LAP in beta thalassemia/HbE should be further investigated.