Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The sialic acid content of the human erythrocyte membrane is higher in homozygous beta-thalassemia than in heterozygous and healthy donors. Analysis of the difference by means of polyacrylamide gel electrophoresis in 1% sodium dodecyl sulphate and PAS staining failed to reveal significant variations in mobility and relative staining intensity of the main glycoprotein components, PAS-1, PAS-2 and PAS-3. The minor, although highly significant, changes which were recognized in the PAS pattern of beta-thalassemia patients compared with normal controls concerned the PAS-4 region and a shoulder trailing band PAS-2, which both increased in staining intensity relatively to the main sialocomponent PAS-1. Moreover, the shoulder was found to be more prominent in profiles from erythrocytes of blood group 0, in both normal and thalassemic subjects. The experimental data are discussed in the light of the presence of blood group ABH specific macroglycolipids in human erythrocytes (Dejter-Juszynski et al. 1978).
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PMID:Minor changes in the sodium dodecyl sulphate-gel electrophoresis periodic acid-Schiff-staining profiles of erythrocyte membranes in beta-thalassemia major. 55 3

Splenectomy was performed on 152 patients with thalassemia (thal), including 90 cases with Hb H disease, 48 cases with homozygous beta-thal, and 14 cases with Hb E-beta-thal. The therapeutic effect in Hb H disease was 83.3%, and 32.3% in Hb E-beta-thal and beta-thal. In Hb H disease, the hemoglobin (Hb) level increased 30 g/l in 14 of 29 cases. After splenectomy, the life-span (T1/2) of 51Cr RBC in Hb H and Hb E-beta-thal increased to 18.2 +/- 2.6 and 18.2 days, respectively. The number of inclusion (Heinz) bodies attached to the RBC membrane increased after surgery. The interdermal delayed supersensitivity reaction became negative in seven of 26 cases of Hb H disease; the IgG also decreased after surgery. The number of PAS positive foam cells in the spleen correlated to Hb level after surgery. Infection is a common complication.
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PMID:Treatment of the thalassemia syndrome with splenectomy. 320 2

In 24 patients affected with thalassaemia of various degrees of seriousness the functional condition of nuclear cells or red serie was investigated in the bone-marrow. The investigation was carried out by analyzing partial erythroblastogrammes, evaluating proliferative activity according to the 3H-thymidine marking index and determining ineffective erythropoiesis by means of nucleated PAS-positive erythroblasts. The findings reveal the degree of seriousness of the disease being directly dependent on the extent of functional disturbances in the cells of the erythropoietic system.
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PMID:[Cytological characteristics of erythropoiesis in thalassemia. I. Functional characteristics of the nucleated elements of erythropoiesis in the bone marrow]. 615 64