| Co-Investigator(Kenkyū-buntansha) |
INOUE Takafumi Research Associate of Medicine, Kawasaki Medical School, 内科学, 助手 (60203238)
神崎 暁郎 川崎医科大学, 内科学, 助手 (40148698)
HASHIMOTO Masashi Lecturer of Medicine, Kawasaki Medical School, 内科学, 講師 (90148693)
SUGIHARA Takashi Lecturer of Medicine, Kawasaki Medical School, 内科学, 講師 (60140505)
YAMADA Osamu Associate Professor of Medicine, Kawasaki Medical School, 内科学, 助教授 (50104790)
OKAMOTO Naoto Research Associate of Medicine, Kawasaki Medical School
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| Research Abstract |
Red cell membrane disorders (315 cases) were studied in the standpoint of cytoskeletal abnormalities, especially on the following topics: (I) Deficiency of red cell membrane protein band 4.2: Band 4.2 deficiency was characterized by (1) ovalostomatocytosis with severe uncompensated hemolysis, (2) complete absence of band 4.2 on SDS-PAGE with no defect of other protein bands, (3) increased Na influx (2.15 mmoles/1 RBC/hour in case 1 and 1.78 in case2(N:1.29 0.14), (4) moderately decreased red cell deformability in intact red cells examined by ektacytometry, (5) immunoblot studies revealed the presence of abnormal bands in this protein. (II) Hematological and membrane characteristics in 33 patients with hereditary stomatocytosis (HSt): Hematological and membrane characteristics were reviewed in 33 patients of hst studied at our institution. the type of the disease was categorized as based on na influx: HST with markedly increased Na influx (Type 1: >5 mmoles/1 RBC/hr), HSt with moderately increased Na influx (Type 2: 1.5-5), and HSt with not increased Na influx (Type 3: 1-1.5), compared to normal control (1.29 0.14). The extent of anemia and hemolytic jaundice was moderate in these 3 types. In Type 1 (n=7), n markedly increased Na influx was characteristic, and in Type 2 (n=15) Na efflux was moderately enhanced, contrary to normal membrane transport in type 3 (n=10). (III) Red cell membrane disoredrs studied by the end of 1988 were 315 cases as total, including 102 cases of hereditary spherocytosis, 49 cases of hereditary elliptocytosis (HE), 33 cases of hereditary stomatocytosis, 28 cases of membrane lipid abnormalties and others. In HE, a case with a molecuar defect of alpha-spectrin, such as HE[Sp alpha^<I/74>], was detected among 49 cases of a common type of HE.
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