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本文报道了一例β地中海贫血症及其家系的分析。先证者汪××、男、汉族四川省籍贯。自五个月时发现贫血,营养欠佳、食欲极差,易上感,其病程表现进行性加重。于1975年11月(患儿10个月)转入我附院治疗。体检发现:双眼间距较宽、鼻根宽、皮肤粘膜苍白,肝脾肿大。实验室检查:血红蛋白2克%,红细胞105万/mm~3,白细胞12,200/mm~3。关于血红蛋白病的检查,由于当时条件所限,只作了抗碱Hb测定、红细胞渗透性试验,综合各项检查诊断为重型β地中海贫血症。患儿经多次输血,切脾治疗,均无明显疗效,出院后死亡。
This article reports an analysis of β-thalassemia and its pedigree. Proof Wang × ×, male, Han native place in Sichuan Province. Since five months found anemia, poor nutrition, poor appetite, easy to feel a progressive increase in the course of its performance. In November 1975 (children 10 months) transferred to my hospital for treatment. Physical examination found: wide binocular distance, wide nasal root, pale skin and mucous membrane, hepatosplenomegaly. Laboratory tests: 2 grams of hemoglobin, red blood cells 1.05 million / mm ~ 3, leukocytes 12,200 / mm ~ 3. About hemoglobin disease examination, due to the conditions at the time limit, made only the anti-alkali Hb determination, erythrocyte permeability test, comprehensive examination of the diagnosis of severe β-thalassemia. Children with multiple transfusions, splenectomy, no significant effect, died after discharge.