丙酮酸激酶缺乏症又称先天性(遗传性)非球型细胞溶血性贫血(Ⅱ型),属常染色体隐性遗传,杂合子无症状,纯合子表现为慢性溶血性贫血。本病多见于英国及北欧血原的白种人中。近年来,我国也有发现。现将我科1976年收治的1例报导如下: 患儿:王×,男孩,5岁,汉族,湖北鄂城县人。因面色苍白5月,发热4天,于1976年5月28日第二次入院。患儿曾因面色发黄,不规则发热伴有轻度黄疸及肝脾肿大,于1976年3～4月间在我科住院。先后疑为黄疸型肝炎、败血症、疟疾、血吸虫病及恶网等病。经检查排除上述疾病后,诊断为溶血性贫血(原因待查),输血150毫升,病情好转出院.一月后,又因持续高热、贫血加重而再次入院。 Pyruvate kinase deficiency, also known as congenital (hereditary) non-spherical hemolytic anemia (type Ⅱ), is autosomal recessive, heterozygous asymptomatic, homozygous for chronic hemolytic anemia. The disease more common in white blood British and northern European. In recent years, our country has also discovered. Now in our department in 1976 admitted 1 case reported as follows: Children: Wang ×, boy, 5 years old, Han, Hubei Echeng people. Due to pale May, fever 4 days, on May 28, 1976 the second admission. Children had yellow complexion, irregular fever accompanied by mild jaundice and hepatosplenomegaly, 1976 March to 4 months in our hospital. Has suspected jaundice hepatitis, sepsis, malaria, schistosomiasis and evil net and other diseases. After examination to exclude the above diseases, the diagnosis of hemolytic anemia (due to be investigated), blood transfusions 150 ml, the condition improved after discharge .After January, due to continued high fever, anemia and re-admission.