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遗传性球形红细胞增多症是一种慢性过程伴急性发作的遗传性溶血性疾患,为常染色体显性遗传病,现将我院收治1例及其家系调查中发现病人7例报告如下:Ⅲ1:先证、女、营业员,Ⅱ1之长女,30岁。1982年6月6日第三次入院。该患自二岁起左上腹扪及包块,渐进肿大,伴头晕、乏力,体力较差。3~4岁起眼黄、尿黄、常于感冒后加重。21岁、24岁二次均因妊娠贫血明显,黄疸加深而住我院内科治疗,对症处理缓解。此次因劳累病情加重第三次入院。
Hereditary spherocytosis is a chronic process associated with acute onset of hereditary hemolytic disorders, autosomal dominant genetic disease, now admitted to our hospital in 1 case and its pedigree investigation found that 7 patients were reported as follows: Ⅲ 1: First card, female, salesperson, eldest daughter Ⅱ1, 30 years old. June 6, 1982 the third admission. The suffering from the left upper quadrant palpable mass since the age of two, progressive enlargement, with dizziness, fatigue, poor physical. 3 to 4 years old, yellow eyes, yellow urine, often aggravating after a cold. 21-year-old, 24-year-old were significantly anemia due to pregnancy, jaundice and live in our hospital medical treatment, symptomatic treatment to ease. The third exacerbation due to exertion to hospital.