北欧国家的重型再生障碍性贫血：一项基于人群的发病率、临床表现、病程及结局的研究。
Severe aplastic anaemia in the Nordic countries: a population based study of incidence, presentation, course, and outcome.
作者信息
Clausen N, Kreuger A, Salmi T, Storm-Mathisen I, Johannesson G
机构信息
Department of Paediatrics, University Hospital of Aarhus, Denmark.
出版信息
Arch Dis Child. 1996 Apr;74(4):319-22. doi: 10.1136/adc.74.4.319.
PURPOSE
Incidence data for severe aplastic anaemia (SAA) in children are scanty and vary. Few population based studies have been reported. A retrospective and prospective study was conducted to determine the incidence and course of SAA.
PATIENTS AND METHODS
All children with a diagnosis of SAA in the Nordic countries from 1982 through 1993 were registered and have been followed up since 1987.
RESULTS
A total of 101 children were diagnosed with SAA. The mean annual child population was 4.31 million. A constant incidence of 1.95/million children/year was found: 2.4 for boys and 1.5 for girls. A non-significant increase of cases occurred from November to March. Possible aetiological agents were noted in 29%. The actuarial survival was 79% after one year and 68% after five years without significant difference between boys and girls.
CONCLUSION
The incidence of SAA in the Nordic countries remains stable with a preponderance among boys. SAA has still a high initial mortality and a risk of late deaths.
目的
儿童重型再生障碍性贫血（SAA）的发病率数据匮乏且存在差异。鲜有基于人群的研究报道。开展了一项回顾性和前瞻性研究以确定SAA的发病率和病程。
患者与方法
对1982年至1993年北欧国家所有诊断为SAA的儿童进行登记，并自1987年起进行随访。
结果
共有101名儿童被诊断为SAA。年儿童平均人口为431万。发现年发病率恒定为每百万儿童1.95例：男孩为2.4例，女孩为1.5例。11月至次年3月病例有非显著性增加。29%的病例发现了可能的病因。1年后精算生存率为79%，5年后为68%，男孩和女孩之间无显著差异。
结论
北欧国家SAA的发病率保持稳定，男性居多。SAA仍有较高的初始死亡率和晚期死亡风险。
Zotero 插件