瑞典的舍格伦-拉尔松综合征。一项临床、遗传和流行病学研究。

Sjögren-Larsson syndrome in Sweden. A clinical, genetic and epidemiological study.

作者信息

Jagell S, Gustavson K H, Holmgren G

出版信息

Clin Genet. 1981 Apr;19(4):233-56. doi: 10.1111/j.1399-0004.1981.tb00704.x.

DOI:10.1111/j.1399-0004.1981.tb00704.x

Abstract

Sjögren-Larsson syndrome (SLS), originally described in Sweden, has been studied in a countrywide survey. A total of 58 SLS patients in 41 families were traced, 35 of them still alive. Thirty patients, 23 alive and seven deceased, have not been reported earlier. The mean incidences per 100,000 in the years 1901-1977 were 0.6 in the whole of Sweden, 10.2 in the county of Västerbotten and 2.7 per 100,000 in the county of Norrbotten. In the above-mentioned areas, the prevalence figures of SLS on 31st December 1978 were estimated to be 0.4, 8.3 and 2.6 per 100,000 persons, the frequencies of SLS gene carriers 0.5, 2.0 and 1.0%, and the gene frequencies 0.002, 0.010 and 0.005, respectively. Of the 58 identified Swedish SLS patients, 45 were born in a restricted area in the northeast of Sweden.

摘要

舍格伦 - 拉尔松综合征（SLS）最初在瑞典被描述，该国进行了一项全国性调查。共追踪了41个家庭中的58例SLS患者，其中35例仍在世。有30例患者此前未被报道，其中23例在世，7例已去世。1901年至1977年期间，瑞典全国每10万人的平均发病率为0.6，西博滕县为10.2，北博滕县为2.7。在上述地区，1978年12月31日SLS的患病率估计分别为每10万人0.4、8.3和2.6，SLS基因携带者的频率分别为0.5%、2.0%和1.0%，基因频率分别为0.002、0.010和0.005。在已确认的58例瑞典SLS患者中，45例出生在瑞典东北部的一个限定区域。