de Braekeleer M, Murray F, Daigneault J, Allard C, Simard F, Aubin G
Institut National d'Etudes Démographiques, Paris, France.
Ann Genet. 1994;37(2):89-92.
Saguenay Lac-Saint-Jean (SLSJ), a geographically isolated region located in northeastern Quebec, has an incidence of cystic fibrosis (CF) of 1 in 902 live births and a carrier rate of 1 in 15 inhabitants. Forty two families among those registered at the local chapters of the Canadian CF Foundation were randomly selected. A multiple-choice questionnaire was applied during an interview. It included 102 questions pertaining to the sociodemographic status of the participants, their knowledge of the disease and their reproductive attitudes. All the educational and socioeconomic levels were represented in the sample; 76% of the respondents were married. Before answering the questionnaire, 86% estimated their knowledge of the transmission of CF to be good or excellent; in fact, the rate of good answers to various questions ranged from 71 to 95%. The birth of a child with CF had a major impact on further pregnancy planning (a 69% reduction or arrest). Seventy one percent of the respondents used a more effective contraception method (including vasectomy and tubal ligation) after the birth of the CF child; in 63% of them, the decision was a direct consequence of the presence of the disease in the family. Thirty four respondents (81%) knew that prenatal diagnosis was available; 90% were in favor of such a prenatal test but only 17% would have required an abortion should the fetus be affected.
萨格奈-圣让湖地区（SLSJ）位于魁北克省东北部，是一个地理上孤立的地区，囊性纤维化（CF）的发病率为每902例活产中有1例，携带者比例为每15名居民中有1例。从加拿大囊性纤维化基金会当地分会登记的家庭中随机选取了42个家庭。在一次访谈中采用了多项选择题问卷。它包括102个与参与者的社会人口统计学状况、他们对该疾病的了解以及他们的生育态度有关的问题。样本涵盖了所有教育和社会经济水平；76%的受访者已婚。在回答问卷之前，86%的人估计他们对囊性纤维化遗传的了解良好或优秀；事实上，对各种问题的正确回答率在71%至95%之间。患有囊性纤维化的孩子的出生对进一步的妊娠计划有重大影响（减少或停止妊娠的比例为69%）。71%的受访者在囊性纤维化患儿出生后采用了更有效的避孕方法（包括输精管切除术和输卵管结扎术）；其中63%的人做出这一决定是因为家庭中存在这种疾病。34名受访者（81%）知道可以进行产前诊断；90%的人赞成这种产前检查，但如果胎儿受影响，只有17%的人会要求堕胎。
