Phipps Julie, Skirton Heather
Oxford Centre for Genomic Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Faculty of Health and Human Sciences, University of Plymouth, Plymouth, UK.
J Genet Couns. 2017 Oct;26(5):1130-1142. doi: 10.1007/s10897-017-0094-7. Epub 2017 Mar 22.
Muenke syndrome constitutes the most common syndromic form of craniosynostosis, occurring in 1 in 30,000 live births. The phenotype is variable, ranging from no clinical findings to complex presentation. Facilitating reproductive decision making for couples at genetic risk of having a child with Muenke syndrome is an important aspect of genetic counselling. Prenatal genetic testing for Muenke syndrome is accurate; however the value of testing is uncertain with a variable phenotype. The purpose of this study was to explore attitudes towards prenatal testing in couples where one partner had tested positive for the Muenke mutation. We used a qualitative approach based on thematic analysis and collected data using individual semi-structured interviews with eight parents. Five key themes were: The Muenke journey; Impact and knowledge of diagnosis; Knowledge and attitude to prenatal testing; Stigma and sharing of information; and Information retention. Knowledge of Muenke syndrome and prenatal testing was poor. Genetic information was provided when treatment of their affected child was their paramount concern. Couples reported not sharing genetic information with family due to fear of stigmatisation. Couples cannot make reproductive decisions if lacking appropriate understanding of the choices: timely genetic counselling regarding prenatal testing is needed when relevant to them.
穆恩克综合征是最常见的综合征型颅缝早闭,在每30000例活产中出现1例。其表型具有多样性,从无临床症状到复杂表现不等。为有生育患穆恩克综合征孩子遗传风险的夫妇提供生殖决策指导是遗传咨询的一个重要方面。对穆恩克综合征进行产前基因检测是准确的;然而,由于表型多样,检测的价值尚不确定。本研究的目的是探讨一方伴侣穆恩克突变检测呈阳性的夫妇对产前检测的态度。我们采用基于主题分析的定性方法,通过对八位父母进行个体半结构化访谈收集数据。五个关键主题为:穆恩克历程;诊断的影响和认知;对产前检测的认知和态度;污名化与信息共享;以及信息保留。对穆恩克综合征和产前检测的了解较差。当对患病孩子的治疗是他们最关心的问题时,才会提供遗传信息。夫妇们表示由于担心被污名化而不与家人分享遗传信息。如果缺乏对选择的适当理解,夫妇们就无法做出生殖决策:在与他们相关时,需要及时提供有关产前检测的遗传咨询。