Levin Trine, Mæhle Lovise
Section on Hereditary Cancer, Oslo University Hospital, PB 4950 Nydalen, 0424, Oslo, Norway.
Fam Cancer. 2017 Apr;16(2):257-265. doi: 10.1007/s10689-016-9939-8.
Germline mutations in the CDKN2A gene are associated with an increased risk of malignant melanoma and pancreatic cancer. In order to find out if the behavior pattern in families with a CDKN2A mutation is similar to what we previously have described in families with a BRCA1 mutation, we have studied the uptake of genetic services in probands and their relatives. We describe whether they attend genetic counseling when invited, whether they want a mutation test after being counseled and whether they adhere to recommendations for surveillance. 66 % (95/144) of first-degree relatives to mutation carriers contacted us within the study period. 98 % (126/128) of all relatives who came for genetic counseling decided on genetic testing for their family's mutation, and 93 % (66/71) of all mutation carriers wanted referral to yearly skin examinations. Female relatives had a significantly higher uptake of genetic services compared to males, similar to the findings in families with a BRCA1 mutation. Uptake of genetic services in general in families with a CDKN2A mutation is high. Females seem to have a higher interest in genetic testing than males, regardless of gene mutated.
CDKN2A基因的种系突变与恶性黑色素瘤和胰腺癌风险增加相关。为了弄清楚携带CDKN2A突变的家族中的行为模式是否与我们之前描述的携带BRCA1突变的家族相似,我们研究了先证者及其亲属对基因检测服务的接受情况。我们描述了他们在收到邀请时是否参加遗传咨询,在接受咨询后是否希望进行突变检测,以及是否遵循监测建议。在研究期间,66%(95/144)的突变携带者的一级亲属与我们取得了联系。所有前来接受遗传咨询的亲属中,98%(126/128)决定针对其家族突变进行基因检测,所有突变携带者中93%(66/71)希望转诊接受每年一次的皮肤检查。与男性相比,女性亲属对基因检测服务的接受程度显著更高,这与携带BRCA1突变的家族中的发现相似。一般而言,携带CDKN2A突变的家族对基因检测服务的接受程度较高。无论突变基因是什么,女性似乎比男性对基因检测更感兴趣。