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[腹主动脉瘤的家族风险及其对选择性检测组织工作的影响]

[Familial risk of abdominal aortic aneurysm and its consequences for organization of selective detection].

作者信息

Limet R

机构信息

Service de Chirurgie Cardio-Vasculaire, Centre Hospitalier Universitaire de Liège, Domaine Universitaire de Sart-Tilman, Belgique.

出版信息

J Mal Vasc. 1995;20(4):285-7.

PMID:8586948
Abstract

Aneurysm of the abdominal aorta (AAA) is multifactorial disease related to acquired factors (arteriosclerosis) and genetic factors (collagen anomalies) with, in some cases, a familial component. The incidence of familial AAA was evaluated in 324 patients in 313 families who underwent surgery for AAA. A family survey was used to determine whether members of the operated patients had AAA or had died due to ruptured aneurysms. There were 276 cases (including 12 females) with sporadic AAA and 81 cases (including 5 females) who were members of families with at least one other member having AAA. Comparison between sporadic AAA (SAAA) and familial AAA (FAAA) demonstrated the following differences: 1) diagnosis was made earlier in FAAA (p < 0.05) than in SAAA (64.1 versus 66 years of age respectively), 2) patients with FAAA were younger at the time of rupture (65.4 versus 75.1 years, p < 0.001), 3) the incidence of rupture was greater in FAAA than in SAAA (32.4% versus 8.7% p < 0.001), and finally 4) the relative risk of developing AAA was 18 times greater in members of a family with AAA than in the reference population. Early screening with ultrasonic examinations in at least patient's brother's over 50 years would not be an unjustified expense. The frequency of later examinations would depend on the size of the aorta initially observed. If FAAA is evidenced, under normal conditions, corrective surgery should be proposed earlier than for sporadic AAA.

摘要

腹主动脉瘤(AAA)是一种多因素疾病,与后天因素(动脉硬化)和遗传因素(胶原蛋白异常)有关,在某些情况下还存在家族因素。对313个家庭中接受AAA手术的324例患者进行了家族性AAA发病率评估。通过家庭调查来确定手术患者的家庭成员是否患有AAA或因动脉瘤破裂死亡。有276例(包括12名女性)散发性AAA患者和81例(包括5名女性)属于至少有一名其他家庭成员患有AAA的家族成员。散发性AAA(SAAA)和家族性AAA(FAAA)之间的比较显示出以下差异:1)FAAA的诊断时间比SAAA早(p < 0.05)(分别为64.1岁和66岁),2)FAAA患者破裂时更年轻(65.4岁对75.1岁,p < 0.001),3)FAAA的破裂发生率高于SAAA(32.4%对8.7%,p < 0.001),最后4)患有AAA的家族成员发生AAA的相对风险比参考人群高18倍。至少对50岁以上患者的兄弟进行超声早期筛查并非不合理的费用。后续检查的频率将取决于最初观察到的主动脉大小。如果证实为FAAA,在正常情况下,应比散发性AAA更早地建议进行矫正手术。

相似文献

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[Familial risk of abdominal aortic aneurysm and its consequences for organization of selective detection].[腹主动脉瘤的家族风险及其对选择性检测组织工作的影响]
J Mal Vasc. 1995;20(4):285-7.
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Aneurysm rupture is independently associated with increased late mortality in those surviving abdominal aortic aneurysm repair.在腹主动脉瘤修复术后存活的患者中,动脉瘤破裂与晚期死亡率增加独立相关。
J Surg Res. 2001 Jan;95(1):50-3. doi: 10.1006/jsre.2000.6037.

引用本文的文献

1
Abdominal Aortic Aneurysm: Evolving Controversies and Uncertainties.腹主动脉瘤:不断演变的争议与不确定性
Int J Angiol. 2018 Jun;27(2):58-80. doi: 10.1055/s-0038-1657771. Epub 2018 May 29.
2
French women from multiplex abdominal aortic aneurysm families should be screened.来自多发性腹主动脉瘤家族的法国女性应该接受筛查。
Ann Surg. 2005 Nov;242(5):739-44. doi: 10.1097/01.sla.0000186168.56571.91.