Ruptured intracranial aneurysm in an adolescent with Alport's syndrome - A new expression of type IV collagenopathy: Case report
Ruptured intracranial aneurysm in an adolescent with Alport's syndrome - A new expression of type IV collagenopathy: Case report
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DOI：
10.1016/s0090-3019(00)00253-6
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发表时间：
2000-07-01
影响因子：
--
通讯作者：
Heary, RF
中科院分区：
文献类型：
--
作者：
Vaicys, C;Hunt, CD;Heary, RF
背景动脉瘤性蛛网膜下腔出血在儿童和青少年年龄组中并不常见。最近对脑血管免疫反应性和脑血管壁分子结构的研究已认识到胶原病，特别是 III 型和 IV 型胶原蛋白在颅内 (i.c) 动脉瘤的发病机制中。阿尔波特综合征是一种遗传性疾病，其特征是 IV 型胶原合成不完善，导致感音神经性听力损失和血尿。血尿是由肾小球基底膜TV型胶原蛋白合成异常引起的。 病例描述 在一次机动车事故后，这名14岁的男性司机出现弥漫性蛛网膜下腔出血，经计算机断层扫描证实。随后的脑血管造影显示左颈动脉分叉部动脉瘤。对患者病史的回顾性回顾显示，肾活检证实患有遗传性奥尔波特综合征。该患者接受了左翼点开颅手术并夹闭了明显破裂的动脉瘤。 结论 作者首次对患有阿尔波特综合征的青少年男性脑动脉瘤破裂进行了临床观察。在本例中，阿尔波特综合征的 IV 型胶原病可能导致了该青少年动脉瘤的发生。颅内动脉瘤在胶原病人群中可能比以前怀疑的更常见，并且无创筛查可能是合适的。 (C) 2000 年，爱思唯尔科学公司。
BACKGROUND Aneurysmal subarachnoid hemorrhage is uncommon in the pediatric and adolescent age groups. Collagenopathies, particularly of collagen type III and IV, have been recognized in the pathogenesis of intracranial (i.c) aneurysms in recent studies of cerebrovascular immunoreactivity and the molecular architecture of the walls of cerebral blood vessels. Alport's syndrome is a genetic disease characterized by imperfect synthesis of type IV collagen leading to sensorineural hearing loss and hematuria. The hematuria results from an abnormal synthesis of the type TV collagen of the glomerular basement membrane.CASE DESCRIPTION After a motor vehicle accident, this 14-year-old male driver presented with diffuse subarachnoid hemorrhage confirmed by computerized tomography. Subsequent cerebral angiography revealed a left carotid artery bifurcation aneurysm. A retrospective review of the patient's history disclosed renal biopsy-proven hereditary Alport's syndrome. The patient underwent left pterional craniotomy and clipping of the aneurysm, which had clearly ruptured.CONCLUSIONS The authors present the first clinical observation of a ruptured cerebral aneurysm in an adolescent male with Alport's syndrome. In this case the type IV collagenopathy of Alport's syndrome may have contributed to the genesis of this adolescent's aneurysm. Intracranial aneurysms may be more common in the population of collagenopathies than previously suspected and non-invasive screening may be appropriate. (C) 2000 by Elsevier Science Inc.