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[马凡综合征。对法医学案例的一项贡献]

[Marfan syndrome. A contribution to forensic medicine cases].

作者信息

Vock R, Schulz E

出版信息

Z Rechtsmed. 1986;96(1):67-78. doi: 10.1007/BF00200861.

DOI:10.1007/BF00200861
PMID:3716650
Abstract

A 19-year-old school boy suffered from fluctuating uncharacteristic chest pain in the last 20 h before his death. He died unexpectedly within a few minutes of a hemopericardium, which resulted from an aneurysmal rupture of the ascending aorta. The patient's past history as well as the autopsy and ultrastructural findings led to the diagnosis of Marfan's syndrome with alterations of the cardiovascular skeletal system but no ophthalmological involvement ("oligosymptomatic" form of Marfan's syndrome). Appraisal of the cause of death is made more difficult by the fact that medical treatment was undertaken on the same day, whereby subtotal liver crushing was established that had resulted from attempts at resuscitation.

摘要

一名19岁的男学生在死前20小时内出现了反复无常的非典型胸痛。他在几分钟内因升主动脉瘤破裂导致心包积血而意外死亡。患者的既往病史以及尸检和超微结构检查结果导致诊断为马凡综合征,伴有心血管骨骼系统改变,但无眼科受累(马凡综合征的“症状轻微”型)。由于当天进行了医疗救治,而复苏尝试导致了肝脏部分挤压伤,这使得死因评估变得更加困难。

相似文献

1
[Marfan syndrome. A contribution to forensic medicine cases].[马凡综合征。对法医学案例的一项贡献]
Z Rechtsmed. 1986;96(1):67-78. doi: 10.1007/BF00200861.
2
Sudden death in Marfan syndrome.马凡综合征中的猝死。
J Forensic Sci. 2014 Jul;59(4):1126-8. doi: 10.1111/1556-4029.12415. Epub 2014 Feb 6.
3
Marfan syndrome: clinical consequences resulting from a medicolegal autopsy of a case of sudden death due to aortic rupture.马凡综合征:一例因主动脉破裂猝死病例的法医学尸检临床后果
Int J Legal Med. 2009 Jan;123(1):55-8. doi: 10.1007/s00414-008-0288-5. Epub 2008 Oct 17.
4
Pulmonary elastic tissue in generalized elastolysis (cutis laxa) and Marfan's syndrome: a light and electron microscopic study.全身弹性组织溶解症(皮肤松弛症)和马方综合征中的肺弹性组织:光镜和电镜研究
J Invest Dermatol. 1975 Nov;65(5):451-7. doi: 10.1111/1523-1747.ep12608193.
5
Fatal aortic dissection in a patient with a family history of Marfan syndrome.
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Spontaneous rupture of the aorta in young people. Its relation to so-called medionecrosis cystica and Marfan's syndrome.年轻人主动脉自发性破裂。其与所谓的囊性中层坏死及马方综合征的关系。
Acta Pathol Microbiol Scand Suppl. 1970;212:Suppl 212:143+.
7
Ultrastructural and histochemical studies of vascular lesions in Marfan's syndrome, with report of 4 autopsy cases.马方综合征血管病变的超微结构和组织化学研究,附4例尸检报告。
Acta Pathol Jpn. 1973 Nov;23(4):847-66. doi: 10.1111/j.1440-1827.1973.tb02780.x.
8
[Marfan's syndrome as a cause of sudden death].[马方综合征作为猝死的一个原因]
Sud Med Ekspert. 1990 Apr-Jun;33(2):50.
9
Spontaneous rupture of the aorta in Marfan's syndrome.
Z Rechtsmed. 1973 Aug;73(1):53-60. doi: 10.1007/BF02114774.
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Floppy aortic valve presenting as sudden death.表现为猝死的松弛型主动脉瓣。
Forensic Sci Int. 1984 Jun;25(2):123-31. doi: 10.1016/0379-0738(84)90021-5.

引用本文的文献

1
Marfan syndrome: clinical consequences resulting from a medicolegal autopsy of a case of sudden death due to aortic rupture.马凡综合征:一例因主动脉破裂猝死病例的法医学尸检临床后果
Int J Legal Med. 2009 Jan;123(1):55-8. doi: 10.1007/s00414-008-0288-5. Epub 2008 Oct 17.

本文引用的文献

1
Arachnodactylia and cardiovascular disease; report of an autopsied case with a summary of previously autopsied cases.蜘蛛指(趾)症与心血管疾病;一例尸检病例报告及既往尸检病例总结
Am Heart J. 1947 Oct;34(4):580-91. doi: 10.1016/0002-8703(47)90534-6.
2
The cardiovascular aspects of Marfan's syndrome: a heritable disorder of connective tissue.马凡氏综合征的心血管方面:一种遗传性结缔组织疾病。
Circulation. 1955 Mar;11(3):321-42. doi: 10.1161/01.cir.11.3.321.
3
[CLINICAL PICTURE OF DISSECTING AORTIC RUPTURE].[主动脉夹层破裂的临床表现]
Dtsch Med Wochenschr. 1965 Jul 30;90:1349-54. doi: 10.1055/s-0028-1113337.
4
[CARDIOVASCULAR FINDINGS IN MARFAN'S SYNDROME].[马凡氏综合征的心血管表现]
Fortschr Geb Rontgenstr Nuklearmed. 1964 May;100:612-21.
5
Successful resection of carotid and abdominal aneurysm in two related patients with Marfan's syndrome.成功切除两名患有马凡氏综合征的相关患者的颈动脉和腹主动脉瘤。
N Engl J Med. 1962 Jul 19;267:141-2. doi: 10.1056/NEJM196207192670306.
6
Ruptured abdominal aneurysm occurring in Marfan's syndrome; attempted repair with the use of a nylon prosthesis.
N Engl J Med. 1959 Apr 16;260(16):821-2. doi: 10.1056/NEJM195904162601608.
7
Genetic factors in diseases of connective tissue; a survey of the present state of knowledge.
Am J Med. 1959 Feb;26(2):283-302. doi: 10.1016/0002-9343(59)90317-1.
8
The Marfan syndrome: report of one case with autopsy, special histological study, and review of the literature.马方综合征:一例尸检、特殊组织学研究及文献复习报告
AMA Arch Intern Med. 1959 Jan;103(1):123-32. doi: 10.1001/archinte.1959.00270010129017.
9
Marfan's syndrome: description of a family.马方综合征:一个家族的描述。
Am J Med. 1957 Sep;23(3):434-44. doi: 10.1016/0002-9343(57)90323-6.
10
[Marfan's syndrome with aneurysm and coarctation of aorta; anatomoclinical study].[伴有主动脉瘤和主动脉缩窄的马方综合征;解剖临床研究]
Schweiz Med Wochenschr. 1957 Jan 19;87(3):49-53.