[Marfan syndrome. A contribution to forensic medicine cases]. 1986

R Vock, and E Schulz

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.

UI MeSH Term Description Entries
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008297 Male Males
D008382 Marfan Syndrome An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2. Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D012151 Resuscitation The restoration to life or consciousness of one apparently dead. (Dorland, 27th ed) Resuscitations
D002305 Cardiac Tamponade Compression of the heart by accumulated fluid (PERICARDIAL EFFUSION) or blood (HEMOPERICARDIUM) in the PERICARDIUM surrounding the heart. The affected cardiac functions and CARDIAC OUTPUT can range from minimal to total hemodynamic collapse. Pericardial Tamponade,Cardiac Tamponades,Pericardial Tamponades,Tamponade, Cardiac,Tamponade, Pericardial,Tamponades, Cardiac,Tamponades, Pericardial
D003645 Death, Sudden The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions. Sudden Death
D004547 Elastic Tissue Connective tissue comprised chiefly of elastic fibers. Elastic fibers have two components: ELASTIN and MICROFIBRILS. Elastic Fibers,Elastic Fiber,Elastic Tissues,Fiber, Elastic,Fibers, Elastic,Tissue, Elastic,Tissues, Elastic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001011 Aorta The main trunk of the systemic arteries. Aortas

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