Karger B, Varchmin-Schultheiss K, Fechner G
Institute of Legal Medicine, University of Münster, Röntgenstrasse 23, 48149 Münster, Germany.
Int J Legal Med. 2005 Jan;119(1):44-6. doi: 10.1007/s00414-004-0482-z. Epub 2004 Sep 16.
A 2.5-year-old boy with known myotubular myopathy (Spiro-Shy-Gonatas syndrome) and gonadorelin intake 9 months ante-mortem was found dead in his bed at home. At autopsy a ruptured subcapsular haematoma of the liver with resulting haemoperitoneum (600 ml) was found. Both lobes of the liver showed numerous circular blood foci <1 mm-2 cm in diameter. Signs of mechanical trauma such as bruising of the abdominal wall were absent. Histologically, the blood cysts were commonly connected to the sinusoids but did not have an endothelial lining and the reticular fibres showed ruptures. These pathomorphological findings are characteristic for peliosis hepatis and the cause of death was therefore determined to be exsanguination due to hepatic haemorrhage from peliosis hepatis instead of from mechanical trauma. To our knowledge this is the youngest casualty from peliosis reported so far.
一名2.5岁男童，已知患有肌管性肌病（斯皮罗 - 施 - 戈纳塔斯综合征），生前9个月摄入过促性腺激素释放素，被发现死于家中床上。尸检发现肝脏有包膜下血肿破裂，导致腹腔积血（600毫升）。肝脏两叶均可见大量直径<1毫米至2厘米的圆形血灶。未发现机械性创伤迹象，如腹壁瘀伤。组织学上，血囊肿通常与肝血窦相连，但无内皮衬里，网状纤维有破裂。这些病理形态学表现是肝紫癜的特征，因此确定死亡原因是肝紫癜引起的肝脏出血导致失血，而非机械性创伤。据我们所知，这是迄今为止报道的最年轻的肝紫癜死亡病例。
