Fatal hepatic haemorrhage in a child—peliosis hepatis versus maltreatment
Fatal hepatic haemorrhage in a child—peliosis hepatis versus maltreatment
复制标题
儿童致命性肝出血——肝紫癜与虐待
DOI：
10.1007/s00414-004-0482-z
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发表时间：
2004
影响因子：
2.1
通讯作者：
G. Fechner
中科院分区：
文献类型：
--
作者：
B. Karger;K. Varchmin;G. Fechner
一名2.5岁男孩被发现死在家中的床上，他患有已知的肌管性肌病（Spiro-Shy-Gonatas综合征），并在死前9个月服用了戈那瑞林。尸检时发现肝包膜下血肿破裂并腹腔积血（600 ml），肝两叶可见大量直径<1 mm-2 cm的圆形血灶。无腹壁瘀伤等机械性创伤体征。在组织学上，血囊肿通常与血窦相连，但没有内皮衬里，网状纤维显示破裂。这些病理形态学发现是肝紫癜的特征，因此确定死亡原因是肝紫癜肝出血引起的失血，而不是机械创伤。据我们所知，这是迄今为止报道的最年轻的紫癜患者。
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.