Takahashi Shirushi, Takada Aya, Saito Kazuyuki, Hara Masaaki, Yoneyama Katsumi, Nakanishi Hiroaki, Takahashi Kei, Moriya Takuya, Funayama Masato
Department of Forensic Medicine, Saitama Medical University, 38 Morohongo, Moroyama-machi, Iruma-gun, Saitama, Japan.
Department of Forensic Medicine, Saitama Medical University, 38 Morohongo, Moroyama-machi, Iruma-gun, Saitama, Japan.
Leg Med (Tokyo). 2015 Jan;17(1):39-42. doi: 10.1016/j.legalmed.2014.08.008. Epub 2014 Sep 10.
An eight-year-old Japanese boy developed abdominal pain, followed by convulsion and loss of consciousness. He was taken to an emergency room but could not be resuscitated. At autopsy, the left main coronary trunk (LMT) demonstrated an increase in caliber with severe luminal narrowing, and the left anterior descending branch (LAD) subsequent to the LMT showed severe stenosis. Microscopically, the intima of the LMT demonstrated severe fibrosis and infiltration of lymphocytes and histiocytes suggesting vasculitis, and the small lumen was occupied by a fresh thrombus. The LAD showed significant intimal thickening with strong lymphocytic inflammation at the edge of the thickening. The left ventricle showed widespread myocardial infarction in the recovery stage. There were no findings of atherosclerosis, vasculitis or fibrocellular changes in the ascending aorta or intravisceral arteries other than the LMT and the LAD under investigation. The increase in the caliber of the LMT and the limitation of arteritis to the LMT and the subsequent branch suggested Kawasaki disease (KD), but it was atypical that the patient had no clinical history consistent with KD. The present case showed no findings suggesting classical polyarteritis nodosa (cPAN) at the acute or scar stage in the other vessels being investigated, and cPAN in childhood is rare compared to KD. A nonspecific inflammatory reaction (single organ vasculitis, SOV) was also considered as a possible cause, but it is difficult to determine whether the cause of the coronary stenosis in the present case was SOV because the sampling of arteries was insufficient. If forensic pathologists make unusual findings suggesting vasculitis at autopsy, the collection of a sufficient number of vessels of various sizes is warranted.
一名八岁日本男孩出现腹痛,随后惊厥并失去意识。他被送往急诊室,但未能复苏。尸检时,左冠状动脉主干(LMT)管径增大,管腔严重狭窄,LMT之后的左前降支(LAD)显示严重狭窄。显微镜下,LMT内膜显示严重纤维化,淋巴细胞和组织细胞浸润,提示血管炎,小腔被新鲜血栓占据。LAD显示内膜显著增厚,增厚边缘有强烈的淋巴细胞炎症。左心室在恢复阶段显示广泛心肌梗死。除了正在研究的LMT和LAD外,升主动脉或内脏动脉未发现动脉粥样硬化、血管炎或纤维细胞改变。LMT管径增大以及动脉炎局限于LMT及其后续分支提示川崎病(KD),但该患者无与KD相符的临床病史,这一点不典型。本病例在正在研究的其他血管的急性或瘢痕阶段未发现提示经典结节性多动脉炎(cPAN)的表现,与KD相比,儿童期cPAN较为罕见。一种非特异性炎症反应(单器官血管炎,SOV)也被认为是可能的病因,但由于动脉取样不足,难以确定本病例中冠状动脉狭窄的病因是否为SOV。如果法医病理学家在尸检时发现提示血管炎的异常表现,则有必要收集足够数量的各种大小的血管。