長期間第XIII因子低下を示した小児頭蓋内出血の1例

Intracerebral Homorrhage Associated with Long-lasting Deficiency of Factor XIII

I．はじめに

　血液凝固第XIII因子は凝固最終段階で形成されるfibrinの安定化に関与する因子である．先天性第XIII因子欠乏症は1960年Duckerら^2）により最初に報告され，これまで200例程度の報告しかみられない稀な疾患である^7）．その約25％に頭蓋内出血を生じ，しばしば死因となることが知られている^2,4）．他の血液凝固因子欠乏症と異なり出血時間，血小板数，全血凝固時間，プロトロンビン時間（PT），部分トロンボプラスチン時間（APTT），トロンビン時間などは正常で，術前診断が困難な場合がある．今回，長時間第XIII因子活性値の低下を示し，先天性第XIII因子欠乏症に伴う頭蓋内出血との鑑別を要した小児例を経験したので文献的考察を加え報告する．

A 5-year-old boy had an intracerebral hematoma evacuated from the left parieto-occipital region at a loc-al hospital. There was a difficulty in hemostasis during the operation. Postopetatively his neurological stategradually deteriorated. CT scans showed a gradual increase in intracerebral hemorrhage at the operated site. He was transferred to our clinic for further treatment.

The preoprative blood coagulation profile showed that the activity of factor XIII was only 4%. Thehematoma was removed without any trouble under administration of factor XIII. Postoperative course wasalso uneventful although the plasma level of factor XIII did not go up higher than 26% in spite of its dailyadministration. Although factor XIII administration was terminated one month after cranioplasty, the pa-tient showed good recovery and the level of factor XIII spontaneously normalized in 6 months.

The cause of the extremely low level of factor XIII in this case is not known, but it could be ascribed tomassive hemorrhage.