臍出血に対し外科的処置を繰り返した先天性XIII因子欠損症の1新生児例 [in Japanese] A Case of Congenital Factor XIII Deficiency With Umbilical Hemorrhage in a Neonate [in Japanese]
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症例は8生日の女児.7生日より臍出血を認め, 硝酸銀治療にて止血困難であった.一般凝固系検査は正常であり, 外科的処置を施行したが, 術後3日目より再出血を来し, Hbは7.8 g/dlまで低下した.血液凝固XIII因子が測定感度以下であり, XIII因子の補充により止血しえた.臍出血はXIII因子欠損症の初発症状として80%の症例に認めているとの報告があるが, 小児外科領域での報告は少ない.診断までに時間を費やした原因は, 血小板数およびPT・APTTが正常であったため, 凝固系の異常を念頭に置かなかった点にあるといえる.このため, 反省および警鐘の意味も含めて報告する.
A seven-day-old girl manifested umbilical hemorrhage after cord-drop. She was treated by coagulation therapy using AgNO_3 several times ; however, it failed. She was admitted to our institute on the eighth day after birth. Routine screening tests for blood coagulation, such as platelet count, bleeding time, PT and APTT were within normal limits. She underwent surgical treatment, including ligations of umbilical vessels, two times on day eight and day 10 after birth. Once hemostasis was obtained, however, hemorrhage started again. Examination of factor XIII activity showed 0%. Umbilical hemorrhage was well controlled by replacement of factor XIII concentrates. The roles of factor XIII (fibrin-stabilizing factor) are stabilizing clotting and cross-linking of the fibrin polymer. The most common symptom of factor XIII deficiency is delayed umbilical hemorrhage, while there are some reports of patients who died of intracranial hemorrhage. Although factor XIII deficiency shows no abnormalities in the routine screening tests for blood coagulation, delayed umbilical bleeding should be recognized as an important sign for early diagnosis.
- Journal of the Japanese Society of Pediatric Surgeons
Journal of the Japanese Society of Pediatric Surgeons 41(1), 18-22, 2005
The Japanese Society of Pediatric Surgeons