日本新生児成育医学会雑誌　第29巻　第1号　126～131頁（2017年）

日本新生児成育医学会雑誌　29(1)：126-131；2017　

受付日：平成27．02．17

受理日：平成28．09．12

帽状腱膜下出血から診断し得た重症血友病Bの新生児例

A Case of Neonatal Severe Hemophilia B Diagnosed with Subgaleal Hemorrhage

長浜赤十字病院　小児科

Department of Pediatrics, Nagahama Red Cross Hospital

小豆澤敬幸・奥野計寿人・山本正仁・浮網聖実・菊岡七恵・清水恭代・成宮正朗

Takayuki AZUKIZAWA，Kazuto OKUNO，Masahito YAMAMOTO，Masami UKIAMI，Nanae KIKUOKA，Yasuyo SHIMIZU，Seirou NARUMIYA

Key Words：hemophilia，subgaleal hemorrhage，newborn

　我々は帽状腱膜下出血を契機に診断した重症血友病Bの新生児例を経験した。症例は在胎41週4日，3,124gで吸引分娩にて出生した男児。日齢2に帽状腱膜下出血に伴う循環血液量減少性ショック，脳室内出血および凝固異常を認め，赤血球濃厚液，新鮮凍結血漿を中心とした治療を行った。日齢7にPT延長を伴わないAPTT延長を認め，第Ⅸ因子活性2％の結果から血友病B と診断した。初期の救命治療後，帽状腱膜下出血と脳室内出血は消退傾向にあり，凝固因子濃縮製剤の補充は行わなかった。生後2か月以降に第Ⅸ因子活性が1％未満となり，重症血友病Bと判明した。凝固因子活性が月齢とともに変動することを考慮し，定期的な検査を行っていくことが必要と考えられる。また，新生児期の予期せぬ急性出血をみた場合，常に先天性血液凝固異常症を念頭において治療に臨む必要がある。本邦における帽状腱膜下出血で発症した新生児血友病の報告例に関する検討を併せて報告する。

　We herein report a case of severe hemophilia B diagnosed with subgaleal hemorrhage. A male infant was born at 41 weeks and four days of gestation via vacuum extraction. The infant’s body weight was 3,124g. At 2 days of age, the infant was admitted with hypovolemic shock and intraventricular hemorrhage associated with coagulation abnormalities resulting in subgaleal hemorrhage. The primary treatment was red blood cell concentrate and fresh frozen plasma. At 7 days of age, coagulation testing showed prolongation of APTT without extension of PT. The patient was diagnosed with hemophilia B according to factor Ⅸ level results that were 2% while the levels of other factors were within normal ranges. Thereafter, the subgaleal and intraventricular hemorrhage resolved and no other findings of bleeding tendencies were observed. At two months after birth, the factor Ⅸ levels were recognized as having decreased to less than 1%. Therefore, the patient was diagnosed with severe form of hemophilia B. According to the fluctuation in factor levels over time, we elected to perform regular blood testing. Physicians should consider the possibility of congenital blood clotting disorders when encountering cases of unexpected acute bleeding during the neonatal period. This report also describes the results of a clinical study of newborn hemophilia causing subgaleal hemorrhage in Japan.