3 型血管性血友病妊娠管理病例报告 2 例
Management of von Willebrand disease type 3 during pregnancy - 2 cases reports.
机构信息
Maternidade Júlio Dinis, Centro Hospitalar do Porto, Porto, Portugal.
出版信息
Eur Rev Med Pharmacol Sci. 2013 Nov;17(21):2857-9.
BACKGROUND
von Willebrand disease type 3, is an extremely rare condition. It can be severe and potentially life-threatening, particularly in pregnant women during labor and subsequently during early puerperium. Due to its rarity, there is no optimal treatment/management during pregnancy.
CASE
We describe two cases of pregnant women with von Willebrand disease type 3, and its successful surveillance and treatment with Haemate P FVIII (human plasma-derived von Willebrand Factor-ristocetin co-factor associated with human coagulation factor VIII), during pregnancy, partum and puerperium.
CONCLUSIONS
Daily prophylaxis with Haemate P FVIII in women with von Willebrand disease type 3, starting 2 hours before caesarean section until the 7th day of puerperium, associated with close analytical and clinical surveillance seems to be a safe clinical option.
背景
血管性血友病 3 型是一种极为罕见的病症。该病可能十分严重，甚至危及生命，尤其是在孕妇分娩及随后的产褥期。由于其罕见性，目前尚无可用于妊娠期的最佳治疗/管理方法。
病例
我们描述了两例血管性血友病 3 型孕妇的病例，她们在妊娠、分娩和产褥期使用 Haemate P FVIII（人血浆衍生的 von Willebrand 因子-瑞斯托菌素辅因子与人凝血因子 VIII 相关）进行成功的监测和治疗。
结论
对于血管性血友病 3 型女性，在剖宫产前 2 小时开始每日预防性使用 Haemate P FVIII，直至产褥期第 7 天，并密切进行分析和临床监测，这似乎是一种安全的临床选择。
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