Schwarz Kathleen B, Zellos Aglaia, Stamato Lisette, Boitnott John, Perlman Elizabeth, Chong Sonny, Casella James F
Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Pediatr Gastroenterol Nutr. 2008 Apr;46(4):423-8. doi: 10.1097/MPG.0b013e31815c1e43.
To develop a protocol for safe performance of percutaneous liver biopsies in children with deficiency of factor VIII (n = 12) or IX (n = 2) and chronic hepatitis C virus infection.
Liver biopsies were performed after administration of factor VIII or IX, before and 24 weeks after cessation of antiviral therapy. To define the optimal means of providing replacement therapy, 10 children were enrolled in a randomized crossover design study of bolus versus continuous factor VIII for performance of the liver biopsy. For the crossover study, all of the patients were given a loading dose of 50 +/- 5 IU recombinant factor (rF)VIII/kg; a minimum of factor VIII activity of > or = 80% 30 to 60 minutes following factor VIII infusion was required for liver biopsy. For the bolus protocol, rFVIII 25 to 50 IU/kg was given 6, 14, 24, 36, 48, and 60 hours after completion of the loading dose. For the continuous protocol, rFVIII was given 3 to 4 IU/kg per hour for 48 hours, followed by a bolus of 25 IU/kg at 60 hours. In patients with factor IX deficiency, a loading dose of 100 IU/kg was followed by a bolus of 50 IU/kg at 3, 15, 27, and 48 hours after the loading dose.
Twenty liver biopsies were performed in children with factor VIII deficiency without major complications. One of the 3 biopsies in the patients with factor IX deficiency was complicated by a hemoperitoneum. Midazolam and fentanyl were used in the first 8 patients. However, postbiopsy pain, presumably secondary to hematoma in 2 patients and hemoperitoneum in 1, prompted us to use ultrasound to locate a suitable biopsy site and to change to propofol; this allowed us to better immobilize the liver, to minimize postbiopsy bleeding. The subsequent 15 biopsies were well tolerated without postbiopsy pain or other complication.
Percutaneous liver biopsy in children with factor VIII deficiency can be safely performed using either bolus or continuous infusion of recombinant factor VIII. A brief general anesthetic and ultrasound guidance are recommended.
制定一项针对患有 VIII 因子缺乏症（n = 12）或 IX 因子缺乏症（n = 2）且感染慢性丙型肝炎病毒的儿童进行经皮肝活检的安全操作方案。
在给予 VIII 因子或 IX 因子后、抗病毒治疗停止前及停止后 24 周进行肝活检。为确定提供替代治疗的最佳方法，10 名儿童参加了一项关于推注与持续输注 VIII 因子用于肝活检的随机交叉设计研究。对于交叉研究，所有患者均给予 50±5 IU 重组因子（rF）VIII/kg 的负荷剂量；肝活检要求在输注 VIII 因子后 30 至 60 分钟时 VIII 因子活性至少≥80%。对于推注方案，在负荷剂量完成后 6、14、24、36、48 和 60 小时给予 rFVIII 25 至 50 IU/kg。对于持续方案，以每小时 3 至 4 IU/kg 的剂量给予 rFVIII 48 小时，然后在 60 小时时给予 25 IU/kg 的推注剂量。对于 IX 因子缺乏的患者，给予 100 IU/kg 的负荷剂量，随后在负荷剂量后 3、15、27 和 48 小时给予 50 IU/kg 的推注剂量。
对患有 VIII 因子缺乏症的儿童进行了 20 次肝活检，未出现重大并发症。在 IX 因子缺乏症患者的 3 次活检中，有 1 次并发腹腔积血。前 8 名患者使用了咪达唑仑和芬太尼。然而，活检后疼痛（推测 2 例继发于血肿，1 例继发于腹腔积血）促使我们使用超声定位合适的活检部位并改用丙泊酚；这使我们能够更好地固定肝脏，将活检后出血降至最低。随后的 15 次活检耐受性良好，未出现活检后疼痛或其他并发症。
对于患有 VIII 因子缺乏症的儿童，使用推注或持续输注重组 VIII 因子均可安全地进行经皮肝活检。建议采用简短的全身麻醉并在超声引导下进行。
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