Estepp Jeremie H, Melloni Chiara, Thornburg Courtney D, Wiczling Paweł, Rogers Zora, Rothman Jennifer A, Green Nancy S, Liem Robert, Brandow Amanda M, Crary Shelley E, Howard Thomas H, Morris Maurine H, Lewandowski Andrew, Garg Uttam, Jusko William J, Neville Kathleen A
Departments of Hematology and Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Division of Clinical Pharmacology, Department of Medicine, Duke University Medical Center, Durham, NC, USA.
J Clin Pharmacol. 2016 Mar;56(3):298-306. doi: 10.1002/jcph.598. Epub 2015 Oct 15.
Hydroxyurea (HU) is a crucial therapy for children with sickle cell anemia, but its off-label use is a barrier to widespread acceptance. We found HU exposure is not significantly altered by liquid vs capsule formulation, and weight-based dosing schemes provide consistent exposure. HU is recommended for all children starting as young as 9 months of age with sickle cell anemia (SCA; HbSS and HbSβspan(0) thalassemia); however; a paucity of pediatric data exists regarding the pharmacokinetics (PK) or the exposure-response relationship of HU. This trial aimed to characterize the PK of HU in children and to evaluate and compare the bioavailability of a liquid vs capsule formulation. This multicenter; prospective; open-label trial enrolled 39 children with SCA who provided 682 plasma samples for PK analysis following administration of HU. Noncompartmental and population PK models are described. We report that liquid and capsule formulations of HU are bioequivalent; weight-based dosing schemes provide consistent drug exposure; and age-based dosing schemes are unnecessary. These data support the use of liquid HU in children unable to swallow capsules and in those whose weight precludes the use of fixed capsule formulations. Taken with existing safety and efficacy literature; these findings should encourage the use of HU across the spectrum of age and weight in children with SCA; and they should facilitate the expanded use of HU as recommended in the National Heart; Lung; and Blood Institute guidelines for individuals with SCA.
羟基脲(HU)是镰状细胞贫血患儿的关键治疗药物,但其非适应证用药是广泛应用的障碍。我们发现,与胶囊剂型相比,液体剂型对HU暴露量的影响不显著,基于体重的给药方案可提供一致的暴露量。对于所有9个月及以上的镰状细胞贫血(SCA;HbSS和HbSβ⁰地中海贫血)患儿,均推荐使用HU;然而,关于HU的药代动力学(PK)或暴露-反应关系,儿科数据匮乏。本试验旨在描述HU在儿童中的PK特征,并评估和比较液体剂型与胶囊剂型的生物利用度。这项多中心、前瞻性、开放标签试验纳入了39例SCA患儿,他们在服用HU后提供了682份血浆样本用于PK分析。描述了非房室和群体PK模型。我们报告,HU的液体剂型和胶囊剂型生物等效;基于体重的给药方案可提供一致的药物暴露量;基于年龄的给药方案没有必要。这些数据支持在无法吞咽胶囊的儿童以及体重不适合使用固定胶囊剂型的儿童中使用液体HU。结合现有的安全性和有效性文献,这些发现应鼓励在SCA患儿的各个年龄和体重范围内使用HU,并应促进按照美国国立心肺血液研究所针对SCA患者的指南扩大HU的使用。