Department of Pediatrics, Division of Pulmonology and Allergy.
Division of Pulmonary and Sleep Medicine.
J Pediatr Hematol Oncol. 2022 Aug 1;44(6):e923-e925. doi: 10.1097/MPH.0000000000002371. Epub 2021 Dec 30.
Individuals with sickle cell disease (SCD) develop a decline in lung function over time. Hydroxyurea (HU) is the most common disease-modifying therapy used in SCD. We hypothesized that children with SCD treated with HU will have a slower decline in pulmonary function. We performed a retrospective chart review of children with HbSS and HbS-beta zero thalassemia referred to pulmonology for respiratory symptoms. We compared the spirometry results at 2 time points between children on HU (HU group) and not on HU (control group). For the HU group, these endpoints were evaluated before and after being on HU. The mean time interval between 2 spirometry studies was not significantly different between the groups (2.6±1.5 y for HU group vs. 3.0±1.8 y for the control group; P =0.33). The mean age of patients in the HU group was 9.8±3.8 years (55% male) and 10.7±4.9 years (50% male) in the control group. The spirometry data was compared within and between the groups using t test. There was a significant increase in forced vital capacity in HU group during follow-up, while children in the control group showed a decline (7.2±17.1 vs. -3.4±18.2; P <0.01). Our study suggests that HU therapy may help preserve lung function over time in children with SCD.
患有镰状细胞病 (SCD) 的个体的肺功能会随时间逐渐下降。羟基脲 (HU) 是 SCD 中最常用的疾病修正治疗方法。我们假设接受 HU 治疗的 SCD 儿童的肺功能下降速度会较慢。我们对因呼吸系统症状而接受肺科评估的 HbSS 和 HbS-beta 零地中海贫血的 SCD 儿童进行了回顾性图表审查。我们比较了 HU 治疗组 (HU 组) 和未接受 HU 治疗组 (对照组) 的 2 个时间点的肺活量测定结果。对于 HU 组，这些终点在接受 HU 治疗前后进行评估。两组之间两次肺活量测定研究之间的平均时间间隔无显著差异 (HU 组为 2.6±1.5 年，对照组为 3.0±1.8 年；P=0.33)。HU 组患者的平均年龄为 9.8±3.8 岁 (55%为男性)，对照组为 10.7±4.9 岁 (50%为男性)。使用 t 检验比较组内和组间的肺活量测定数据。在 HU 组中，随访期间用力肺活量显著增加，而对照组儿童的用力肺活量则下降 (7.2±17.1 与-3.4±18.2；P<0.01)。我们的研究表明，HU 治疗可能有助于 SCD 儿童的肺功能随时间保持稳定。
