Pediatric Hematology Oncology and BMT Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Faculty of Medicine, Ain Shams University Research Institute-Clinical Research Center (MASRI-CRC), Cairo, Egypt.
Ann Hematol. 2024 Sep;103(9):3493-3506. doi: 10.1007/s00277-024-05877-8. Epub 2024 Jul 19.
To evaluate the safety and efficacy of L-glutamine in reducing vaso-occlusive crisis (VOC) and improving cerebral arterial blood flow in children with sickle cell disease (SCD). This is an interventional randomized controlled trial that recruited sixty SCD patients, aged 9.2 ± 3.7 years, who had at least two VOCs during the last 12 months and on a stable dose of hydroxyurea. They were randomly assigned in a 1:1 ratio to receive glutamine (0.3 gm/kg/dose/12h) orally for 24 weeks or the standard of care (SOC). All patients had VOCs in the last year > 3, those on glutamine had a higher number of VOCs and hospitalization for VOC in the last year. There was a decreasing trend in the number, severity, and hospitalization of VOC and a significantly lower cumulative number of VOCs and hospitalizations in the glutamine group than in SOC (p = 0.008, p < 0.001 respectively). Time-averaged mean maximum velocity for the glutamine group had a marginal increase in both middle cerebral arteries, all values remained normal within a normal range, and in both internal carotid arteries, values increased from abnormally low to normal ranges at week 24. Glutamine reduced the number of VOCs and severity and may have a potentially favorable impact on the cerebral arterial flow velocities.
评估 L-谷氨酰胺在减少镰状细胞病(SCD)患儿血管阻塞性危象(VOC)和改善脑动脉血流中的安全性和有效性。这是一项干预性随机对照试验,招募了 60 名年龄为 9.2±3.7 岁的 SCD 患者,他们在过去 12 个月中至少有 2 次 VOC,并稳定剂量服用羟基脲。他们以 1:1 的比例随机分配接受谷氨酰胺(0.3 gm/kg/剂量/12h)口服治疗 24 周或标准治疗(SOC)。所有患者在过去一年中都有超过 3 次的 VOC,服用谷氨酰胺的患者在过去一年中有更多的 VOC 和因 VOC 住院的次数。在谷氨酰胺组中,VOC 的次数、严重程度和住院率呈下降趋势,谷氨酰胺组的累积 VOC 次数和住院率明显低于 SOC(p=0.008,p<0.001)。谷氨酰胺组的大脑中动脉的平均最大流速有一定程度的增加,所有值仍在正常范围内,而颈内动脉的平均值从异常低值增加到正常范围在第 24 周。谷氨酰胺减少了 VOC 的次数和严重程度,可能对脑动脉血流速度有潜在的有利影响。