الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Patients with sickle cell disease (SCD) suffer from recurrent and unpredictable episodes of acute pain due to Vaso-occlusive crisis (VOC) which may require hospitalization. VOC represents the cause of almost 95% of hospitalizations for patients with SCD. In 2017, L-glutamine oral powder was approved for reducing sickle cell crisis episodes, especially acute painful crises.
In this phase IV open-label randomized controlled trial, we evaluated the safety and efficacy of L-glutamine in reducing the number of VOCs in patients with SCD, as well as the effect of glutamine on the cerebral arterial blood flow as assessed by transcranial Doppler (TCD). Sixty participants were randomly assigned in a 1:1 ratio to receive glutamine for 24 weeks and the standard of care (SOC) without glutamine. Glutamine was provided as a powder that was mixed with beverage or food orally twice a day, (0.3 grams per kilogram per dose) maximum of 15 g/dose.
All patients were subjected to a detailed history including demographic characteristics, history of VOCs, other complications and comorbidities, detailed transfusion history, and drug history including hydroxyurea and iron chelation. Assessment of health-related quality of life (HRQoL) at the beginning and the end of the study using the Peds QL™ 3.0 Sickle Cell Disease Module, Arabic version questionnaire. Transcranial Doppler (TCD) was performed for all participants with the calculation of the Time Averaged Mean Maximum Velocity (TAMMV) in the right and left middle cerebral artery (MCA), right and left anterior cerebral artery (ACA), and right and left internal carotid artery (ICA) at three time points: before the start of treatment, after 12 weeks (three months) of glutamine, and at the end of the study.
The primary endpoints were the number, severity, and hospitalizations for VOC. At baseline, all patients in both groups had three or more VOCs in the past year. Although patients receiving glutamine had more VOCs in the past year and were more likely to be hospitalized for VOCs, VOC severity was comparable between the two groups at baseline.
The primary endpoints were that the number, severity, and hospitalizations of VOCs tended to decrease in the glutamine arm and the cumulative number of VOCs and hospitalizations in the glutamine arm was significantly lower than in SOC.
We did not find a difference between both study groups when comparing the hemoglobin concentration and the markers of hemolysis, except at week 12, the retics were higher those on SOC.
When comparing TAMV for the glutamine group at baseline and week 24, we found that both MCA had a marginal increase in TAMV although all values remained normal within a range of 100 – 118 cm/sec, while both ICA showed a significant increase from an abnormally low value of less than 70 cm/sec to normal ranges.
As regards quality of life, for patients in the glutamine arm, there were significant improvements in pain, how to deal with pain, anxiety, anger, and treatment problems. These findings were consistent among patients and parent groups.
Regarding the safety profile, few patients developed nausea, mild abdominal pain, and loose stool. Most side effects started to develop at week four of l-glutamine therapy and all of them resolved spontaneously.
In conclusion, glutamine reduced the number of VOCs and hospitalizations for VOCs in our cohort with SCD. It has a potential favorable impact on the cerebral arterial flow velocities, but more studies are needed to confirm this impact.