The Medical Letter on Drugs and Therapeutics
L-Glutamine (Endari) for Sickle Cell Disease
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The FDA has approved an oral powder formulation of the amino acid L-glutamine (Endari – Emmaus) to reduce the acute complications of sickle cell disease in patients ≥5 years old. Endari is only the second drug to be approved for use in sickle cell disease; the antimetabolite hydroxyurea (Droxia) was approved 20 years ago. L-glutamine oral powder is also available by prescription as NutreStore for treatment of short bowel syndrome and over the counter as a dietary supplement.

SICKLE CELL DISEASE — The elongated or sickle-shaped red blood cells of sickle cell disease obstruct small blood vessels, causing ischemic tissue injury, episodes of severe pain (sickle cell crises), and many other complications. The only other available treatments have been hydroxyurea and chronic transfusions. Hydroxyurea, which increases fetal hemoglobin levels, has reduced the number of painful ... more      

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