Maapliv

Maapliv (REC 0545) is a specialized intravenous solution of 16 branched-chain amino acid (BCAA)-free amino acids developed by Recordati Rare Diseases. It is specifically designed for the management of acute decompensation episodes in patients with maple syrup urine disease (MSUD), also known as leucinosis, from birth onwards. In MSUD, a deficiency in the branched-chain alpha-keto acid dehydrogenase complex leads to the toxic accumulation of leucine, isoleucine, and valine. Maapliv provides a concentrated source of non-branched amino acids to stimulate protein synthesis (anabolism), which consumes the excess circulating BCAAs for protein building, thereby rapidly reducing neurotoxic leucine levels. It is used in emergency settings for patients who are not eligible for or cannot tolerate oral or enteral BCAA-free formulations.

Brand names

Maapliv

Other names

BCAA-free amino acid solution