TY - CHAP M1 - Book, Section TI - HHH Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - HHH is an acronym that stands for Hyperornithinemia-Hyperammonemia-Homocitrullinuria. Homocitrullinuria is a key feature of the HHH Syndrome. It is a genetically transmitted inborn error of metabolism caused by a defect in the transport of ornithine into the mitochondrial matrix characterized clinically by early growth retardation, learning disabilities, periodic confusion, and ataxia. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164072961 ER -