Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia

• Published in: Amino acids Vol. 21; no. 3; pp. 303 - 318
• Main Authors: Mühling, J, Dehne, M G, Fuchs, M, Sablotzki, A, Weiss, S, Spatz, J, Hempelmann, G
• Format: Journal Article
• Language: English
• Published: Austria Springer Nature B.V 01.01.2001
• Subjects: Adult; Amino acids; Amino Acids - blood; Ammonia - blood; Anesthesia; Blood Coagulation Tests; Cerebrospinal fluid; Citrulline - analogs & derivatives; Citrulline - urine; Clinical Chemistry Tests; Female; Humans; Hyperammonemia; Leukocytes; Medical treatment; Metabolism; Monitoring, Physiologic - methods; Monitoring, Physiologic - standards; Ornithine; Ornithine - blood; Patients; Syndrome

Currently we know not more than 50 patients who show an interesting combination of increased plasma ornithine concentrations, postprandial hyperammonemia, and homocitrullinuria (HHH-syndrome). Since exact knowledge of this severe, although rare syndrome is important for any perioperative or intensiv...