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

Amino Acids

Volumn 21, Issue 3, 2001, Pages 303-318

  Muhling J ^a   Dehne, M G ^a   Fuchs, M ^b   Sablotzki, A ^c   Weiss, S ^a   Spatz, J ^a   Hempelmann, G ^a  

^a JUSTUS LIEBIG UNIVERSITY   (Germany)

^b Dr Ing Herbert Knauer GmbH   (Germany)

^c MARTIN LUTHER UNIVERSITY HALLE WITTENBERG   (Germany)

Author keywords

Amino acids; Coagulation analyses; HHH syndrome; Liquor; PMN; Urine

Indexed keywords

BIOLOGICAL MARKER; ORNITHINE;

ADULT; AMINO ACID ANALYSIS; AMINO ACID BLOOD LEVEL; AMINO ACID METABOLISM; AMINO ACID URINE LEVEL; ARTICLE; BIOLOGICAL MONITORING; CASE REPORT; CELL FUNCTION; CELL LEVEL; CITRULLINEMIA; CLINICAL FEATURE; CONTROLLED STUDY; DISEASE COURSE; FEMALE; GENERAL ANESTHESIA; HUMAN; HYPERAMMONEMIA; HYPERORNITHINEMIA; NEUTROPHIL; PATHOPHYSIOLOGY; PERIOPERATIVE PERIOD; POSTPRANDIAL STATE; PRIORITY JOURNAL; PROTEIN CEREBROSPINAL FLUID LEVEL;

ADULT; AMINO ACIDS; AMMONIA; ANESTHESIA; BLOOD COAGULATION TESTS; CITRULLINE; CLINICAL CHEMISTRY TESTS; FEMALE; HUMANS; MONITORING, PHYSIOLOGIC; ORNITHINE; SYNDROME;

EID: 0034750103     PISSN: 09394451     EISSN: None     Source Type: Journal    
DOI: 10.1007/s007260170016     Document Type: Article

References (27)

1. Comparison of ornithine metabolism in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts
2. Further evidence for a separate enzymatic entity for the synthesis of homocitrulline, distinct from the regular ornithine transcarbamylase
3. Splanchnic circulation during nitrous oxide anaesthesia and hypocarbia in normal man
4. Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: Low creatine excretion and effect of citrulline, arginine or ornithine supplement
5. Ornithinemia, hyperammonemia and homocitrullinuria
6. Free amino acid concentrations in blood cells of two brothers with gyrate atrophy of the choroid and retina with hyperornithinaemia
7. Liver circulation and function during isoflurane and halothane anaesthesia
8. Hepatic circulation during surgical stress and anaesthesia with halothane, isoflurane or fentanyl
9. The hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: An ornithine transport defect remediable with ornithine supplements
10. Separation of ornithine and lysine activities of the ornithine-carbamylase-catalyzed reaction
11. Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria)
12. Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria
13. Effects of morphine on splanchnic bloodflow
14. Hyperammonemia-hyperornithinemia-homocitrullinuria syndrome: Neurologic, ophtalmologic and neuropsychologic examination of six patients
15. Intracellular amino acid levels as a predictor of protein synthesis
16. H-H-H syndrome: A rare disease also relevant in anesthesiology
17. Quantitative determination of free intracellular amino acids in single human polymorphonuclear leucocytes. Recent developments in sample preparation and high-performance liquid chromatography
18. Clinical biochemical and ultrastructural study on the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
19. Anaesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome
20. The mechanisms of hyperammonemia and hyperornithinemia in the syndrome of hyperornithinaemia-hyperammonaemia with homocitrullinuria
21. Hereditary orotic aciduria. An urinary screening test
22. A case of hyperammonemia-hyperornithinemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs
23. Metabolic defect in hyperornithinaemia
24. Hyperornithinemia, hyperammonemia, homocitrullinuria. A new disorder of amio acid metabolism associated with myoclonic seizures and mental retardation
25. Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria
26. Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: Presentation as acute liver disease with coagulopathy