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[Hyperlysinemia and hyperammonemia].

D Rabier1, P Parvy, J Bardet

  • 1Laboratoire de Biochemie Médicale B, Hôpital Necker-Enfants Malades, Paris.

Annales De Biologie Clinique
|January 1, 1991
PubMed
Summary
This summary is machine-generated.

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Hyperlysinemia, an increase in plasma lysine, is not always present in hyperammonemia. This condition was observed in specific metabolic disorders like propionic aciduria and Reye's syndrome.

Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Clinical Chemistry

Context:

  • Hyperammonemia is a condition characterized by elevated ammonia levels in the blood.
  • Plasma lysine levels have been frequently associated with hyperammonemia.
  • Understanding the relationship between lysine and ammonia is crucial for diagnosing and managing metabolic disorders.

Purpose:

  • To investigate the association between hyperlysinemia and various types of hyperammonemia.
  • To determine if elevated plasma lysine is a consistent marker for hyperammonemia.
  • To identify specific conditions where hyperlysinemia occurs alongside hyperammonemia.

Summary:

  • This retrospective study analyzed patients with different forms of hyperammonemia.
  • Results indicate that hyperlysinemia (expressed as a percentage of total aminoacidemia) is not invariably linked to hyperammonemia.

Related Experiment Videos

  • Hyperlysinemia was notably observed in neonatal propionic aciduria, methylmalonic aciduria, and Reye's syndrome, and to a lesser extent in ornithine transcarbamylase deficiency.

    • Impact:

    • Challenges the assumption of a direct, automatic link between elevated lysine and ammonia levels.
    • Provides valuable insights into the specific metabolic pathways and genetic disorders associated with hyperlysinemia.
    • Aids in refining diagnostic criteria and understanding the metabolic profiles of patients with urea cycle defects and organic acidemias.