ALMIBA®

| bottle # 125 ml

 

PHARMACOLOGIC PROPERTIES

ATC Code: A16AA01, Amino acids and derivatives

Levocarnitine is present as a natural constituent in animal tissues, micro-organisms and plants. In man the physiological metabolic requirements are met both by the consumption of food containing carnitine and the endogenous synthesis in the liver and kidneys from lysine with methionine serving as the methyl donor. Only the L-isomer is biologically active, playing an essential role in lipid metabolism as well as in the metabolism of ketone bodies as branched chain-amino-acids. Levocarnitine as a factor is necessary in the transport of long-chain fatty acids into the mitochondria – facilitating the oxidation of fatty acids rather than their incorporation into triglycerides. By releasing CoA from its thioesters, through the action of CoA; carnitine acetyl transferase, levocarnitine also enhances the metabolic flux in the Kreb’s cycle; with the same mechanism it stimulates the activity of pyruvate dehydrogenase and in skeletal muscle, the oxidation of branched chain-amino acids. Levocarnitine is thus involved, directly or indirectly in several pathways so that its availability should be an important factor controlling not only the oxidative utilization of fatty acids and ketone bodies but also that of glucose and some amino acids to provide the organism with energy and support normal metabolism processes.

 

THERAPEUTIC INDICATIONS

ALMIBA® is indicated for the treatment of primary and secondary carnitine deficiency in adults, children, infants and neonates.

ALMIBA® is indicated in the treatment of primary systemic carnitine deficiency. In the reported cases, the clinical presentation consisted of recurrent episodes of Reye-like encephalopathy, hypoketotic hypoglycemia, and/or cardiomyopathy. Associated symptoms included hypotonia, muscle weakness and failure to thrive.

ALMIBA® is also indicated for acute and chronic treatment of patients with an inborn error of metabolism which results in a secondary carnitine deficiency.

Also ALMIBA® is also indicated for the therapy of carnitine deficiency in haemodialysis patients.

Secondary carnitine deficiency should be suspected in long-term haemodialysis patients who have the following conditions:

  1. Severe and persistent muscle cramps and/or hypotensive episodes during dialysis.
  2. Lack of energy causing a significant negative effect on the quality of life.
  3. Skeletal muscle weakness and/or myopathy.
  4. Cardiomyopathy.
  5. Anaemia of uraemia unresponsive to or requiring large doses of erythropoietin.
  6. Muscle mass loss caused by malnutrition.

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