title: Clinical applications of creatine supplementation on paediatrics.
abstract: Creatine plays a central role in energy metabolism and is synthesized in the liver, kidney and pancreas. In healthy patients, it is transported via the blood stream to the muscles, heart and brain with high and fluctuating energy demands by the molecule creatine transporter. Creatine, although naturally synthesized in the human body, can be ingested in the form of supplements and is commonly used by athletes. The purpose of this review was to assess the clinical applications of creatine supplementation on paediatrics. Creatine metabolism disorders have so far been described at the level of two synthetic steps, guanidinoacetate N-methyltransferase (GAMT) and arginine: glycine amidinotransferase (AGAT), and at the level of the creatine transporter 1(CrT1). GAMT and AGAT deficiency respond positively to substitutive treatment with creatine monohydrate whereas in CrT1 defect, it is not able to replenish creatine in the brain with oral creatine supplementation. There are also data concerning the short and long-term therapeutic benefit of creatine supplementation in children and adults with gyrate atrophy (a result of the inborn error of metabolism with ornithine delta- aminotransferase activity), muscular dystrophy (facioscapulohumeral dystrophy, Becker dystrophy, Duchenne dystrophy and sarcoglycan deficient limb girdle muscular dystrophy), McArdle's disease, Huntington's disease and mitochondria-related diseases. Hypoxia and energy related brain pathologies (brain trauma, cerebral ischemia, prematurity) might benefit from Cr supplementation. This review covers also the basics of creatine metabolism and proposed mechanisms of action.
authors.affiliation: 4th Department of Pediatrics, Aristotle University, Thessaloniki, Greece. aeevange@auth.gr
authors.forename: Athanasios
authors.initials: A
authors.lastname: Evangeliou
authors.forename: Konstantina
authors.initials: K
authors.lastname: Vasilaki
authors.forename: Paraskevi
authors.initials: P
authors.lastname: Karagianni
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Nikolaidis
mesh_terms: D000818:Animals; D002648:Child; D003401:Creatine / Q000096:biosynthesis / Q000378:metabolism / Q000494:pharmacology*; D019587:Dietary Supplements; D006801:Humans; D008659:Metabolic Diseases / Q000188:drug therapy; D008661:Metabolism, Inborn Errors / Q000188:drug therapy; D018696:Neuroprotective Agents / Q000737:chemistry / Q000494:pharmacology / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic
chemical_list: D018696:Neuroprotective Agents; D003401:Creatine