Treatment of Mitochondrial Encephalomyopathies With a Xanthine Oxidase Inhibitor
Autor: | Blanco-Barca Mo, M I Novo-Rodríguez, Félix Camiña, Santiago Rodríguez-Segade, Manuel Castro-Gago, Jesús Eirís-Puñal, Carmen Gómez-Lado |
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Rok vydání: | 2006 |
Předmět: |
Adenosine monophosphate
Xanthine Oxidase medicine.medical_specialty medicine.drug_class Allopurinol Biology Drug Administration Schedule chemistry.chemical_compound Developmental Neuroscience Mitochondrial Encephalomyopathies Internal medicine medicine Humans Lactic Acid Energy charge Child Xanthine oxidase Xanthine oxidase inhibitor Adenine Nucleotides Infant Adenosine diphosphate Treatment Outcome Endocrinology Neurology chemistry Child Preschool Pediatrics Perinatology and Child Health Female Neurology (clinical) Energy Metabolism Adenosine triphosphate Follow-Up Studies medicine.drug |
Zdroj: | Pediatric Neurology. 35:318-322 |
ISSN: | 0887-8994 |
DOI: | 10.1016/j.pediatrneurol.2006.05.012 |
Popis: | Five females with mitochondrial encephalomyopathies were treated for 3 to 7 years with a xanthine oxidase inhibitor (allopurinol, oral route, 20 mg/kg/day, in 2 or 3 doses daily). Clinical course was monitored in all patients. In addition, various metabolic variables, namely blood lactic acid, blood adenosine triphosphate, adenosine diphosphate, and adenosine monophosphate were monitored, as well as energy charge. Data obtained were compared with data for an age-matched control group of 10 healthy children. Four of the five patients manifested clinical improvement, and the remaining patient exhibited slower disease progression. Three of the four patients who exhibited clinical improvement also had normalization of blood lactic acid level. All five patients had an increase in blood adenosine triphosphate levels and a decline in blood adenosine monophosphate; four of the five manifested a decline in blood adenosine diphosphate and increased energy charge. Mean blood adenosine triphosphate was significantly increased with respect to pretreatment levels and with respect to the control group; mean energy charge displayed an increase, though this was not statistically significant. In one patient, reduction of the allopurinol dose to 10 mg/kg/day was followed by a decline in both blood adenosine triphosphate level and energy charge, and by clinical worsening. In conclusion, the xanthine oxidase inhibitor allopurinol appears to have had beneficial effects in these patients in terms of both energy metabolism and clinical course. |
Databáze: | OpenAIRE |
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