Treatment of two mitochondrial disease patients with a combination of febuxostat and inosine that enhances cellular ATP.
J Hum Genet
; 64(4): 351-353, 2019 Apr.
Article
en En
| MEDLINE
| ID: mdl-30631120
ABSTRACT
Since mitochondria are energy-generating micro-organisms, most of the disorders in patients with mitochondrial diseases (mt-disease) are considered secondary to defects in ATP synthesis, although some other factors such as reactive oxygen species may be involved. A simultaneous oral administration of febuxostat and inosine was reported to elevate both hypoxanthine and ATP levels in peripheral blood. Based on those results, we attempted co-administration of febuxostat and inosine in two patients with mitochondrial disease one patient with mitochondrial cardiomyopathy and the other patient with mitochondrial diabetes. In the former case, brain natriuretic peptide (BNP), which is a specific marker for heart failure, was decreased by 31%, and in the latter case, the insulinogenic index increased 3.1 times, suggesting the favorable action of the treatment. Considering that there is no effective treatment available for this disorder, the present therapy may be quite useful for the management of patients with mitochondrial diseases.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Adenosina Trifosfato
/
Péptido Natriurético Encefálico
/
Enfermedades Mitocondriales
/
Mitocondrias
Límite:
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Año:
2019
Tipo del documento:
Article