Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: a case report.
J Pediatr Endocrinol Metab
; 34(3): 407-410, 2021 Mar 26.
Article
em En
| MEDLINE
| ID: mdl-33675205
ABSTRACT
OBJECTIVES:
The efficacy of sodium phenylbutyrate (SPB) for hyperammonemia associated with congenital portosystemic shunt (CPSS) remains unknown. We show the effectiveness of oral SPB. CASE PRESENTATION Our patient had CPSS with severe hypoplasia of extrahepatic portal veins. At 9 months of age, to assess the efficacy of oral SPB, we evaluated the 24 h fluctuations of venous ammonia levels. In the first two days without SPB, ammonia levels were above 80 µmol/L for half a day. On the third and fourth days, administration of oral SPB three times a day decreased ammonia to acceptable levels, except at midnight. On the fifth day, another oral SPB administration at 8 pm decreased ammonia at midnight. Low levels of branched-chain amino acids, as well as coagulation disturbances, were observed without apparent symptoms. At 12 months of age, he showed normal psychomotor development.CONCLUSIONS:
Oral SPB may be effective for hyperammonemia associated with CPSS.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Fenilbutiratos
/
Sistema Porta
/
Hiperamonemia
Tipo de estudo:
Risk_factors_studies
Limite:
Humans
/
Infant
/
Male
Idioma:
En
Revista:
J Pediatr Endocrinol Metab
Assunto da revista:
ENDOCRINOLOGIA
/
PEDIATRIA
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Japão