The Therapeutic Hypothermia in Treatment of Hyperammonemic Encephalopathy due to Urea Cycle Disorders and Organic Acidemias.
Klin Padiatr
; 231(2): 74-79, 2019 Mar.
Article
en En
| MEDLINE
| ID: mdl-30870873
ABSTRACT
BACKGROUND:
Hyperammonemic encephalopathy in newborns with urea cycle disorders and certain organic acidurias can cause severe brain injury, coma and death. Standard therapy includes protein restriction, nitrogen-scavenging drugs, prevention of catabolism and hemodialysis. Neuroprotective hypothermia as part of the treatment has been reported only 3 times. It has been suggested that mild systemic hypothermia can contribute to better neurological outcomes in hyperammonemic encephalopathy. However, the limited experience precludes accurate conclusions on safety and efficacy.METHODS:
Whole body therapeutic hypothermia was included in the standard treatment of hyperammonemic encephalopathy in 4 neonates with urea cycle disorder or organic aciduria.RESULTS:
Two patients survived the initial crisis. One patient has a developmental quotient of 0.8, while the other shows severe developmental delay. The cooling protocol had to be discontinued in 3 patients due to the otherwise untreatable complications (hypotension and hemorrhage).CONCLUSION:
The efficacy and safety of therapeutic hypothermia in the treatment of neonatal hyperammonemic encephalopathy depend on various factors, requiring further evaluation.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Urea
/
Hipoxia-Isquemia Encefálica
/
Hiperamonemia
/
Trastornos Innatos del Ciclo de la Urea
/
Hipotermia Inducida
Tipo de estudio:
Guideline
Límite:
Humans
/
Newborn
Idioma:
En
Revista:
Klin Padiatr
Año:
2019
Tipo del documento:
Article
País de afiliación:
Croacia