Naslov
The Therapeutic Hypothermia in Treatment of Hyperammonemic Encephalopathy due to Urea Cycle Disorders and Organic Acidemias

Autori
Ninković, Dorotea ; Mustapić, Željka ; Bartoniček, Dorotea ; Benjak, Vesna ; Ćuk, Mario ; Dasović Buljević, Andrea ; Grčić, Boris ; Fumić, Ksenija ; Grizelj, Ruža ; Lehman, Ivan ; Ramadža Petković, Danijela ; Sarnavka, Vladimir ; Slaviček, Jasna ; Stipanović Kastelić, Jasminka ; Barišić, Nina ; Barić, Ivo

Izvornik
Klinische Pädiatrie (0300-8630) 231 (2019), 02; 74-79

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, stručni

Ključne riječi
hyperammonemic coma ; encephalopathy ; urea cycle disorders ; organic acidurias ; therapeutic hypothermia ; hypotension

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA