A Case with Transient Hyperammonemia of Newborn.
- Author:
Hee Kyung LEE
1
;
Beom Hee LEE
;
Hye Young JIN
;
Gu Hwan KIM
;
Jin Ho CHOI
;
Han Wook YOO
Author Information
1. Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. hwyoo@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Hyperammonemia;
THAN;
Urea cycle disorder
- MeSH:
Diet, Protein-Restricted;
Humans;
Hyperammonemia;
Infant, Newborn;
Renal Replacement Therapy;
Urea Cycle Disorders, Inborn
- From:Journal of Genetic Medicine
2010;7(1):87-90
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Hyperammonemia in the newborn often leads to severe fatal illness associated with hyperammonemic encephalopathy. Transient hyperammonemia in newborns (THAN) is characterized by self-limiting, transient hyperammonemia during the neonatal period. THAN may have favorable long-term outcomes if it is diagnosed early and appropriately managed. However, severe hyperammonemia can develop even in newborns with THAN, which may require emergent management. Here we report a case of THAN with severe hyperammonemia during the neonatal period that was successfully treated with continuous renal replacement therapy and nitrogen-scavenging medications. Our patient went on to develop normally and has not re-experienced a hyperammonemic episode until 9 months of age without the administration of a protein restricted diet or medications.
