Advanced Search
CASE REPORT
Korean J Pediatr 2010 April;53(4) :598-602.
doi:https://doi.org/10.3345/kjp.2010.53.4.598
A case of severe transient hyperammonemia in a newborn
Min Woo Hwang (Hwang MW), Seung Taek Yu (Yu ST), Yeon Kyun Oh (Oh YK)
Department of Pediatrics, Wonkwang University School of Medicine, Iksan, Korea
Corresponding Author: Yeon Kyun Oh ,Email: oyk5412@wonkwang.ac.kr
Copyright © 2010 by The Korean Pediatric Society
ABSTRACT
Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea cycle. Hyperammonemic coma may develop within 2-3 days of life, although its etiology is unknown. Laboratory studies reveal marked hyperammonemia (>4,000 µmol/ L). The degree of neurologic impairment and developmental delay in this disorder depends on the duration of hyperammonemic coma. Moreover, the infant may succumb to the disease if treatment is not started immediately and continued vigorously. Hyperammonemic coma as a medical emergency requires dialysis therapy. Here, we report a case of severe transient hyperammonemia in a preterm infant (35 week of gestation) presented with respiratory distress, seizure, and deep coma within 48 hours and required ventilatory assistance and marked elevated plasma ammonia levels. He survived with aggressive therapy including peritoneal dialysis, and was followed 2 years later without sequelae.
Keywords: Transient hyperammonemia | Hyperammonemic coma | Newborn
TOOLS
PDF Links  PDF Links
Full text via DOI  Full text via DOI
Download Citation  Download Citation
Supplementary Material  Supplementary Material
  E-Mail
Share:      
METRICS
1,145
View
3
Download
A case of reactive arthritis after Salmonella enteritis in a 12-year-old boy  2011 July;54(7)
A Case of Chylous Mesenteric Cyst in a Neonate  2005 May;48(5)
A Case of Netherton's Syndrome in a Newborn  2003 April;46(4)
A Case Report of Congenital Hyperekplexia in Twin  2002 October;45(10)
A Case of Transient Hyperphosphatasemia  2000 June;43(6)
Register for e-submission
Register here to access the e-submission system of Korean J Pediatr for authors and reviewers.
Manuscript Submission
To submit a manuscript, please visit the Korean J Pediatr e-submission management system at http://submit.kjp.or.kr, read the Instructions for Authors, and log into the Korean J Pediatr e-submission system. For assistance with manuscript submission, please contact: kjpped@gmail.com.
Free archive
Anyone may access any past or current articles without logging in.
Korean Pediatric Society Office
#1606, Seocho World Officetel, 19 Seoun-ro, Seocho-gu, Seoul 137-070, Korea
TEL : +82-2-3473-7305    FAX : +82-2-3473-7307   E-mail: kjpped@gmail.com
BrowseCurrent IssueFor Authors and ReviewersAbout
Copyright© The Korean Pediatric Society. All right reserved.