Link to the University of Pittsburgh Homepage
Link to the University Library System Homepage Link to the Contact Us Form

Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy

Hasegawa, T and Tzakis, AG and Todo, S and Reyes, J and Nour, B and Finegold, DN and Starzl, TE (1995) Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy. Journal of Pediatric Surgery, 30 (6). 863 - 865. ISSN 0022-3468

[img]
Preview
PDF
Accepted Version
Available under License : See the attached license file.

Download (438kB) | Preview
[img] Plain Text (licence)
Available under License : See the attached license file.

Download (1kB)

Abstract

Ornithine transcarbamylase (OTC) deficiency is an X chromosome-linked disorder causing hyperammonemic encephalopathy with a very poor prognosis. We describe here two patients with OTC deficiency, one a late-onset female patient (case 1) and the other a neonatal-onset male patient (case 2), who were successfully treated with orthotopic liver transplantation (OLTx). The OTC activity in the excised liver was 10% and 0% of control respectively. Hyperammonemic encephalopathy was controlled with medical therapy in case 1 until the age 5 years, but the complicated course in case 2 in which hyperammonemia required peritoneal dialysis and hemodialysis in the neonatal period necessitated OLTx with a reduced-size liver at the age of 80 days. Both patients had restoration of serum ammonia level to normal in 2 and 3 days after liver replacement, and both patients have normal neurological and developmental status after 2 and 0.5 years of postoperative follow-up. These cases illustrate not only the metabolic cure of this disorder, but also the need to preserve neurological integrity by aggressive medical management of the hyperammonemia preoperatively and early surgical intervention when indicated, even if this is required very early in life. © 1995.


Share

Citation/Export:
Social Networking:
Share |

Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Hasegawa, T
Tzakis, AG
Todo, S
Reyes, J
Nour, B
Finegold, DN
Starzl, TEtes11@pitt.eduTES11
Centers: Other Centers, Institutes, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 January 1995
Date Type: Publication
Journal or Publication Title: Journal of Pediatric Surgery
Volume: 30
Number: 6
Page Range: 863 - 865
DOI or Unique Handle: 10.1016/0022-3468(95)90766-1
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0022-3468
Other ID: uls-drl:31735062126242, Starzl CV No. 1768
Date Deposited: 08 Apr 2010 17:30
Last Modified: 02 Feb 2019 14:56
URI: http://d-scholarship.pitt.edu/id/eprint/5154

Metrics

Monthly Views for the past 3 years

Plum Analytics

Altmetric.com


Actions (login required)

View Item View Item