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
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: |
|
Details
| Item Type: |
Article
|
| Status: |
Published |
| Creators/Authors: |
| Creators | Email | Pitt Username | ORCID  |
|---|
| Hasegawa, T | | | | | Tzakis, AG | | | | | Todo, S | | | | | Reyes, J | | | | | Nour, B | | | | | Finegold, DN | | | | | Starzl, TE | tes11@pitt.edu | TES11 | |
|
| Centers: |
Other Centers, Institutes, Offices, 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 |
![[img]](https://d-scholarship.pitt.edu/style/images/fileicons/text_plain.png)
![[feed]](/images/feed-icon-32x32.png){kind=icon}