Abstract | OBJECTIVE: METHOD: RESULT: Four girls were diagnosed with ornithine carbamoyl transferase deficiency by genetic test. They had the clinical onset at the age of 1.5 to 3.0 years. Liver transplantation had been performed at their age of 53.9 months, 40.6 months, 40.3 months and 22.8 months, respectively. The grafts of case 1 and case 2 were from left lateral lobe of liver of cadaveric donor, the graft of case 3 was from left lateral lobe of liver of a living donor, the graft of case 4 was a whole liver of a dead child. The liver function of 4 patients gradually returned to normal, blood ammonia levels were normal and restored the normal diet, 4 children were discharged on postoperative 25-30 days. Regular follow-up was done, the liver function, biochemical features and growth status have been followed up for 162.2 months, 124.2 months, 12.0 months and 4.8 months after liver transplantation, respectively. Now, all the four cases are healthy and growth is normal. CONCLUSION:
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Authors | Zhijun Zhu, Liying Sun, Lin Wei, Wei Qu, Zhigui Zeng, Ying Liu, Liang Zhang, Enhui He, Dong Wang |
Journal | Zhonghua er ke za zhi = Chinese journal of pediatrics
(Zhonghua Er Ke Za Zhi)
Vol. 53
Issue 2
Pg. 136-9
(Feb 2015)
ISSN: 0578-1310 [Print] China |
PMID | 25876690
(Publication Type: Case Reports, Journal Article)
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Topics |
- Child
- Child, Preschool
- Female
- Humans
- Hyperammonemia
(etiology, surgery)
- Liver
- Liver Function Tests
- Liver Transplantation
- Living Donors
- Ornithine Carbamoyltransferase Deficiency Disease
(complications)
- Prognosis
- Retrospective Studies
- Tissue Donors
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