Living donor liver transplantation for ornithine transcarbamylase deficiency.

摘要: Ornithine transcarbamylase deficiency, the most common urea cycle disorder, causes hyperammonemic encephalopathy and has a poor prognosis. Recently, LT was introduced as radical OTCD treatment, yielding favorable outcomes. We retrospectively analyzed results for at our facility. Twelve children with (six boys six girls) accounted 7.1% of 170 who underwent LDLT department between May 2001 April 2010. Ages ranged from nine months to 11 yr seven months. Post-operative follow-up period 3-97 The post-operative survival rate 91.7%. One patient died. Two patients had neurological impairment preoperatively showed no alleviation after LT. All other than those died or failed show recovery achieved satisfactory quality-of-life improvement outcomes treatment have been satisfactory. However, associated hyperammonemia is unlikely subside even It desirable henceforth that more objective concrete guidelines management be established facilitate optimal timing while avoiding risk episodes.