[Show abstract][Hide abstract] ABSTRACT: Liver transplantation is now an established technique to treat children with end-stage liver disease. Implantation of left lateral segment grafts (Couidaud's segments II and III) can be a problem in small infants because of a large-for-size graft. Reduced left lateral segmental liver transplantation has been recently introduced for small infants to mitigate the problem of large-for-size graft. Further reduction of the left lateral segment graft increases the possibility of supplying an adequate hyperreduced left lateral segment graft as an alternative surgical technique. We report 3 cases of our experience of transplantation using hyperreduced left lateral segment grafts from living donors.
Journal of Pediatric Surgery 09/2008; 43(8):1575-8. DOI:10.1016/j.jpedsurg.2008.02.056 · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Neonatal hepatitis, a syndrome occurring in children, has various etiologies, such as viral infection, unidentified disorders of bile salt synthesis, and other poorly understood metabolic diseases. It is characterized by jaundice, giant cell hepatitis, and, rarely, liver failure necessitating liver transplantation. We experienced 3 cases of idiopathic neonatal hepatitis with unusual progressive fibrosis presenting with retrograde portal flow and portal-systemic shunt. Clinical manifestations were hyperammonemia, hyperbilirubinemia, and coagulopathy. Characteristic histological findings were giant cell transformation of hepatocytes and progressive severe fibrosis. Two patients underwent living donor liver transplantation. We consider that liver transplantation is indicated in cases of neonatal hepatitis with hepatofugal portal flow and collateral vein formation.
[Show abstract][Hide abstract] ABSTRACT: Application of liver transplantation to methylmalonic acidemia (MMAemia) is controversial because MMAemia is caused by a systemic defect of methylmalonyl-CoA mutase. The clinical courses of seven pediatric patients with MMAemia undergoing living donor liver transplantation (LDLT) were reviewed. Serum and urinary methylmalonic acid (MMA) levels were found to be significantly decreased after LDLT, whereas serum and urinary MMA levels did not return to normal in any patient. One patient died of sepsis 44 days after LDLT. The other six patients are currently doing well. All patients had preoperative history of acute metabolic decompensation and/or metabolic stroke. However, no episode of acute metabolic decompensation or metabolic stroke was observed postoperatively in any surviving patients. In the preoperative period, all patients showed lethargy and cognitive deficit, both of which were eradicated after LDLT in all surviving patients. Preoperatively, all patients were subjected to dietary protein intake restriction and tube feeding, and were administered several metabolism-correcting medications. The metabolism-correcting medications being administered remained mostly unchanged after LDLT, whereas protein restriction was liberalized and tube feeding became unnecessary in all surviving patients. In addition, physical and neurodevelopmental growth delay remained in all surviving patients during the observation period, which ranged from 4 to 21 months with a median of 10.5 months.
American Journal of Transplantation 01/2008; 7(12):2782-7. DOI:10.1111/j.1600-6143.2007.01986.x · 5.68 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% +/- 9.2% (range 1.25-26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.