To address the current role of liver transplantation (LT) for urea cycle disorders (UCDs), we reviewed the worldwide English literature on the outcomes of LT for UCD as well as 13 of our own cases of living donor liver transplantation (LDLT) for UCD. The total number of cases was 51, including our 13 cases. The overall cumulative patient survival rate is presumed to be more than 90% at 5 years.

We newly developed a sheet-type macroencapsulation device entrapping rat islets from 3% polyvinyl alcohol (PVA) dissolved in Euro-Collins solution containing 10% fetal bovine serum and 5% dimethyl sulfoxide (PVA + EC) using a freezing/thawing technique. The same encapsulation technique but with 3% PVA dissolved only in double-distilled water (PVA) and a culture of free islets were served as controls. After 14-day culture in the CMRL-1066 medium, the islet recovery rate, morphological changes, insulin content, and insulin secretion were evaluated in vitro to prove the feasibility of this method of encapsulation. We also xenotransplanted the device into the peritoneal cavity of diabetic C57BL/6 mice to check its function in vivo. After 1-day culture, the islet recovery rate and insulin content in the PVA group were significantly lower than that in the PVA + EC and free islet groups. After 14-day culture, only the islets in the PVA+EC group maintained a normal morphology and effective insulin secretory response to high glucose while the response was not observed in the PVA group after 1-day culture and no longer observed in the free islets after 7-day culture. After transplantation of rat islets encapsulated in the PVA + EC device to diabetic C57BL/6 mice, nonfasting blood glucose levels showed a rapid decrease from high glucose levels of pre-transplantation, maintaining significantly lower glucose levels during the whole course of study in comparison with the sham-operated group. Our results indicated that this freezing/thawing macroencapsulation technique using 3% PVA + EC was effective for xenotransplantation of islet cells.

Smaller-size infants undergoing living-donor liver transplantation (LDLT) are at increased risks of vascular complications because of their smaller vascular structures in addition to vascular pedicles of insufficient length for reconstruction. Out of 585 child patients transplanted between June 1990 and March 2005, 64 (10%) weighing less than 6 kg underwent 65 LDLTs. Median age and weight were 6.9 months (range: 1-16 months) and 5 kg (range: 2.8-5.9 kg), respectively. Forty-five lateral segment, 12 monosegment, and 8 reduced monosegment grafts were adopted, and median graft-to-recipient weight ratio was 4.4% (range: 2.3-9.7). Outflow obstruction occurred in only 1 patient (1.5%). Portal vein complication occurred in 9 (14%) including 5 with portal vein thrombosis. Hepatic artery thrombosis (HAT) occurred in 5 (7.7%). Patient and graft survivals were 73% and 72% at 1 yr, and 69% and 68% at 5 yr after LDLT, respectively. Thirteen of 22 grafts (58%) lost during the follow-up period occurred within the first 3 months posttransplantation. Overall graft survival in patients with and without portal vein complication was 67% and 65%, respectively (P ϭ 0.54). Overall graft survival in patients with and without HAT was 40% and 67%, respectively. HAT significantly affected graft survival (P ϭ 0.04). In conclusion, our surgical technique for smaller-size recipients resulted in an acceptable rate of vascular complications. Overcoming early posttransplantation complications will further improve outcomes in infantile LDLT. Liver Transpl 12:1224-1232, 2006 Liver transplantation (LT) is an established curative therapy for children with end-stage chronic liver disease or acute liver failure. Outcomes following LT in children have significantly improved over the past 2 decades, because of advances in surgical procedures, preservation technology, immunosuppressive management, and perioperative care.1 Shortage of full-size grafts from pediatric donors once produced high waiting-list mortality in the pediatric population, especially in children younger than 5 yr old, and prompted the identification of alternative graft sources for pediatric patients.2,3 To increase the supply of appropriate-sized organs for pediatric recipients, the techniques of reduced, split, and living-donor liver transplantation (LDLT) was developed. This technological innovation expanded the potential donor pool, and led to a significant decrease in waiting-list mortality for children. [4][5][6] Although LT in small infants provides similar results as those in older groups. 7,8 it is more challenging technically because of the smaller vascular structures. Additionally, pediatric LT from living donors faces problems of size mismatch of vessels between adult donors and pediatric recipients, accompanied by technical difficulties arising from insufficient vascular pedicles for reconstruction. More importantly, LDLT in infants is often limited by the large-for-size graft, even when using a left-lateral segment graft. This is attributed to the small size of the infan...

ObjectiveThe objective of this study was to determine whether oral glutamine supplements can protect lymphocyte and gut barrier function in patients with advanced esophageal cancer undergoing radiochemotherapy. Summary Background DataGlutamine supplements improved protein metabolism in tumor bearing rats who underwent chemotherapy and reduced the toxicity of chemotherapy through an enhancement of glutathione production in rats. MethodsThirteen patients with esophageal cancer were randomly placed in either a control or a glutamine group. Glutamine was administered orally (30 g/day) at the start of radiochemotherapy and for the subsequent 28 days. All patients underwent mediastinal irradiation and chemotherapy consisting of 5-fluorouracil and cisplatin. The lymphocyte count was determined, and blast formation was assessed after stimulation with phytohemagglutinin and concanavalin A. Gut barrier function was assessed by measuring the total amount of phenolsulfonphthalein excreted in the urine after the oral administration of phenolsulfonphthalein. ResultsGlutamine supplements prevented a reduction in the lymphocyte count (control: 567 ± 96/mm3 vs. glutamine: 1007 + 151, p < 0.05), and blast formation of lymphocyte (phytohemagglutinin, control: 19478 ± 2121