We identify a single common modification, glycogen hyperphosphorylation, as the root cause of Lafora body pathogenesis.

No abstract

Background: Living donor liver transplantation is an important strategy of procuring segmental liver allografts for pediatric patients with liver failure, as suitably sized whole donor organs are scarce. The early pediatric living donor liver transplantation experience was associated with high rates of hepatic artery thrombosis, graft loss, and mortality. Collaboration with microsurgeons for hepatic artery anastomosis in pediatric living donor liver transplantation has decreased rates of arterial complications; however, reported outcomes are limited. Methods: A 14-year retrospective review was undertaken of children at the authors’ institution who underwent living donor liver transplantation with hepatic artery anastomosis performed by a single microsurgeon using an operating microscope. Data were collected on demographics, cause of liver failure, graft donor, vessel caliber, vessel anastomosis, arterial complications, and long-term follow-up. Results: Seventy-three children with end-stage liver failure underwent living donor liver transplantation with microvascular hepatic artery anastomosis. The commonest cause for liver failure was biliary atresia (63 percent). A total of 83 end-to-end hepatic artery anastomoses were completed using an operating microscope. Hepatic artery complications occurred in five patients, consisting of three cases of kinked anastomoses that were revised without complications and two cases of hepatic artery thrombosis (3 percent), of which one resulted in graft loss and patient death. Patient survival was 94 percent at 1 year and 90 percent at 5 years. Conclusions: Microvascular hepatic artery anastomosis in pediatric patients undergoing living donor liver transplantation is associated with a low hepatic artery complication rate and excellent long-term liver graft function. Collaboration between microsurgeons and transplant surgeons can significantly reduce technical complications and improve patient outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.