Living-donor liver transplantation with hyperreduced left lateral segment grafts: a single-center experience.

Background: In the setting of liver transplantation in small infants who receive left lateral segment (LLS) grafts, problems are encountered related to graft-size mismatching in the form of so-called "large-for-size" grafts. To address these problems, the feasibility of further reducing the size of LLS grafts to form hyperreduced LLS (HRLLS) grafts was investigated.

Methods: Of the 175 pediatric living-donor liver transplantations performed between November 2005 and December 2011 at our institute, 31 cases were performed using HRLLS grafts.

Incidentally detected cholangiocarcinoma in an explanted liver with biliary atresia after Kasai operation.

This report presents the case of a 30-yr-old woman with BA who developed incidental cholangiocarcinoma following the Kasai operation. She showed progressive liver dysfunction and cirrhosis at the age of 30 yr and underwent LDLT. A 4-cm-diameter liver tumor in the anastomotic site of portoenterostomy was incidentally found as a result of a pathological examination of the explanted native liver.

Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein.

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. A 14-year-old male with CAPV underwent living donor liver transplantation with a left lobe graft from his father. The portal vein reconstruction was achieved with a renoportal anastomosis using an interpositional graft from the native collateral vein, because portal venous system directly drains to the left renal vein without constructing the confluence of superior mesenteric vein and splenic vein.

Living donor liver transplantation using grafts with hepatic cysts.

Cystic lesions in the liver are often found through the evaluation of liver donors. Multiple cysts are worrisome, and donor candidates with multiple cysts may be unacceptable as liver donors, especially when their recipients have fibrocystic disease (FCD), which is an inherited disorder. This study reviewed 183 cases of living donor liver transplantation.

Diaphragmatic hernia in infants following living donor liver transplantation: report of three cases and a review of the literature.

DH is a rare complication following LT. This report presents three cases of right-sided DH after LT using a left-sided graft. All of the patients were younger than one yr of age, and they were critically ill owing to their original disease, characterized by biliary atresia, progressive familiar intrahepatic cholestasis, and acute liver failure.

Glycemic management in living donor liver transplantation for patients with glycogen storage disease type 1b.

GSD type 1b is an autosomal recessive inborn error of carbohydrate metabolism caused by defects of the G6Pase translocase (G6PT). Patients with GSD1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, and neutropenia. LT has been indicated for severe glucose intolerance.

Portal vein reconstruction in pediatric living donor liver transplantation for patients younger than 1 year with biliary atresia.

Background/purpose: Infants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis.

Method: Of 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique.

Pediatric liver-kidney transplantation for hepatorenal fibrocystic disease from a living donor.

The indications for and the timing of LT and/or KT for the patients with HRFCD are based on the severity of liver and kidney involvement. Most organs come from living donors, because the number of deceased donors is extremely low in Japan. Therefore, patients with HRFCD may need two organs from living donors.

Living-donor liver transplantation for propionic acidemia.

Propionic acidemia is a rare autosomal recessive disorder affecting the catabolism of branched-chain amino acids because of a genetic defect in PCC. Despite the improvements in medical treatment with protein restriction, sufficient caloric intake, supplementation of l-carnitine, and metronidazole, patients with the severe form of propionic acidemia have life-threatening metabolic acidosis, hyperammonemia, and cardiomyopathy, which results in serious neurologic sequelae and sometimes death. This study retrospectively reviewed three children with neonatal-onset propionic acidemia who received LDLT.

Micro-particle transporting system using galvanotactically stimulated apo-symbiotic cells of Paramecium bursaria.

It is well known that Paramecium species including green paramecia (Paramecium bursaria) migrate towards the anode when exposed to an electric field in a medium. This type of a cellular movement is known as galvanotaxis. Our previous study revealed that an electric stimulus given to P bursaria is converted to a galvanotactic cellular movement by involvement of T-type calcium channel on the plasma membrane [Aonuma et al.

Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation.

LDLT is an effective treatment modality in patients with congenial metabolic liver disease. PA is a rare autosomal recessive disorder caused by deficiency in propionyl-CoA carboxylase. The present study demonstrates a two-yr-old girl with PA who was admitted for metabolic decompensation and immediately treated with CHD and protein intake restriction at 46 days of age.