Current management of biliary atresia based on 35 years of experience at a single center.

Objective: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy.

Methods: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016.

Comparison of the Results of Living Donor Liver Transplantation Due to Acute Liver Failure and Biliary Atresia in a Quaternary Center.

Objective: The objective of this study was to compare the complications, outcomes, and survival prevalence in patients undergoing living donor liver transplantation due to biliary atresia (BA) or acute liver failure (ALF).

Results: In the period of June 1998-July 2016, 199 children underwent living transplantation due to BA or ALF. Of these 199, 184 were included in the analysis.

Pollen concentrations and prevalence of asthma and allergic rhinitis in Italy: Evidence from the GEIRD study.

Background: Pollen exposure has acute adverse effects on sensitized individuals. Information on the prevalence of respiratory diseases in areas with different pollen concentrations is scanty.

Aim: We performed an ecologic analysis to assess whether the prevalence of allergic rhinitis and asthma in young adults varied across areas with different pollen concentrations in Italy.

Living donor liver transplantation in children: should the adult donor be operated on by an adult or pediatric surgeon? Experience of a single pediatric center.

Background/purpose: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation.

Methods: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013.

Centrilobular necrosis as a manifestation of venous outflow block in pediatric malnourished liver transplant recipients--case reports.

CLN is a frequent histological finding in biopsies after pediatric: LT, and its pathogenesis has not yet been fully clarified and has different causes. Among the vascular causes, VOB is sometimes difficult to diagnose, especially when technical variants such as split-liver, reduced-liver, or living-related LT are utilized. Three liver-transplanted malnourished children (ages 12, 20, and 28 months) developed altered LFTs and post-operative ascites with right pleural effusion (two cases) and jaundice (one case).

Transmission of clear cell tumor in a graft liver from cadaveric donor: case report.

Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor-recipient serostatus, especially in relation to the Epstein-Barr virus. The patient was a two-yr-old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor.

The role of interleukin-6, endothelins, and apoptotic genes in small bowel transplantation, in a swine model of ischemia and reperfusion injury.

IRI is closely related to sepsis in ITx setting. Complete understanding of the mechanisms involved in IRI development may improve outcomes. Ortothopic ITx without immunosuppression was performed in order to characterize IRI-associated mucosal damage.

Which is the best technique for hepatic venous reconstruction in pediatric living-donor liver transplantation? Experience from a single center.

Background/purpose: The introduction of the piggyback technique for reconstruction of the liver outflow in reduced-size liver transplants for pediatric patients has increased the incidence of hepatic venous outflow block (HVOB). Here, we proposed a new technique for hepatic venous reconstruction in pediatric living-donor liver transplantation.

Methods: Three techniques were used: direct anastomosis of the orifice of the donor hepatic veins and the orifice of the recipient hepatic veins (group 1); triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins (group 2); and a new technique, which is a wide longitudinal anastomosis performed at the anterior wall of the inferior vena cava (group 3).

Rex shunt for acute portal vein thrombosis after pediatric liver transplantation in children with biliary atresia.

Background/purpose: Posttransplantation portal vein thrombosis (PVT) can have severe health consequences, and portal hypertension and other consequences of the long-term privation of portal inflow to the graft may be hazardous, especially in young children. The Rex shunt has been used successfully to treat PVT patients since 1998. In 2007, we started to perform this surgery in patients with idiopathic PVT and late posttransplantation PVT.

Orthotopic liver transplantation in biliary atresia: a single-center experience.

Introduction: Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures.

Objective: The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes.

Hepatic artery graft in pediatric liver transplantation: single-center experience with 58 cases.

Introduction: The use of arterial grafts (AG) in pediatric orthotopic liver transplantation (OLT) is an alternative in cases of poor hepatic arterial inflow, small or anomalous recipient hepatic arteries, and retransplantations (re-OLT) due to hepatic artery thrombosis (HAT). AG have been crucial to the success of the procedure among younger children. Herein we have reported our experience with AG.

Living related donor liver transplantation in children.

Objective: The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors.

Methods: We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010.

Results: Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3).

Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt?

Background: Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients.

Methods: We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft.

Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects.

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one.

Multiple clinical presentations of lymphoproliferative disorders in pediatric liver transplant recipients: a single-center experience.

Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication following solid organ transplantation that has been linked to Epstein-Barr virus (EBV) infection. The aim of this article was to describe a single-center experience with the multiplicity of clinical presentations of PTLD. Among 350 liver transplantations performed in 303 children, 13 survivor children displayed a histological diagnosis of PTLD (13/242 survivors; 5.

Surgical treatment of hepatic tumors in children: lessons learned from liver transplantation.

Purpose: Hepatectomy remains a complex operation even in experienced hands. The objective of the present study was to describe our experience in liver resections, in the light of liver transplantation, emphasizing the indications for surgery, surgical techniques, complications, and results.

Methods: The medical records of 53 children who underwent liver resection for primary or metastatic hepatic tumors were reviewed.

Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality.

Background: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO.

Long-term results of percutaneous bilioenteric anastomotic stricture treatment in liver-transplanted children.

The purpose of this study was to evaluate the mid- and long-term results of percutaneous transhepatic cholangiography (PTC) and biliary drainage in children with isolated bilioenteric anastomotic stenosis (BAS) after pediatric liver transplantation. Sixty-four children underwent PTC from March 1993 to May 2008. Nineteen cholangiograms were normal; 10 showed intrahepatic biliary stenosis and BAS, and 35 showed isolated BAS.

"Rex shunt" for the treatment of portal vein thrombosis after pediatric liver transplantation: a case report.

Background And Purpose: Late portal vein thrombosis (PVT) can be extremely well tolerated, although portal hypertension and other consequences of the long-term deprivation of portal inflow to the graft may be hazardous, especially in young children. Recently, the "Rex shunt" has been used successfully to treat these patients. We now report the initial experience with this novel technique.

Sirolimus in pediatric liver transplantation: a single-center experience.

Background And Aims: Liver transplantation (OLT) in children has seen significant improvements in recent years. Long-term immunosuppressive strategies have focused on avoiding the risks of long-term immunosuppression, particularly nephrotoxicity, de novo malignancy and late infections. Since its introduction in renal transplantation in 1999, sirolimus (SRL) has been used by an increasing number of liver transplant centers.

Immunohistochemical studies of stellate cells in experimental cholestasis in newborn and adult rats.

Background And Aims: Although there is much known about liver diseases, some aspects remain unclear, such as the nature of the differences between the diseases observed in newborn infants and those in adults. For example, how do newborns respond to duct epithelial cell injury? Do the stellate cells in newborns respond similarly to those in adults during biliary obstruction?

Methods: Ninety newborn Wistar rats aged six days, weighing 8.0 - 13.

Midterm and long-term results of percutaneous endovascular treatment of venous outflow obstruction after pediatric liver transplantation.

Purpose: To evaluate retrospectively the midterm and long-term results of percutaneous endovascular treatment of venous outflow obstruction after pediatric liver transplantation.

Materials And Methods: During a 9-year period, 18 children with obstruction of a hepatic vein (HV) or inferior vena cava (IVC) anastomosis underwent percutaneous transluminal angioplasty (PTA) with balloon dilation or stent placement in case of PTA failure after liver transplantation. Patients' body weights ranged from 7.

Long-term follow-up after successful transjugular intrahepatic portosystemic shunt placement in a pediatric patient with Budd-Chiari syndrome.

Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.

Bile duct ligation in neonatal rats: is it a valid experimental model for biliary atresia studies?

BA is the most important disease requiring liver transplantation in children. Common BDL in rats is a classic experimental model to study biliary obstruction. The response of the neonatal animal to BDL has yet to be completely understood and few reports have focused on the behavioral differences of the liver between neonatal and adult animals.