Intraoperative renal replacement therapy during liver transplantation in children: Safety, efficacy and impact on survival.

Background: Intraoperative Continuous Renal Replacement Therapy (iCRRT) can prevent life-threatening complications, facilitate fluid management, and maintain metabolic homeostasis during liver transplantation (LT) in adults. There is a paucity of data in pediatric LT. We evaluated the safety, efficacy, and impact on survival of iCRRT in pediatric LT.

Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report.

Thrombocytopenia absent radius (TAR) syndrome is a rare genetic disorder that has been associated with food protein-induced allergic proctocolitis and transient leukemoid reactions, among other manifestations. There has been no prior reports of its association with autoimmune disease, more specifically, autoimmune hepatitis (AIH) or the development of pediatric acute liver failure (PALF). We present a case of an 8-month-old infant with TAR syndrome who presented with PALF, secondary to AIH with elevated liver-kidney microsomal antibody (>1:2560).

Development of a national Zoom-based lectureship for pediatric transplant hepatology trainees.

Background: Previous publications identified a gap in standard education on topics related to advanced hepatology and liver transplantation for pediatric transplant hepatology trainees. The Society of Pediatric Liver Transplantation (SPLIT) Education Committee designed a Zoom-based lectureship series for all advanced pediatric transplant hepatology trainees. We aim to describe the educational series and feedback from fellow participants.

Acute-on-chronic liver failure before liver transplantation does not impact post-transplant survival in children with biliary atresia.

Acute-on-chronic liver failure (ACLF) occurs in children with biliary atresia (BA) awaiting liver transplantation (LT). However, data on transplant outcomes in ACLF are limited. Our aim was to characterize ACLF and determine its effect on transplant outcome and resource utilization.

Beyond Varices: Complications of Cirrhotic Portal Hypertension in Pediatrics.

Complications of cirrhotic portal hypertension (PHTN) in children are broad and include clinical manifestations ranging from variceal hemorrhage, hepatic encephalopathy (HE), ascites, spontaneous bacterial peritonitis (SBP), and hepatorenal syndrome (HRS) to less common conditions such as hepatopulmonary syndrome, portopulmonary hypertension, and cirrhotic cardiomyopathy. The approaches to the diagnosis and management of these complications have become standard of practice in adults with cirrhosis with many guidance statements available. However, there is limited literature on the diagnosis and management of these complications of PHTN in children with much of the current guidance available focused on variceal hemorrhage.

Neonates with acute liver failure have higher overall mortality but similar posttransplant outcomes as older infants.

Neonatal acute liver failure (ALF) carries a high mortality rate; however, little data exist on its peritransplant hospital course. This project aimed to identify factors associated with outcomes in neonates with ALF using large multicenter databases. Patients with International Classification of Diseases, Ninth Revision/International Classification of Diseases, Tenth Revision codes for liver failure (2004-2018) from linked Pediatric Health Information System and Scientific Registry of Transplant Recipients databases were assigned to two groups: neonates aged ≤30 days or older infants aged 31-120 days at admission.

Our evolution in the treatment of hepatic artery and portal vein thrombosis in pediatric liver transplantation: Success with catheter-directed therapies.

Background: In pediatric liver transplant recipients, hepatic artery thrombosis and portal vein thrombosis are major causes of acute graft failure and mortality within 30 days of transplantation. There is, however, a strong possibility of graft salvage if flow can be re-established to reduce ischemic injury. The current standard treatment is surgical revascularization, and if unsuccessful, retransplantation.

A Case of Syphilitic Hepatitis in an Adolescent.

The incidence of syphilis is rising among adolescents necessitating improved provider awareness and screening practices. We present a case of an adolescent with acute hepatitis ultimately diagnosed with secondary syphilitic hepatitis. Clinical presentation, laboratory abnormalities, and histologic features of syphilitic hepatitis are nonspecific, with diagnosis relying on clinical suspicion and targeted testing.

Bivalirudin for the prevention of hepatic artery thrombosis in pediatric liver transplantation.

Background: Early hepatic artery thrombosis (HAT) after liver transplantation is a serious complication that frequently results in graft loss and the need for retransplantation. Although studies have reported on various operative and endovascular treatment approaches, pharmacologic strategies for the prevention or management of HAT are not well defined. Patients with blood clotting disorders, those with a contraindication to heparin, and those who have previously developed HAT represent unique challenges in management.

Transient Elastography in Pediatric Liver Disease.

Transient elastography is an imaging technique utilizing shear wave technology to measure liver stiffness. Recent studies have shown success in utilizing this technique in children. Transient elastography is useful in estimating degree of fibrosis in various pediatric liver diseases, including biliary atresia, alpha-1-antitrypsin deficiency, Alagille syndrome, cystic fibrosis-related liver disease, and non-alcoholic steatohepatitis among others.

Applying an Age-specific Definition to Better Characterize Etiologies and Outcomes in Neonatal Acute Liver Failure.

Objective: Neonatal acute liver failure (ALF) is a rare disease with high mortality for which no standard age-specific definition exists. To advance the understanding of neonatal ALF, we characterize the etiology, presenting features, treatment, and outcomes in infants within 1 month of life.

Methods: We performed a single-center 11-year retrospective chart review of neonates ≤30 days of life with ALF as defined by an INR of ≥2.

Dexmedetomidine leading to profound bradycardia in a pediatric liver transplant recipient.

Dexmedetomidine, an α -agonist, is used in the PICU for its sedative properties as it minimally affects respiratory status. However, hemodynamic instability is one of its known side effects. There is limited published experience with its use in pediatric liver transplant.

Admission Characteristics Identify Risk of Pediatric Acute-on-Chronic Liver Failure.

Objectives: Acute-on-chronic liver failure (ACLF) is well-studied in adults and characterized by decompensated cirrhosis, multi-organ failure, and early mortality. Studies of ACLF in children are limited. We sought to characterize the prevalence and clinical factors associated with pediatric ACLF (PACLF).

Therapeutic Endoscopy for the Control of Nonvariceal Upper Gastrointestinal Bleeding in Children: A Case Series.

Objectives: Gastrointestinal bleeding is one of the most common indications for urgent endoscopy in the pediatric setting. The majority of these procedures are performed for control of variceal bleeding, with few performed for nonvariceal upper gastrointestinal (NVUGI) bleeding. The data on therapeutic endoscopy for NVUGI are sparse.

Resolution of Hepatic Artery Thrombosis in 2 Pediatric Liver Transplant Patients.

Hepatic artery thrombosis (HAT) is a serious complication after liver transplantation. This is the first report of spontaneous resolution of HAT in pediatric liver transplant patients on low molecular weight heparin therapy. A total of 2 patients, a 26-month-old boy who presented with acute liver failure and required emergent liver transplantation and a 15-year-old boy with ulcerative colitis and autoimmune hepatitis-primary sclerosing cholangitis overlap underwent liver transplantation for progressive cirrhosis; both developed HAT during the postoperative period.