Long-Term Islet Graft Functional Assessments in More than 500 Patients Undergoing Total Pancreatectomy with Intraportal Islet Autotransplantation.

Background: Total pancreatectomy and intraportal islet cell auto transplantation (TPIAT) is increasingly being offered to patients with refractory chronic pancreatitis. Understanding factors that impact islet function over time is critical.

Study Design: We evaluated factors associated with islet function over 12 years post TPIAT using mixed meal tolerance testing (MMTT).

Precision Medicine Proof-of-Concept Study of a TNF Inhibitor in FSGS and Treatment-Resistant Minimal Change Disease.

Background: Focal segmental glomerulosclerosis (FSGS) and treatment-resistant minimal change disease (TR-MCD) are heterogeneous disorders with subgroups defined by distinct underlying mechanisms of glomerular and tubulointerstitial injury. A non-invasive urinary biomarker profile has been generated to identify patients with intra-kidney tumor necrosis factor (TNF)-activation and to predict response to anti-TNF treatment. We conducted this proof-of-concept, multi-center, open-label clinical trial to test the hypothesis that in patients with FSGS or TR-MCD and evidence of intra-renal TNF activation based on their biomarker profile, short-term treatment with adalimumab would reverse the elevated urinary excretion of MCP-1 and TIMP-1.

Successful treatment of an intrahepatic congenital portosystemic shunt causing dysfunction in a pediatric liver transplant graft.

A 5-month-old child developed severe liver dysfunction after a liver transplant with a partial liver graft. Ultrasound examination revealed a communication between the left portal vein and the left hepatic vein, a rare congenital portosystemic shunt. Here we describe the successful management of this shunt with an endovascular closure device.

Prolonged magnesium sulfate infusion as adjuvant analgesia in postoperative transplant patients in the pediatric ICU: Preliminary results of a feasibility study.

The opioid crisis has emphasized identification of opioid-sparing analgesics. This study was designed as a prospective trial with retrospective control group to determine feasibility for implementing a high-dose prolonged magnesium sulfate infusion for adjuvant analgesia in the pediatric intensive care unit. Approval was granted for study of children receiving total pancreatectomy with islet cell autotransplantation and liver transplantation ages 3-18 years.

Long-term Outcomes of Liver Transplantation for Inborn Errors of Metabolism in Children.

Background: Liver transplantation for inborn errors of metabolism is increasingly common and has historically had positive outcomes. However, this therapeutic modality is not without risks, and patient post-transplant quality of life should be part of the consideration.

Methods: This retrospective, observational cohort study included all pediatric patients receiving liver transplant from 2010 through 2020 at a single center.

Islet Isolation Outcomes in Patients Undergoing Total Pancreatectomy With Islet Autotransplantation in the POST Consortium.

Background: In total pancreatectomy with islet autotransplantation (TPIAT), a greater number of islets transplanted produces more favorable outcomes. We aimed to determine predictors of islet isolation outcomes.

Methods: We investigated factors associated with islet isolation outcomes expressed as islet number (IN), islet equivalents (IEQ; standardized to an islet with 150 μm diameter), IN/kg, or IEQ/kg using data from the multicenter Prospective Observational Study of TPIAT.

The landscape of liver transplantation for patients with alcohol-associated liver disease in the United States.

Indications for liver transplants have expanded to include patients with alcohol-associated liver disease (ALD) over the last decade. Concurrently, the liver allocation policy was updated in February 2020 replacing the Donor Service Area with Acuity Circles (ACs). The aim is to compare the transplantation rate, waitlist outcomes, and posttransplant survival of candidates with ALD to non-ALD and assess differences in that effect after the implementation of the AC policy.

Essential Fatty Acid Deficiency Is Common After Total Pancreatectomy and Islet Autotransplantation.

Objectives: Total pancreatectomy and islet autotransplantation (TPIAT) for pancreatitis may induce risk for essential fatty acid deficiency (EFAD) due to exocrine pancreatic insufficiency and intestinal alterations. The prevalence of EFAD post-TPIAT is currently unknown.

Methods: We abstracted essential fatty acid (EFA) profiles (n = 332 samples) for 197 TPIAT recipients (72% adult, 33% male).

Valacyclovir or valganciclovir for cytomegalovirus prophylaxis: A randomized controlled trial in adult and pediatric kidney transplant recipients.

Background: Valganciclovir (valG), a cytomegalovirus (CMV) prophylactic agent, has dose-limiting side effects. The tolerability and effectiveness of valacyclovir (valA) as CMV prophylaxis is unknown.

Methods: We conducted a randomized, open-label, single-center trial of valA versus valG for all posttransplant CMV prophylaxis in adult and pediatric kidney recipients.

Addressing long-term mortality risk in patients undergoing total pancreatectomy with islet autotransplant (TPIAT): causes of death and risk factors.

Background: Total pancreatectomy with islet autotransplant (TPIAT) can improve quality of life for individuals with pancreatitis but creates health risks including diabetes, exocrine insufficiency, altered intestinal anatomy and function, and asplenia.

Methods: We studied survival and causes of death for 693 patients who underwent TPIAT between 2001 and 2020, using the National Death Index with medical records to ascertain survival after TPIAT, causes of mortality, and risk factors for death. We used Kaplan Meier curves to examine overall survival, and Cox regression and competing-risks methods to determine pre-TPIAT factors associated with all-cause and cause-specific post-TPIAT mortality.

Early Metabolic Measures Predict Long-term Insulin Independence in Recipients of Total Pancreatectomy and Islet Autotransplantation.

Background: Although diabetes after total pancreatectomy and islet autotransplantation (TP-IAT) is one of the biggest concerns for TP-IAT recipients and physicians, reliable prediction of post-TP-IAT glycemic control remains unestablished. This study was conducted to identify early predictors of insulin independence and goal glycemic control by hemoglobin A1c (HbA1c) ≤ 6.5% after TP-IAT.

Should children receive a kidney transplant before 2 years of age?

Background: The optimal age of kidney transplantation for infants and toddlers with kidney failure is unclear. We aimed to evaluate the patient survival associated with kidney transplantation before 2 years of age versus remaining on the waitlist until ≥2 years.

Method: We used the Scientific Registry of Transplant Recipients to identify all children added to the deceased-donor waitlist before 2 years of age between 1/1/2000 and 4/30/2020.

Highlights from the 12th congress of the international pediatric transplant association, Austin, Texas 2023.

The 12th Congress of the (IPTA) event in Austin, Texas, had over 400 attendees from 40 countries. The attendees included a diverse mix of pediatric transplant professionals from several specialties including physicians, surgeons, scientists, nurses, organ procurement personnel, advance transplant providers, pharmacists, administrators, fellows, residents, and students. The 4-day event featured nearly 200 abstracts, 90 oral presentations, 24 mini oral presentations, and more than 80 poster presentations.

Total Pancreatectomy with Islet Auto-Transplantation: Surgical Procedure, Outcomes, and Quality of Life.

Chronic pancreatitis is a progressive and irreversible process of pancreatic inflammation and fibrosis that can lead to intractable abdominal pain and severely impaired quality of life (QoL). Often patients are refractory to standard medical or endoscopic treatments. Total pancreatectomy (TP) and islet auto-transplantation (TP-IAT) can offer pain relief to patients by removing the entire pancreas and the auto-transplant component ameliorates the resulting diabetes.

Protocolized screening and detection of occult alcohol use before and after liver transplant: Lessons learned from a quality improvement initiative.

Introduction: Detection of alcohol (ETOH) use with biomarkers provides an opportunity to intervene and treat patients with alcohol use disorder before and after liver transplant (LT). We describe our center's experience using urine ethyl glucuronide (EtG) and serum phosphatidylethanol (PEth) in alcohol screening protocols.

Methods: Single-center, retrospective review of patients presenting for LT evaluation, patients waitlisted for LT for alcohol-associated liver disease (ALD), and patients who received a LT for ALD over a 12-month period, from October 1, 2019 through September 30, 2020.

Sudden Onset Iron Overload Cardiomyopathy After Liver Transplantation.

Iron overload cardiomyopathy has been described in patients who develop acute heart failure after liver transplantation but few reports of this are available. We present a case of a patient with end-stage liver disease who underwent a deceased donor liver transplantation and developed acute onset systolic heart failure with reduced left ventricular ejection fraction. A cardiac magnetic resonance image demonstrated late gadolinium enhancement with diffuse enhancement globally and T1 mapping with severely decreased pre-contrast T1 values suggesting iron overload cardiomyopathy.

American Society for Gastrointestinal Endoscopy guideline on management of post-liver transplant biliary strictures: methodology and review of evidence.

This clinical practice guideline from the American Society for Gastrointestinal Endoscopy provides an evidence-based approach for strategies to manage biliary strictures in liver transplant recipients. This document was developed using the Grading of Recommendations Assessment, Development and Evaluation framework. The guideline addresses the role of ERCP versus percutaneous transhepatic biliary drainage and covered self-expandable metal stents (cSEMSs) versus multiple plastic stents for therapy of strictures, use of MRCP for diagnosing post-transplant biliary strictures, and administration of antibiotics versus no antibiotics during ERCP.

Use of Cidofovir for Safe Transplantation in a Toddler with Acute Liver Failure and Adenovirus Viremia.

Background: Since October 2021, there have been more than 500 cases of severe hepatitis of unknown origin in children reported worldwide, including 180 cases in the U.S. The most frequently detected potential pathogen to date has been adenovirus, typically serotype 41.

When is it safe for the liver donor to be discharged home and prevent unnecessary re-hospitalizations? - A systematic review of the literature and expert panel recommendations.

Background: Few data are available on discharge criteria after living liver donation (LLD).

Objectives: To identify the features for fit for discharge checklist after LLD to prevent unnecessary re-hospitalizations and to provide international expert recommendations.

Data Sources: Ovid MEDLINE, Embase, Scopus, Google Scholar, and Cochrane Central.

Stenting of ductus venosus as a palliation for portal hypertension because of neonatal cirrhosis.

Portal hypertension because of liver cirrhosis is a significant cause of morbidity and mortality. Treatment options in these patients include liver transplant, symptomatic treatment of oesophageal varices via endoscopic treatment and symptomatic management of ascites. Portosystemic shunt creation can be challenging in newborns and infants.