Elizabeth B. Rand
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View article: Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the <scp>GALA</scp> Study Group
Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the <span>GALA</span> Study Group Open
Background and Aim Alagille syndrome (ALGS) is a rare disorder characterised by cholestasis and extrahepatic manifestations. Given the current era of ileal bile acid transporter (IBAT) inhibitor therapies that reduce serum bile acid (SBA) …
View article: Treatment of intractable pruritus with maralixibat in patients with Alagille syndrome before and after reversal of biliary diversion
Treatment of intractable pruritus with maralixibat in patients with Alagille syndrome before and after reversal of biliary diversion Open
Alagille syndrome (ALGS) is a rare, cholestatic, multisystemic disorder characterized by bile duct paucity. Cholestatic pruritus is a common, and often severe, symptom of ALGS and is the leading cause of liver transplantation. The treatmen…
View article: Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation
Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation Open
We describe the clinical characteristics, treatment, and survival in patients with FALD-HCC. Results suggest that adults with FALD-HCC diagnosed with early stage disease may have survival benefit. Our findings underscore the importance of …
View article: Liver Transplant From a Deceased Donor With Cystinosis: A Case Report
Liver Transplant From a Deceased Donor With Cystinosis: A Case Report Open
Many inherited metabolic disorders (IMD) are associated with end‐organ damage necessitating organ transplantation. Although utilization of deceased donors with history of IMD warrants caution, there may be circumstances under which such do…
View article: Single‐Center Survey of Prevalence of Viruses in Pediatric Liver Explants
Single‐Center Survey of Prevalence of Viruses in Pediatric Liver Explants Open
Background Adenovirus has been implicated as a cause of acute liver failure (ALF) following a global outbreak of adenovirus‐associated ALF in 2021. To understand any association between viral infection and ALF, it is important to know the …
View article: Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease
Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease Open
The Fontan operation is the current standard of care for single-ventricle congenital heart disease. Individuals with a Fontan circulation (FC) exhibit central venous hypertension and face life-threatening complications of hepatic fibrosis,…
View article: Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA
Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA Open
Background and Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for chol…
View article: Hepatocellular carcinoma in survivors after Fontan operation: a case–control study
Hepatocellular carcinoma in survivors after Fontan operation: a case–control study Open
Journal Article Hepatocellular carcinoma in survivors after Fontan operation: a case–control study Get access Yuli Y Kim, Yuli Y Kim Division of Cardiology, Hospital of the University of Pennsylvania, Perelman Center for Advanced Medicine,…
View article: Liver Biopsy Leads to Serendipitous Diagnosis of Glycogen Storage Disease Type IX in a Patient With Fontan‐Associated Liver Disease
Liver Biopsy Leads to Serendipitous Diagnosis of Glycogen Storage Disease Type IX in a Patient With Fontan‐Associated Liver Disease Open
Fontan‐associated liver disease (FALD) is a form of congestive hepatopathy resulting from Fontan palliation procedures in patients with single ventricle physiology. Although there is variation between pediatric centers, the surveillance fo…
View article: Safety and Immunogenicity of Live Viral Vaccines in a Multicenter Cohort of Pediatric Transplant Recipients
Safety and Immunogenicity of Live Viral Vaccines in a Multicenter Cohort of Pediatric Transplant Recipients Open
Importance Live vaccines (measles-mumps-rubella [MMR] and varicella-zoster virus [VZV]) have not been recommended after solid organ transplant due to concern for inciting vaccine strain infection in an immunocompromised host. However, the …
View article: Live Vaccines in Pediatric Liver Transplant Recipients: “To Give or Not to Give”
Live Vaccines in Pediatric Liver Transplant Recipients: “To Give or Not to Give” Open
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View article: Center Variability in Acute Rejection and Biliary Complications After Pediatric Liver Transplantation
Center Variability in Acute Rejection and Biliary Complications After Pediatric Liver Transplantation Open
Transplant center performance and practice variation for pediatric post–liver transplantation (LT) outcomes other than survival are understudied. This was a retrospective cohort study of pediatric LT recipients who received transplants bet…
View article: Multiple organ involvement and ICU considerations for the care of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in children
Multiple organ involvement and ICU considerations for the care of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in children Open
Liver disease results in approximately 15,000 pediatric hospitalizations per year in the United States and is a significant burden to child health. Major etiologies of liver failure and indications for transplantation in children include: …
View article: Early detection of SARS‐CoV‐2 and other infections in solid organ transplant recipients and household members using wearable devices
Early detection of SARS‐CoV‐2 and other infections in solid organ transplant recipients and household members using wearable devices Open
The increasing global prevalence of SARS-CoV-2 and the resulting COVID-19 disease pandemic pose significant concerns for clinical management of solid organ transplant recipients (SOTR). Wearable devices that can measure physiologic changes…
View article: Hepatic Involvement in Aicardi-Goutières Syndrome
Hepatic Involvement in Aicardi-Goutières Syndrome Open
Aicardi-Goutières syndrome (AGS) is a monogenic type-I interferonopathy that results in neurologic injury. The systemic impact of sustained interferon activation is less well characterized. Liver inflammation is known to be associated with…
View article: End‐Organ Function and Exercise Performance in Patients With Fontan Circulation: What Characterizes the High Performers?
End‐Organ Function and Exercise Performance in Patients With Fontan Circulation: What Characterizes the High Performers? Open
Background The physiologic hallmarks of the Fontan circulation—chronically elevated central venous pressures and low cardiac output—have significant effects not only on cardiovascular status but also impact other organ systems. Exercise ca…
View article: 1396. Live Virus Vaccination Following Pediatric Liver Transplantation: Results from Two Academic Children’s Hospitals
1396. Live Virus Vaccination Following Pediatric Liver Transplantation: Results from Two Academic Children’s Hospitals Open
Background Guidelines for immunization following solid organ transplantation discourage live virus vaccination (LVV) in most recipients. Single-center studies support LVV as safe and effective in orthotopic liver transplant (OLT) recipient…
View article: Efficacy and Safety of Immunosuppression Withdrawal in Pediatric Liver Transplant Recipients: Moving Toward Personalized Management
Efficacy and Safety of Immunosuppression Withdrawal in Pediatric Liver Transplant Recipients: Moving Toward Personalized Management Open
Background and Aims Tolerance is transplantation’s holy grail, as it denotes allograft health without immunosuppression and its toxicities. Our aim was to determine, among stable long‐term pediatric liver transplant recipients, the efficac…
View article: Protein Losing Enteropathy After Fontan Operation: Glimpses of Clarity Through the Lifting Fog
Protein Losing Enteropathy After Fontan Operation: Glimpses of Clarity Through the Lifting Fog Open
Fontan-associated protein losing enteropathy is a challenge to treat and is a major contributor to morbidity and mortality in patients with palliated single ventricle. Numerous strategies for management have been proposed, with confusion a…
View article: Alagille syndrome mutation update: Comprehensive overview of<i>JAG1</i>and<i>NOTCH2</i>mutation frequencies and insight into missense variant classification
Alagille syndrome mutation update: Comprehensive overview of<i>JAG1</i>and<i>NOTCH2</i>mutation frequencies and insight into missense variant classification Open
Alagille syndrome is an autosomal dominant disease with a known molecular etiology of dysfunctional Notch signaling caused primarily by pathogenic variants in JAGGED1 (JAG1), but also by variants in NOTCH2. The majority of JAG1 variants re…
View article: Cost-effectiveness of Universal Hepatitis C Virus Screening of Pregnant Women in the United States
Cost-effectiveness of Universal Hepatitis C Virus Screening of Pregnant Women in the United States Open
Background Hepatitis C virus’ (HCV) chronic prevalence among pregnant women in the United States doubled nationally from 2009–2014 (~0.7%), yet many cases remain undiagnosed. Screening pregnant women is not recommended by the Society of Ma…
View article: Evidence of Chronic Allograft Injury in Liver Biopsies From Long-term Pediatric Recipients of Liver Transplants
Evidence of Chronic Allograft Injury in Liver Biopsies From Long-term Pediatric Recipients of Liver Transplants Open
In an analysis of biopsies from an apparently homogeneous group of stable, long-term pediatric liver transplant recipients with consistently normal liver test results, we found evidence of chronic graft injury (inflammation and/or fibrosis…
View article: Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study
Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study Open
Background Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. Methods and Results Liver biopsies and cardiac catherizations were performed as …