Ryan Himes
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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: Liver disease and transplantation in telomere biology disorders: An international multicenter cohort
Liver disease and transplantation in telomere biology disorders: An international multicenter cohort Open
Background: Patients with telomere biology disorders (TBD) develop hepatic disease, including hepatitis, cirrhosis, and hepatopulmonary syndrome. No specific treatment exists for TBD-related liver disease, and the role of liver transplanta…
View article: Real‐world experience of maralixibat in Alagille syndrome: Novel findings outside of clinical trials
Real‐world experience of maralixibat in Alagille syndrome: Novel findings outside of clinical trials Open
Objective Maralixibat, an ileal bile acid transporter inhibitor, is the first drug approved by the U.S. Food and Drug Administration for the treatment of cholestatic pruritus in patients aged ≥3 months with Alagille syndrome (ALGS). Approv…
View article: P142: Efficacy, safety and tolerability of chenodeoxycholic acid in adult patients with cerebrotendinous xanthomatosis (RESTORE): A randomized, placebo-controlled phase 3 study*
P142: Efficacy, safety and tolerability of chenodeoxycholic acid in adult patients with cerebrotendinous xanthomatosis (RESTORE): A randomized, placebo-controlled phase 3 study* Open
Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive metabolic disorder of lipid-storage and bile acid synthesis caused by biallelic pathogenic variants in the CYP27A1 gene, which encodes the sterol 27-hydroxylase enzyme. Def…
View article: Diagnostic challenges and disease management in patients with a mild Zellweger spectrum disorder phenotype
Diagnostic challenges and disease management in patients with a mild Zellweger spectrum disorder phenotype Open
Peroxisome Biogenesis Disorders-Zellweger spectrum disorder (PBD-ZSD) is a rare, autosomal recessive peroxisome biogenesis disorder that presents with variable symptoms. In patients with PBD-ZSD, pathogenic variants in the PEX family of ge…
View article: Human Herpesvirus 6 Infection in Pediatric Liver Transplantation: Single-Center Study of Incidence, Outcomes, and Management
Human Herpesvirus 6 Infection in Pediatric Liver Transplantation: Single-Center Study of Incidence, Outcomes, and Management Open
Background Distinctions between HHV-6 primary infection in seronegative patients and HHV-6 reactivation in seropositive patients remains largely undescribed in pediatric liver transplant (LT) recipients. Methods We implemented pretransplan…
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: Evaluation of liver failure in a pediatric transplant recipient of a liver allograft with inherited chromosomally integrated HHV‐6B
Evaluation of liver failure in a pediatric transplant recipient of a liver allograft with inherited chromosomally integrated HHV‐6B Open
Background Active infections of human herpesvirus 6B (HHV‐6B) are frequent in immunocompromised recipients after transplantation. Nevertheless, they need to be distinguished from latent inherited chromosomally integrated genomes (iciHHV‐6)…
View article: Factors Associated With Frequent Opioid Use in Children With Acute Recurrent and Chronic Pancreatitis
Factors Associated With Frequent Opioid Use in Children With Acute Recurrent and Chronic Pancreatitis Open
Objectives: The aim of the study was to understand the association of frequent opioid use with disease phenotype and pain pattern and burden in children and adolescents with acute recurrent (ARP) or chronic pancreatitis (CP). Methods: Cros…
View article: Diabetes Mellitus in Children with Acute Recurrent and Chronic Pancreatitis
Diabetes Mellitus in Children with Acute Recurrent and Chronic Pancreatitis Open
Objectives: Adults with chronic pancreatitis (CP) have a high risk for developing pancreatogenic diabetes mellitus (DM), but little is known regarding potential risk factors for DM in children with acute recurrent pancreatitis (ARP) or CP.…
View article: Risk Factors for Rapid Progression From Acute Recurrent to Chronic Pancreatitis in Children
Risk Factors for Rapid Progression From Acute Recurrent to Chronic Pancreatitis in Children Open
Objective: The aim of the study was to determine the rate of progression from acute recurrent pancreatitis (ARP) to chronic pancreatitis (CP) in children and assess risk factors. Study Design: Data were collected from the IN ternational S …
View article: Central line-associated bloodstream infection among children with biliary atresia listed for liver transplantation
Central line-associated bloodstream infection among children with biliary atresia listed for liver transplantation Open
There is substantial risk for CLABSI among children with BA listed for liver transplantation. No clinical, demographic, or laboratory factor we tested emerged as an independent predictor of CLABSI. While our data did not show an impact of …
View article: Chronic Pancreatitis
Chronic Pancreatitis Open
Objectives: The aim of the present study was to investigate the natural history of chronic pancreatitis (CP); patients in the North American Pancreatitis Study2 (NAPS2, adults) and INternational Study group of Pediatric Pancreatitis: In se…
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: Impact of Obesity on Pediatric Acute Recurrent and Chronic Pancreatitis
Impact of Obesity on Pediatric Acute Recurrent and Chronic Pancreatitis Open
Objective The aim of this study was to assess the impact of obesity on pediatric acute recurrent pancreatitis or chronic pancreatitis (CP). Methods We determined body mass index (BMI) status at enrollment in INSPPIRE ( IN ternational S tud…
View article: Pancreas Divisum in Pediatric Acute Recurrent and Chronic Pancreatitis
Pancreas Divisum in Pediatric Acute Recurrent and Chronic Pancreatitis Open
Introduction: The significance of pancreas divisum (PD) as a risk factor for pancreatitis is controversial. We analyzed the characteristics of children with PD associated with acute recurrent or chronic pancreatitis to better understand it…
View article: Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood
Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood Open
Objectives: Autoimmune pancreatitis (AIP) represents a complex immune‐mediated pancreas disorder. Pediatric AIP (P‐AIP) is rare. We have recently summarized the characteristic features of P‐AIP. We now aim to develop recommendation stateme…