A5061086403- Pancreatitis Pathology and Treatment
- Pediatric Hepatobiliary Diseases and Treatments
- Liver Disease Diagnosis and Treatment
- Pancreatic and Hepatic Oncology Research
- Liver Diseases and Immunity
- Gallbladder and Bile Duct Disorders
- Cystic Fibrosis Research Advances
- Liver Disease and Transplantation
- Childhood Cancer Survivors' Quality of Life
- Inflammatory Bowel Disease
- Gastrointestinal disorders and treatments
- IgG4-Related and Inflammatory Diseases
- Hepatitis Viruses Studies and Epidemiology
- Diabetes and associated disorders
- Child Nutrition and Water Access
- Hepatitis B Virus Studies
- Infant Nutrition and Health
- Hepatitis C virus research
- Neonatal Respiratory Health Research
- Neuroendocrine Tumor Research Advances
- Digestive system and related health
- Prostate Cancer Treatment and Research
- Monoclonal and Polyclonal Antibodies Research
- Connexins and lens biology
- Amoebic Infections and Treatments
Massachusetts General Hospital
2013-2024
MaineGeneral Medical Center
2011-2024
Henry Ford Health System
2024
Harvard University
2012-2023
The University of Texas MD Anderson Cancer Center
2022
Primary Children's Hospital
2020
University of Utah
2020
Stanford University
2019
Harvard University Press
2018-2019
University of Minnesota Children's Hospital
2018
Background and Aims Many children with primary sclerosing cholangitis (PSC) receive oral vancomycin therapy (OVT) or ursodeoxycholic acid (UDCA). There is a paucity of data on whether these medications improve outcomes. Approach Results We analyzed retrospective from the Pediatric PSC Consortium. Children treated OVT were matched 1:1:1 to those UDCA managed observation (no treatment) based closest propensity score, ensuring similar baseline characteristics. Two hundred sixty‐four patients...
Background and Aims: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, real‐world lacking. This study aimed to elucidate the natural history of liver disease in contemporary, international cohort children with ALGS. Approach Results: was multicenter retrospective clinically and/or genetically confirmed ALGS diagnosis, born between January 1997 August 2019. Native survival (NLS) event‐free rates...
Summary Background Data on oral vancomycin for primary sclerosing cholangitis (PSC)‐associated inflammatory bowel disease (IBD) are limited. Aims Using data from the Paediatric PSC Consortium, to examine effect of IBD activity. Methods In this retrospective multi‐centre cohort study, we matched vancomycin‐treated and untreated patients (1:3) based duration at time outcome assessment. The was Physician Global Assessment (PGA) clinical activity after 1 year (±6 months) vancomycin. We used...
We explored pancreatic neuroendocrine tumors (PanNETs) associated with tuberous sclerosis complex (TSC) to determine their incidence in the TSC population; define clinical, radiological, and pathological characteristics; investigate association underlying genotypes. Retrospectively reviewed abdominal imaging of 219 patients TSC, evaluating incidence, size, architecture lesions. Pathology records at Massachusetts General Hospital (MGH) were for all PanNET diagnoses TSC. Literature was...
ABSTRACT Objectives: The aim of the present study was to investigate natural history chronic pancreatitis (CP); patients in North American Pancreatitis Study2 (NAPS2, adults) and INternational Study group Pediatric Pancreatitis: In search for a cuRE (INSPPIRE, pediatric) were compared. Methods: Demographics, risk factors, disease duration, management outcomes 224 children 1063 adults compared using appropriate statistical tests categorical continuous variables. Results: Alcohol 53% 1% ( P...
Background and Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, approved pharmacologic therapy for cholestatic in ALGS. Since long-term placebo-controlled studies are not feasible or ethical children rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials aligned harmonized natural history cohort the G lobal AL agille A lliance...
The aim of the study was to determine rate progression from acute recurrent pancreatitis (ARP) chronic (CP) in children and assess risk factors.Data were collected INternational Study group Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) cohort. Kaplan-Meier curves constructed calculate duration initial attack (AP) CP. Log-rank test used compare survival (nonprogression) probability distribution between groups. Cox proportional hazard regression models fitted obtain ratio (with 95%...
The significance of pancreas divisum (PD) as a risk factor for pancreatitis is controversial. We analyzed the characteristics children with PD associated acute recurrent or chronic to better understand its impact.We compared without in well-phenotyped INSPPIRE (INternational Study group Pediatric Pancreatitis: In search cuRE) cohort. Differences were using 2-sample t test Wilcoxon rank sum continuous variables, Pearson χ Fisher exact categorical variables.PD was found 52 359 (14.5%)...
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. now aim to develop recommendation statements standardize diagnostic and therapeutic approach P-AIP facilitate future research in field.A panel pediatric gastroenterologists participating International Study Group Pancreatitis: In search for cuRE was formed discuss then vote on 15 statements. A consensus at least...
We created the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE (INSPPIRE 2) cohort to study risk factors, natural history, and outcomes pediatric acute recurrent pancreatitis chronic (CP). Patient physician questionnaires collect information on demographics, clinical family social disease outcomes. Health-related quality life, depression, anxiety are measured using validated questionnaires. Information entered paper is transferred into database managed by Consortium...
Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not children. We aimed to create a tool that accounts the biochemical phenotypic features early disease stage of pediatric PSC.
In young children hepatitis A virus (HAV) infections are usually subclinical events. However, HAV is also associated with progressive hepatic failure and even death in some patients. This study was undertaken to characterize the course of A-related acute liver from a developing country where endemic produces significant morbidity. Patients <15 years age confirmed A, seen at Aga Khan University Hospital between January 1991 August 1998 were identified using patient registry. Of 2735...
Abstract Introduction The objective of this study was to evaluate the feasibility shear wave elastography ( SWE ), as a non‐invasive means assessing liver fibrosis stage in paediatric and adolescent patients. Materials Methods Consecutive subjects scheduled for biopsy LB ) evaluation known or suspected diffuse disease were included after informed guardian consent subject assent IRB ‐approved single institution study. Elastograms acquired prior biopsy, from under breath‐hold normal...
Adults with chronic pancreatitis (CP) have a high risk for developing pancreatogenic diabetes mellitus (DM), but little is known regarding potential factors DM in children acute recurrent (ARP) or CP. We compared demographic and clinical features of ARP CP, without DM, the INternational Study Group Pediatric Pancreatitis: In Search CuRE (INSPPIRE) registry.We reviewed INSPPIRE database presence absence physician-diagnosed 397 children, excluding those total pancreatectomy islet...
Primary intestinal lymphangiectasia (PIL) is a rare protein‐losing enteropathy characterized by congenital malformation of the lymphatic vessels small intestine causing insufficient drainage and leakage lymph fluid. Tuberous sclerosis complex (TSC) an autosomal dominant genetic disorder benign hamartomas in multiple organ systems. While system has been implicated TSC through lymphangioleiomyomatosis (LAM) lymphedema, this paper reports first case PIL TSC, female patient with TSC2 mutation....