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Diagnostic and therapeutic role of endoscopic retrograde cholangiopancreatography in biliary rhabdomyosarcoma

World journal of Gastroenterology 2008年第30期14卷 4823-4825页

作者： Ryan W Himes Isaac Raijman milton j.finegold Heidi V Russell Douglas S Fishman Section of Pediatric Gastroenterology Hepatology & Nutrition Baylor College of Medicine Texas Children's Hospital Digestive Disease Associates of Houston Department of Pathology Baylor College of Medicine Texas Children’s Hospital Texas Children’s Cancer Center Baylor College of Medicine Section of Pediatric Gastroenterology Hepatology & Nutrition Baylor College of Medicine Texas Children’s Hospital

Biliary rhabdomyosarcoma (BRMS) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMS, in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used both diagnostically and therapeutically, thus obviating the need for surgery and its attendant risks of morbidity and mortality. We conclude that ERCP is an effective alternative to surgery for BRMS in some patients.

关键词： Pancreaticobiliary Malignancy Pediatric Endoscopic retrograde cholangiopancreatography jaundice

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Wilson disease:Histopathological correlations with treatment on follow-up liver biopsies

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World journal of Gastroenterology 2010年第12期16卷 1487-1494页

作者： Sandy Cope-Yokoyama milton j.finegold Giacomo Carlo Sturniolo Kyoungmi Kim Claudia Mescoli Massimo Rugge Valentina Medici Department of Pathology University of Texas Southwestern Medical Center and Children's Medical Center Department of Pathology Baylor College of Medicine and Texas Children's Hospital Department of Surgical and Gastroenterological Sciences University of Padua Division of Biostatistics Department of Public Health ScienceUniversity of California DavisOne Shields Avenue Davis Department of Diagnostic Medical Sciences and Special Therapies Pathology UnitUniversity of Padua Division of Gastroenterology and Hepatology Department of Internal MedicineUniversity of California Davis Medical Center

AIM:To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease(WD)***:We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent multiple follow-up liver ***,clinical and laboratory data were gathered and all patients underwent an initial biopsy and at least one repeat ***:Time to repeat biopsy ranged from 2 to 12 *** patients(non-progressors)showed stable hepatic histology or *** one case,we observed improvement of fibrosis from stage 2 to *** patients(progressors)had worsening of *** was no significant correlation between the histological findings and serum aminotransferases or copper me-tabolism *** hepatic copper concentration reached normal levels in only two patients:one from the non-progressors and one from the progressors *** estimated rate of progression of hepatic fibrosis in the entire group was 0 units per year in the time frame between the first and the second liver biopsy(4 years),and 0.25 between the second and the third(3 years).In the progressors group,the rate of progression of liver fibrosis was estimated at 0.11 fibrosis units per year between the first and second biopsy and,0.6 fibrosis units between the second and third ***:The inability of clinical tools to detect fibrosis progression in WD suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered.

关键词： Wilson disease Copper Liver biopsy Histopathology

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Progressive familial intrahepatic cholestasis—farnesoid X receptor deficiency due to NR1H4 mutation:A case report

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World journal of Clinical Cases 2021年第15期9卷 3631-3636页

作者： Piotr Czubkowski Richard j Thompson Irena jankowska A S Knisely milton finegold Pamela Parsons joanna Cielecka-Kuszyk Sandra Strautnieks joanna Pawłowska Laura N Bull Department of Gastroenterology HepatologyNutritional Disorders and PediatricsThe Children’s Memorial Health InstituteWarsaw 04-730Poland Institute of Liver Studies King's College London HospitalLondon SE59RSUnited Kingdom Institut für Pathologie Medizinische Universität GrazGraz 8010Austria Department of Pathology and Immunology Baylor College of MedicineHoustonTX 77030United States Texas Children’s Hospital HoustonTX 77030United States Department of Pathology The Children’s Memorial Health InstituteWarsaw 04-730Poland Department of Medicine and Institute for Human Genetics UCSF Liver Center LaboratoryUniversity of California San FranciscoSan FranciscoCA 94143United States

BACKGROUND Functioning farnesoid X receptor(FXR;encoded by NR1H4)is key to normal bile acid *** mutations in NR1H4 are reported in a few children with intrahepatic *** describe a boy with progressive familial intrahepatic cholestasis and homozygous mutation in *** SUMMARY A boy had severe neonatal cholestasis with moderate hypercholanemia and persistently elevated *** medical treatment,coagulopathy was uncontrollable,prompting liver transplantation at age 8 mo with incidental *** patient experienced catch-up growth with good liver function and did not develop allograft ***,1 year after transplant,he died from an acute infection,considered secondary to immunosuppression and asplenia.A homozygous protein-truncating mutation,c.547C>T,p.(Arg183Ter),was subsequently identified in NR1H4,and both parents were shown to be heterozygous *** of FXR and of bile salt export pump expression was confirmed by immunostaining of explanted *** Severe cholestasis with persistently high alpha-fetoprotein and modest elevation of serum bile acid levels may suggest FXR *** patients with FXR deficiency may not develop allograft steatosis and may respond well to liver transplantation.

关键词： Neonatal cholestasis Progressive familial intrahepatic cholestasis Bile salt export pump Liver transplantation Alpha-fetoprotein Case report

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