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Practical insights into chronic management of hepatic Wilson’s disease

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World Journal of Clinical Cases 2022年第14期10卷 4334-4347页

作者： Erica Nicola Lynch Claudia Campani Tommaso innocenti gabriele Dragoni Paolo Forte Andrea Galli Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences“Mario Serio”University of FlorenceFlorence 50134Italy Department of Experimental and Clinical Medicine University of FlorenceFlorence 50134Italy Department of Medical Biotechnologies University of SienaSiena 53100Italy Division of Gastroenterology University Hospital“Careggi”Florence 50134Italy

Wilson’s disease(WD)is a rare inherited disorder of human copper metabolism,with an estimated prevalence of 1:30000-1:50000 and a broad spectrum of hepatic and neuropsychiatric *** healthy individuals,the bile is the main route of elimination of *** WD patients,copper accumulates in the liver,it is released into the bloodstream,and is excreted in *** can also be accumulated in the brain,kidneys,heart,and osseous matter and causes damage due to direct toxicity or oxidative *** WD is commonly but not exclusively diagnosed in childhood or young ***,non-cirrhotic WD patients seem to have a normal life ***,chronic management of patients with Wilson’s disease is challenging,as available biochemical tests have many limitations and do not allow a clear identification of non-compliance,overtreatment,or treatment *** provide optimal care,clinicians should have a complete understanding of these limitations and counterbalance them with a thorough clinical *** aim of this review is to provide clinicians with practical tools and suggestions which may answer doubts that can arise during chronic management of patients with hepatic *** particular,it summarises current knowledge on Wilson’s disease clinical and biochemical monitoring and *** also analyses available evidence on pregnancy and the role of low-copper diet in *** research should focus on trying to provide new copper metabolism tests which could help to guide treatment adjustments.

关键词： Wilson’s disease Urinary copper excretion Non-ceruloplasmin-bound copper D-penicillamine Trientine Zinc salts

Understanding fatigue in primary biliary cholangitis:From pathophysiology to treatment perspectives

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World Journal of Hepatology 2022年第6期14卷 1111-1119页

作者： Erica Nicola Lynch Claudia Campani Tommaso innocenti gabriele Dragoni Maria Rosa Biagini Paolo Forte Andrea Galli Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences“Mario Serio”University of FlorenceFlorence 50134Italy Department of Experimental and Clinical Medicine University of FlorenceFlorence 50134Italy Department of Medical Biotechnologies University of SienaSiena 53100Italy Division of Gastroenterology University Hospital“Careggi”Florence 50134Italy

Fatigue is considered one of the most frequent and debilitating symptoms in primary biliary cholangitis(PBC),affecting over 50%of PBC *** in five patients with PBC suffer from severe fatigue,which significantly impairs quality of *** is made up of a central and a peripheral component,whose pathophysiology is still greatly *** fatigue is characterised by a lack of self-motivation and can manifest both in physical and mental activities(lack of intention).Peripheral fatigue includes neuromuscular dysfunction and muscle weakness(lack of ability).Peripheral fatigue could be explained by an excessive deviation from aerobic to anaerobic metabolism leading to excessive lactic acid accumulation and therefore accelerated decline in muscle function and prolonged recovery *** opposed to itching,and with the exception of endstage liver disease,fatigue is not related to disease *** objective of this review is to outline current understanding regarding the pathophysiology of fatigue,the role of comorbidities and contributing factors,the main tools for fatigue assessment,the failed therapeutic options,and future treatment perspectives for this disabling *** fatigue is an extremely common and debilitating symptom and there is still no licensed therapy for fatigue in PBC patients,further research is warranted to understand its causative mechanisms and to find an effective treatment.

关键词： Fatigue Primary biliary cholangitis Treatment Pathophysiology Central fatigue Peripheral fatigue

Main factors influencing long-term outcomes of liver transplantation in 2022

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World Journal of Hepatology 2023年第3期15卷 321-352页

作者： Elisa Fuochi Lorenzo Anastasio Erica Nicola Lynch Claudia Campani gabriele Dragoni Stefano Milani Andrea Galli Tommaso innocenti Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences“Mario Serio”University of FlorenceFlorence 50134Italy Department of Experimental and Clinical Medicine University of FlorenceFlorence 50134Italy Department of Medical Biotechnologies University of SienaSiena 53100Italy

Liver transplant(LT)outcomes have markedly improved in the recent decades,even if long-term morbidity and mortality are still *** of late deaths are independent from graft function and different comorbidities,including complications of metabolic syndrome and de novo neoplasms,seem to play a key role in determining long-term outcomes in LT *** review discusses the main factors associated with late mortality and suggests possible strategies to improve long-term management and follow-up after liver *** particular,the reduction of drug toxicity,the use of tools to identify high-risk patients,and setting up a multidisciplinary team also for long-term management of LT recipients may further improve survival after liver transplantation.

关键词： Alcohol Liver transplantation Long term survival Metabolic syndrome Renal dysfunction Therapy adherence

Higher volume growth rate is associated with development of worrisome features in patients with branch duct-intraductal papillary mucinous neoplasms

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World Journal of Clinical Cases 2022年第17期10卷 5667-5679页

作者： Tommaso innocenti Ginevra Danti Erica Nicola Lynch gabriele Dragoni Matteo Gottin Filippo Fedeli Daniele Palatresi Maria Rosa Biagini Stefano Milani Vittorio Miele Andrea Galli Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences"Mario Serio"University of FlorenceFlorence 50134Italy Emergency Radiology Unit Department of ServicesCareggi University HospitalFlorence 50134Italy Department of Medical Biotechnologies University of SienaSiena 53100Italy

BACKGROUND Branch duct-intraductal papillary mucinous neoplasms(BD-IPMNs)are the most common pancreatic cystic tumours and have a low risk of malignant *** guidelines only evaluate cyst diameter as an important risk factor but it is not always easy to measure,especially when comparing different *** the other side,cyst volume is a new parameter with low interobserver variability and is highly reproducible over *** To assess both diameter and volume growth rate of BD-IPMNs and evaluate their correlation with the development of malignant *** Computed tomography scans and magnetic resonance imaging exams were retrospectively *** diameter was measured on three planes,while the volume was calculated by segmentation:The volume of the entire cyst was determined by manually drawing a region of interest along the edge of the neoplasm on each consecutive slice covering the whole lesion;therefore,a threedimensional volume of interest was finally obtained with the calculated value expressed in cm^(3).Changes in size over time were *** development of worrisome features was *** We evaluated exams of 98 patients across a 40.5-mo median follow-up *** patients developed worrisome *** at baseline were significantly larger in patients who developed worrisome features(diameters P=0.0035,P=0.00652,P=0.00424;volume P=0.00222).Volume growth rate was significantly higher in patients who developed worrisome features(1.12 cm^(3)/year vs 0 cm^(3)/year,P=0.0001);diameter growth rate was higher as well,but the difference did not always reach statistical *** but not diameter growth rate in the first year of follow-up was higher in patients who developed worrisome features(0.46 cm^(3)/year vs 0 cm^(3)/year,P=0.00634).CONCLUSION The measurement of baseline volume and its variation over time is a reliable tool for the follow-up of *** measurement could be a better tool than

关键词： Intraductal papillary mucinous neoplasms Cyst Volume Growth Worrisome features Malignancies