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Practical insights into chronic management of hepatic Wilson’s disease

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 manifestations.In healthy individuals,the bile is the main route of elimination of copper.In WD patients,copper accumulates in the liver,it is released into the bloodstream,and is excreted in urine.Copper can also be accumulated in the brain,kidneys,heart,and osseous matter and causes damage due to direct toxicity or oxidative stress.Hepatic WD is commonly but not exclusively diagnosed in childhood or young adulthood.Adherent,non-cirrhotic WD patients seem to have a normal life expectancy.Nevertheless,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 goals.To provide optimal care,clinicians should have a complete understanding of these limitations and counterbalance them with a thorough clinical assessment.The 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 WD.In particular,it summarises current knowledge on Wilson’s disease clinical and biochemical monitoring and treatment.It also analyses available evidence on pregnancy and the role of low-copper diet in WD.Future 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

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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 patients.One in five patients with PBC suffer from severe fatigue,which significantly impairs quality of life.Fatigue is made up of a central and a peripheral component,whose pathophysiology is still greatly unresolved.Central 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 time.As opposed to itching,and with the exception of endstage liver disease,fatigue is not related to disease progression.The 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 symptom.Since 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

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Predictors of survival in patients with established cirrhosis and hepatocellular carcinoma treated with sorafenib

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World Journal of Gastroenterology 2014年第3期20卷 786-794页

作者： Andrea L Inghilesi Donatella Gallori Lorenzo Antonuzzo Paolo Forte Daniela Tomcikova Umberto Arena Stefano Colagrande Silvia Pradella Bernardo Fani Elena Gianni Luca Boni Giacomo Laffi Francesco Di Costanzo Fabio Marra Dipartimento di Medicina Sperimentale e Clinica Università degli Studi di Firenze Oncologia Medica Azienda Ospedaliero-Universitaria Careggi Gastroenterologia 2 Azienda Ospedaliero-Universitaria Careggi Centro per il Coordinamento delle Sperimentazioni Cliniche Istituto Toscano Tumoriand Azienda Ospedaliero-Universitaria Careggi Dipartimento di Scienze Biomediche Sperimentali e ClinicheUniversità degli Studi di Firenze

AIM:To investigate in greater detail the efficacy and safety of sorafenib for the treatment of hepatocellular carcinoma(HCC)in patients with established cirrhosis.METHODS:From October 2009 to July 2012 patients with an established diagnosis of cirrhosis and HCC treated with sorafenib were consecutively enrolled.According to the Barcelona Clinic Liver Cancer(BCLC)classification,patients were in the advanced stage(BCLC-C)or in the intermediate stage(BCLC-B)but unfit or unresponsive to other therapeutic strategies.Treatment was evaluated performing a 4-phase computed tomography or magnetic resonance imaging scan every 2-3 mo,and analyzed according to the modified Response Evaluation Criteria in Solid Tumors.Sorafenib was administered at 800 mg/d,until radiological progression or occurrence of unacceptable adverse events(AEs).Univariate and multivariate analyses identified predictors of 16-wk clinical benefit and overall survival.RESULTS:Forty-four patients were enrolled,15 had intermediate HCC and 14 a Child-Pugh score of B7.AEs caused treatment interruption in 19 patients(43%),and median treatment duration was shorter in this subset(5 wk vs 19 wk,P<0.001)and in the BCLC-C subgroup(13 wk vs 40 wk,P=0.015).No significant differences in the reason for treatment interruption or in treatment duration were found comparing patients in Child-Pugh class A vs B or in patients older or younger than 70 years.After 16 wk of treatment,18 patients(41%)had stable disease or partial response.Patients with viral infection or BCLC-C were at higher risk of disease progression.ECOG,extrahepatic spread,macrovascular invasion,alpha-fetoprotein or alkaline phosphatase levels at admission were independent predictors of overall survival.CONCLUSION:In patients with cirrhosis and HCC treated with sorafenib,AEs are a common cause of early treatment withdrawal.Vascular invasion and extrahepatic spread condition early response to treatment and survival.Baseline biochemical parameters may be helpful to identify patients at higher risk of shorter

关键词： Hepatocellular carcinoma Sorafenib Cirrhosis Adver

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Impact of living with bipolar patients: Making sense of caregivers' burden

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World Journal of Psychiatry 2014年第1期4卷 1-12页

作者： Maurizio Pompili Désirée Harnic Xenia Gonda Alberto forte Giovanni Dominici Marco Innamorati Konstantinos N Fountoulakis Gianluca Serafini Leo Sher Luigi Janiri Zoltan Rihmer Mario Amore paolo Girardi Department of Neurosciences Mental Health and Sensory Organs Suicide Prevention Center Sant’Andrea Hospital Sapienza University of Rome 00189 Rome Italy Department of Psychiatry Catholic University Medical School 00189 Rome Italy Department of Clinical and Theoretical Mental Health and Department of Pharmacodynamics Semmelweis University H-1086 Budapest Hungary Neuropsychopharmacology and Neurochemistry Research Group National Academy of Sciences and Semmelweis University H-1086 Budapest Hungary National Institute of Psychiatry and Addictions Laboratory for Suicide Research and Prevention H-1135 Buda-pest Hungary 3rd Department of Psychiatry Aristotle University of Thessaloniki GR-54006 Thessaloniki Greece Department of Psychiatry Icahn School of Medicine at Mount Sinai New York NY 10029 United States Department of Clinical and Theoretical Mental Health Semmelweis University H-1125 Budapest Hungary Department of Neuroscience Rehabilitation Ophthalmology Genetics Maternal and Child Health Section of Psychiatry University of Genova I-16146 Genova Italy

The aim of the present review was to examine objective and subjective burdens in primary caregivers(usually family members) of patients with bipolar disorder(BD) and to list which symptoms of the patients are considered more burdensome by the caregivers. In order to provide a critical review about caregiver's burden in patients with bipolar disorder, we performed a detailed Pub Med, Bio Med Central, ISI Web of Science, Psyc INFO, Elsevier Science Direct and Cochrane Library search to identify all papers and book chapters in English published during the period between 1963 and November 2011. The highest levels of distress were caused by the patient's behavior and the patient's role dysfunction(work, education and social relationships). Furthermore, the caregiving role compromises other social roles occupied by the caregiver, becoming part of the heavy social cost of bipolar affective disorder. There is a need to better understand caregivers' views and personal perceptions of the stresses and demands arising from caring for someone with BD in order to develop practical appropriate interventions and to improve the training of caregivers.

关键词： Bipolar disorder Caregivers Burden Prevention

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Clinical epidemiology of chronic viral hepatitis B:A Tuscany real-world large-scale cohort study

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World Journal of Hepatology 2018年第5期10卷 409-416页

作者： Cristina Stasi Caterina Silvestri Roberto Berni Maurizia Rossana Brunetto Anna Linda Zignego Cristina Orsini Stefano Milani Liana Ricciardi Andrea De Luca Pierluigi Blanc Cesira Nencioni Donatella Aquilini Alessandro Bartoloni Giampaolo Bresci Santino Marchi Franco Filipponi Piero Colombatto Paolo Forte Andrea Galli Sauro Luchi Silvia Chigiotti Alessandro Nerli Giampaolo Corti Rodolfo Sacco Paola Carrai Angelo Ricchiuti Massimo Giusti paolo Almi Andrea Cozzi Silvia Carloppi Giacomo Laffi Fabio Voller Francesco Cipriani Observatory of Epidemiology Regional Health Agency of TuscanyFlorence 50141Italy Center for Systemic Manifestations of Hepatitis Viruses(MaSVE) Internal Medicine and Liver UnitDepartment of Experimental and Clinical MedicineCareggi University HospitalFlorence 50134Italy Web Solutions Data Visualization and Scientific DocumentationRegional Health Agency of TuscanyFlorence 50141Italy Hepatology Unit Department of Clinical and Experimental MedicineUniversity Hospital of PisaPisa 56100Italy Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences“Mario Serio”Careggi University HospitalFlorence 50134Italy Infectious Disease Unit Hospital of LuccaLucca 55100Italy Infectious Diseases Unit Department of Medical BiotechnologiesSiena University HospitalSiena 53100Italy Infectious Disease Unit “S.Maria Annunziata”HospitalPonte a Niccheri 50012Italy Infectious Disease Unit Hospital of GrossetoGrosseto 58100Italy Infectious Disease Unit Hospital of PratoPrato 59100Italy Infectious and Tropical Diseases Unit Department of Experimental and Clinical MedicineCareggi University HospitalFlorence 50134Italy Gastroenterology and Metabolic Disorders Department of SurgeryCisanello University HospitalPisa 56100Italy Gastroenterology Unit Department of Translational Research and New Technologies in Medicine and SurgeryCisanello University Hospital of PisaPisa 56100Italy Liver Surgery and Transplantation Unit Department of Surgical PathologyMedicineMolecular and Critical AreaCisanello University HospitalPisa 56100Italy Internal Medicine Unit “San Jacopo”HospitalPistoia 51100Italy Infectious Diseases and Hepatology Unit Department of Internal and Specialized MedicineUniversity Hospital of SienaSiena 53100Italy Gastroenterology Unit San Giuseppe HospitalEmpoli 50053Italy Internal Medicine and Liver Unit Department of Experimental and Clinical MedicineCareggi University HospitalFlorence 50135Italy Department of Prevention Central Tuscany Local UnitFlorence 5

AIM To build a regional database of chronic patients to define the clinical epidemiology of hepatitis B virus(HBV)-infected patients in the Tuscan public health care system.METHODS This study used a cross-sectional cohort design. We evaluated chronic viral hepatitis patients with HBV referred to the outpatient services of 16 hospital units. Information in the case report forms included main demographic data, blood chemistry data, viral hepatitis markers, instrumental evaluations, and eligibility for treatment or ongoing therapy and liver transplantation. RESULTS Of 4015 chronic viral hepatitis patients, 1096(27.3%) were HBV infected. The case report form was correctly completed for only 833 patients(64% males, 36% females; mean age 50.1 ± 15.4). Of these HBV-infected patients, 73% were Caucasian, 21% Asian, 4% Central African, 1% North African and 1% American. Stratifying patients by age and nationality, we found that 21.7% of HBV-infected patients were aged < 34 years(only 2.8% were Italian). The most represented routes of transmission were nosocomial/dental procedures(23%), mother-to-child(17%) and sexual transmission(12%). The most represented HBV genotypes were D(72%) and A(14%). Of the patients, 24.7% of patients were HBe Ag positive, and 75.3% were HBe Ag negative. Of the HBV patients 7% were anti-HDV positive. In the whole cohort, 26.9% were cirrhotic(35.8% aged < 45 years), and 47% were eligible for or currently undergoing treatment, of whom 41.9 % were cirrhotic. CONCLUSION Only 27.3% of chronic viral hepatitis patients were HBV infected. Our results provide evidence of HBV infection in people aged < 34 years, especially in the foreign population not protected by vaccination. In our cohort of patients, liver cirrhosis was also found in young adults.

关键词： Hepatitis B virus infection Liver fibrosis Cirrhosis Public health Epidemiology

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