Practical insights into chronic management of hepatic Wilson’s disease
作者机构：Gastroenterology Research UnitDepartment of Experimental and Clinical Biomedical Sciences“Mario Serio”University of FlorenceFlorence 50134Italy Department of Experimental and Clinical MedicineUniversity of FlorenceFlorence 50134Italy Department of Medical BiotechnologiesUniversity of SienaSiena 53100Italy Division of GastroenterologyUniversity Hospital“Careggi”Florence 50134Italy
出 版 物：《World Journal of Clinical Cases》 (世界临床病例杂志)
年 卷 期：2022年第10卷第14期
页 面：4334-4347页
核心收录：
学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学]
主 题：Wilson’s disease Urinary copper excretion Non-ceruloplasmin-bound copper D-penicillamine Trientine Zinc salts
摘 要：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.
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