Open Access
EASL Clinical Practice Guidelines: Wilson's disease
Peter Ferenci,Anna Członkowska,Wolfgang Stremmel,Roderick H. J. Houwen,William Rosenberg,Michael L. Schilsky,P Jansen,Darius Moradpour,J D Gitlin +8 more
TLDR
There is not a single randomized controlled trial conducted in Wilson's disease which has an optimal design so it is impossible to assign a high or even a moderate quality of evidence to any of the questions dealt with in these guidelines.Abstract:
This Clinical Practice Guideline (CPG) has been developed to assist physicians and other healthcare providers in the diagnosis and management of patients with Wilson's disease. The goal is to describe a number of generally accepted approaches for diagnosis, prevention, and treatment of Wilson's disease. Recommendations are based on a systematic literature review in the Medline (PubMed version), Embase (Dialog version), and the Cochrane Library databases using entries from 1966 to 2011. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system used in other EASL CPGs was used and set against the somewhat different grading system used in the AASLD guidelines (Table 1A and B). Unfortunately, there is not a single randomized controlled trial conducted in Wilson's disease which has an optimal design. Thus, it is impossible to assign a high or even a moderate quality of evidence to any of the questions dealt with in these guidelines. The evaluation is mostly based on large case series which have been reported within the last decades.read more
Citations
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Burden of liver diseases in the world
TL;DR: The global prevalence of viral hepatitis remains high, while drug-induced liver injury continues to increase as a major cause of acute hepatitis.
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The Metabolic Basis Of Inherited Disease.
TL;DR: This volume, more than most, explains the contributions of the laboratory to clinical medicine, and shedding light on fundamental metabolic sequences and biologic mechanisms.
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ACG Clinical Guideline: The Diagnosis and Management of Idiosyncratic Drug-Induced Liver Injury
Naga Chalasani,Paul H. Hayashi,Herbert L. Bonkovsky,Victor J. Navarro,William M. Lee,Robert J. Fontana +5 more
TL;DR: This ACG Clinical Guideline is presented an evidence-based approach to diagnosis and management of DILI with special emphasis on DILi due to herbal and dietary supplements and DilI occurring in individuals with underlying liver disease.
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EASL Clinical Practice Guidelines: Liver transplantation
Patrizia Burra,Andrew K. Burroughs,Ivo Graziadei,Jacques Pirenne,Juan Carlos Garcia Valdecasas,Paolo Muiesan,Didier Samuel,Xavier Forns +7 more
TL;DR: This Clinical Practice Guideline has been developed to assist physicians and other healthcare providers during the evaluation process of candidates for LT and to help them in the correct management of patients after LT.
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Wilson's disease and other neurological copper disorders.
TL;DR: Disordered copper metabolism is also associated with other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations and the late-onset neurodegenerative disorders Alzheimer's disease and Parkinson's disease.
References
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GRADE: an emerging consensus on rating quality of evidence and strength of recommendations
Gordon H. Guyatt,Andrew D Oxman,Gunn Elisabeth Vist,Regina Kunz,Yngve Falck-Ytter,Pablo Alonso-Coello,Holger J. Schünemann +6 more
TL;DR: The advantages of the GRADE system are explored, which is increasingly being adopted by organisations worldwide and which is often praised for its high level of consistency.
Book
The metabolic basis of inherited disease
TL;DR: The metabolic basis of inherited disease, the metabolic basis for inherited disease as mentioned in this paper, The metabolic basis in inherited disease and inherited diseases, and inherited disease diagnosis and management, in the context of inherited diseases
Journal ArticleDOI
Absorption, transport, and hepatic metabolism of copper and zinc: special reference to metallothionein and ceruloplasmin.
Book
Diseases of the liver
Leon Schiff,Eugene R. Schiff +1 more
TL;DR: This chapter examines Anatomy; Function; Differences between dogs and cats; Investigating liver disease; Pathophysiology of acute and chronic Liver disease; Acute liver disease ; Chronic liver disease%; and Treatment of chronic liver disease.