|1.||Liver Diseases (Liver Disease)
|4.||Hepatolenticular Degeneration (Wilson's Disease)
|1.||Lutsenko, Svetlana: 38 articles (09/2015 - 01/2002)|
|2.||Stremmel, Wolfgang: 29 articles (09/2015 - 09/2004)|
|3.||Brewer, George J: 27 articles (01/2015 - 03/2002)|
|4.||Członkowska, Anna: 25 articles (10/2015 - 02/2006)|
|5.||Ferenci, Peter: 23 articles (07/2015 - 02/2002)|
|6.||Schilsky, Michael L: 22 articles (09/2015 - 02/2002)|
|7.||Merle, Uta: 19 articles (09/2015 - 04/2006)|
|8.||Weiss, Karl Heinz: 18 articles (06/2015 - 04/2006)|
|9.||Litwin, Tomasz: 17 articles (10/2015 - 04/2008)|
|10.||Loudianos, Georgios: 14 articles (09/2015 - 01/2003)|
10/01/2011 - "In 4 young pediatric patients with presymptomatic Wilson disease, we found zinc monotherapy beginning at time of diagnosis to be safe and highly effective for follow-up intervals between 1 and 2 years. "
12/01/2015 - "Zinc therapy is considered a good option in Wilson disease (WD), as a first-line treatment in presymptomatic children and a maintenance therapy after the initial chelator therapy. "
05/01/2009 - "Zinc is an effective treatment for Wilson disease. "
08/04/1984 - "Effective treatment of Wilson's disease with oral zinc sulphate: two case reports."
10/01/1982 - "Effective treatment of Wilson's disease with oral zinc."
12/01/1978 - "Urinary copper excretion was significantly elevated in response to 30 mg/kg of DMS, suggesting that the drug might also be useful for the treatment of Wilson's disease."
02/01/2007 - "Chelating agent is the mainstay of treatment in Wilson's disease, which is an inherited disorder of hepatic copper metabolism. "
03/01/2005 - "[Genetic disorders of copper transport--diagnosis and new treatment for the patients of Wilson's disease]."
06/01/1998 - "An improved method for the study of copper metabolism in Wilson's disease, using a stable, rather than radioactive, copper isotope (65Cu) has recently been described. "
11/01/1996 - "Although results of neurologic examination, speech pathology examination, and 24-h urine copper level analysis in symptomatic Wilson's disease patients improved during the study period, KF ring regression did not correlate with the improvement in these clinical parameters (p > 0.05). "
|3.||Penicillamine (Cuprimine)FDA Link
05/01/2009 - "Nevertheless, according to currently available data, patients with hepatic presentation of Wilson disease are probably most effectively treated by D-penicillamine. "
12/01/1984 - "The best treatment for fulminant Wilson's disease is prevention by diagnosis in a pre-symptomatic stage and institution of carefully supervised life-long therapy with penicillamine."
04/12/1986 - "A patient who presented with severe hepatic Wilson's disease at age 14 responded well to treatment with penicillamine and remained in good health for a further 20 years, although she became somewhat careless about her medication towards the end of this period. "
06/28/1999 - "Treatment with penicillamine in Indian childhood disease, which probable has the same etiology, is effective when it is started early enough as well as in Wilson's disease. "
09/01/1993 - "D-penicillamine, an orally-administered chelating agent, is effective for Wilson's disease (WD). "
|4.||tetrathiomolybdate (ammonium tetrathiomolybdate)IBA
02/10/2009 - "Tetrathiomolybdate (TTM) is a promising new treatment for Wilson's disease which has been demonstrated both in an animal model and in clinical trials. "
05/01/1992 - "A new experimental drug, tetrathiomolybdate, shows promise in the initial treatment of patients with Wilson disease. "
05/01/2004 - "Studies of the use of ammonium tetrathiomolybdate as an alternative chelating agent for the initial treatment of neurologic Wilson's disease are still in the experimental phase. "
03/01/2003 - "Open-label study of 55 untreated patients (22 of them new) presenting with neurologic Wilson disease treated with tetrathiomolybdate varying from 120 to 410 mg/d for 8 weeks and then followed up for 3 years. "
10/01/2014 - "Tetrathiomolybdate (TM) is a unique anticopper drug developed for the treatment of the neurologic presentation of Wilson's disease, for which it is excellent. "
|5.||Trientine (Trien)FDA Link
07/09/2011 - "Trientine (TETA dihydrochloride) has been used since the mid-1980s as a second-line treatment for Wilson's disease, and our recent clinical studies have reinforced the impression that it is likely to be safe for long-term use in patients with diabetes and related metabolic disorders. "
04/23/1999 - "In this study, trientine and ascorbate, known to have a beneficial effect in an animal model of Wilson disease, were administered to transgenic mice overexpressing a mutated human SOD1 (G93A). "
09/01/1993 - "[A study of trientine therapy in Wilson's disease with neurological symptoms]."
02/01/2016 - "Wilson's disease with cognitive impairment and without extrapyramidal signs: improvement of neuropsychological performance and reduction of MRI abnormalities with trientine treatment."
05/01/2015 - "To prospectively evaluate once-daily trientine as therapy for Wilson disease. "
|6.||Zinc AcetateFDA Link
12/01/2010 - "The authors conclude that zinc acetate is an effective and safe treatment for Japanese patients with Wilson disease."
01/01/2007 - "The safety of the zinc acetate allowed us to avoid other potentially toxic chelating drugs; this observation is in line with the growing evidence on the efficacy of the drug in the treatment of Wilson's disease. "
03/01/2001 - "The objectives were to evaluate appropriate doses of zinc acetate and its efficacy for the maintenance management of Wilson's disease in pediatric cases. "
12/01/2010 - "The 37 patients that comprise this study were found to have Wilson disease using clinical and biochemical tests and were administrated zinc acetate for 48 weeks. "
12/01/2010 - "In this study, the authors used zinc acetate to treat Japanese patients with Wilson disease and investigated its efficacy. "
11/01/2009 - "Our data suggest that the enzymatic ceruloplasmin assay is superior to the immunologic assay in diagnosing Wilson's disease and should become the preferred method."
01/01/1979 - "Clinical amelioration, clearance of Kayser-Fleischer rings and rising of ceruloplasmin concentration are described in a patient with the classical findings of Wilson's disease. "
08/01/2008 - "In this study, we evaluated various decision thresholds of serum ceruloplasmin concentration in the diagnosis of Wilson disease based on genotype-verified Wilson disease patients, carriers, and normal individuals. "
09/01/2006 - "Our study goals were to determine the feasibility of a population screening for Wilson disease using dried blood spots and to characterize the base-line ceruloplasmin concentration in newborn blood spots of patients with Wilson disease. "
04/14/2006 - "Patients with an established diagnosis of Wilson disease but normal ceruloplasmin blood levels were chosen for our study (n = 14). "
03/23/1958 - "[Pure hepatic form of Wilson's disease in infant; successful therapy with chelating agents]."
06/01/1990 - "We conclude that long-term Zn treatment in Wilson's disease can be a safe and effective alternative to Cu chelating agents. "
01/01/1968 - "[Anatomo-clinical study of a case of Wilson's disease treated with chelating agents for 5 years]."
03/31/1959 - "[Clinical and biological study of a case of Wilson's disease in pure hepatic form in an infant; favorable effects of therapy with chelating agents]."
01/01/1959 - "[Clinical and biological study of Wilson's disease with pure hepatic form in an infant; successful results of therapy with chelating agents]."
06/01/2010 - "Therefore, the present study was carried out to elucidate iron metabolism in Atp7b(-/-) mice, an animal model of Wilson disease. "
11/01/2001 - "Four male patients with Wilson's disease were enrolled in this study of pre- and post-treatment iron metabolism. "
05/01/2000 - "Increased cerebral iron uptake in Wilson's disease: a 52Fe-citrate PET study."
05/01/2012 - "Wilson's disease: does iron metabolism impact phenotypic presentation?"
01/01/2012 - "Iron metabolism and the role of HFE gene polymorphisms in Wilson disease."
02/01/2003 - "The combined therapy with penicillamine and zinc salts was effective in treatment of patients with Wilson's disease."
02/01/2003 - "In this study, the effect of the therapy with combined penicillamine and zinc salts was evaluated based on the follow-up observations of 21 patients with Wilson's disease. "
02/01/2003 - "[Treatment of Wilson's disease with penicillamine and zinc salts: a follow-up study]."
09/01/2000 - "The group investigated comprised 45 individuals with Wilson's disease treated with penicillamine or zinc salts and a control group of 36 healthy individuals. "
10/01/2009 - "Treatments for Wilson disease include chelating agents and zinc salts and liver transplantation. "
12/01/1990 - "The only effective treatment for fulminant Wilson's disease is liver transplantation, which may lead to reversal of the underlying disease. "
03/01/2008 - "Liver transplantation is a safe and effective treatment for both acute and chronic presentations of Wilson's disease. "
01/01/1998 - "Although neuropsychiatric manifestations are prominent in some patients with Wilson disease, there is little published information regarding the efficacy of liver transplantation for these patients. "
11/01/1993 - "Several metabolic disorders that produce liver injury, such as Wilson's disease and alpha-1-antitrypsin deficiency, are cured by liver transplantation. "
04/01/1990 - "Improvement in chronic hepatocerebral degeneration following liver transplantation."
|2.||Transplantation (Transplant Recipients)
01/01/2011 - "Recent studies have demonstrated that normal bone marrow (BM) cells transplantation can correct liver injury in a mouse model of Wilson disease (WD). "
12/01/1996 - "This study investigated whether intrahepatic transplantation of normal hepatocytes can prevent Wilson's disease in LEC rats. "
05/01/2014 - "Current status of human hepatocyte transplantation and its potential for Wilson's disease."
12/01/2013 - "Transient liver elastography data showed some change from 65 kPa, 33 kPa, 34.8 kPa and undetectable to 46.4 kPa, 19.8 kPa, 29.1 kPa and 67.8 kPa at 4 weeks after transplantation in a patient with Wilson disease, a patient with HCV, and two patients with HBV. "
03/01/2012 - "There are many problems in transplantation for Wilson's disease. "
|3.||Chelation Therapy (Therapy, Chelation)
12/01/2007 - "Lifelong chelation therapy is essential in all Wilson's disease patients. "
03/01/1995 - "Psychosis in an adolescent patient with Wilson's disease: effects of chelation therapy."
12/01/1994 - "We report three young women with Wilson's disease; one had a fulminant form and was transplanted and the other two responded to chelation therapy. "
04/01/1983 - "The effect of chelation therapy on the amino aciduria and peptiduria of Wilson's disease."
04/01/2014 - "Most patients stabilized (35%) or improved on chelation therapy (26% fully recovered, 24% improved), but 15% deteriorated; 8% required a liver transplant, and 7.4% died within the observation period (71% of deaths were related to Wilson disease). "
|4.||Tissue Therapy (Cell Therapy)
05/01/2009 - "We concluded that pro-oxidant perturbations were uniquely effective for cell therapy in Wilson disease because of the nature of preexisting hepatic damage."
05/01/2014 - "Nonetheless, cell therapy studies in animal models elicited major differences in the mechanisms driving liver repopulation with transplanted hepatocytes in Wilson's disease versus nondiseased settings. "
05/01/2014 - "Recently, noninvasive imaging was developed to demonstrate Cu removal from the liver, including after cell therapy in Wilson's disease. "
05/01/2014 - "The ability to repopulate the liver with healthy hepatocytes raises the possibility of cell therapy in Wilson's disease. "
05/01/2014 - "To achieve permanent correction of Wilson's disease by a cell therapy approach, replacement of diseased hepatocytes with healthy hepatocytes is desirable. "
01/01/2009 - "Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange."
12/07/1999 - "Plasma exchange for hemolytic crisis in Wilson disease."
04/01/1992 - "Plasma exchange for fulminant Wilson disease."
07/01/2006 - "Successful plasma exchange treatment in hemolytic crisis of Wilson's disease preventing liver transplantation."
10/01/2003 - "Bridging use of plasma exchange and continuous hemodiafiltration before living donor liver transplantation in fulminant Wilson's disease."