A fit-for-purpose copper speciation method for the determination of exchangeable copper relevant to Wilson’s disease

2021 ◽  
Author(s):  
M. Estela del Castillo Busto ◽  
Susana Cuello-Nunez ◽  
Christian Ward-Deitrich ◽  
Tim Morley ◽  
Heidi Goenaga-Infante
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Copper Speciation ◽  
Fit For Purpose

THE LIVER IN JUVENILE WILSON'S DISEASE

PEDIATRICS ◽  
1962 ◽  
Vol 30 (3) ◽  
pp. 402-413 ◽  
Author(s):  
Mervin Silverberg ◽  
Sydney S. Gellis
Keyword(s):  
Wilson’S Disease ◽  
Punch Biopsy ◽  
Wilson's Disease ◽  
Careful Examination ◽  
Unknown Etiology ◽  
Surgical Biopsy ◽  
Rubeanic Acid ◽  
Reliable Methods ◽  
Determination Of Copper

Twelve cases of juvenile Wilson's disease are reviewed. Eight presented with hepatic manifestations, and six of these showed a predominance of liver symptomatology throughout their entire illness. It appears that this type of onset is not uncommon when the disease begins in the pre-adolescent period. In childhood, chronic liver disease of unknown etiology should always be screened for Wilson's disease. Careful examination for Kayser-Fleischer rings should repeatedly be made. The finding of cupruria, aminoaciduria without glycosuria or albuminuria, and decreased blood uric acid levels are strongly suggestive of Wilson's disease. Punch biopsy of the liver with visualization of copper by means of an improved modification of the histo-chemical rubeanic acid method or Howell's newer histo-chemical method, or determination of copper content as well as histochemical analysis of liver obtained by surgical biopsy appear to be the only reliable methods at present for proof of diagnosis. [See Table III in Source Pdf.]

Direct determination of Cu isotope ratios in dried urine spots by means of fs-LA-MC-ICPMS. Potential to diagnose Wilson's disease

2013 ◽  
Vol 28 (1) ◽  
pp. 98-106 ◽  
Author(s):  
Martín Resano ◽  
Maite Aramendía ◽  
Luis Rello ◽  
Mª Luisa Calvo ◽  
Sylvain Bérail ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Direct Determination ◽  
Isotope Ratios ◽  
Wilson's Disease ◽  
Cu Isotope

scholarly journals Formation constants of copper(i) complexes with cysteine, penicillamine and glutathione: implications for copper speciation in the human eye

2015 ◽  
Vol 44 (47) ◽  
pp. 20413-20425 ◽  
Author(s):  
Lan-Chi Königsberger ◽  
Erich Königsberger ◽  
Glenn Hefter ◽  
Peter M. May
Keyword(s):  
Wilson’S Disease ◽  
Formation Constants ◽  
Wilson's Disease ◽  
Copper Deposition ◽  
Human Eye ◽  
Copper Speciation ◽  
Speciation Modelling ◽  
Redox Equilibria

Speciation modelling incorporating redox equilibria provides a plausible explanation for copper deposition in the human eye associated with Wilson's disease.

Determination of the serum metallothionein (MT)1/2 concentration in patients with Wilson's disease and Menkes disease

2014 ◽  
Vol 28 (4) ◽  
pp. 441-447 ◽  
Author(s):  
Kyoumi Nakazato ◽  
Satoru Tomioka ◽  
Katsuyuki Nakajima ◽  
Hidetoshi Saito ◽  
Mihoko Kato ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Menkes Disease ◽  
Wilson's Disease

Determination of trace elements in hair of Wilson's disease patients using PIXE

1987 ◽  
Vol 22 (1-3) ◽  
pp. 191-192 ◽  
Author(s):  
Sha Yin ◽  
Liu Pingsheng ◽  
Zhang Runhua ◽  
Liu Guilin ◽  
Zhang Zhengxin ◽  
...  
Keyword(s):  
Trace Elements ◽  
Wilson’S Disease ◽  
Wilson's Disease
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