Long Term Urinary Copper Excretion on Chelation Therapy in Children with Wilson Disease

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Atchariya Chanpong ◽  
Anil Dhawan
2019 ◽  
Vol 42 (2) ◽  
pp. 371-380 ◽  
Author(s):  
Jan Pfeiffenberger ◽  
Christine Marie Lohse ◽  
Daniel Gotthardt ◽  
Christian Rupp ◽  
Markus Weiler ◽  
...  

2010 ◽  
Vol 42 ◽  
pp. S373
Author(s):  
G. Ranucci ◽  
E. Nicastro ◽  
C. Della Corte ◽  
M. Tufano ◽  
P. Vajro ◽  
...  

1986 ◽  
Vol 44 (6) ◽  
pp. 899-906 ◽  
Author(s):  
M G Boosalis ◽  
J T McCall ◽  
L D Solem ◽  
D H Ahrenholz ◽  
C J McClain

The Lancet ◽  
1972 ◽  
Vol 300 (7786) ◽  
pp. 1090
Author(s):  
JohnK Vyden ◽  
DavidH Curnow ◽  
AlanB Beck ◽  
CliveA.P Boundy

2021 ◽  
Vol 5 (2) ◽  
pp. 161-167
Author(s):  
O. A. Zhigaltsova-Kuchinskaya ◽  
◽  
N. N. Silivontchik ◽  
S. A. Likhachev ◽  
I. V. Pleshko ◽  
...  

Bacground. The optimization of Wilson’s disease (WD) diagnosis is one of the most disputable problem. Objective. The retrospective study of initial assessment findings under clinical suspicion for WD in 102 patients with the confirmed diagnosis. Material and methods. The results of laboratory tests and Kaiser-Fleischer rings (KF rings) identification under clinical suspicion for WD in 102 patients with the confirmed diagnosis. Results. At stage I, 17 patients (16.7%; 95% CI 10.7–25.1) were defined as having clinically definitive WD based on the combination of low serum ceruloplasmin and KF rings, 4 patients (3.9%; 95% CI 1.5–9.7) – based on the drop of ceruloplasmin level. After stage II, involving 24-hour urinary copper excretion evaluation, the rate of definitive diagnosis of WD reached 24,5% (95% CI 17.2 33.7). After stage III (genotyping for carriage of ATP7B gene mutations) – 56.9% (95% CI 47.2–66.0). Serum free copper increase was found in 54.9% (95% CI 41.4 67.7) of cases. Conclusions. Under clinical suspicion for WD, initial structured ophthalmological, laboratory and molecular-genetic assessment ensured the diagnosis of WD only in 56.9% (95% CI 56.9; 47.2–66.1). Frequent detection of serum free copper increase (54.9%, 95% CI 41.4 67.7) allows to use this test due to its greater availability as compared with 24-hour urinary copper excretion evaluation in WD diagnostics.


1975 ◽  
Vol 34 (4) ◽  
pp. 340-345 ◽  
Author(s):  
W McMurray ◽  
V M Martin ◽  
P Scudder ◽  
J Stocks ◽  
A G White ◽  
...  

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