PA76 CORRELATION BETWEEN URINARY COPPER EXCRETION AND LIVER COPPER CONTENT WITH AGE IN CHILDREN WITH WILSON DISEASE

2010 ◽  
Vol 42 ◽  
pp. S373
Author(s):  
G. Ranucci ◽  
E. Nicastro ◽  
C. Della Corte ◽  
M. Tufano ◽  
P. Vajro ◽  
...  
2019 ◽  
Vol 42 (2) ◽  
pp. 371-380 ◽  
Author(s):  
Jan Pfeiffenberger ◽  
Christine Marie Lohse ◽  
Daniel Gotthardt ◽  
Christian Rupp ◽  
Markus Weiler ◽  
...  

Author(s):  
C.J. Botha ◽  
A.S. Shakespeare ◽  
R. Gehring ◽  
D. Van der Merwe

The efficacy of a molybdate formulation and a zinc oxide bolus as prophylactic agents for enzootic icterus was evaluated in sheep. Before copper loading, liver biopsies were performed on 12 male, 6-month-old, Mutton Merino sheep to determine hepatic copper (Cu) and zinc (Zn) concentrations. The animals were restrictively randomised according to liver copper concentrations to 3 treatment groups (n = 4) to achieve similar mean liver copper concentrations per group. All sheep received 4 m /kg of a 0.5 %aqueous solution of CuSO4·5H2O intraruminally 7 days per week for 10 weeks. On Day 0 the sheep in the Mo-group were injected subcutaneously with 42 mg molybdenum (Mo) contained in a commercial molybdate formulation. The animals in the Zn-group each received a zinc oxide bolus, containing 43 g zinc oxide, via a rumen cannula. Treatment was repeated on Day 42. Four animals served as untreated controls. Urinary copper excretion, plasma copper concentration, haematocrit and glutamate dehydrogenase (GLDH) activity were determined throughout the trial. The animals were sacrificed after 10 weeks and liver samples were submitted for histopathological examination. Liver and kidney copper and zinc concentrations were determined. Neither the molybdate treatment nor the zinc oxide boluses prevented hepatic copper accumulation. The urinary copper excretion, plasma copper concentration, haematocrit and GLDH activity were not significantly different (P > 0.05) from the controls.


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.


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