scholarly journals The pattern of urinary copper excretion and its response to treatment in patients with Wilson's disease

QJM ◽  
2011 ◽  
Vol 104 (9) ◽  
pp. 775-778 ◽  
Author(s):  
J. M. Walshe
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Response To Treatment ◽  
Copper Excretion ◽  
Urinary Copper ◽  
Urinary Copper Excretion

scholarly journals INITIAL ASSESSMENT FINDINGS IN PATIENTS WITH CONFIRMED WILSON’S DISEASE

2021 ◽  
Vol 5 (2) ◽  
pp. 161-167
Author(s):  
O. A. Zhigaltsova-Kuchinskaya ◽  
◽  
N. N. Silivontchik ◽  
S. A. Likhachev ◽  
I. V. Pleshko ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Molecular Genetic ◽  
Wilson's Disease ◽  
Clinical Suspicion ◽  
Initial Assessment ◽  
Serum Free ◽  
Copper Excretion ◽  
Serum Ceruloplasmin ◽  
Urinary Copper ◽  
Urinary Copper Excretion

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.

Usefulness of penicillamine-stimulated urinary copper excretion in the diagnosis of adult Wilson's disease

2008 ◽  
Vol 43 (5) ◽  
pp. 597-603 ◽  
Author(s):  
José Ramón Foruny ◽  
Daniel Boixeda ◽  
Antonio López-Sanroman ◽  
Enrique Vázquez-Sequeiros ◽  
Mónica Villafruela ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Copper Excretion ◽  
Urinary Copper ◽  
Urinary Copper Excretion

scholarly journals Value of Kayser-Fleischer ring as a diagnostic tool for Wilson’s disease in children

2021 ◽  
Vol 3 (Number 1) ◽  
pp. 20-23
Author(s):  
Sadika Kadir ◽  
S M Bazlul Karim ◽  
Mohammed Ashraful Haque ◽  
Rafiqul Islam ◽  
Asif Imran ◽  
...  
Keyword(s):  
Liver Disease ◽  
Diagnostic Tool ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Female Ratio ◽  
Copper Excretion ◽  
Group I ◽  
Urinary Copper ◽  
F Ring ◽  
Urinary Copper Excretion

The Kayser-Fleischer(K-F) ring is the hallmark of Wilson’s disease (WD). In most adults or older children, the diagnosis of Wilson’s disease may be made easily when K-Frings and low ceruloplasmin levels are present. In this study presence of K-F ring has been evaluated among children with liver disease in Bangladesh to improve the management of Chronic liver disease due to WD and reduce complications. This cross-sectional study was carried out at the Department of Paediatric Gastroenterology and Nutrition, BSMMU, Dhaka on 60 children presented with liver disease. Thirty children over three years of age considered as cases (Group-I) and thirty children with non- Wilsonian liver disease as control (Group-II). Slit lamp examination for K-F ring and twenty-four hour urinary copper excretion after giving one gram d-penicillamine 12-hour apart were done in each patient. The efficacy of K-F ring was studied. Mean age of WD patients was 8.9± 2.78 years, with a male female ratio of 1.3: 1. There was significant low level of serum ceruloplasmin in 93.33% of cases (p<.001). After penicillamine challenge, 24-hour urinary copper excretion was found significantly higher in patients with WD (median 3626.5±1698 μg/24h, range 1262- 195000) than non-Wilsonian liver disease (median 450±278.09 μg/24-h, range 47- 2062 μg/24h), (p<.001). K-F ring was found in 15 (50%) patients, absent in all patients of non-Wilsonian liver disease group and the difference was statistically significant (p<.001). Evaluation of Kayser-Fleischer ring is still a very essential diagnostic tool and is a non-invasive, affordable way to assist in the diagnosis of a potentially fatal disease.

scholarly journals Appropriate Administration Schedule of D-Penicillamine for Pediatric Wilson's Disease Patients Based on Urinary Copper Excretion

2002 ◽  
Vol 122 (8) ◽  
pp. 585-588
Author(s):  
Noriyasu FUKUOKA ◽  
Shushi MORITA ◽  
Shizuka HAMATANI ◽  
Hitoshi OKADA ◽  
Mayumi KONDOH ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Administration Schedule ◽  
Copper Excretion ◽  
Urinary Copper ◽  
Urinary Copper Excretion

Urinary copper excretion before and after oral intake of d-penicillamine in parents of patients with Wilson's disease

2012 ◽  
Vol 44 (4) ◽  
pp. 323-327 ◽  
Author(s):  
Jakeliny Vieira ◽  
Pedro Vitoriano Oliveira ◽  
Yara Juliano ◽  
Karim Repsold Jorge Warde ◽  
Marta Mitiko Deguti ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Oral Intake ◽  
Wilson's Disease ◽  
Copper Excretion ◽  
Urinary Copper ◽  
Before And After ◽  
Urinary Copper Excretion

Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease

Hepatology ◽  
1992 ◽  
Vol 15 (4) ◽  
pp. 609-615 ◽  
Author(s):  
Claudia Martins Da Costa ◽  
Dianne Baldwin ◽  
Bernard Portmann ◽  
Yvette Lolin ◽  
Alex P. Mowat ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Copper Excretion ◽  
Urinary Copper ◽  
Urinary Copper Excretion

scholarly journals Therapeutic Response of Wilson’s disease to D-Penicillamine in Paediatric Population: A one year follow-up study

1970 ◽  
Vol 18 (1) ◽  
pp. 37-43
Author(s):  
NC Saha ◽  
A Sultana ◽  
MAH Mollah ◽  
T Begum ◽  
AKMM Rahman ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Paediatric Population ◽  
Wilson's Disease ◽  
Response To Treatment ◽  
College Hospital ◽  
Medical College ◽  
Urinary Copper ◽  
F Ring ◽  
One Year

Objectives: The objective of this study was to observe the outcome of patients treated with penicillamine. Design: Intervention type of study Setting: Department of Paediatrics, Dhaka Medical College Hospital Study period: January 2007 to December 2008. Study subjects: Sixteen diagnosed cases of Wilson's disease as per inclusion criteria. Intervention: D-penicillamine was started in a low dose, which was titrated gradually. The clinical and biochemical parameters were evaluated to look for the response to treatment. Results: A total of 16 cases were included. Among them 12 were male and 4 were female. The mean (± SD) of age of the patients was 10 (± 2.34) years. Consanguinity between parents was present in 44% (n=7). The hepatic and neurological variety of WD were 56 % (n=9) and 44% (n=7) respectively. The K-F ring was present in 75% (n=12/16) of WD cases. The excretion of 24 hrs urinary copper was steadily increased from discharge till second follow-up in response with increasing dose of penicillamine, thereafter the value was declining gradually till final follow-up at 1 year. Regarding outcome, 7 patients improved of which 4 were in hepatic and 3 in neurological group, 3 of hepatic WD expired and 2 developed neurological manifestations. One patients developed proteinuria while penicillamine treatment .About half of patients with WD were improved. Adequate cupriuresis occurred at three months. All the symptoms and biochemical markers WD improved gradually. No significant side effect was seen. Key words: Wilson's disease; penicillamine; urinary copper. DOI: 10.3329/jdmc.v18i1.6304 J Dhaka Med Coll. 2009; 18(1) : 37-43

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