scholarly journals Wilson’s Disease in an Elderly Patient

1995 ◽  
Vol 9 (2) ◽  
pp. 78-80 ◽  
Author(s):  
Maziar Badii ◽  
Henry Wong ◽  
Urs P Steinbrecher ◽  
Hugh J Freeman

A 65-year-old man with Fanconi’s syndrome was investigated for the cause of chronic liver disease. Wilson’s disease was diagnosed based on the detection of bilateral Kayser-Fleischer rings, a low serum ceruloplasmin level, increased urine copper excretion and positive histochemical stains of his liver for copper. This case is unusual because of the patient’s elderly age at the time of diagnosis and the absence of neurological changes due to Wilson’s disease in spite of advanced hepatic disease and the presence of Kayser-Fleischer rings. Even in the elderly patient, Wilson’s disease should be considered a possible cause of chronic liver disease.

PEDIATRICS ◽  
1963 ◽  
Vol 31 (1) ◽  
pp. 160-161
Author(s):  
I. HERBERT SCHINBERG

The point made in the article by Silverberg and Gellis (Pediatrics, 30:402, 1962) is excellent and important: "In childhood, chronic liver disease of unknown etiology should always be screened for Wilson's disease." But why wait until chronic liver disease has developed? The diagnosis can be made while the patient is still asymptomatic as the authors' own case material demonstrates. E.M. was examined carefully in 1952 at 8 years of age, when she was asymptomatic, because her sister M.M., then 15 years old, had Wilson's disease.


2014 ◽  
Vol 38 (2) ◽  
pp. 86-93 ◽  
Author(s):  
Farhana Bayes ◽  
ASM Bazlul Karim ◽  
Laila Helaly ◽  
Fahmida Bayes ◽  
Md Rukunuzzaman ◽  
...  

Background: The incidence of Wilson’s disease (WD) is increasing day by day in every ethnic group worldwide. WD has been found as a common cause of chronic liver disease in children. This study was undertaken to find out the occurrence and different types of hepatic presentation of Wilson’s disease in children admitted with liver diseases at a tertiary care centre of Bangladesh. Methodology: This cross sectional descriptive study was carried out at the department of Paediatric Gastroenterology and Nutrition, BSMMU during the period from March 2008 through April 2010. A total number of 71 children of both sexes aged 3-15 years, who had the features of liver disease (jaundice with or without hepatomegaly / splenomegaly and / or raised serum ALT), were enrolled in this study. For the purpose of the study, the diagnosis of WD was made by the presence of any 2 of the 3 features: presence of K-F ring by slit lamp examination, low serum ceruloplasmin level (<20 mg/dL) and urinary copper excretion of >1600 ?gm /24 hours after penicillamine challenge. Results: Wilson’s disease was found in 31 (43.7%) of 71 children. Among them chronic liver diseases were 18 (58%), acute hepatitis 6 (19.4%), acute liver failure 6 (19.4%) and asymptomatic WD case was 1 (3.2%). The mean age ±SD of WD cases at presentation was 9.87±2.6 years and 22 (70%) cases were male. Maximum numbers of WD cases were found in children below 10 year of age. The two common presenting features of WD cases were jaundice 28 (90.3%) and ascitis (58.1%). Other features were K-F ring, 25 (80.6%) and hepatomegaly, 24 (77.4%). Biochemical findings showed low serum ceruloplasmin level (done in 20 patients) in 20 cases and 24 hours urinary copper excretion of >1600 mg/day in 23 cases. About one third of children presented with liver diseases were diagnosed as Wilson’s disease and about 50% of WD cases presented with chronic liver diseases. Conclusion: Wilson’s disease is a common cause of chronic liver disease. Penicillamine challenge is a reliable diagnostic test for Wilson’s disease. DOI: http://dx.doi.org/10.3329/bjch.v38i2.21142 Bangladesh J Child Health 2014; VOL 38 (2) : 79-85


2017 ◽  
Vol 8 (4) ◽  
pp. 285-286 ◽  
Author(s):  
R.G. Manappallil ◽  
U. Karadan ◽  
P. Kumar ◽  
M.C. Sabir ◽  
R.N. Supreeth

1987 ◽  
Vol 15 (5) ◽  
pp. 276-281 ◽  
Author(s):  
M. Zuin ◽  
P. M. Battezzati ◽  
M. Camisasca ◽  
D. Riebenfeld ◽  
M. Podda

A preparation containing a standardized ginseng extract which has been shown to exert anti-hepatotoxic activity in vitro, combined with trace elements and multi-vitamins was compared to placebo in 24 elderly out-patients with toxin-induced (alcohol and drugs) chronic liver disease in order to evaluate its effect on liver function. Each patient was blindly treated either with the preparation containing ginseng extract or placebo for 12 weeks. The preparation containing ginseng extract significantly modified bromsulphthalein retention and blood zinc levels when compared to pre-treatment levels and to placebo. Serum bile acids, and γ-glutamyl transpeptidase before and after a fatty meal were significantly reduced after treatment with the test preparation and not with placebo. When the two treatment groups were compared, however, no significant difference in these parameters was observed. These results suggest that treatment with the preparation containing ginseng extract could improve the detoxifying activity of the liver in elderly patients with toxin-induced chronic liver disease.


F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 608
Author(s):  
Sudeep Adhikari ◽  
Prashant Kumar Shah ◽  
Yuba Raj Sharma

We present a case of a 36-year-old female patient who presented with subacute liver disease with a history of alcohol abuse. On basic liver function tests (LFT), she had aspartate transaminase / alanine transaminase > 2.2 and alkaline phosphatase / total bilirubin < 4. This pattern in acute liver failure patients signifies Wilson’s disease. Its presence in our patient with subacute liver disease also prompted us to suspect Wilson’s disease and we extended the liver disease screen to include slit lamp eye examination for Kayser-Fleischer rings, serum ceruloplasmin and 24-hour urinary copper level, which led to the diagnosis. She improved clinically and biochemically with zinc acetate therapy.  As screening for rare diseases is not always possible in low-income countries, this case demonstrates the usefulness of the basic LFT as a guide for suspecting Wilson’s disease in patients with liver disease.


2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Nebiyu Bekele ◽  
Frew Ewnetu ◽  
Tigest Hailu ◽  
Zerubabel Tegegne ◽  
Abilo Tadesse

Background. Wilson’s disease is an inherited autosomal recessive disorder of copper metabolism. Clinical signs, biochemical parameters, histologic findings, and/or ATP7B genetic testing are required to diagnose Wilson’s disease. Case Presentation. 25-year-old and 22-year-old young women (siblings) presented to the University of Gondar Hospital, Northwest Ethiopia, with difficulty of keeping balance of 3-year duration and progressive extremity weakness of 5-year duration, respectively. Both siblings had visible ocular Kayser–Fleischer rings, low serum ceruloplasmin level and increased urinary copper content, ultrasound-evidenced cirrhotic liver disease, and axial T2-weighted MRI hyperintensities in basal ganglia, thalamus, and brainstem (midbrain and pons). Diagnosis of Wilson’s disease was established in both patients using a diagnostic scoring system proposed by “8th International Meeting on Wilson Disease and Menkes Disease, Leipzig (2001).” Treatment with D-penicillamine as a chelator and zinc sulphate as a metalothionein-inductor was started. Screening of their family members was recommended. Conclusion. Wilson’s disease, declared to be an orphan disease, requires clinical acumen of physicians and expensive investigation modalities for prompt recognition and is inaccessible as required, lifelong drugs for treatment.


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