scholarly journals Cardiac arrhythmia in Wilson’s disease: An oversighted and overlooked entity!

2016 ◽  
Vol 7 (04) ◽  
pp. 587-589 ◽  
Author(s):  
Bhupender Kumar Bajaj ◽  
Ankur Wadhwa ◽  
Richa Singh ◽  
Saurabh Gupta

ABSTRACTWilson’s disease is a multisystem disorder which manifests with hepatic, neurological, musculoskeletal, hematological, renal, and cardiac symptoms. The hepatic and neurological manifestations often overshadow the other system involvement including cardiac symptoms and signs, which may prove fatal. We report a case of a young female who presented with progressive parkinsonian features and dystonia for around 4 months followed 2 months later by the complaint of episodes of light-headedness. She was diagnosed to have Wilson’s disease based on the presence of Kayser–Fleischer ring and laboratory parameters of copper metabolism. Electrocardiography of the patient incidentally revealed 2nd degree Mobitz type-1 atrioventricular block explaining her episodes of light-headedness. She was started on penicillamine and trihexyphenidyl. The heart block improved spontaneously. Cardiac autonomic function tests including blood pressure response to standing and heart rate response to standing were observed to be normal. We review the literature on cardiac manifestations of Wilson’s disease and emphasize that patients with Wilson’s disease should be assessed for cardiac arrhythmia and cardiac dysfunction as these may have therapeutic and prognostic implications.

2021 ◽  
pp. 152692482110028
Author(s):  
Alberto Ferrarese ◽  
Patrizia Burra

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.


Hepatology ◽  
1986 ◽  
Vol 6 (3) ◽  
pp. 427-432 ◽  
Author(s):  
Surjit K. S. Srai ◽  
Andrew K. Burroughs ◽  
Bernard Wood ◽  
Owen Epstein

1991 ◽  
Vol 83 (6) ◽  
pp. 364-366 ◽  
Author(s):  
R. Siegemund ◽  
J. Lößner ◽  
K. Günther ◽  
H.-J. Kühn ◽  
H. Bachmann

2006 ◽  
Vol 349 (3) ◽  
pp. 1079-1086 ◽  
Author(s):  
Noriko Fujiwara ◽  
Hiroyuki Iso ◽  
Nobue Kitanaka ◽  
Junichi Kitanaka ◽  
Hironobu Eguchi ◽  
...  

Author(s):  
Vrinda Vijayakumari ◽  
Kaliyannan Mayilananthi ◽  
Durga Krishnan ◽  
Ramprasath Anbazhagan ◽  
Gaurav Narayanan

Wilson’s disease is one the rare autosomal recessive disorders of copper metabolism due to mutation in ATP7B gene located in chromosome 13. The mutations of this gene cause accumulation of copper in different tissues such as brain, liver, and eyes. The clinical presentation usually reflects this tissue distribution and varies from asymptomatic patients to those with hepatic or neuro-psychiatric manifestations. Here, we report an interesting case of Wilson’s disease which presented with mild persistent hemolysis leading to pre hepatic and post hepatic jaundice. He also had hepatocellular jaundice due to liver injury.


1961 ◽  
Vol 40 (3) ◽  
pp. 445-453 ◽  
Author(s):  
James L. German ◽  
Alexander G. Bearn

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