Understanding Hepatic vs. Neuro Wilson Disease
Explore the differences between Hepatic and Neuro Wilson Disease through clinical observations and the impact of copper overload syndromes on liver and brain functions. Discover the pathophysiology and treatment options for these conditions.
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Presentation Transcript
Hepatic and Neuro Wilson disease Is there a difference? Talk: John Matthai Chairpersons: Fazal Nabi, Rukmini Mridula
Hepatic and Neuro Wilson disease Is there a difference?
Pathophysiology of hepatic vs. neuro. involvement of WD : not well understood
Some clinical observations : clues to pathophysiology 1. Do all copper overload syndromes result in hepatic and in neurological disease ?
Copper overload syndromes Organs affected Reference Wilson s disease Liver / Brain / both
Copper overload syndromes Organs affected Reference Wilson s disease Indian Childhood cirrhosis Liver / Brain / both Patra et al. Indian J Pediatr 2013 Aug Liver Ramakrishna B et al. Ann Trop Pediatr Sep 1995 Atypical copper cirrhosis Liver Muller et al. Lancet 1996 Mar Endemic Tyrrolian cirrhosis Liver in chronic cholestasis Liver
Copper overload syndromes Organs affected Reference Wilson s disease Indian Childhood cirrhosis Liver / Brain / both Patra et al. Indian J Pediatr 2013 Aug Liver Ramakrishna B et al. Ann Trop Pediatr Sep 1995 Atypical copper cirrhosis Liver Muller et al. Lancet 1996 Mar Endemic Tyrrolian cirrhosis Liver in chronic cholestasis Liver
WD - clinical observations : clues to pathophysiology 1. Do all copper overload syndromes result in hepatic and in neurological disease ? 2. In an individual WD patient, can neurological disease occur without hepatic disease ?
WD - clinical observations : clues to pathophysiology 1. Do all copper overload syndromes result in hepatic and in neurological disease ? 2. In an individual WD patient, can neurological disease occur without hepatic disease ? 3. Does copper chelating treatment improve hepatic and neurological involvement ?
WD - clinical observations : clues to pathophysiology 1. Do all copper overload syndromes result in hepatic and in neurological disease ? 2. In an individual WD patient, can neurological disease occur without hepatic disease ? 3. Does copper chelating treatment improve hepatic and neurological involvement ? 4. Why are basal ganglia the preferred site for copper deposition in the brain ?
WD - clinical observations : clues to pathophysiology 1. Do all copper overload syndromes result in hepatic and in neurological disease ? 2. In an individual WD patient, can neurological disease occur without hepatic disease ? 3. Does copper chelating treatment improve hepatic and neurological involvement ? 4. Why are basal ganglia the preferred site for copper deposition in the brain ? 5. Worsening of neurological signs in a few patients after starting Penicillamine
