WILSON'S DISEASE
Third Orphan disease symposium
Bruxelles- UCL St Luc

Genetics and diagnostics: possibilities and limitations

28/11/2003

Peter Ferenci, Wien , Austria
peter.ferenci@akh-wien.ac.at

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Table des matières

Genetics and diagnostics: possibilities and limitations

Interpretation of molecular genetic test results

Diapositive PPT

Diapositive PPT

Consequence of gene mutations

Consequence of gene mutations

proteinomics

Diapositive PPT

ATP7B (Wilson´s disease gene-product)

Diapositive PPT

Methods

Genotype - phenotype study in WD patients - country of origin

phenotypic classification

Diagnosis of Wilson´s disease

Kayser-Fleischer rings at diagnosis of Wilson disease

Diapositive PPT

Hepatic copper content for diagnosis of Wilson disease

Liver copper as diagnostic test for Wilson disease

mutation detection strategy

mutations detected

H1069Q homozygotes and compound heterozygotes

H1069Q mutation in Wilson disease

other homozygotes

Distribution of WD mutations according to the clinical presentation

Distribution of WD mutations according to the clinical presentation

Clinical presentation in pts. with common mutations

Distribution of WD mutations according to the age at onset of symptoms

Distribution of WD mutations according to the age at onset of symptoms

Distribution of WD mutations according to the age at onset of neurologic symptoms

Distribution of WD mutations according to the age at onset of neurologic symptoms

Distribution of WD mutations according to the age at onset of hepatic symptoms

Diapositive PPT

Summary

Mutation analysis for diagnosis of Wilson disease

Mutation analysis for diagnosis of WD

Diapositive PPT

Factors modulating copper toxicity in Wilson disease

Summary

Info orphan symposia: sokal@pedi.ucl.ac.be

Page d'accueil: http://www.pediatrie.be