Take the quiz
Question 1
Which organs are commonly affected by Fabry disease?
Question 2
What are symptoms of Fabry disease?
Question 3
What is a therapeutic goal for Fabry disease patients with cardiac involvement?
Question 4
Which substrate accumulates in Fabry disease?
Skin, liver and nervous system
Liver, splean and eyes
Heart, kidneys and nervous system
The gastrointestinal tract,
lungs and skin
lungs and skin
Correct!
The heart, kidneys and nervous system are the organs most commonly affected by Fabry disease.
Other frequently affected organs are the skin, eyes and gastrointestinal tract.
Incorrect!
The heart, kidneys and nervous system are the organs most commonly affected by Fabry disease.
Other frequently affected organs are the skin, eyes and gastrointestinal tract.
Next Question
Unexplained left ventricular
Hypertrophy,
Myocardial fibrosis, arrhythmias
Hypertrophy,
Myocardial fibrosis, arrhythmias
Thrombocytopenia,
Anemia, Leukopenia
Anemia, Leukopenia
Splenomegaly,
Hepatomegaly, Thrombocytopenia
Hepatomegaly, Thrombocytopenia
Proximal muscle weakness,
Scoliosis, Orthopnea
Scoliosis, Orthopnea
Correct!
In Fabry disease patients with cardiac involvement, the accumulation of the substrate leads to left ventricular hypertrophy, myocardial fibrosis, and consequently, arrhythmias.
Incorrect!
In Fabry disease patients with cardiac involvement, the accumulation of the substrate leads to left ventricular hypertrophy, myocardial fibrosis, and consequently, arrhythmias.
Next Question
Increase in blood pressure
Prevention of left ventricular hypertrophy (LVH) or its stabilization
Pacemaker implantation
Reduction of heart rate
Correct!
The therapeutic goal for Fabry disease patients with cardiac involvement should be the prevention or stabilization of left ventricular hypertrophy (LVH). If left untreated, this condition continues to progress.
Incorrect!
The therapeutic goal for Fabry disease patients with cardiac involvement should be the prevention or stabilization of left ventricular hypertrophy (LVH). If left untreated, this condition continues to progress. An increase in blood pressure and the necessity for implantation of a pacemaker should be avoided. Reducing heart rate is not a therapeutic goal, since Fabry disease patients typically suffer from bradycardia.
Next Question
GL-1
Glucose
Uric acid
GL-3
Correct!
GL-3 accumulates in Fabry disease patients because the enzyme α-galactosidase has no or only reduced activity. GL-3 can also be used as a biomarker in diagnostics.
Incorrect!
GL-3 accumulates in Fabry disease patients because the enzyme α-galactosidase has no or only reduced activity. GL-3 can also be used as a biomarker in diagnostics.
To the results
Congratulations!
You are now very close to becoming a Fabry disease expert.
You can find more information about Fabry disease, diagnosis and therapy here:
MAT-BE-2501046-v1.0-102025