Scheie-hurler disease E76.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Hereditary mucopolysaccharidosis. Variant of Pfaundler-Hurler's disease and Scheie's disease with clinically significantly milder course, later manifestation and slower progression than in Pfaundler-Hurler disease.

Manifestation
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Mostly three-nine. LJ.

Progression/forecast
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Mostly survival into the 2nd or 3rd decade.

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Lysosomal storage diseases;

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Last updated on: 29.10.2020