What domains does the Gaucher SSI evaluate?

The SSI scores bone disease (pain, avascular necrosis, fractures), visceral involvement (hepatosplenomegaly), and hematological parameters (hemoglobin, platelet count) to produce a composite severity score from 0–30.

When is enzyme replacement therapy started in Gaucher disease?

ERT (imiglucerase, velaglucerase) is indicated for symptomatic Type 1 Gaucher disease, particularly in patients with significant cytopenias, visceral enlargement, or bone disease. SSI scores ≥9 generally indicate treatment.

Gaucher Disease Severity Index (SSI)

Calculate the Severity Score Index (SSI) for Gaucher disease based on bone, visceral, and hematological involvement to guide treatment decisions.

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Frequently Asked Questions

What domains does the Gaucher SSI evaluate?

When is enzyme replacement therapy started in Gaucher disease?