Glycogen storage disease type I
ICD-10 E74.0 · ICD-11 5C51.3.6

Treatment of Glycogen Storage Disease Type I with Gout and Hyperuricemia

In patients with Glycogen Storage Disease Type I, elevated uric acid is a recognised complication that can progress to hyperuricemia and recurrent gout attacks. Addressing this complication requires both dietary and pharmacological management.

Clinical Scenario

GSD Type I patients with a tendency toward gout attacks present with hyperuricemia as a compounding metabolic burden. Dietary modification — specifically a low-purine diet — is a cornerstone alongside targeted urate-lowering therapy.

Treatment Approach (Partial Overview)

The protocol incorporates urate-lowering therapy directed at reducing serum uric acid levels. When conventional approaches are insufficient, a newer class of urate-lowering agent is considered. The complete sequencing, criteria, and regimen details are in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1038/gim.2014.128

For those with a tendency toward gout attacks, a low-purine diet is prescribed in addition to allopurinol.

Newer agents, such as pegloticase, have been used in situations where the use of other agents has failed.

View source ↗