In patients aged 10 years and older with Glycogen Storage Disease Type I, elevated triglycerides and cholesterol above normal ranges may persist despite appropriate dietary treatment, warranting pharmacological intervention.
Elevated triglycerides and cholesterol above the normal ranges may persist in some patients with GSD I despite appropriate dietary treatment. Management of hyperlipidemia with medications is generally considered in patients who are at least 10 years old.
The protocol involves targeted lipid-lowering drug therapy. The specific agents indicated and their sequencing are detailed in the full evidence-based regimen.
Maintain serum lipid levels as close to normal as possible.
DOI: 10.1038/gim.2014.128
Management of hyperlipidemia with medications usually does not begin until the patient is at least 10 years old.
Elevated triglycerides and cholesterol above the normal ranges may persist in some patients with GSD I, despite appropriate dietary treatment.
Hyperlipidemia in GSD Ia can be managed with lipid-lowering drugs such as 3-hydroxy-3-methyl-glutaryl-CoA reductase inhibitors and fibrates.
Good metabolic control is the best management option for maintaining serum lipid levels as close to normal as possible, thereby reducing the risk of acute pancreatitis and long-term development of atherosclerosis.
View source ↗