PracticeUpdate Conference Series - SSIEM 2018

" …notably,

non-HDL cholesterol, triglyceride/HDL cholesterol ratio, non-HDL cholesterol/ HDL cholesterol ratio, and plasma leptin are reliable markers of insulin resistance and metabolic syndrome. "

Notably, non-HDL cholesterol, triglyc- eride/HDL cholesterol ratio, non-HDL cholesterol/HDL cholesterol ratio, and plasma leptin are reliable markers of insu- lin resistance and metabolic syndrome. GSDI, or von Gierke disease, is the most common of the glycogen storage diseases. This genetic disease results from a deficiency of the enzyme glucose- 6-phosphatase and has a US incidence of approximately one in 50,000 to 100,000 births. The most common forms of GSDI are designated GSDIa and GSDIb, the former accounting for over 80% of diagnosed cases and the latter for <20%. A few rarer forms have been described. The metabolic characteristics of GSDIa and Ib are similar. The deficiency impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis.

Because these are the two principal metabolic mechanisms by which the liver supplies glucose to the rest of the body during periods of fasting, it causes severe hypoglycemia and results in increased glycogen storage in liver and kidneys. Both organs function normally in childhood but are susceptible to a variety of problems in adult years. Other metabolic derangements include lactic acidosis and hyperlipidemia. Frequent or continuous feedings of cornstarch or other carbohydrates are the principal treatment. Other therapeutic measures may be needed for associated problems. Dr. Rossi concluded that lipid markers are employed to assess metabolic risk in the general population. The present findings suggest that non-HDL cholesterol, triglyceride/HDL cholesterol

ratio, and non-HDL cholesterol/HDL cholesterol ratio may be useful tools for early detection of the risk of insulin resistance and metabolic syndrome in patients with GSDIa. Correlation data in GSDIa, together with normal lipid markers in patients with GSDIb, support the hypothesis that chronic hyperlipidemia and adipose tissue dysfunction may play a role in insulin resistance and metabolic syndrome in patients with GSDIa. “The results suggested that lipid markers might be helpful to detect risk for insulin resistance early in patients with GSD1a,” Dr. Rossi noted. “Further research should dissect the link between hyperlipidemia and insulin resistance,” he added.

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SSIEM 2018 • PRACTICEUPDATE CONFERENCE SERIES