Abstract
The very low post-heparin lipase activity (PHLA) and the hypertriglyceridemia of familial hyperchylomicronemia have previously been found unresponsive to drug therapy. In this study, 2 siblings with the disease were treated with oxandrolone 7.5 mg daily during the last week of a 2-week hospital admission. PHLA was normalized during treatment, but serum triglycerides were unaffected. We suggest that the increase of PHLA does not signify a correction of the deficiency in the chylomicron-clearing mechanism of these patients.