Elevated plasma triglyceride levels are associated with human protoporphyria.

Six patients with protoporphyria had mildly elevated triglyceride levels (200 to 300 mg/dl) on serum chemistry screening panels. Measurement of fasting plasma lipid profiles indicated that triglyceride levels were mildly elevated in 22 patients with protoporphyria compared with the values of a age- and sex-matched population of the Lipid Research Clinics prevalence study (p = 0.021). The effect of ingestion of the retinoid precursor beta-carotene on plasma triglyceride levels was assessed in 13 of these patients, both during carotene therapy and during therapy-free intervals. There was no significant increase in plasma triglyceride levels during administration of carotene; eight of 13 patients had lower levels during therapy. There was no significant correlation between plasma triglyceride levels at p less than or equal to 0.05 and serum carotene or blood protoporphyrin levels. Our results indicate that mild hypertriglyceridemia occurs with increased frequency in patients with protoporphyria, but not as a direct result of beta-carotene therapy.