June 1984

Primary Hypertriglyceridemia

Author Affiliations

H. G. H. McMaster Lipid Clinic Hamilton General Hospital 237 Barton St E Hamilton, Ontario, Canada L8L 2X2

Am J Dis Child. 1984;138(6):607. doi:10.1001/archpedi.1984.02140440091026

Sir.—The article by Deckelbaum et al1 on primary hypertriglyceridemia in childhood interests me because of our large experience of severe hypertriglyceridemia in adult patients.2,3 The experience of my colleagues and me now extends to 184 patients, all 19 years or older with fasting plasma triglyceride levels greater than 1,000 mg/dL and 43% with levels greater than 2,000 mg/dL. None have shown the clinical feature of intermittent swelling of the limbs and scrotum. Deckelbaum et al1 described the episodic abdominal pain, eruptive xanthomas, and hepatosplenomegaly at times when the triglyceride levels exceeded 2,000 mg/dL. The swelling of the scrotum could appear on its own, or with swelling and blueness of the legs. The uniqueness of this syndrome might be questioned in view of a case report by Simons et al.4

There are sufficient similarities between the two reports to warrant the following comparison (Deckelbaum and colleagues'