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Family Study and Discussion of Lipid Transport

CAPT. MILES H. SIGLER, MC; MAJOR MILTON E. RUBINI, MC

Arch Intern Med. 1961;107(6):894-902.

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Idiopathic or essential hyperlipemia is an uncommon inherited disorder of fat metabolism in which elevated plasma triglyceride levels lend a characteristic milky appearance to fasting plasma. A wide spectrum of clinical findings in this disorder has been described,^1-3 but little is known concerning the mechanism of the biochemical abnormality. Thannhauser⁴ attributed the high blood lipid levels to delayed plasma clearance of exogenous fat. Schrade et al.⁵ concluded that the defect is an abnormal metabolism of endogenous glycerides, since completely normal blood lipid levels could not be obtained by dietary restriction of fat. A lipemia-clearing factor was deficient in some cases of essential hyperlipemia⁶ but not in others.⁷ Klatskin and Gordon⁸ noted the coexistence of pancreatitis and the syndrome of essential hyperlipemia, but which was primary is not clear. The problem of pathogenesis is especially important because of the proclivity of patients with this disorder . . . [Full Text PDF of this Article]

Author Affiliations
USA; USA

From U.S. Army Tropical Research Medical Laboratory and the Department of Medicine, University of Puerto Rico School of Medicine.; Ward Officer (Capt. Sigler) and Chief (Major Rubini), Department of Metabolism, U.S. Army Tropical Research Medical Laboratory; Instructor (Capt. Sigler) and Assistant Professor (Major Rubini) of Medicine, University of Puerto Rico School of Medicine.

Footnotes
Submitted for publication April 28, 1960.

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