A 12-year-old girl with homozygous type-II hyperlipoproteinæmia, which was refractory to medical treatment, had significant improvement in her serum lipid abnormalities during a trial of parenteral hyperalimentation. Subsequently, end-to-side portacaval shunt caused striking reduction of the serum-cholesterol and low-density (beta) lipoprotein elevations, regression or disappearance of xanthomatous skin and tendinous lesions during the 61/2 months since operation, and relief of severe cardiac symptoms apparently as resorption occurred of xanthomatous deposits in the aortic valve and around the coronary arteries. Liver biopsy at 51/2 months showed several changes, including depletion of rough endoplasmic reticulum; this finding was consistent with the possibility that the portal diversion caused the desired therapeutic effects by inhibiting synthesis of hepatic cholesterol or low-density lipoprotein.
|Original language||English (US)|
|Number of pages||5|
|State||Published - Oct 27 1973|
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