Nutritional Support, Including Intravenous Alimentation, for the Infant with Wolman’s Disease
Wolman’s disease was identified 15 years ago as a hereditary syndrome involving an inborn error of lipid metabolism. The visible expression of this handicap is an accumulation of ester cholesterol and triglyceride in foam cells in the viscera, and a resultant severe compromise in general body nutrition and development, for the homozygously affected infant. An early terminology for the condition, “Primary familial xanthomatosis with involvement and calcification of the adrenals” (Wolman et al, 1961), pointed out the remarkable adrenal changes; the significance of the functional effects from this pathology has remained uncertain. Although Wolman’s disease appears to be uncommon, a world-wide distribution is now documented, without an apparent increase in gene frequency in any particular population group. Families have been reported from Israel (both Jewish and Arab), Japan, New Zealand, England, Belgium, Canada (Toronto, Montreal), and the United States (Boston, Philadelphia, Cleveland, Los Angeles, Albany, and Charleston, W. Va.)
KeywordsFoam Cell Nutritional Support Blood Stream Infection Serum Protein Level General Clinical Research Center
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