Nutritional Support, Including Intravenous Alimentation, for the Infant with Wolman’s Disease

  • Allen C. Crocker
  • Joseph N. Fisher
  • Robert M. Filler
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 19)

Abstract

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.)

Keywords

Cholesterol Catheter Foam Corticosteroid Tuberculosis 

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References

  1. Crocker, A.C., Vawter, G.F., Neuhauser, E.B.D., and Rosowsky, A.: Wolman’s disease; three new patients with a recently described lipidosis. Pediatrics, 35: 627, 1965.PubMedGoogle Scholar
  2. Filler, R.N., and Eraklis, A.J.: Care of the critically ill Child: intravenous alimentation. Pediatrics, 46: 456, 1970.PubMedGoogle Scholar
  3. Filler, R.N., Eraklis, A.J., Rubin, V.G., and Das, J.B.: Long-term total parenteral nutrition in infants. New Eng.J.Med., 281: 589, 1969.PubMedCrossRefGoogle Scholar
  4. Wolman, M., Sterk, V.V., Gatt, S., and Frenkel, M.: Primary familial xanthomatosis with involvement and calcification of the adrenals. Pediatrics, 28: 742, 1961.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1972

Authors and Affiliations

  • Allen C. Crocker
    • 1
    • 2
  • Joseph N. Fisher
    • 1
    • 2
  • Robert M. Filler
    • 1
    • 2
  1. 1.Departments of Medicine and SurgeryChildren’s Hospital Medical CenterBostonUSA
  2. 2.Departments of Pediatrics and SurgeryHarvard Medical SchoolBostonUSA

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