Abstract
Summary
The sera of 10 children with Niemann-Pick disease, 9 of whom had hepatomegaly, were analyzed by paper electropho-resis with a Tris borate buffer. The alpha-2 globulin fraction was absent in all 10 sera. This alpha globulin fraction was also absent in 13 out of 14 control patients with primary or secondary liver involvement. The absence of serum alpha-2 globulin appears to be a sensitive indicator of liver involvement, since it is absent even when serum glutamic oxalo-acetic transaminase and serum glutamic pyru-vic transaminase are normal. Six of the remaining 46 controls with no evidence of clinical liver disease also had absent serum alpha-2 globulin. Three of these were parents of the children with Niemann-Pick disease.
Get full access to this article
View all access options for this article.