Article Sidebar
Article Details
Copyright remains in the Author’s name. The work is licensed under a Creative Commons Attribution - Noncommercial Works License. Authors are required to complete and sign an Author Agreement form that outlines Author and Publisher rights and terms of publication. The Agreement form should be uploaded along with other submissions files and any submission will be considered incomplete without it [forthcoming].
Material submitted for publication in the SAMJ is accepted provided it has not been published or submitted for publication elsewhere. Please inform the editorial team if the main findings of your paper have been presented at a conference and published in abstract form, to avoid copyright infringement. The SAMJ does not hold itself responsible for statements made by the authors.
Previously published images
If an image/figure has been previously published, permission to reproduce or alter it must be obtained by the authors from the original publisher and the figure legend must give full credit to the original source. This credit should be accompanied by a letter indicating that permission to reproduce the image has been granted to the author/s. This letter should be uploaded as a supplementary file during submission.
Main Article Content
Familial Type 11 Hyperlipoproteinaemia
Abstract
Thirty-four patients with familial type 11 hyperlipoproteinaemia were seen over an 8-month period at a lipid disorders clinic for children and young adults. Of the 34 patients, 5 were classified as homozygous, 27 as heterozygous type lIa, and 2 as heterozygous type lib. Clinical manifestations of the disease were present in all 5 homozygous patients and in 12 of the 29 heterozygotes. The most common physical finding was Achilles tendon xanthoma. The high incidence of physical signs in our patients stresses the importance of such features in the young. For the purpose of treatment the heterozygotes were divided into 2 groups: (a) children under the age of 15 years; (b) young adults from 16 to 25 years of age. Patients in both groups received a minimum of 6 weeks' dietary treatment followed by combined dietary and cholestyramine therapy (Questran; Mead Johnson). The decrease in serum cholesterol on diet alone was similar in both groups. On combined therapy, the children showed a decrease in serum cholesterol of 27% compared with 15% in the young adults. A general fall in the serum cholesterol of 36% was achieved in the children, compared with 19% in the young adults. In 3 homozygotes diet alone produced a fall in serum cholesterol similar to that found in the heterozygotes. In 2 patients who had cholestyramine added to the diet, further decrease of 20% in serum cholesterol was achieved.
