Article Text

Download PDFPDF

Dietary treatment of hyperlysinaemia.
  1. J W Gregory,
  2. N Beail,
  3. N A Boyle,
  4. C Dobrowski,
  5. P Jackson
  1. Department of Paediatrics, Huddersfield Royal Infirmary.


    We describe the lysine restricted, dietary management of three out of four siblings who were identified as having hyperlysinaemia. The diets, started in the neonatal period, were maintained for varying periods with unpredictable success. The propositus, who was not treated, was diagnosed at the age of 5 years, by which time he was already severely handicapped, presumably because of his metabolic disorder. Tentative recommendations are put forward for the management of this seemingly rare disorder. Mild chronic ammonia toxicity may be a factor in the pathogenesis of this condition.

    Statistics from

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.