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What is Homocystinuria?
Classical Homocystinuria is an inherited disorder caused by Cystathione β-synthetase deficiency resulting in mental retardation, eye problems, and risk of thrombosis. The aim of dietary management is to prevent the accumulation of methionine by means of a low methionine diet (in the UK this is achieved with the use of methionine exchanges). Protein requirements are met by supplementing the diet with a methionine free amino acid mixture, which contains cystine.
Cystine levels should be monitored and may need supplementing depending upon the patients individual tolerance. Cystine is available as a supplement from SHS.
Betaine is often used to assist in remethylation. Milder variants of Homocystinuria may be partially or totally vitamin B6 responsive. Folic acid supplementation may also be required.
Bibliography
Yap S, Naughten E. Homocystinuria due to cystathionine b-synthase deficiency in
ireland: 25 years experience of a newborn screened and treated population with
reference to clinical outcome and biochemical control. J Inher Metab Dis. 1998;21738-747.
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The content provided by lowproteinLIVING is for information purposes only and is in no way intended to be a substitute for medical consultation with your doctor, dietitian or healthcare professional. The information, opinions, and recommendations presented in these pages are not intended to replace the care of your own doctor, dietitian or healthcare professional. Before you make any changes in the management of your diet / treatment or any other persons diet /treatment you should always consult your doctor, dietitian or healthcare professional. Although we carefully review our content, lowproteinLIVING cannot guarantee or take responsibility for the medical accuracy of documents we publish, nor can shs-nutrition assume any liability for the content of any web site linked to our site. © 2008 SHS International. All rights reserved.
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