LowproteinLIVING

What is Isovaleric Acidaemia and Disorders of Leucin Metabolism?

Isovaleric Acidaemia (IVA) is a rare inherited disorder affecting leucine metabolism caused by a deficiency of isovaleryl-CoA dehydrogenase.

The disease can present itself in a variety of forms from a severe presentation to a much milder form.  Symptoms will vary from individual to individual and include drowsiness, rapid breathing and vomiting.  A spell in hospital is often necessary to stabilise the infant/child and the hospital team will give you information and guidance on how to manage your child’s condition.

The aim of dietary management is to limit dietary leucine intake and minimise formation of isovaleric acid by means of a low protein diet. The protein requirements are met by supplementing the diet with a leucine free amino acid mixture if necessary.

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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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