MSUD Concept

The dietary management consists of a diet low in branched chain amino acids (i.e. low in native protein) with addition of amino acids supplements free from Leucine, Isoleucine and Valine.

Disorders in Branched Chain Amino Acid Metabolism (e.g. MSUD)

Background:
Maple Syrup Urine Disease (MSUD) is an inherited rare disorder in metabolism of the branched chain amino acids Leucine, Isoleucine and Valine, leading to an accumulation of these amino acids and their corresponding oxo acids in all body fluids. If not treated, this condition results in severe irreversible neurological damages and finally death.

COMIDAMED

Dr. Schär AG 


Winkelau 9
I - 39014 Burgstall / Postal


Tel. +39 0473 293 300
Fax +39 0473 293 399
info.it@drschaer.com

 

 

COMIDACLOUD

Aminosäurenmischung | diätetische Behandlung von Phenylketonurie | erstes Lebensjahr | Erwachsene | glutenfrei | HPA | Hyperphenylalaninämie | Jugendliche | Kinder | Kleinkinder | Kohlenhydrate | Mineralstoffe | phenylalaninfreie | Proteinsupplemente | Phenylketonurie | PKU | Schwangere |Spurenelemente | Säuglinge | Vitamine

COMIDATAGS

Germany | Entwicklung | Herstellung | spezielle Ernährung | medizinisch | Nährstoffe | Rosbach |