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[post_parent] => 426 [post_title] => Ornithine Transcarbamylase Deficiency (OTC) [post_name] => ornithine-transcarbamylase-deficiency-otc [date] => 2009-10-30 ) [29] => stdClass Object ( [ID] => 953 [post_parent] => 9 [post_title] => Psychologists' Resources [post_name] => psychologists-resources [date] => 2009-12-24 ) [30] => stdClass Object ( [ID] => 443 [post_parent] => 426 [post_title] => Carbamyl Phosphate Synthetase Deficiency (CPS) [post_name] => carbamyl-phosphate-synthetase-deficiency-cps [date] => 2009-10-30 ) [31] => stdClass Object ( [ID] => 445 [post_parent] => 426 [post_title] => Argininosuccinic Acid Synthetase Deficiency (Citullinemia) (AS) [post_name] => argininosuccinic-acid-synthetase-deficiency-citullinemia-as [date] => 2009-10-30 ) [32] => stdClass Object ( [ID] => 447 [post_parent] => 426 [post_title] => Argininosuccinic Acid Lyase Deficiency (ASA) [post_name] => argininosuccinic-acid-lyase-deficiency-asa [date] => 2009-10-30 ) [33] => stdClass Object ( [ID] => 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For Professionals
For Families

Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD)

This protocol is for the sick infant/child who has been previously diagnosed with Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD). MCADD is the most frequent of the fatty acid oxidation disorders (FAOD) and one of the most frequently identified inborn errors of metabolism. The incidence of MCADD may be as high as 1/10,000 with mortality rates of 13-43% at initial crises. It is caused by an intramitochondrial defect in the b-oxidation of fatty acids and is a major cause of hypoketotic hypoglycemia. MCADD is also a cause for lethargy, liver dysfunction with hepatomegaly, metabolic acidosis, hyperammonemia and sudden death.

Click to view the complete protocol as a PDF

Disclaimer
The care of patients with metabolic disorders is complex and it is essential that a metabolic specialist be contacted as rapidly as possible. This protocol is only a guideline and should not be used for definitive treatment without metabolic consultation. Please read our Terms of Use.