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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

Isovaleric Acidemia

This protocol is for the sick infant/child who has been previously diagnosed with isovaleric acidemia. The organic acidemias comprise a group of metabolic disorders in which the defect produces an accumulation of organic acids. The central emergency features of the organic acid disorders are profound metabolic ketoacidosis and hypoglycemia. The inheritance of isovaleric acidemia is autosomal recessive. It is often referred to as the “sweaty foot syndrome” due to the characteristic odor body and body fluids odor produced by it.

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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.