5C50.E0 Classical organic aciduria

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

This a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present.

code elsewhere

• Ketoacidosis due to beta-ketothiolase deficiency  (5C50.DY)

synonyms

• Classical organic aciduria
• Methylmalonic aciduria - homocystinuria
• Methylmalonic acidaemia - homocystinuria
• Methylmalonic aciduria - homocystinuria type cbl C
• Methylmalonic acidaemia - homocystinuria type cbl C
• Methylmalonic aciduria - homocystinuria type cbl D
• Methylmalonic acidaemia - homocystinuria type cbl D
• Methylmalonic aciduria - homocystinuria type cbl F
• Methylmalonic acidaemia - homocystinuria type cbl F
• Methylmalonic aciduria
• Methylmalonic acidaemia
• Methylmalonic aciduria, vitamin B12 unresponsive
• Complete deficiency of methylmalonyl-CoA mutase
• Mutase0 methylmalonic acidaemia
• Mutase0 methylmalonic aciduria
• Partial deficiency of methylmalonyl-CoA mutase
• Mutase methylmalonic acidaemia
• Mutase methylmalonic aciduria
• Methylmalonyl-CoA epimerase deficiency
• Methylmalonyl-CoA racemase deficiency
• Methylmalonyl-CoA epimerase deficiency with sepiapterin reductase deficiency
• Methylmalonyl-CoA racemase deficiency with sepiapterin reductase deficiency
• Methylmalonic aciduria, vitamin B12 responsive
• Adenosylcobalamin deficiency
• Vitamin B12 responsive methylmalonic aciduria type cbl A
• Vitamin B12 responsive methylmalonic aciduria type cbl B
• Vitamin B12 responsive methylmalonic aciduria type cbl mut-
• Propionic aciduria
• Propionic acidaemia
• Propionyl-CoA carboxylase deficiency
• Ketotic glycinaemia
• Ketotic hyperglycinaemia
• Hyperglycinaemia with ketosis and leucopaenia
• Isovaleric aciduria
• Isovaleric acidaemia
• Isovaleric acid CoA dehydrogenase deficiency
• Multiple carboxylase deficiency
• Multiple carboxylase deficiency due to biotinidase deficiency
• Multiple carboxylase deficiency due to holocarboxylase synthetase deficiency
• Neurodegeneration due to 3-hydroxyisobutyryl-CoA hydrolase deficiency
• Beta-hydroxyisobutyryl-CoA deacylase deficiency
• Methacrylic aciduria
• 3-hydroxyisobutyric aciduria
• 3-hydroxyisobutyrate dehydrogenase deficiency
• 3-hydroxy-3-methylglutaric aciduria
• 3-methylcrotonylglycinuria
• 3-methylglutaconic aciduria
• MGA - [3-methylglutaconic aciduria]
• 3-methylglutaconic aciduria type 1
• 3-methylglutaconyl-CoA hydratase deficiency
• 3MG-CoA hydratase deficiency
• MGA1- [3-methylglutaconic aciduria type 1]
• Barth syndrome
• 3-methylglutaconic aciduria type 2
• Tafazzin gene defect
• MGA2 - [3-methylglutaconic aciduria type 2]
• Cardioskeletal myopathy-neutropaenia
• 3-methylglutaconic aciduria type 3
• Infantile optic atrophy with chorea and spastic paraplegia
• Autosomal recessive optic atrophy type 3
• Costeff optic atrophy syndrome
• Optic atrophy plus syndrome
• Costeff syndrome
• MGA3 - [3-methylglutaconic aciduria type 3]
• 3-methylglutaconic aciduria type 4
• MGA4 - [3-methylglutaconic aciduria type 4]
• 3-methylglutaconic aciduria type 5
• Dilated cardiomyopathy with ataxia
• DCMA - [Dilated cardiomyopathy with ataxia] syndrome
• 3-methylglutaconic aciduria with normal3 -methylglutaconyl-Co A hydratase
• 2-methylbutyric aciduria
• Short/branched-chain acyl-coA dehydrogenase deficiency
• 2-methylbutyryl glycinuria
• Developmental delay due to 2-methylbutyryl-CoA dehydrogenase deficiency
• SBCAD - [Short/branched-chain acyl-coA dehydrogenase] deficiency
• Isobutyric aciduria
• Isobutyric acidaemia
• Isobutyryl-CoA dehydrogenase deficiency
• Methylmalonate semialdehyde dehydrogenase deficiency
• Glutaric aciduria type 3
• Glutaric acidaemia type 3
• Glutaryl-CoA oxidase deficiency

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