MS-DRG 642
CMS Version 40.1 Compliance
Inborn And Other Disorders Of Metabolism
Complete mapping of the 202 ICD-10 diagnosis and procedure codes currently associated with this Medicare Severity Diagnosis Related Group classification.
Associated ICD-10 Classifications
C96.5
Multifocal and unisystemic Langerhans-cell histiocytosis
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C96.6
Unifocal Langerhans-cell histiocytosis
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D81.30
Adenosine deaminase deficiency, unspecified
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D81.31
Severe combined immunodeficiency due to adenosine deaminase deficiency
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D81.32
Adenosine deaminase 2 deficiency
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D81.39
Other adenosine deaminase deficiency
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D81.5
Purine nucleoside phosphorylase [PNP] deficiency
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D81.810
Biotinidase deficiency
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D84.1
Defects in the complement system
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E70.0
Classical phenylketonuria
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E70.1
Other hyperphenylalaninemias
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E70.20
Disorder of tyrosine metabolism, unspecified
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E70.21
Tyrosinemia
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E70.29
Other disorders of tyrosine metabolism
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E70.30
Albinism, unspecified
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E70.310
X-linked ocular albinism
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E70.311
Autosomal recessive ocular albinism
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E70.318
Other ocular albinism
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E70.319
Ocular albinism, unspecified
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E70.320
Tyrosinase negative oculocutaneous albinism
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E70.321
Tyrosinase positive oculocutaneous albinism
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E70.328
Other oculocutaneous albinism
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E70.329
Oculocutaneous albinism, unspecified
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E70.330
Chediak-Higashi syndrome
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E70.331
Hermansky-Pudlak syndrome
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E70.338
Other albinism with hematologic abnormality
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E70.339
Albinism with hematologic abnormality, unspecified
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E70.39
Other specified albinism
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E70.40
Disorders of histidine metabolism, unspecified
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E70.41
Histidinemia
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E70.49
Other disorders of histidine metabolism
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E70.5
Disorders of tryptophan metabolism
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E70.81
Aromatic L-amino acid decarboxylase deficiency
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E70.89
Other disorders of aromatic amino-acid metabolism
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E70.9
Disorder of aromatic amino-acid metabolism, unspecified
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E71.0
Maple-syrup-urine disease
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E71.110
Isovaleric acidemia
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E71.111
3-methylglutaconic aciduria
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E71.118
Other branched-chain organic acidurias
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E71.120
Methylmalonic acidemia
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E71.121
Propionic acidemia
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E71.128
Other disorders of propionate metabolism
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E71.19
Other disorders of branched-chain amino-acid metabolism
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E71.2
Disorder of branched-chain amino-acid metabolism, unspecified
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E71.30
Disorder of fatty-acid metabolism, unspecified
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E71.310
Long chain/very long chain acyl CoA dehydrogenase deficiency
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E71.311
Medium chain acyl CoA dehydrogenase deficiency
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E71.312
Short chain acyl CoA dehydrogenase deficiency
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E71.313
Glutaric aciduria type II
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E71.314
Muscle carnitine palmitoyltransferase deficiency
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E71.318
Other disorders of fatty-acid oxidation
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E71.32
Disorders of ketone metabolism
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E71.39
Other disorders of fatty-acid metabolism
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E71.40
Disorder of carnitine metabolism, unspecified
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E71.41
Primary carnitine deficiency
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E71.42
Carnitine deficiency due to inborn errors of metabolism
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E71.43
Iatrogenic carnitine deficiency
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E71.440
Ruvalcaba-Myhre-Smith syndrome
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E71.448
Other secondary carnitine deficiency
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E71.50
Peroxisomal disorder, unspecified
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E71.510
Zellweger syndrome
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E71.511
Neonatal adrenoleukodystrophy
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E71.518
Other disorders of peroxisome biogenesis
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E71.520
Childhood cerebral X-linked adrenoleukodystrophy
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E71.521
Adolescent X-linked adrenoleukodystrophy
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E71.522
Adrenomyeloneuropathy
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E71.528
Other X-linked adrenoleukodystrophy
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E71.529
X-linked adrenoleukodystrophy, unspecified type
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E71.53
Other group 2 peroxisomal disorders
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E71.540
Rhizomelic chondrodysplasia punctata
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E71.541
Zellweger-like syndrome
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E71.542
Other group 3 peroxisomal disorders
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E71.548
Other peroxisomal disorders
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E72.00
Disorders of amino-acid transport, unspecified
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E72.01
Cystinuria
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E72.02
Hartnup's disease
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E72.03
Lowe's syndrome
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E72.04
Cystinosis
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E72.09
Other disorders of amino-acid transport
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E72.10
Disorders of sulfur-bearing amino-acid metabolism, unspecified
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E72.11
Homocystinuria
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E72.12
Methylenetetrahydrofolate reductase deficiency
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E72.19
Other disorders of sulfur-bearing amino-acid metabolism
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E72.20
Disorder of urea cycle metabolism, unspecified
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E72.21
Argininemia
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E72.22
Arginosuccinic aciduria
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E72.23
Citrullinemia
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E72.29
Other disorders of urea cycle metabolism
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E72.3
Disorders of lysine and hydroxylysine metabolism
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E72.4
Disorders of ornithine metabolism
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E72.50
Disorder of glycine metabolism, unspecified
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E72.51
Non-ketotic hyperglycinemia
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E72.52
Trimethylaminuria
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E72.53
Primary hyperoxaluria
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E72.59
Other disorders of glycine metabolism
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E72.81
Disorders of gamma aminobutyric acid metabolism
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E72.89
Other specified disorders of amino-acid metabolism
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E72.9
Disorder of amino-acid metabolism, unspecified
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E74.00
Glycogen storage disease, unspecified
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E74.01
von Gierke disease
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E74.02
Pompe disease
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E74.03
Cori disease
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E74.04
McArdle disease
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E74.05
Lysosome-associated membrane protein 2 [LAMP2] deficiency
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E74.09
Other glycogen storage disease
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E74.20
Disorders of galactose metabolism, unspecified
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E74.21
Galactosemia
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E74.29
Other disorders of galactose metabolism
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E74.4
Disorders of pyruvate metabolism and gluconeogenesis
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E74.810
Glucose transporter protein type 1 deficiency
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E74.818
Other disorders of glucose transport
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E74.819
Disorders of glucose transport, unspecified
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E74.89
Other specified disorders of carbohydrate metabolism
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E74.9
Disorder of carbohydrate metabolism, unspecified
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E75.21
Fabry (-Anderson) disease
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E75.22
Gaucher disease
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E75.240
Niemann-Pick disease type A
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E75.241
Niemann-Pick disease type B
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E75.242
Niemann-Pick disease type C
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E75.243
Niemann-Pick disease type D
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E75.244
Niemann-Pick disease type A/B
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E75.248
Other Niemann-Pick disease
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E75.249
Niemann-Pick disease, unspecified
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E75.3
Sphingolipidosis, unspecified
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E75.5
Other lipid storage disorders
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E75.6
Lipid storage disorder, unspecified
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E76.01
Hurler's syndrome
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E76.02
Hurler-Scheie syndrome
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E76.03
Scheie's syndrome
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E76.1
Mucopolysaccharidosis, type II
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E76.210
Morquio A mucopolysaccharidoses
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E76.211
Morquio B mucopolysaccharidoses
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E76.219
Morquio mucopolysaccharidoses, unspecified
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E76.22
Sanfilippo mucopolysaccharidoses
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E76.29
Other mucopolysaccharidoses
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E76.3
Mucopolysaccharidosis, unspecified
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E76.8
Other disorders of glucosaminoglycan metabolism
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E76.9
Glucosaminoglycan metabolism disorder, unspecified
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E77.0
Defects in post-translational modification of lysosomal enzymes
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E77.1
Defects in glycoprotein degradation
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E77.8
Other disorders of glycoprotein metabolism
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E77.9
Disorder of glycoprotein metabolism, unspecified
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E78.00
Pure hypercholesterolemia, unspecified
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E78.01
Familial hypercholesterolemia
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E78.1
Pure hyperglyceridemia
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E78.2
Mixed hyperlipidemia
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E78.3
Hyperchylomicronemia
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E78.41
Elevated Lipoprotein(a)
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E78.49
Other hyperlipidemia
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E78.5
Hyperlipidemia, unspecified
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E78.6
Lipoprotein deficiency
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E78.70
Disorder of bile acid and cholesterol metabolism, unspecified
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E78.79
Other disorders of bile acid and cholesterol metabolism
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E78.81
Lipoid dermatoarthritis
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E78.89
Other lipoprotein metabolism disorders
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E78.9
Disorder of lipoprotein metabolism, unspecified
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E79.1
Lesch-Nyhan syndrome
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E79.2
Myoadenylate deaminase deficiency
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E79.81
Aicardi-Goutières syndrome
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E79.82
Hereditary xanthinuria
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E79.89
Other specified disorders of purine and pyrimidine metabolism
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E79.9
Disorder of purine and pyrimidine metabolism, unspecified
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E80.0
Hereditary erythropoietic porphyria
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E80.1
Porphyria cutanea tarda
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E80.20
Unspecified porphyria
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E80.21
Acute intermittent (hepatic) porphyria
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E80.29
Other porphyria
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E80.3
Defects of catalase and peroxidase
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E83.00
Disorder of copper metabolism, unspecified
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E83.01
Wilson's disease
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E83.09
Other disorders of copper metabolism
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E83.10
Disorder of iron metabolism, unspecified
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E83.110
Hereditary hemochromatosis
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E83.111
Hemochromatosis due to repeated red blood cell transfusions
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E83.118
Other hemochromatosis
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E83.119
Hemochromatosis, unspecified
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E83.19
Other disorders of iron metabolism
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E83.30
Disorder of phosphorus metabolism, unspecified
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E83.31
Familial hypophosphatemia
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E83.32
Hereditary vitamin D-dependent rickets (type 1) (type 2)
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E83.39
Other disorders of phosphorus metabolism
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E83.89
Other disorders of mineral metabolism
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E83.9
Disorder of mineral metabolism, unspecified
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E88.01
Alpha-1-antitrypsin deficiency
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E88.1
Lipodystrophy, not elsewhere classified
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E88.2
Lipomatosis, not elsewhere classified
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E88.40
Mitochondrial metabolism disorder, unspecified
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E88.41
MELAS syndrome
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E88.42
MERRF syndrome
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E88.43
Disorders of mitochondrial tRNA synthetases
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E88.49
Other mitochondrial metabolism disorders
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E88.810
Metabolic syndrome
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E88.811
Insulin resistance syndrome, Type A
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E88.818
Other insulin resistance
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E88.819
Insulin resistance, unspecified
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E88.89
Other specified metabolic disorders
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E88.9
Metabolic disorder, unspecified
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E88.A
Wasting disease (syndrome) due to underlying condition
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H49.811
Kearns-Sayre syndrome, right eye
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H49.812
Kearns-Sayre syndrome, left eye
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H49.813
Kearns-Sayre syndrome, bilateral
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H49.819
Kearns-Sayre syndrome, unspecified eye
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