DRG 642
Medical DRGInborn and Other Disorders of Metabolism
What is MS-DRG 642?
MS-DRG 642 is a Medical Medicare Severity Diagnosis Related Group under Major Diagnostic Category 10 (MDC 10). It maps to 216 ICD-10-CM diagnosis codes. Hospitals use this grouping to determine reimbursement for inpatient stays under Medicare.
Linked ICD-10-CM Diagnosis Mappings
- C96.5
Multifocal and unisystemic Langerhans-cell histiocytosis
BillableChapter 2 - C96.6
Unifocal Langerhans-cell histiocytosis
BillableChapter 2 - D81.30
Adenosine deaminase deficiency, unspecified
BillableChapter 3 - D81.31
Severe combined immunodeficiency due to adenosine deaminase deficiency
BillableChapter 3 - D81.32
Adenosine deaminase 2 deficiency
BillableChapter 3 - D81.39
Other adenosine deaminase deficiency
BillableChapter 3 - D81.5
Purine nucleoside phosphorylase [PNP] deficiency
BillableChapter 3 - D81.810
Biotinidase deficiency
BillableChapter 3 - D84.1
Defects in the complement system
BillableChapter 3 - E70.0
Classical phenylketonuria
BillableChapter 4 - E70.1
Other hyperphenylalaninemias
BillableChapter 4 - E70.20
Disorder of tyrosine metabolism, unspecified
BillableChapter 4 - E70.21
Tyrosinemia
BillableChapter 4 - E70.29
Other disorders of tyrosine metabolism
BillableChapter 4 - E70.30
Albinism, unspecified
BillableChapter 4 - E70.310
X-linked ocular albinism
BillableChapter 4 - E70.311
Autosomal recessive ocular albinism
BillableChapter 4 - E70.318
Other ocular albinism
BillableChapter 4 - E70.319
Ocular albinism, unspecified
BillableChapter 4 - E70.320
Tyrosinase negative oculocutaneous albinism
BillableChapter 4 - E70.321
Tyrosinase positive oculocutaneous albinism
BillableChapter 4 - E70.328
Other oculocutaneous albinism
BillableChapter 4 - E70.329
Oculocutaneous albinism, unspecified
BillableChapter 4 - E70.330
Chediak-Higashi syndrome
BillableChapter 4 - E70.331
Hermansky-Pudlak syndrome
BillableChapter 4 - E70.338
Other albinism with hematologic abnormality
BillableChapter 4 - E70.339
Albinism with hematologic abnormality, unspecified
BillableChapter 4 - E70.39
Other specified albinism
BillableChapter 4 - E70.40
Disorders of histidine metabolism, unspecified
BillableChapter 4 - E70.41
Histidinemia
BillableChapter 4 - E70.49
Other disorders of histidine metabolism
BillableChapter 4 - E70.5
Disorders of tryptophan metabolism
BillableChapter 4 - E70.81
Aromatic L-amino acid decarboxylase deficiency
BillableChapter 4 - E70.89
Other disorders of aromatic amino-acid metabolism
BillableChapter 4 - E70.9
Disorder of aromatic amino-acid metabolism, unspecified
BillableChapter 4 - E71.0
Maple-syrup-urine disease
BillableChapter 4 - E71.110
Isovaleric acidemia
BillableChapter 4 - E71.111
3-methylglutaconic aciduria
BillableChapter 4 - E71.118
Other branched-chain organic acidurias
BillableChapter 4 - E71.120
Methylmalonic acidemia
BillableChapter 4 - E71.121
Propionic acidemia
BillableChapter 4 - E71.128
Other disorders of propionate metabolism
BillableChapter 4 - E71.19
Other disorders of branched-chain amino-acid metabolism
BillableChapter 4 - E71.2
Disorder of branched-chain amino-acid metabolism, unspecified
BillableChapter 4 - E71.30
Disorder of fatty-acid metabolism, unspecified
BillableChapter 4 - E71.310
Long chain/very long chain acyl CoA dehydrogenase deficiency
BillableChapter 4 - E71.311
Medium chain acyl CoA dehydrogenase deficiency
BillableChapter 4 - E71.312
Short chain acyl CoA dehydrogenase deficiency
BillableChapter 4 - E71.313
Glutaric aciduria type II
BillableChapter 4 - E71.314
Muscle carnitine palmitoyltransferase deficiency
BillableChapter 4 - E71.318
Other disorders of fatty-acid oxidation
BillableChapter 4 - E71.32
Disorders of ketone metabolism
BillableChapter 4 - E71.39
Other disorders of fatty-acid metabolism
BillableChapter 4 - E71.40
Disorder of carnitine metabolism, unspecified
BillableChapter 4 - E71.41
Primary carnitine deficiency
BillableChapter 4 - E71.42
Carnitine deficiency due to inborn errors of metabolism
BillableChapter 4 - E71.43
Iatrogenic carnitine deficiency
BillableChapter 4 - E71.440
Ruvalcaba-Myhre-Smith syndrome
BillableChapter 4 - E71.448
Other secondary carnitine deficiency
BillableChapter 4 - E71.50
Peroxisomal disorder, unspecified
BillableChapter 4 - E71.510
Zellweger syndrome
BillableChapter 4 - E71.511
Neonatal adrenoleukodystrophy
BillableChapter 4 - E71.518
Other disorders of peroxisome biogenesis
BillableChapter 4 - E71.520
Childhood cerebral X-linked adrenoleukodystrophy
BillableChapter 4 - E71.521
Adolescent X-linked adrenoleukodystrophy
BillableChapter 4 - E71.522
Adrenomyeloneuropathy
BillableChapter 4 - E71.528
Other X-linked adrenoleukodystrophy
BillableChapter 4 - E71.529
X-linked adrenoleukodystrophy, unspecified type
BillableChapter 4 - E71.53
Other group 2 peroxisomal disorders
BillableChapter 4 - E71.540
Rhizomelic chondrodysplasia punctata
BillableChapter 4 - E71.541
Zellweger-like syndrome
BillableChapter 4 - E71.542
Other group 3 peroxisomal disorders
BillableChapter 4 - E71.548
Other peroxisomal disorders
BillableChapter 4 - E72.00
Disorders of amino-acid transport, unspecified
BillableChapter 4 - E72.01
Cystinuria
BillableChapter 4 - E72.02
Hartnup's disease
BillableChapter 4 - E72.03
Lowe's syndrome
BillableChapter 4 - E72.04
Cystinosis
BillableChapter 4 - E72.09
Other disorders of amino-acid transport
BillableChapter 4 - E72.10
Disorders of sulfur-bearing amino-acid metabolism, unspecified
BillableChapter 4 - E72.11
Homocystinuria
BillableChapter 4 - E72.12
Methylenetetrahydrofolate reductase deficiency
BillableChapter 4 - E72.19
Other disorders of sulfur-bearing amino-acid metabolism
BillableChapter 4 - E72.20
Disorder of urea cycle metabolism, unspecified
BillableChapter 4 - E72.21
Argininemia
BillableChapter 4 - E72.22
Arginosuccinic aciduria
BillableChapter 4 - E72.23
Citrullinemia
BillableChapter 4 - E72.29
Other disorders of urea cycle metabolism
BillableChapter 4 - E72.3
Disorders of lysine and hydroxylysine metabolism
BillableChapter 4 - E72.4
Disorders of ornithine metabolism
BillableChapter 4 - E72.50
Disorder of glycine metabolism, unspecified
BillableChapter 4 - E72.51
Non-ketotic hyperglycinemia
BillableChapter 4 - E72.52
Trimethylaminuria
BillableChapter 4 - E72.530
Primary hyperoxaluria, type 1
BillableChapter 4 - E72.538
Other specified primary hyperoxaluria
BillableChapter 4 - E72.539
Primary hyperoxaluria, unspecified
BillableChapter 4 - E72.59
Other disorders of glycine metabolism
BillableChapter 4 - E72.81
Disorders of gamma aminobutyric acid metabolism
BillableChapter 4 - E72.89
Other specified disorders of amino-acid metabolism
BillableChapter 4 - E72.9
Disorder of amino-acid metabolism, unspecified
BillableChapter 4 - E74.00
Glycogen storage disease, unspecified
BillableChapter 4 - E74.01
von Gierke disease
BillableChapter 4 - E74.02
Pompe disease
BillableChapter 4 - E74.03
Cori disease
BillableChapter 4 - E74.04
McArdle disease
BillableChapter 4 - E74.05
Lysosome-associated membrane protein 2 [LAMP2] deficiency
BillableChapter 4 - E74.09
Other glycogen storage disease
BillableChapter 4 - E74.20
Disorders of galactose metabolism, unspecified
BillableChapter 4 - E74.21
Galactosemia
BillableChapter 4 - E74.29
Other disorders of galactose metabolism
BillableChapter 4 - E74.4
Disorders of pyruvate metabolism and gluconeogenesis
BillableChapter 4 - E74.810
Glucose transporter protein type 1 deficiency
BillableChapter 4 - E74.818
Other disorders of glucose transport
BillableChapter 4 - E74.819
Disorders of glucose transport, unspecified
BillableChapter 4 - E74.820
SLC13A5 Citrate Transporter Disorder
BillableChapter 4 - E74.829
Other disorders of citrate metabolism
BillableChapter 4 - E74.89
Other specified disorders of carbohydrate metabolism
BillableChapter 4 - E74.9
Disorder of carbohydrate metabolism, unspecified
BillableChapter 4 - E75.21
Fabry (-Anderson) disease
BillableChapter 4 - E75.22
Gaucher disease
BillableChapter 4 - E75.240
Niemann-Pick disease type A
BillableChapter 4 - E75.241
Niemann-Pick disease type B
BillableChapter 4 - E75.242
Niemann-Pick disease type C
BillableChapter 4 - E75.243
Niemann-Pick disease type D
BillableChapter 4 - E75.244
Niemann-Pick disease type A/B
BillableChapter 4 - E75.248
Other Niemann-Pick disease
BillableChapter 4 - E75.249
Niemann-Pick disease, unspecified
BillableChapter 4 - E75.3
Sphingolipidosis, unspecified
BillableChapter 4 - E75.5
Other lipid storage disorders
BillableChapter 4 - E75.6
Lipid storage disorder, unspecified
BillableChapter 4 - E76.01
Hurler's syndrome
BillableChapter 4 - E76.02
Hurler-Scheie syndrome
BillableChapter 4 - E76.03
Scheie's syndrome
BillableChapter 4 - E76.1
Mucopolysaccharidosis, type II
BillableChapter 4 - E76.210
Morquio A mucopolysaccharidoses
BillableChapter 4 - E76.211
Morquio B mucopolysaccharidoses
BillableChapter 4 - E76.219
Morquio mucopolysaccharidoses, unspecified
BillableChapter 4 - E76.22
Sanfilippo mucopolysaccharidoses
BillableChapter 4 - E76.29
Other mucopolysaccharidoses
BillableChapter 4 - E76.3
Mucopolysaccharidosis, unspecified
BillableChapter 4 - E76.8
Other disorders of glucosaminoglycan metabolism
BillableChapter 4 - E76.9
Glucosaminoglycan metabolism disorder, unspecified
BillableChapter 4 - E77.0
Defects in post-translational modification of lysosomal enzymes
BillableChapter 4 - E77.1
Defects in glycoprotein degradation
BillableChapter 4 - E77.8
Other disorders of glycoprotein metabolism
BillableChapter 4 - E77.9
Disorder of glycoprotein metabolism, unspecified
BillableChapter 4 - E78.00
Pure hypercholesterolemia, unspecified
BillableChapter 4 - E78.010
Homozygous familial hypercholesterolemia [HoFH]
BillableChapter 4 - E78.011
Heterozygous familial hypercholesterolemia [HeFH]
BillableChapter 4 - E78.019
Familial hypercholesterolemia, unspecified
BillableChapter 4 - E78.1
Pure hyperglyceridemia
BillableChapter 4 - E78.2
Mixed hyperlipidemia
BillableChapter 4 - E78.3
Hyperchylomicronemia
BillableChapter 4 - E78.41
Elevated Lipoprotein(a)
BillableChapter 4 - E78.49
Other hyperlipidemia
BillableChapter 4 - E78.5
Hyperlipidemia, unspecified
BillableChapter 4 - E78.6
Lipoprotein deficiency
BillableChapter 4 - E78.70
Disorder of bile acid and cholesterol metabolism, unspecified
BillableChapter 4 - E78.79
Other disorders of bile acid and cholesterol metabolism
BillableChapter 4 - E78.81
Lipoid dermatoarthritis
BillableChapter 4 - E78.89
Other lipoprotein metabolism disorders
BillableChapter 4 - E78.9
Disorder of lipoprotein metabolism, unspecified
BillableChapter 4 - E79.1
Lesch-Nyhan syndrome
BillableChapter 4 - E79.2
Myoadenylate deaminase deficiency
BillableChapter 4 - E79.81
Aicardi-Goutieres syndrome
BillableChapter 4 - E79.82
Hereditary xanthinuria
BillableChapter 4 - E79.89
Other specified disorders of purine and pyrimidine metabolism
BillableChapter 4 - E79.9
Disorder of purine and pyrimidine metabolism, unspecified
BillableChapter 4 - E80.0
Hereditary erythropoietic porphyria
BillableChapter 4 - E80.1
Porphyria cutanea tarda
BillableChapter 4 - E80.20
Unspecified porphyria
BillableChapter 4 - E80.21
Acute intermittent (hepatic) porphyria
BillableChapter 4 - E80.29
Other porphyria
BillableChapter 4 - E80.3
Defects of catalase and peroxidase
BillableChapter 4 - E83.00
Disorder of copper metabolism, unspecified
BillableChapter 4 - E83.01
Wilson's disease
BillableChapter 4 - E83.09
Other disorders of copper metabolism
BillableChapter 4 - E83.10
Disorder of iron metabolism, unspecified
BillableChapter 4 - E83.110
Hereditary hemochromatosis
BillableChapter 4 - E83.111
Hemochromatosis due to repeated red blood cell transfusions
BillableChapter 4 - E83.118
Other hemochromatosis
BillableChapter 4 - E83.119
Hemochromatosis, unspecified
BillableChapter 4 - E83.19
Other disorders of iron metabolism
BillableChapter 4 - E83.30
Disorder of phosphorus metabolism, unspecified
BillableChapter 4 - E83.31
Familial hypophosphatemia
BillableChapter 4 - E83.32
Hereditary vitamin D-dependent rickets (type 1) (type 2)
BillableChapter 4 - E83.39
Other disorders of phosphorus metabolism
BillableChapter 4 - E83.822
ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
BillableChapter 4 - E83.824
ABCC6 deficiency causing pseudoxanthoma elasticum
BillableChapter 4 - E83.89
Other disorders of mineral metabolism
BillableChapter 4 - E83.9
Disorder of mineral metabolism, unspecified
BillableChapter 4 - E88.01
Alpha-1-antitrypsin deficiency
BillableChapter 4 - E88.10
Lipodystrophy, unspecified
BillableChapter 4 - E88.11
Partial lipodystrophy
BillableChapter 4 - E88.12
Generalized lipodystrophy
BillableChapter 4 - E88.13
Localized lipodystrophy
BillableChapter 4 - E88.14
HIV-associated lipodystrophy
BillableChapter 4 - E88.19
Other lipodystrophy, not elsewhere classified
BillableChapter 4 - E88.2
Lipomatosis, not elsewhere classified
BillableChapter 4 - E88.40
Mitochondrial metabolism disorder, unspecified
BillableChapter 4 - E88.41
MELAS syndrome
BillableChapter 4 - E88.42
MERRF syndrome
BillableChapter 4 - E88.43
Disorders of mitochondrial tRNA synthetases
BillableChapter 4 - E88.49
Other mitochondrial metabolism disorders
BillableChapter 4 - E88.810
Metabolic syndrome
BillableChapter 4 - E88.811
Insulin resistance syndrome, Type A
BillableChapter 4 - E88.818
Other insulin resistance
BillableChapter 4 - E88.819
Insulin resistance, unspecified
BillableChapter 4 - E88.82
Obesity due to disruption of MC4R pathway
BillableChapter 4 - E88.89
Other specified metabolic disorders
BillableChapter 4 - E88.9
Metabolic disorder, unspecified
BillableChapter 4 - E88.A
Wasting disease (syndrome) due to underlying condition
BillableChapter 4 - H49.811
Kearns-Sayre syndrome, right eye
BillableChapter 7 - H49.812
Kearns-Sayre syndrome, left eye
BillableChapter 7 - H49.813
Kearns-Sayre syndrome, bilateral
BillableChapter 7 - H49.819
Kearns-Sayre syndrome, unspecified eye
BillableChapter 7