MS-DRG v44.0 · MDC 10: Endocrine, Nutritional and Metabolic Diseases and Disorders
642 — Inborn and Other Disorders of Metabolism
Source: CMS MS-DRG v44.0 and FY2027 IPPS final rule. Page updated September 29, 2026.
- Relative weight
- 1.3219
- Geometric mean LOS
- 3.2 days
- Arithmetic mean LOS
- 4.5 days
- Type
- Medical
About MS-DRG 642
MS-DRG 642, Inborn and Other Disorders of Metabolism, is a medical DRG in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders) under MS-DRG version 44.0, which applies to inpatient discharges from October 1, 2026 through September 30, 2027.
Its FY2027 relative weight is 1.3219, so Medicare's base payment for this DRG is about 1.32 times the average inpatient case, before hospital-specific adjustments. The geometric mean length of stay is 3.2 days and the arithmetic mean is 4.5 days.
216 ICD-10-CM diagnosis codes are part of this DRG's grouping logic; they are listed below.
ICD-10-CM codes that group to DRG 642 (216)
Chapter 2: Neoplasms
Chapter 3: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
- D81.30Adenosine deaminase deficiency, unspecified
- D81.31Severe combined immunodeficiency due to adenosine deaminase deficiency
- D81.32Adenosine deaminase 2 deficiency
- D81.39Other adenosine deaminase deficiency
- D81.5Purine nucleoside phosphorylase [PNP] deficiency
- D81.810Biotinidase deficiency
- D84.1Defects in the complement system
Chapter 4: Endocrine, nutritional and metabolic diseases
- E70.0Classical phenylketonuria
- E70.1Other hyperphenylalaninemias
- E70.20Disorder of tyrosine metabolism, unspecified
- E70.21Tyrosinemia
- E70.29Other disorders of tyrosine metabolism
- E70.30Albinism, unspecified
- E70.310X-linked ocular albinism
- E70.311Autosomal recessive ocular albinism
- E70.318Other ocular albinism
- E70.319Ocular albinism, unspecified
- E70.320Tyrosinase negative oculocutaneous albinism
- E70.321Tyrosinase positive oculocutaneous albinism
- E70.328Other oculocutaneous albinism
- E70.329Oculocutaneous albinism, unspecified
- E70.330Chediak-Higashi syndrome
- E70.331Hermansky-Pudlak syndrome
- E70.338Other albinism with hematologic abnormality
- E70.339Albinism with hematologic abnormality, unspecified
- E70.39Other specified albinism
- E70.40Disorders of histidine metabolism, unspecified
- E70.41Histidinemia
- E70.49Other disorders of histidine metabolism
- E70.5Disorders of tryptophan metabolism
- E70.81Aromatic L-amino acid decarboxylase deficiency
- E70.89Other disorders of aromatic amino-acid metabolism
- E70.9Disorder of aromatic amino-acid metabolism, unspecified
- E71.0Maple-syrup-urine disease
- E71.110Isovaleric acidemia
- E71.1113-methylglutaconic aciduria
- E71.118Other branched-chain organic acidurias
- E71.120Methylmalonic acidemia
- E71.121Propionic acidemia
- E71.128Other disorders of propionate metabolism
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.30Disorder of fatty-acid metabolism, unspecified
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
- E71.311Medium chain acyl CoA dehydrogenase deficiency
- E71.312Short chain acyl CoA dehydrogenase deficiency
- E71.313Glutaric aciduria type II
- E71.314Muscle carnitine palmitoyltransferase deficiency
- E71.318Other disorders of fatty-acid oxidation
- E71.32Disorders of ketone metabolism
- E71.39Other disorders of fatty-acid metabolism
- E71.40Disorder of carnitine metabolism, unspecified
- E71.41Primary carnitine deficiency
- E71.42Carnitine deficiency due to inborn errors of metabolism
- E71.43Iatrogenic carnitine deficiency
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.50Peroxisomal disorder, unspecified
- E71.510Zellweger syndrome
- E71.511Neonatal adrenoleukodystrophy
- E71.518Other disorders of peroxisome biogenesis
- E71.520Childhood cerebral X-linked adrenoleukodystrophy
- E71.521Adolescent X-linked adrenoleukodystrophy
- E71.522Adrenomyeloneuropathy
- E71.528Other X-linked adrenoleukodystrophy
- E71.529X-linked adrenoleukodystrophy, unspecified type
- E71.53Other group 2 peroxisomal disorders
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
- E72.00Disorders of amino-acid transport, unspecified
- E72.01Cystinuria
- E72.02Hartnup's disease
- E72.03Lowe's syndrome
- E72.04Cystinosis
- E72.09Other disorders of amino-acid transport
- E72.10Disorders of sulfur-bearing amino-acid metabolism, unspecified
- E72.11Homocystinuria
- E72.12Methylenetetrahydrofolate reductase deficiency
- E72.19Other disorders of sulfur-bearing amino-acid metabolism
- E72.20Disorder of urea cycle metabolism, unspecified
- E72.21Argininemia
- E72.22Arginosuccinic aciduria
- E72.23Citrullinemia
- E72.29Other disorders of urea cycle metabolism
- E72.3Disorders of lysine and hydroxylysine metabolism
- E72.4Disorders of ornithine metabolism
- E72.50Disorder of glycine metabolism, unspecified
- E72.51Non-ketotic hyperglycinemia
- E72.52Trimethylaminuria
- E72.530Primary hyperoxaluria, type 1
- E72.538Other specified primary hyperoxaluria
- E72.539Primary hyperoxaluria, unspecified
- E72.59Other disorders of glycine metabolism
- E72.81Disorders of gamma aminobutyric acid metabolism
- E72.89Other specified disorders of amino-acid metabolism
- E72.9Disorder of amino-acid metabolism, unspecified
- E74.00Glycogen storage disease, unspecified
- E74.01von Gierke disease
- E74.02Pompe disease
- E74.03Cori disease
- E74.04McArdle disease
- E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiency
- E74.09Other glycogen storage disease
- E74.20Disorders of galactose metabolism, unspecified
- E74.21Galactosemia
- E74.29Other disorders of galactose metabolism
- E74.4Disorders of pyruvate metabolism and gluconeogenesis
- E74.810Glucose transporter protein type 1 deficiency
- E74.818Other disorders of glucose transport
- E74.819Disorders of glucose transport, unspecified
- E74.820SLC13A5 Citrate Transporter Disorder
- E74.829Other disorders of citrate metabolism
- E74.89Other specified disorders of carbohydrate metabolism
- E74.9Disorder of carbohydrate metabolism, unspecified
- E75.21Fabry (-Anderson) disease
- E75.22Gaucher disease
- E75.240Niemann-Pick disease type A
- E75.241Niemann-Pick disease type B
- E75.242Niemann-Pick disease type C
- E75.243Niemann-Pick disease type D
- E75.244Niemann-Pick disease type A/B
- E75.248Other Niemann-Pick disease
- E75.249Niemann-Pick disease, unspecified
- E75.3Sphingolipidosis, unspecified
- E75.5Other lipid storage disorders
- E75.6Lipid storage disorder, unspecified
- E76.01Hurler's syndrome
- E76.02Hurler-Scheie syndrome
- E76.03Scheie's syndrome
- E76.1Mucopolysaccharidosis, type II
- E76.210Morquio A mucopolysaccharidoses
- E76.211Morquio B mucopolysaccharidoses
- E76.219Morquio mucopolysaccharidoses, unspecified
- E76.22Sanfilippo mucopolysaccharidoses
- E76.29Other mucopolysaccharidoses
- E76.3Mucopolysaccharidosis, unspecified
- E76.8Other disorders of glucosaminoglycan metabolism
- E76.9Glucosaminoglycan metabolism disorder, unspecified
- E77.0Defects in post-translational modification of lysosomal enzymes
- E77.1Defects in glycoprotein degradation
- E77.8Other disorders of glycoprotein metabolism
- E77.9Disorder of glycoprotein metabolism, unspecified
- E78.00Pure hypercholesterolemia, unspecified
- E78.010Homozygous familial hypercholesterolemia [HoFH]
- E78.011Heterozygous familial hypercholesterolemia [HeFH]
- E78.019Familial hypercholesterolemia, unspecified
- E78.1Pure hyperglyceridemia
- E78.2Mixed hyperlipidemia
- E78.3Hyperchylomicronemia
- E78.41Elevated Lipoprotein(a)
- E78.49Other hyperlipidemia
- E78.5Hyperlipidemia, unspecified
- E78.6Lipoprotein deficiency
- E78.70Disorder of bile acid and cholesterol metabolism, unspecified
- E78.79Other disorders of bile acid and cholesterol metabolism
- E78.81Lipoid dermatoarthritis
- E78.89Other lipoprotein metabolism disorders
- E78.9Disorder of lipoprotein metabolism, unspecified
- E79.1Lesch-Nyhan syndrome
- E79.2Myoadenylate deaminase deficiency
- E79.81Aicardi-Goutieres syndrome
- E79.82Hereditary xanthinuria
- E79.89Other specified disorders of purine and pyrimidine metabolism
- E79.9Disorder of purine and pyrimidine metabolism, unspecified
- E80.0Hereditary erythropoietic porphyria
- E80.1Porphyria cutanea tarda
- E80.20Unspecified porphyria
- E80.21Acute intermittent (hepatic) porphyria
- E80.29Other porphyria
- E80.3Defects of catalase and peroxidase
- E83.00Disorder of copper metabolism, unspecified
- E83.01Wilson's disease
- E83.09Other disorders of copper metabolism
- E83.10Disorder of iron metabolism, unspecified
- E83.110Hereditary hemochromatosis
- E83.111Hemochromatosis due to repeated red blood cell transfusions
- E83.118Other hemochromatosis
- E83.119Hemochromatosis, unspecified
- E83.19Other disorders of iron metabolism
- E83.30Disorder of phosphorus metabolism, unspecified
- E83.31Familial hypophosphatemia
- E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)
- E83.39Other disorders of phosphorus metabolism
- E83.822ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
- E83.824ABCC6 deficiency causing pseudoxanthoma elasticum
- E83.89Other disorders of mineral metabolism
- E83.9Disorder of mineral metabolism, unspecified
- E88.01Alpha-1-antitrypsin deficiency
- E88.10Lipodystrophy, unspecified
- E88.11Partial lipodystrophy
- E88.12Generalized lipodystrophy
- E88.13Localized lipodystrophy
- E88.14HIV-associated lipodystrophy
- E88.19Other lipodystrophy, not elsewhere classified
- E88.2Lipomatosis, not elsewhere classified
- E88.40Mitochondrial metabolism disorder, unspecified
- E88.41MELAS syndrome
- E88.42MERRF syndrome
- E88.43Disorders of mitochondrial tRNA synthetases
- E88.49Other mitochondrial metabolism disorders
- E88.810Metabolic syndrome
- E88.811Insulin resistance syndrome, Type A
- E88.818Other insulin resistance
- E88.819Insulin resistance, unspecified
- E88.82Obesity due to disruption of MC4R pathway
- E88.89Other specified metabolic disorders
- E88.9Metabolic disorder, unspecified
- E88.AWasting disease (syndrome) due to underlying condition
Chapter 7: Diseases of the eye and adnexa
Common questions about DRG 642
What is the relative weight of DRG 642?
- 1.3219 for FY2027 (MS-DRG v44.0), from the IPPS final rule Table 5.
What is the average length of stay for DRG 642?
- The geometric mean length of stay is 3.2 days; the arithmetic mean is 4.5 days.
Is DRG 642 medical or surgical?
- DRG 642 is a medical DRG.