Inborn Errors of Metabolism

Principal Diagnosis - Pdx

  • C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
  • C96.6 Unifocal Langerhans-cell histiocytosis
  • D81.30 Adenosine deaminase deficiency, unspecified
  • D81.31 Severe combined immunodeficiency due to adenosine deaminase deficiency
  • D81.32 Adenosine deaminase 2 deficiency
  • D81.39 Other adenosine deaminase deficiency
  • D81.5 Purine nucleoside phosphorylase [PNP] deficiency
  • D81.810 Biotinidase deficiency
  • D84.1 Defects in the complement system
  • E70.0 Classical phenylketonuria
  • E70.1 Other hyperphenylalaninemias
  • E70.20 Disorder of tyrosine metabolism, unspecified
  • E70.21 Tyrosinemia
  • E70.29 Other disorders of tyrosine metabolism
  • E70.30 Albinism, unspecified
  • E70.310 X-linked ocular albinism
  • E70.311 Autosomal recessive ocular albinism
  • E70.318 Other ocular albinism
  • E70.319 Ocular albinism, unspecified
  • E70.320 Tyrosinase negative oculocutaneous albinism
  • E70.321 Tyrosinase positive oculocutaneous albinism
  • E70.328 Other oculocutaneous albinism
  • E70.329 Oculocutaneous albinism, unspecified
  • E70.330 Chediak-Higashi syndrome
  • E70.331 Hermansky-Pudlak syndrome
  • E70.338 Other albinism with hematologic abnormality
  • E70.339 Albinism with hematologic abnormality, unspecified
  • E70.39 Other specified albinism
  • E70.40 Disorders of histidine metabolism, unspecified
  • E70.41 Histidinemia
  • E70.49 Other disorders of histidine metabolism
  • E70.5 Disorders of tryptophan metabolism
  • E70.81 Aromatic L-amino acid decarboxylase deficiency
  • E70.89 Other disorders of aromatic amino-acid metabolism
  • E70.9 Disorder of aromatic amino-acid metabolism, unspecified
  • E71.0 Maple-syrup-urine disease
  • E71.110 Isovaleric acidemia
  • E71.111 3-methylglutaconic aciduria
  • E71.118 Other branched-chain organic acidurias
  • E71.120 Methylmalonic acidemia
  • E71.121 Propionic acidemia
  • E71.128 Other disorders of propionate metabolism
  • E71.19 Other disorders of branched-chain amino-acid metabolism
  • E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
  • E71.30 Disorder of fatty-acid metabolism, unspecified
  • E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
  • E71.311 Medium chain acyl CoA dehydrogenase deficiency
  • E71.312 Short chain acyl CoA dehydrogenase deficiency
  • E71.313 Glutaric aciduria type II
  • E71.314 Muscle carnitine palmitoyltransferase deficiency
  • E71.318 Other disorders of fatty-acid oxidation
  • E71.32 Disorders of ketone metabolism
  • E71.39 Other disorders of fatty-acid metabolism
  • E71.40 Disorder of carnitine metabolism, unspecified
  • E71.41 Primary carnitine deficiency
  • E71.42 Carnitine deficiency due to inborn errors of metabolism
  • E71.43 Iatrogenic carnitine deficiency
  • E71.440 Ruvalcaba-Myhre-Smith syndrome
  • E71.448 Other secondary carnitine deficiency
  • E71.50 Peroxisomal disorder, unspecified
  • E71.510 Zellweger syndrome
  • E71.511 Neonatal adrenoleukodystrophy
  • E71.518 Other disorders of peroxisome biogenesis
  • E71.520 Childhood cerebral X-linked adrenoleukodystrophy
  • E71.521 Adolescent X-linked adrenoleukodystrophy
  • E71.522 Adrenomyeloneuropathy
  • E71.528 Other X-linked adrenoleukodystrophy
  • E71.529 X-linked adrenoleukodystrophy, unspecified type
  • E71.53 Other group 2 peroxisomal disorders
  • E71.540 Rhizomelic chondrodysplasia punctata
  • E71.541 Zellweger-like syndrome
  • E71.542 Other group 3 peroxisomal disorders
  • E71.548 Other peroxisomal disorders
  • E72.00 Disorders of amino-acid transport, unspecified
  • E72.01 Cystinuria
  • E72.02 Hartnup's disease
  • E72.03 Lowe's syndrome
  • E72.04 Cystinosis
  • E72.09 Other disorders of amino-acid transport
  • E72.10 Disorders of sulfur-bearing amino-acid metabolism, unspecified
  • E72.11 Homocystinuria
  • E72.12 Methylenetetrahydrofolate reductase deficiency
  • E72.19 Other disorders of sulfur-bearing amino-acid metabolism
  • E72.20 Disorder of urea cycle metabolism, unspecified
  • E72.21 Argininemia
  • E72.22 Arginosuccinic aciduria
  • E72.23 Citrullinemia
  • E72.29 Other disorders of urea cycle metabolism
  • E72.3 Disorders of lysine and hydroxylysine metabolism
  • E72.4 Disorders of ornithine metabolism
  • E72.50 Disorder of glycine metabolism, unspecified
  • E72.51 Non-ketotic hyperglycinemia
  • E72.52 Trimethylaminuria
  • E72.53 Primary hyperoxaluria
  • E72.59 Other disorders of glycine metabolism
  • E72.81 Disorders of gamma aminobutyric acid metabolism
  • E72.89 Other specified disorders of amino-acid metabolism
  • E72.9 Disorder of amino-acid metabolism, unspecified
  • E74.00 Glycogen storage disease, unspecified
  • E74.01 von Gierke disease

Operating / Non-Operating Room Procedures

There are no procedure / surgical codes for MS-DRG 642