DRG 642

Inborn and other disorders of metabolism

  • C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
  • C96.6 Unifocal Langerhans-cell histiocytosis
  • D81.3 Adenosine deaminase [ADA] 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
  • E70.8 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
  • E74.02 Pompe disease
  • E74.03 Cori disease
  • E74.04 McArdle disease
  • E74.09 Other glycogen storage disease
  • E74.20 Disorders of galactose metabolism, unspecified
  • E74.21 Galactosemia
  • E74.29 Other disorders of galactose metabolism
  • E74.4 Disorders of pyruvate metabolism and gluconeogenesis
  • E74.8 Other specified disorders of carbohydrate metabolism
  • E74.9 Disorder of carbohydrate metabolism, unspecified
  • E75.21 Fabry (-Anderson) disease
  • E75.22 Gaucher disease
  • E75.240 Niemann-Pick disease type A
  • E75.241 Niemann-Pick disease type B
  • E75.242 Niemann-Pick disease type C
  • E75.243 Niemann-Pick disease type D
  • E75.248 Other Niemann-Pick disease
  • E75.249 Niemann-Pick disease, unspecified
  • E75.3 Sphingolipidosis, unspecified
  • E75.5 Other lipid storage disorders
  • E75.6 Lipid storage disorder, unspecified
  • E76.01 Hurler's syndrome
  • E76.02 Hurler-Scheie syndrome
  • E76.03 Scheie's syndrome
  • E76.1 Mucopolysaccharidosis, type II
  • E76.210 Morquio A mucopolysaccharidoses
  • E76.211 Morquio B mucopolysaccharidoses
  • E76.219 Morquio mucopolysaccharidoses, unspecified
  • E76.22 Sanfilippo mucopolysaccharidoses
  • E76.29 Other mucopolysaccharidoses
  • E76.3 Mucopolysaccharidosis, unspecified
  • E76.8 Other disorders of glucosaminoglycan metabolism
  • E76.9 Glucosaminoglycan metabolism disorder, unspecified
  • E77.0 Defects in post-translational modification of lysosomal enzymes
  • E77.1 Defects in glycoprotein degradation
  • E77.8 Other disorders of glycoprotein metabolism
  • E77.9 Disorder of glycoprotein metabolism, unspecified
  • E78.00 Pure hypercholesterolemia, unspecified
  • E78.01 Familial hypercholesterolemia
  • E78.1 Pure hyperglyceridemia
  • E78.2 Mixed hyperlipidemia
  • E78.3 Hyperchylomicronemia
  • E78.41 Elevated Lipoprotein(a)
  • E78.49 Other hyperlipidemia
  • E78.5 Hyperlipidemia, unspecified
  • E78.6 Lipoprotein deficiency
  • E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
  • E78.79 Other disorders of bile acid and cholesterol metabolism
  • E78.81 Lipoid dermatoarthritis
  • E78.89 Other lipoprotein metabolism disorders
  • E78.9 Disorder of lipoprotein metabolism, unspecified
  • E79.1 Lesch-Nyhan syndrome
  • E79.2 Myoadenylate deaminase deficiency
  • E79.8 Other disorders of purine and pyrimidine metabolism
  • E79.9 Disorder of purine and pyrimidine metabolism, unspecified
  • E80.0 Hereditary erythropoietic porphyria
  • E80.1 Porphyria cutanea tarda
  • E80.20 Unspecified porphyria
  • E80.21 Acute intermittent (hepatic) porphyria
  • E80.29 Other porphyria
  • E80.3 Defects of catalase and peroxidase
  • E83.00 Disorder of copper metabolism, unspecified
  • E83.01 Wilson's disease
  • E83.09 Other disorders of copper metabolism
  • E83.10 Disorder of iron metabolism, unspecified
  • E83.110 Hereditary hemochromatosis
  • E83.111 Hemochromatosis due to repeated red blood cell transfusions
  • E83.118 Other hemochromatosis
  • E83.119 Hemochromatosis, unspecified
  • E83.19 Other disorders of iron metabolism
  • E83.30 Disorder of phosphorus metabolism, unspecified
  • E83.31 Familial hypophosphatemia
  • E83.32 Hereditary vitamin D-dependent rickets (type 1) (type 2)
  • E83.39 Other disorders of phosphorus metabolism
  • E83.89 Other disorders of mineral metabolism
  • E83.9 Disorder of mineral metabolism, unspecified
  • E88.01 Alpha-1-antitrypsin deficiency
  • E88.1 Lipodystrophy, not elsewhere classified
  • E88.2 Lipomatosis, not elsewhere classified
  • E88.40 Mitochondrial metabolism disorder, unspecified
  • E88.41 MELAS syndrome
  • E88.42 MERRF syndrome
  • E88.49 Other mitochondrial metabolism disorders
  • E88.81 Metabolic syndrome
  • E88.89 Other specified metabolic disorders
  • E88.9 Metabolic disorder, unspecified
  • H49.811 Kearns-Sayre syndrome, right eye
  • H49.812 Kearns-Sayre syndrome, left eye
  • H49.813 Kearns-Sayre syndrome, bilateral
  • H49.819 Kearns-Sayre syndrome, unspecified eye