| E70 |
Disorders of aromatic amino-acid metabolism [+]
|
|
| E70.0 |
Classical phenylketonuria
|
| E70.1 |
Other hyperphenylalaninemias
|
| E70.2 |
Disorders of tyrosine metabolism [+]
| Excludes 1: |
transitory tyrosinemia of newborn (P74.5)
|
|
|
| E70.20 |
Disorder of tyrosine metabolism, unspecified
|
| E70.21 |
Tyrosinemia
Hypertyrosinemia
|
| E70.29 |
Other disorders of tyrosine metabolism
Alkaptonuria
Ochronosis
|
|
| E70.3 |
Albinism [+]
|
|
| E70.30 |
Albinism, unspecified
|
| E70.31 |
Ocular albinism [+]
|
|
| E70.310 |
X-linked ocular albinism
|
| E70.311 |
Autosomal recessive ocular albinism
|
| E70.318 |
Other ocular albinism
|
| E70.319 |
Ocular albinism, unspecified
|
|
| E70.32 |
Oculocutaneous albinism [+]
| Excludes 1: |
Chediak-Higashi syndrome (E70.330)
Hermansky-Pudlak syndrome (E70.331)
|
|
|
| E70.320 |
Tyrosinase negative oculocutaneous albinism
Albinism I
Oculocutaneous albinism ty-neg
|
| E70.321 |
Tyrosinase positive oculocutaneous albinism
Albinism II
Oculocutaneous albinism ty-pos
|
| E70.328 |
Other oculocutaneous albinism
Cross syndrome
|
| E70.329 |
Oculocutaneous albinism, unspecified
|
|
| E70.33 |
Albinism with hematologic abnormality [+]
|
|
| 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
Piebaldism
|
|
| E70.4 |
Disorders of histidine metabolism [+]
|
|
| 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 [+]
|
|
| E70.81 |
Aromatic L-amino acid decarboxylase deficiency
AADC deficiency
|
| E70.89 |
Other disorders of aromatic amino-acid metabolism
|
|
| E70.9 |
Disorder of aromatic amino-acid metabolism, unspecified
|
|
| E71 |
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism [+]
|
|
| E71.0 |
Maple-syrup-urine disease
|
| E71.1 |
Other disorders of branched-chain amino-acid metabolism [+]
|
|
| E71.11 |
Branched-chain organic acidurias [+]
|
|
| E71.110 |
Isovaleric acidemia
|
| E71.111 |
3-methylglutaconic aciduria
|
| E71.118 |
Other branched-chain organic acidurias
|
|
| E71.12 |
Disorders of propionate metabolism [+]
|
|
| 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
Hyperleucine-isoleucinemia
Hypervalinemia
|
|
| E71.2 |
Disorder of branched-chain amino-acid metabolism, unspecified
|
| E71.3 |
Disorders of fatty-acid metabolism [+]
| Excludes 1: |
peroxisomal disorders (E71.5)
Refsum's disease (G60.1)
Schilder's disease (G37.0)
|
| Excludes 2: |
carnitine deficiency due to inborn error of metabolism (E71.42)
|
|
|
| E71.30 |
Disorder of fatty-acid metabolism, unspecified
|
| E71.31 |
Disorders of fatty-acid oxidation [+]
|
|
| E71.310 |
Long chain/very long chain acyl CoA dehydrogenase deficiency
LCAD deficiency
VLCAD deficiency
|
| E71.311 |
Medium chain acyl CoA dehydrogenase deficiency
MCAD deficiency
|
| E71.312 |
Short chain acyl CoA dehydrogenase deficiency
SCAD deficiency
|
| E71.313 |
Glutaric aciduria type II
Glutaric aciduria type II A
Glutaric aciduria type II B
Glutaric aciduria type II C
| Excludes 1: |
glutaric aciduria (type 1) NOS (E72.3)
|
|
| 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
|
|
| E71.4 |
Disorders of carnitine metabolism [+]
| Excludes 1: |
Muscle carnitine palmitoyltransferase deficiency (E71.314)
|
|
|
| E71.40 |
Disorder of carnitine metabolism, unspecified
|
| E71.41 |
Primary carnitine deficiency
|
| E71.42 |
Carnitine deficiency due to inborn errors of metabolism
| Code also |
associated inborn error or metabolism
|
|
| E71.43 |
Iatrogenic carnitine deficiency
Carnitine deficiency due to hemodialysis
Carnitine deficiency due to Valproic acid therapy
|
| E71.44 |
Other secondary carnitine deficiency [+]
|
|
| E71.440 |
Ruvalcaba-Myhre-Smith syndrome
|
| E71.448 |
Other secondary carnitine deficiency
|
|
|
| E71.5 |
Peroxisomal disorders [+]
| Excludes 1: |
Schilder's disease (G37.0)
|
|
|
| E71.50 |
Peroxisomal disorder, unspecified
|
| E71.51 |
Disorders of peroxisome biogenesis [+]
Group 1 peroxisomal disorders
| Excludes 1: |
Refsum's disease (G60.1)
|
|
|
| E71.510 |
Zellweger syndrome
|
| E71.511 |
Neonatal adrenoleukodystrophy
| Excludes 1: |
X-linked adrenoleukodystrophy (E71.42-)
|
|
| E71.518 |
Other disorders of peroxisome biogenesis
|
|
| E71.52 |
X-linked adrenoleukodystrophy [+]
|
|
| E71.520 |
Childhood cerebral X-linked adrenoleukodystrophy
|
| E71.521 |
Adolescent X-linked adrenoleukodystrophy
|
| E71.522 |
Adrenomyeloneuropathy
|
| E71.528 |
Other X-linked adrenoleukodystrophy
Addison only phenotype adrenoleukodystrophy
Addison-Schilder adrenoleukodystrophy
|
| E71.529 |
X-linked adrenoleukodystrophy, unspecified type
|
|
| E71.53 |
Other group 2 peroxisomal disorders
|
| E71.54 |
Other peroxisomal disorders [+]
|
|
| E71.540 |
Rhizomelic chondrodysplasia punctata
| Excludes 1: |
chondrodysplasia punctata NOS (Q77.3)
|
|
| E71.541 |
Zellweger-like syndrome
|
| E71.542 |
Other group 3 peroxisomal disorders
|
| E71.548 |
Other peroxisomal disorders
|
|
|
|
| E72 |
Other disorders of amino-acid metabolism [+]
| Excludes 1: |
disorders of:
aromatic amino-acid metabolism (E70.-)
branched-chain amino-acid metabolism (E71.0-E71.2)
fatty-acid metabolism (E71.3)
purine and pyrimidine metabolism (E79.-)
gout (M1A.-, M10.-)
|
|
|
| E72.0 |
Disorders of amino-acid transport [+]
| Excludes 1: |
disorders of tryptophan metabolism (E70.5)
|
|
|
| E72.00 |
Disorders of amino-acid transport, unspecified
|
| E72.01 |
Cystinuria
|
| E72.02 |
Hartnup's disease
|
| E72.03 |
Lowe's syndrome
| Use additional |
code for associated glaucoma (H42) |
|
| E72.04 |
Cystinosis
Fanconi (-de Toni) (-Debré) syndrome with cystinosis
| Excludes 1: |
Fanconi (-de Toni) (-Debré) syndrome without cystinosis (E72.09)
|
|
| E72.09 |
Other disorders of amino-acid transport
Fanconi (-de Toni) (-Debré) syndrome, unspecified
|
|
| E72.1 |
Disorders of sulfur-bearing amino-acid metabolism [+]
| Excludes 1: |
cystinosis (E72.04)
cystinuria (E72.01)
transcobalamin II deficiency (D51.2)
|
|
|
| E72.10 |
Disorders of sulfur-bearing amino-acid metabolism, unspecified
|
| E72.11 |
Homocystinuria
Cystathionine synthase deficiency
|
| E72.12 |
Methylenetetrahydrofolate reductase deficiency
|
| E72.19 |
Other disorders of sulfur-bearing amino-acid metabolism
Cystathioninuria
Methioninemia
Sulfite oxidase deficiency
|
|
| E72.2 |
Disorders of urea cycle metabolism [+]
| Excludes 1: |
disorders of ornithine metabolism (E72.4)
|
|
|
| E72.20 |
Disorder of urea cycle metabolism, unspecified
Hyperammonemia
| Excludes 1: |
hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4
transient hyperammonemia of newborn (P74.6)
|
|
| 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
Glutaric aciduria NOS
Glutaric aciduria (type I)
Hydroxylysinemia
Hyperlysinemia
| Excludes 1: |
glutaric aciduria type II (E71.313)
Refsum's disease (G60.1)
Zellweger syndrome (E71.510)
|
|
| E72.4 |
Disorders of ornithine metabolism
Hyperammonemia-Hyperornithinemia-Homocitrullinemia syndrome
Ornithinemia (types I, II)
Ornithine transcarbamylase deficiency
| Excludes 1: |
hereditary choroidal dystrophy (H31.2-)
|
|
| E72.5 |
Disorders of glycine metabolism [+]
|
|
| E72.50 |
Disorder of glycine metabolism, unspecified
|
| E72.51 |
Non-ketotic hyperglycinemia
|
| E72.52 |
Trimethylaminuria
|
| E72.53 |
Primary hyperoxaluria
Oxalosis
Oxaluria
|
| E72.59 |
Other disorders of glycine metabolism
D-glycericacidemia
Hyperhydroxyprolinemia
Hyperprolinemia (types I, II)
Sarcosinemia
|
|
| E72.8 |
Other specified disorders of amino-acid metabolism [+]
|
|
| E72.81 |
Disorders of gamma aminobutyric acid metabolism
4-hydroxybutyric aciduria
Disorders of GABA metabolism
GABA metabolic defect
GABA transaminase deficiency
GABA-T deficiency
Gamma-hydroxybutyric aciduria
SSADHD
Succinic semialdehyde dehydrogenase deficiency
|
| E72.89 |
Other specified disorders of amino-acid metabolism
Disorders of beta-amino-acid metabolism
Disorders of gamma-glutamyl cycle
|
|
| E72.9 |
Disorder of amino-acid metabolism, unspecified
|
|
| E73 |
Lactose intolerance [+]
|
|
| E73.0 |
Congenital lactase deficiency
|
| E73.1 |
Secondary lactase deficiency
|
| E73.8 |
Other lactose intolerance
|
| E73.9 |
Lactose intolerance, unspecified
|
|
| E74 |
Other disorders of carbohydrate metabolism [+]
| Excludes 1: |
diabetes mellitus (E08-E13)
hypoglycemia NOS (E16.2)
increased secretion of glucagon (E16.3)
mucopolysaccharidosis (E76.0-E76.3)
|
|
|
| E74.0 |
Glycogen storage disease [+]
|
|
| E74.00 |
Glycogen storage disease, unspecified
|
| E74.01 |
von Gierke disease
Type I glycogen storage disease
|
| E74.02 |
Pompe disease
Cardiac glycogenosis
Type II glycogen storage disease
|
| E74.03 |
Cori disease
Forbes disease
Type III glycogen storage disease
|
| E74.04 |
McArdle disease
Type V glycogen storage disease
|
| E74.05 |
Lysosome-associated membrane protein 2 [LAMP2] deficiency
Danon disease
| Code also |
, if applicable, associated manifestations such as:
dilated cardiomyopathy (I42.0)
obstructive hypertrophic cardiomyopathy (I42.1)
|
|
| E74.09 |
Other glycogen storage disease
Andersen disease
Glycogen storage disease, types 0, IV, VI-XI
Hers disease
Liver phosphorylase deficiency
Muscle phosphofructokinase deficiency
Tauri disease
|
|
| E74.1 |
Disorders of fructose metabolism [+]
| Excludes 1: |
muscle phosphofructokinase deficiency (E74.09)
|
|
|
| E74.10 |
Disorder of fructose metabolism, unspecified
|
| E74.11 |
Essential fructosuria
Fructokinase deficiency
|
| E74.12 |
Hereditary fructose intolerance
Fructosemia
|
| E74.19 |
Other disorders of fructose metabolism
Fructose-1, 6-diphosphatase deficiency
|
|
| E74.2 |
Disorders of galactose metabolism [+]
|
|
| E74.20 |
Disorders of galactose metabolism, unspecified
|
| E74.21 |
Galactosemia
|
| E74.29 |
Other disorders of galactose metabolism
Galactokinase deficiency
|
|
| E74.3 |
Other disorders of intestinal carbohydrate absorption [+]
| Excludes 2: |
lactose intolerance (E73.-)
|
|
|
| E74.31 |
Sucrase-isomaltase deficiency
|
| E74.39 |
Other disorders of intestinal carbohydrate absorption
Disorder of intestinal carbohydrate absorption NOS
Glucose-galactose malabsorption
Sucrase deficiency
|
|
| E74.4 |
Disorders of pyruvate metabolism and gluconeogenesis
Deficiency of phosphoenolpyruvate carboxykinase
Deficiency of pyruvate carboxylase
Deficiency of pyruvate dehydrogenase
| Excludes 1: |
disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
Leigh's syndrome (G31.82)
|
|
| E74.8 |
Other specified disorders of carbohydrate metabolism [+]
|
|
| E74.81 |
Disorders of glucose transport, not elsewhere classified [+]
|
|
| E74.810 |
Glucose transporter protein type 1 deficiency
De Vivo syndrome
Glucose transport defect, blood-brain barrier
Glut1 deficiency
GLUT1 deficiency syndrome 1, infantile onset
GLUT1 deficiency syndrome 2, childhood onset
|
| E74.818 |
Other disorders of glucose transport
(Familial) renal glycosuria
|
| E74.819 |
Disorders of glucose transport, unspecified
|
|
| E74.89 |
Other specified disorders of carbohydrate metabolism
Essential pentosuria
|
|
| E74.9 |
Disorder of carbohydrate metabolism, unspecified
|
|
| E75 |
Disorders of sphingolipid metabolism and other lipid storage disorders [+]
| Excludes 1: |
mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)
|
|
|
| E75.0 |
GM2 gangliosidosis [+]
|
|
| E75.00 |
GM2 gangliosidosis, unspecified
|
| E75.01 |
Sandhoff disease
|
| E75.02 |
Tay-Sachs disease
|
| E75.09 |
Other GM2 gangliosidosis
Adult GM2 gangliosidosis
Juvenile GM2 gangliosidosis
|
|
| E75.1 |
Other and unspecified gangliosidosis [+]
|
|
| E75.10 |
Unspecified gangliosidosis
Gangliosidosis NOS
|
| E75.11 |
Mucolipidosis IV
|
| E75.19 |
Other gangliosidosis
GM1 gangliosidosis
GM3 gangliosidosis
|
|
| E75.2 |
Other sphingolipidosis [+]
| Excludes 1: |
adrenoleukodystrophy [Addison-Schilder] (E71.528)
|
|
|
| E75.21 |
Fabry (-Anderson) disease
|
| E75.22 |
Gaucher disease
|
| E75.23 |
Krabbe disease
|
| E75.24 |
Niemann-Pick disease [+]
Acid sphingomyelinase deficiency (ASMD)
|
|
| E75.240 |
Niemann-Pick disease type A
Acid sphingomyelinase deficiency type A (ASMD type A)
Infantile neurovisceral acid sphingomyelinase deficiency
|
| E75.241 |
Niemann-Pick disease type B
Acid sphingomyelinase deficiency type B (ASMD type B)
Chronic visceral acid sphingomyelinase deficiency
|
| E75.242 |
Niemann-Pick disease type C
|
| E75.243 |
Niemann-Pick disease type D
|
| E75.244 |
Niemann-Pick disease type A/B
Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
Chronic neurovisceral acid sphingomyelinase deficiency
|
| E75.248 |
Other Niemann-Pick disease
|
| E75.249 |
Niemann-Pick disease, unspecified
Acid sphingomyelinase deficiency (ASMD) NOS
|
|
| E75.25 |
Metachromatic leukodystrophy
|
| E75.26 |
Sulfatase deficiency
Multiple sulfatase deficiency (MSD)
|
| E75.27 |
Pelizaeus-Merzbacher disease
|
| E75.28 |
Canavan disease
|
| E75.29 |
Other sphingolipidosis
Farber's syndrome
Sulfatide lipidosis
|
|
| E75.3 |
Sphingolipidosis, unspecified
|
| E75.4 |
Neuronal ceroid lipofuscinosis
Batten disease
Bielschowsky-Jansky disease
Kufs disease
Spielmeyer-Vogt disease
|
| E75.5 |
Other lipid storage disorders
Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
Wolman's disease
|
| E75.6 |
Lipid storage disorder, unspecified
|
|
| E76 |
Disorders of glycosaminoglycan metabolism [+]
|
|
| E76.0 |
Mucopolysaccharidosis, type I [+]
|
|
|
| E76.1 |
Mucopolysaccharidosis, type II
Hunter's syndrome
|
| E76.2 |
Other mucopolysaccharidoses [+]
|
|
| E76.21 |
Morquio mucopolysaccharidoses [+]
|
|
| E76.210 |
Morquio A mucopolysaccharidoses
Classic Morquio syndrome
Morquio syndrome A
Mucopolysaccharidosis, type IVA
|
| E76.211 |
Morquio B mucopolysaccharidoses
Morquio-like mucopolysaccharidoses
Morquio-like syndrome
Morquio syndrome B
Mucopolysaccharidosis, type IVB
|
| E76.219 |
Morquio mucopolysaccharidoses, unspecified
Morquio syndrome
Mucopolysaccharidosis, type IV
|
|
| E76.22 |
Sanfilippo mucopolysaccharidoses
Mucopolysaccharidosis, type III (A) (B) (C) (D)
Sanfilippo A syndrome
Sanfilippo B syndrome
Sanfilippo C syndrome
Sanfilippo D syndrome
|
| E76.29 |
Other mucopolysaccharidoses
beta-Glucuronidase deficiency
Maroteaux-Lamy (mild) (severe) syndrome
Mucopolysaccharidosis, types VI, VII
|
|
| E76.3 |
Mucopolysaccharidosis, unspecified
|
| E76.8 |
Other disorders of glucosaminoglycan metabolism
|
| E76.9 |
Glucosaminoglycan metabolism disorder, unspecified
|
|
| E77 |
Disorders of glycoprotein metabolism [+]
|
|
| E77.0 |
Defects in post-translational modification of lysosomal enzymes
Mucolipidosis II [I-cell disease]
Mucolipidosis III [pseudo-Hurler polydystrophy]
|
| E77.1 |
Defects in glycoprotein degradation
Aspartylglucosaminuria
Fucosidosis
Mannosidosis
Sialidosis [mucolipidosis I]
|
| E77.8 |
Other disorders of glycoprotein metabolism
|
| E77.9 |
Disorder of glycoprotein metabolism, unspecified
|
|
| E78 |
Disorders of lipoprotein metabolism and other lipidemias [+]
| Excludes 1: |
sphingolipidosis (E75.0-E75.3)
|
|
|
| E78.0 |
Pure hypercholesterolemia [+]
|
|
| E78.00 |
Pure hypercholesterolemia, unspecified
Fredrickson's hyperlipoproteinemia, type IIa
Hyperbetalipoproteinemia
Low-density-lipoprotein-type [LDL] hyperlipoproteinemia
(Pure) hypercholesterolemia NOS
|
| E78.01 |
Familial hypercholesterolemia
|
|
| E78.1 |
Pure hyperglyceridemia
Elevated fasting triglycerides
Endogenous hyperglyceridemia
Fredrickson's hyperlipoproteinemia, type IV
Hyperlipidemia, group B
Hyperprebetalipoproteinemia
Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinemia
|
| E78.2 |
Mixed hyperlipidemia
Broad- or floating-betalipoproteinemia
Combined hyperlipidemia NOS
Elevated cholesterol with elevated triglycerides NEC
Fredrickson's hyperlipoproteinemia, type IIb or III
Hyperbetalipoproteinemia with prebetalipoproteinemia
Hypercholesteremia with endogenous hyperglyceridemia
Hyperlipidemia, group C
Tubo-eruptive xanthoma
Xanthoma tuberosum
| Excludes 1: |
cerebrotendinous cholesterosis [van Bogaert-Scherer- Epstein] (E75.5)
familial combined hyperlipidemia (E78.49)
|
|
| E78.3 |
Hyperchylomicronemia
Chylomicron retention disease
Fredrickson's hyperlipoproteinemia, type I or V
Hyperlipidemia, group D
Mixed hyperglyceridemia
|
| E78.4 |
Other hyperlipidemia [+]
|
|
| E78.41 |
Elevated Lipoprotein(a)
Elevated Lp(a)
|
| E78.49 |
Other hyperlipidemia
Familial combined hyperlipidemia
|
|
| E78.5 |
Hyperlipidemia, unspecified
|
| E78.6 |
Lipoprotein deficiency
Abetalipoproteinemia
Depressed HDL cholesterol
High-density lipoprotein deficiency
Hypoalphalipoproteinemia
Hypobetalipoproteinemia (familial)
Lecithin cholesterol acyltransferase deficiency
Tangier disease
|
| E78.7 |
Disorders of bile acid and cholesterol metabolism [+]
| Excludes 1: |
Niemann-Pick disease type C (E75.242)
|
|
|
| E78.70 |
Disorder of bile acid and cholesterol metabolism, unspecified
|
| E78.71 |
Barth syndrome
|
| E78.72 |
Smith-Lemli-Opitz syndrome
|
| E78.79 |
Other disorders of bile acid and cholesterol metabolism
|
|
| E78.8 |
Other disorders of lipoprotein metabolism [+]
|
|
| E78.81 |
Lipoid dermatoarthritis
|
| E78.89 |
Other lipoprotein metabolism disorders
|
|
| E78.9 |
Disorder of lipoprotein metabolism, unspecified
|
|
| E79 |
Disorders of purine and pyrimidine metabolism [+]
| Excludes 1: |
Ataxia-telangiectasia (Q87.19)
Bloom's syndrome (Q82.8)
Cockayne's syndrome (Q87.19)
calculus of kidney (N20.0)
combined immunodeficiency disorders (D81.-)
Fanconi's anemia (D61.09)
gout (M1A.-, M10.-)
orotaciduric anemia (D53.0)
progeria (E34.8)
Werner's syndrome (E34.8)
xeroderma pigmentosum (Q82.1)
|
|
|
| E79.0 |
Hyperuricemia without signs of inflammatory arthritis and tophaceous disease
Asymptomatic hyperuricemia
|
| E79.1 |
Lesch-Nyhan syndrome
HGPRT deficiency
|
| E79.2 |
Myoadenylate deaminase deficiency
|
| E79.8 |
Other disorders of purine and pyrimidine metabolism [+]
|
|
| E79.81 |
Aicardi-Goutières syndrome
|
| E79.82 |
Hereditary xanthinuria
|
| E79.89 |
Other specified disorders of purine and pyrimidine metabolism
|
|
| E79.9 |
Disorder of purine and pyrimidine metabolism, unspecified
|
|
| E80 |
Disorders of porphyrin and bilirubin metabolism [+]
| Includes: |
defects of catalase and peroxidase
|
|
|
| E80.0 |
Hereditary erythropoietic porphyria
Congenital erythropoietic porphyria
Erythropoietic protoporphyria
|
| E80.1 |
Porphyria cutanea tarda
|
| E80.2 |
Other and unspecified porphyria [+]
|
|
| E80.20 |
Unspecified porphyria
Porphyria NOS
|
| E80.21 |
Acute intermittent (hepatic) porphyria
|
| E80.29 |
Other porphyria
Hereditary coproporphyria
|
|
| E80.3 |
Defects of catalase and peroxidase
Acatalasia [Takahara]
|
| E80.4 |
Gilbert syndrome
|
| E80.5 |
Crigler-Najjar syndrome
|
| E80.6 |
Other disorders of bilirubin metabolism
Dubin-Johnson syndrome
Rotor's syndrome
|
| E80.7 |
Disorder of bilirubin metabolism, unspecified
|
|
| E83 |
Disorders of mineral metabolism [+]
| Excludes 1: |
dietary mineral deficiency (E58-E61)
parathyroid disorders (E20-E21)
vitamin D deficiency (E55.-)
|
|
|
| E83.0 |
Disorders of copper metabolism [+]
|
|
| E83.00 |
Disorder of copper metabolism, unspecified
|
| E83.01 |
Wilson's disease
| Code also |
associated Kayser Fleischer ring (H18.04-)
|
|
| E83.09 |
Other disorders of copper metabolism
Menkes' (kinky hair) (steely hair) disease
|
|
| E83.1 |
Disorders of iron metabolism [+]
| Excludes 1: |
iron deficiency anemia (D50.-)
sideroblastic anemia (D64.0-D64.3)
|
|
|
| E83.10 |
Disorder of iron metabolism, unspecified
|
| E83.11 |
Hemochromatosis [+]
| Excludes 1: |
GALD (P78.84)
Gestational alloimmune liver disease (P78.84)
Neonatal hemochromatosis (P78.84)
|
|
|
| E83.110 |
Hereditary hemochromatosis
Bronzed diabetes
Pigmentary cirrhosis (of liver)
Primary (hereditary) hemochromatosis
|
| E83.111 |
Hemochromatosis due to repeated red blood cell transfusions
Iron overload due to repeated red blood cell transfusions
Transfusion (red blood cell) associated hemochromatosis
|
| E83.118 |
Other hemochromatosis
|
| E83.119 |
Hemochromatosis, unspecified
|
|
| E83.19 |
Other disorders of iron metabolism
| Use additional |
code, if applicable, for idiopathic pulmonary hemosiderosis (J84.03) |
|
|
| E83.2 |
Disorders of zinc metabolism
Acrodermatitis enteropathica
|
| E83.3 |
Disorders of phosphorus metabolism and phosphatases [+]
| Excludes 1: |
adult osteomalacia (M83.-)
osteoporosis (M80.-)
|
|
|
| E83.30 |
Disorder of phosphorus metabolism, unspecified
|
| E83.31 |
Familial hypophosphatemia
Vitamin D-resistant osteomalacia
Vitamin D-resistant rickets
| Excludes 1: |
vitamin D-deficiency rickets (E55.0)
|
|
| E83.32 |
Hereditary vitamin D-dependent rickets (type 1) (type 2)
25-hydroxyvitamin D 1-alpha-hydroxylase deficiency
Pseudovitamin D deficiency
Vitamin D receptor defect
|
| E83.39 |
Other disorders of phosphorus metabolism
Acid phosphatase deficiency
Hypophosphatasia
|
|
| E83.4 |
Disorders of magnesium metabolism [+]
|
|
| E83.40 |
Disorders of magnesium metabolism, unspecified
|
| E83.41 |
Hypermagnesemia
|
| E83.42 |
Hypomagnesemia
|
| E83.49 |
Other disorders of magnesium metabolism
|
|
| E83.5 |
Disorders of calcium metabolism [+]
| Excludes 1: |
autoimmune hypoparathyroidism (E20.812)
autosomal dominant hypocalcemia (E20.810)
chondrocalcinosis (M11.1-M11.2)
hungry bone syndrome (E83.81)
hyperparathyroidism (E21.0-E21.3)
secondary hypoparathyroidism in diseases classified elsewhere (E20.811)
|
|
|
| E83.50 |
Unspecified disorder of calcium metabolism
|
| E83.51 |
Hypocalcemia
|
| E83.52 |
Hypercalcemia
Familial hypocalciuric hypercalcemia
|
| E83.59 |
Other disorders of calcium metabolism
|
|
| E83.8 |
Other disorders of mineral metabolism [+]
|
|
| E83.81 |
Hungry bone syndrome
|
| E83.89 |
Other disorders of mineral metabolism
|
|
| E83.9 |
Disorder of mineral metabolism, unspecified
|
|
| E84 |
Cystic fibrosis [+]
| Includes: |
mucoviscidosis
|
| Code also |
exocrine pancreatic insufficiency (K86.81)
|
|
|
| E84.0 |
Cystic fibrosis with pulmonary manifestations
| Use additional |
code to identify any infectious organism present, such as: |
| Use additional |
Pseudomonas (B96.5) |
|
| E84.1 |
Cystic fibrosis with intestinal manifestations [+]
|
|
| E84.11 |
Meconium ileus in cystic fibrosis
| Excludes 1: |
meconium ileus not due to cystic fibrosis (P76.0)
|
|
| E84.19 |
Cystic fibrosis with other intestinal manifestations
Distal intestinal obstruction syndrome
|
|
| E84.8 |
Cystic fibrosis with other manifestations
|
| E84.9 |
Cystic fibrosis, unspecified
|
|
| E85 |
Amyloidosis [+]
| Excludes 2: |
Alzheimer's disease (G30.0-)
|
|
|
| E85.0 |
Non-neuropathic heredofamilial amyloidosis
Hereditary amyloid nephropathy
| Code also |
associated disorders, such as:
autoinflammatory syndromes (M04.-)
|
| Excludes 2: |
Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)
|
|
| E85.1 |
Neuropathic heredofamilial amyloidosis
Amyloid polyneuropathy (Portuguese)
Transthyretin-related (ATTR) familial amyloid polyneuropathy
|
| E85.2 |
Heredofamilial amyloidosis, unspecified
|
| E85.3 |
Secondary systemic amyloidosis
Hemodialysis-associated amyloidosis
|
| E85.4 |
Organ-limited amyloidosis
Localized amyloidosis
Transthyretin-related (ATTR) familial amyloid cardiomyopathy
|
| E85.8 |
Other amyloidosis [+]
|
|
| E85.81 |
Light chain (AL) amyloidosis
|
| E85.82 |
Wild-type transthyretin-related (ATTR) amyloidosis
Senile systemic amyloidosis (SSA)
|
| E85.89 |
Other amyloidosis
|
|
| E85.9 |
Amyloidosis, unspecified
|
|
| E86 |
Volume depletion [+]
| Use additional |
code(s) for any associated disorders of electrolyte and acid-base balance (E87.-) |
| Excludes 1: |
dehydration of newborn (P74.1)
postprocedural hypovolemic shock (T81.19)
traumatic hypovolemic shock (T79.4)
|
| Excludes 2: |
hypovolemic shock NOS (R57.1)
|
|
|
| E86.0 |
Dehydration
|
| E86.1 |
Hypovolemia
Depletion of volume of plasma
|
| E86.9 |
Volume depletion, unspecified
|
|
| E87 |
Other disorders of fluid, electrolyte and acid-base balance [+]
| Excludes 1: |
diabetes insipidus (E23.2)
electrolyte imbalance associated with hyperemesis gravidarum (O21.1)
electrolyte imbalance following ectopic or molar pregnancy (O08.5)
familial periodic paralysis (G72.3)
metabolic acidemia in newborn, unspecified (P19.9)
|
|
|
| E87.0 |
Hyperosmolality and hypernatremia
Sodium [Na] excess
Sodium [Na] overload
| Excludes 1: |
diabetes with hyperosmolarity (E08, E09, E11, E13 with final characters .00 or .01)
|
|
| E87.1 |
Hypo-osmolality and hyponatremia
Sodium [Na] deficiency
| Excludes 1: |
syndrome of inappropriate secretion of antidiuretic hormone (E22.2)
|
|
| E87.2 |
Acidosis [+]
| Excludes 1: |
diabetic acidosis - see categories E08-E10, E11, E13 with ketoacidosis
|
|
|
| E87.20 |
Acidosis, unspecified
Lactic acidosis NOS
Metabolic acidosis NOS
| Code also |
, if applicable, respiratory failure with hypercapnia (J96. with 5th character 2)
|
|
| E87.21 |
Acute metabolic acidosis
Acute lactic acidosis
|
| E87.22 |
Chronic metabolic acidosis
Chronic lactic acidosis
| Code first |
underlying etiology, if applicable
|
|
| E87.29 |
Other acidosis
Respiratory acidosis NOS
| Excludes 2: |
acute respiratory acidosis (J96.02)
chronic respiratory acidosis (J96.12)
|
|
|
| E87.3 |
Alkalosis
Alkalosis NOS
Metabolic alkalosis
Respiratory alkalosis
|
| E87.4 |
Mixed disorder of acid-base balance
|
| E87.5 |
Hyperkalemia
Potassium [K] excess
Potassium [K] overload
|
| E87.6 |
Hypokalemia
Potassium [K] deficiency
|
| E87.7 |
Fluid overload [+]
| Excludes 1: |
edema NOS (R60.9)
fluid retention (R60.9)
|
|
|
| E87.70 |
Fluid overload, unspecified
|
| E87.71 |
Transfusion associated circulatory overload
Fluid overload due to transfusion (blood) (blood components)
TACO
|
| E87.79 |
Other fluid overload
|
|
| E87.8 |
Other disorders of electrolyte and fluid balance, not elsewhere classified
Electrolyte imbalance NOS
Hyperchloremia
Hypochloremia
|
|
| E88 |
Other and unspecified metabolic disorders [+]
| Use additional |
codes for associated conditions |
| Excludes 1: |
histiocytosis X (chronic) (C96.6)
|
|
|
| E88.0 |
Disorders of plasma-protein metabolism, not elsewhere classified [+]
| Excludes 1: |
monoclonal gammopathy (of undetermined significance) (D47.2)
polyclonal hypergammaglobulinemia (D89.0)
Waldenström macroglobulinemia (C88.0)
|
| Excludes 2: |
disorder of lipoprotein metabolism (E78.-)
|
|
|
| E88.01 |
Alpha-1-antitrypsin deficiency
AAT deficiency
|
| E88.02 |
Plasminogen deficiency
Dysplasminogenemia
Hypoplasminogenemia
Type 1 plasminogen deficiency
Type 2 plasminogen deficiency
| Code also |
, if applicable, ligneous conjunctivitis (H10.51)
|
| Use additional |
code for associated findings, such as: |
| Use additional |
hydrocephalus (G91.4) |
| Use additional |
otitis media (H67.-) |
| Use additional |
respiratory disorder related to plasminogen deficiency (J99) |
|
| E88.09 |
Other disorders of plasma-protein metabolism, not elsewhere classified
Bisalbuminemia
|
|
| E88.1 |
Lipodystrophy, not elsewhere classified
Lipodystrophy NOS
| Excludes 1: |
Whipple's disease (K90.81)
|
|
| E88.2 |
Lipomatosis, not elsewhere classified
Lipomatosis NOS
Lipomatosis (Check) dolorosa [Dercum]
|
| E88.3 |
Tumor lysis syndrome
Tumor lysis syndrome (spontaneous)
Tumor lysis syndrome following antineoplastic drug chemotherapy
| Use additional |
code for adverse effect, if applicable, to identify drug (T45.1X5) |
|
| E88.4 |
Mitochondrial metabolism disorders [+]
| Excludes 1: |
disorders of pyruvate metabolism (E74.4)
Kearns-Sayre syndrome (H49.81)
Leber's disease (H47.22)
Leigh's encephalopathy (G31.82)
Mitochondrial myopathy, NEC (G71.3)
Reye's syndrome (G93.7)
|
|
|
| E88.40 |
Mitochondrial metabolism disorder, unspecified
|
| E88.41 |
MELAS syndrome
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
|
| E88.42 |
MERRF syndrome
Myoclonic epilepsy associated with ragged-red fibers
| Code also |
progressive myoclonic epilepsy (G40.3-)
|
|
| E88.43 |
Disorders of mitochondrial tRNA synthetases
|
| E88.49 |
Other mitochondrial metabolism disorders
|
|
| E88.8 |
Other specified metabolic disorders [+]
|
|
| E88.81 |
Metabolic syndrome and other insulin resistance [+]
| Use additional |
codes for associated manifestations, such as: |
| Use additional |
obesity (E66.-) |
|
|
| E88.810 |
Metabolic syndrome
Dysmetabolic syndrome
|
| E88.811 |
Insulin resistance syndrome, Type A
|
| E88.818 |
Other insulin resistance
Insulin resistance syndrome, Type B
|
| E88.819 |
Insulin resistance, unspecified
|
|
| E88.89 |
Other specified metabolic disorders
Launois-Bensaude adenolipomatosis
| Excludes 1: |
adult pulmonary Langerhans cell histiocytosis (J84.82)
|
|
|
| E88.9 |
Metabolic disorder, unspecified
|
| E88.A |
Wasting disease (syndrome) due to underlying condition
Cachexia due to underlying condition
| Code first |
underlying condition
|
| Excludes 1: |
cachexia NOS (R64)
nutritional marasmus (E41)
|
| Excludes 2: |
failure to thrive (R62.51, R62.7)
|
|
|