A00-B99 • C00-D48 • D50-D89 • E00-E90 • F00-F99 • G00-G99 • H00-H59 • H60-H95 • I00-I99 • J00-J99 • K00-K93
L00-L99 • M00-M99 • N00-N99 • O00-O99 • P00-P96 • Q00-Q99 • R00-R99 • S00-T98 • V01-Y98 • Z00-Z99 • U00-U99

E70-E90 Metabolic disorders




 
Chapter IV

Endocrine, nutritional and metabolic diseases
(E00-E90)

Metabolic disorders
(E70-E90)

Excludes:
* androgen resistance syndrome (E34.5)
* congenital adrenal hyperplasia (E25.0)
* Ehlers-Danlos syndrome (Q79.6)
* haemolytic anaemias due to enzyme disorders (D55.-)
* Marfan's syndrome (Q87.4)
* 5-alpha-reductase deficiency (E29.1)

E70Disorders of aromatic amino-acid metabolism
Latin: Disordines metabolismi aminoacidorum aromaticorum
E70.0Classical phenylketonuria
E70.1Other hyperphenylalaninaemias
E70.2Disorders of tyrosine metabolism
Alkaptonuria
Hypertyrosinaemia
Ochronosis
Tyrosinaemia
Tyrosinosis
E70.3Albinism
Albinism:
· ocular
· oculocutaneous
Syndrome:
· Chediak(-Steinbrinck)-Higashi
· Cross
· Hermansky-Pudlak
E70.8Other disorders of aromatic amino-acid metabolism
Disorders of:
· histidine metabolism
· tryptophan metabolism
E70.9Disorder of aromatic amino-acid metabolism, unspecified

E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Latin: Disordines metabolismi aminoacidorum catenosorum et metabolismi acidorum stearicorum
E71.0Maple-syrup-urine disease
E71.1Other disorders of branched-chain amino-acid metabolism
Hyperleucine-isoleucinaemia
Hypervalinaemia
Isovaleric acidaemia
Methylmalonic acidaemia
Propionic acidaemia
E71.2Disorder of branched-chain amino-acid metabolism, unspecified
E71.3Disorders of fatty-acid metabolism
Adrenoleukodystrophy [Addison-Schilder]
Muscle carnitine palmityltransferase deficiency
Excludes:Schilder's disease (G37.0)

E72Other disorders of amino-acid metabolism
Latin: Disordines metabolismi aminoacidorum, alii
Excludes:abnormal findings without manifest disease (R70-R89)
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 (M10.-)
E72.0Disorders of amino-acid transport
Cystinosis
Cystinuria
Fanconi(-de Toni)(-Debré) syndrome
Hartnup's disease
Lowe's syndrome
Excludes:disorders of tryptophan metabolism (E70.8)
E72.1Disorders of sulfur-bearing amino-acid metabolism
Cystathioninuria
Homocystinuria
Methioninaemia
Sulfite oxidase deficiency
Excludes:transcobalamin II deficiency (D51.2)
E72.2Disorders of urea cycle metabolism
Argininaemia
Argininosuccinic aciduria
Citrullinaemia
Hyperammonaemia
Excludes:disorders of ornithine metabolism (E72.4)
E72.3Disorders of lysine and hydroxylysine metabolism
Glutaric aciduria
Hydroxylysinaemia
Hyperlysinaemia
Excludes:Refsum's disease (G60.1)
Zellweger's syndrome (Q87.8)
E72.4Disorders of ornithine metabolism
Ornithinaemia (types I, II)
E72.5Disorders of glycine metabolism
Hyperhydroxyprolinaemia
Hyperprolinaemia (types I, II)
Non-ketotic hyperglycinaemia
Sarcosinaemia
E72.8Other specified disorders of amino-acid metabolism
Disorders of:
· Beta-amino-acid metabolism
· Gamma-glutamyl cycle
E72.9Disorder of amino-acid metabolism, unspecified

E73Lactose intolerance
Latin: Intolerantia lactosi
E73.0Congenital lactase deficiency
E73.1Secondary lactase deficiency
E73.8Other lactose intolerance
E73.9Lactose intolerance, unspecified

E74Other disorders of carbohydrate metabolism
Latin: Disordines metabolismi carbohydratorum alii
Excludes:increased secretion of glucagon (E16.3)
diabetes mellitus (E10-E14)
hypoglycaemia NOS (E16.2)
mucopolysaccharidosis (E76.0-E76.3)
E74.0Glycogen storage disease
Cardiac glycogenosis
Disease:
· Andersen
· Cori
· Forbes
· Hers
· McArdle
· Pompe
· Tauri
· von Gierke
Liver phosphorylase deficiency
E74.1Disorders of fructose metabolism
Essential fructosuria
Fructose-1,6-diphosphatase deficiency
Hereditary fructose intolerance
E74.2Disorders of galactose metabolism
Galactokinase deficiency
Galactosaemia
E74.3Other disorders of intestinal carbohydrate absorption
Glucose-galactose malabsorption
Sucrase deficiency
Excludes:lactose intolerance (E73.-)
E74.4Disorders of pyruvate metabolism and gluconeogenesis
Deficiency of:
· phosphoenolpyruvate carboxykinase
· pyruvate:
  · carboxylase
  · dehydrogenase
Excludes:with anaemia (D55.-)
E74.8Other specified disorders of carbohydrate metabolism
Essential pentosuria
Oxalosis
Oxaluria
Renal glycosuria
E74.9Disorder of carbohydrate metabolism, unspecified

E75Disorders of sphingolipid metabolism and other lipid storage disorders
Latin: Disordines metabolismi sphingolipidi et depositionis lipidorum alii
Excludes:mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)
E75.0GM2 gangliosidosis
Disease:
· Sandhoff
· Tay-Sachs
GM2 gangliosidosis:
· NOS
· adult
· juvenile
E75.1Other gangliosidosis
Gangliosidosis:
· NOS
· GM1
· GM3
Mucolipidosis IV
E75.2Other sphingolipidosis
Disease:
· Fabry(-Anderson)
· Gaucher
· Krabbe
· Niemann-Pick
Farber's syndrome
Metachromatic leukodystrophy
Sulfatase deficiency
Excludes:adrenoleukodystrophy [Addison-Schilder] (E71.3)
E75.3Sphingolipidosis, unspecified
E75.4Neuronal ceroid lipofuscinosis
Disease:
· Batten
· Bielschowsky-Jansky
· Kufs
· Spielmeyer-Vogt
E75.5Other lipid storage disorders
Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
Wolman's disease
E75.6Lipid storage disorder, unspecified

E76Disorders of glycosaminoglycan metabolism
Latin: Disordines metabolismi glycosaminoglycanorum
E76.0Mucopolysaccharidosis, type I
Syndrome:
· Hurler
· Hurler-Scheie
· Scheie
E76.1Mucopolysaccharidosis, type II
Hunter's syndrome
E76.2Other mucopolysaccharidoses
Beta-glucuronidase deficiency
Mucopolysaccharidosis, types III, IV, VI, VII
Syndrome:
· Maroteaux-Lamy (mild)(severe)
· Morquio(-like)(classic)
· Sanfilippo (type B)(type C)(type D)
E76.3Mucopolysaccharidosis, unspecified
E76.8Other disorders of glucosaminoglycan metabolism
E76.9Disorder of glucosaminoglycan metabolism, unspecified

E77Disorders of glycoprotein metabolism
Latin: Disordines metabolismi glycoproteini
E77.0Defects in post-translational modification of lysosomal enzymes
Mucolipidosis II [I-cell disease]
Mucolipidosis III [pseudo-Hurler polydystrophy]
E77.1Defects in glycoprotein degradation
Aspartylglucosaminuria
Fucosidosis
Mannosidosis
Sialidosis [mucolipidosis I]
E77.8Other disorders of glycoprotein metabolism
E77.9Disorder of glycoprotein metabolism, unspecified

E78Disorders of lipoprotein metabolism and other lipidaemias
Latin: Disordines metabolismi lipoproteiniet lipidaemiae alii
Excludes:sphingolipidosis (E75.0-E75.3)
E78.0Pure hypercholesterolaemia
Familial hypercholesterolaemia
Fredrickson's hyperlipoproteinaemia, type IIa
Hyperbetalipoproteinaemia
Hyperlipidaemia, group A
Low-density-lipoprotein-type [LDL] hyperlipoproteinaemia
E78.1Pure hyperglyceridaemia
Endogenous hyperglyceridaemia
Fredrickson's hyperlipoproteinaemia, type IV
Hyperlipidaemia, group B
Hyperprebetalipoproteinaemia
Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinaemia
E78.2Mixed hyperlipidaemia
Broad- or floating-betalipoproteinaemia
Fredrickson's hyperlipoproteinaemia, type IIb or III
Hyperbetalipoproteinaemia with prebetalipoproteinaemia
Hypercholesterolaemia with endogenous hyperglyceridaemia
Hyperlipidaemia, group C
Tubero-eruptive xanthoma
Xanthoma tuberosum
Excludes:cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein] (E75.5)
E78.3Hyperchylomicronaemia
Fredrickson's hyperlipoproteinaemia, type I or V
Hyperlipidaemia, group D
Mixed hyperglyceridaemia
E78.4Other hyperlipidaemia
Familial combined hyperlipidaemia
E78.5Hyperlipidaemia, unspecified
E78.6Lipoprotein deficiency
Abetalipoproteinaemia
High-density lipoprotein deficiency
Hypoalphalipoproteinaemia
Hypobetalipoproteinaemia (familial)
Lecithin cholesterol acyltransferase deficiency
Tangier disease
E78.8Other disorders of lipoprotein metabolism
E78.9Disorder of lipoprotein metabolism, unspecified

E79Disorders of purine and pyrimidine metabolism
Latin: Disordines metabolismi purini et pyrimidini
Excludes:calculus of kidney (N20.0)
combined immunodeficiency disorders (D81.-)
gout (M10.-)
orotaciduric anaemia (D53.0)
xeroderma pigmentosum (Q82.1)
E79.0Hyperuricaemia without signs of inflammatory arthritis and tophaceous disease
Asymptomatic hyperuricaemia
E79.1Lesch-Nyhan syndrome
E79.8Other disorders of purine and pyrimidine metabolism
Hereditary xanthinuria
E79.9Disorder of purine and pyrimidine metabolism, unspecified

E80Disorders of porphyrin and bilirubin metabolism
Latin: Porphiria et disordines metabolismi bilirubini
Includes:defects of catalase and peroxidase
E80.0Hereditary erythropoietic porphyria
Congenital erythropoietic porphyria
Erythropoietic protoporphyria
E80.1Porphyria cutanea tarda
E80.2Other porphyria
Hereditary coproporphyria
Porphyria:
· NOS
· acute intermittent (hepatic)
Use additional external cause code (Chapter XX), if desired, to identify cause.
E80.3Defects of catalase and peroxidase
Acatalasia [Takahara]
E80.4Gilbert's syndrome
E80.5Crigler-Najjar syndrome
E80.6Other disorders of bilirubin metabolism
Dubin-Johnson syndrome
Rotor's syndrome
E80.7Disorder of bilirubin metabolism, unspecified

E83Disorders of mineral metabolism
Latin: Disordines metabolismi mineralium
Excludes:dietary mineral deficiency (E58-E61)
parathyroid disorders (E20-E21)
vitamin D deficiency (E55.-)
E83.0Disorders of copper metabolism
Menkes' (kinky hair)(steely hair) disease
Wilson's disease
E83.1Disorders of iron metabolism
Haemochromatosis
Excludes:anaemia:
· iron deficiency (D50.-)
· sideroblastic (D64.0-D64.3)
E83.2Disorders of zinc metabolism
Acrodermatitis enteropathica
E83.3Disorders of phosphorus metabolism
Acid phosphatase deficiency
Familial hypophosphataemia
Hypophosphatasia
Vitamin-D-resistant:
· osteomalacia
· rickets
Excludes:adult osteomalacia (M83.-)
osteoporosis (M80-M81)
E83.4Disorders of magnesium metabolism
Hypermagnesaemia
Hypomagnesaemia
E83.5Disorders of calcium metabolism
Familial hypocalciuric hypercalcaemia
Idiopathic hypercalciuria
Excludes:chondrocalcinosis (M11.1-M11.2)
hyperparathyroidism (E21.0-E21.3)
E83.8Other disorders of mineral metabolism
E83.9Disorder of mineral metabolism, unspecified

E84Cystic fibrosis
Latin: Fibrosis cystica
Includes:mucoviscidosis
E84.0Cystic fibrosis with pulmonary manifestations
E84.1Cystic fibrosis with intestinal manifestations
Meconium ileus+ (P75*)
Excludes:meconium obstruction in cases where cystic fibrosis is known not to be present (P76.0)
E84.8Cystic fibrosis with other manifestations
Cystic fibrosis with combined manifestations
E84.9Cystic fibrosis, unspecified

E85Amyloidosis
Latin: Amyloidosis
Excludes:Alzheimer's disease (G30.-)
E85.0Non-neuropathic heredofamilial amyloidosis
Familial Mediterranean fever
Hereditary amyloid nephropathy
E85.1Neuropathic heredofamilial amyloidosis
Amyloid polyneuropathy (Portuguese)
E85.2Heredofamilial amyloidosis, unspecified
E85.3Secondary systemic amyloidosis
Haemodialysis-associated amyloidosis
E85.4Organ-limited amyloidosis
Localized amyloidosis
E85.8Other amyloidosis
E85.9Amyloidosis, unspecified

E86Volume depletion
Latin: Dehydratio et hypovolaemia
Dehydration
Depletion of volume of plasma or extracellular fluid
Hypovolaemia
Excludes:dehydration of newborn (P74.1)
hypovolaemic shock:
· NOS (R57.1)
· postoperative(T81.1)
· traumatic (T79.4)

E87Other disorders of fluid, electrolyte and acid-base balance
Latin: Disordines fluidi, electrolyti et aequilibrii acidobasis alii
E87.0Hyperosmolality and hypernatraemia
Sodium [Na] excess
Sodium [Na] overload
E87.1Hypo-osmolality and hyponatraemia
Sodium [Na] deficiency
Excludes:Syndrome of inappropriate secretion of antidiuretic hormone (E22.2)
E87.2Acidosis
Acidosis:
· NOS
· lactic
· metabolic
· respiratory
Excludes:diabetic acidosis (E10-E14 with common fourth character .1)

E87.3Alkalosis
Alkalosis:
· NOS
· metabolic
· respiratory
E87.4Mixed disorder of acid-base balance
E87.5Hyperkalaemia
Potassium [K] excess
Potassium [K] overload
E87.6Hypokalaemia
Potassium [K] deficiency
E87.7Fluid overload
Excludes:oedema (R60.-)
E87.8Other disorders of electrolyte and fluid balance, not elsewhere classified
Electrolyte imbalance NOS
Hyperchloraemia
Hypochloraemia

E88Other metabolic disorders
Latin: Disordines metabolismi alii
Use additional external cause code (Chapter XX), if desired, to identify drug, if drug-induced.
Excludes:histiocytosis X (chronic) (D76.0)
E88.0Disorders of plasma-protein metabolism, not elsewhere classified
Alpha-1-antitrypsin deficiency
Bisalbuminaemia
Excludes:disorder of lipoprotein metabolism (E78.-)
monoclonal gammopathy (D47.2)
polyclonal hypergammaglobulinaemia (D89.0)
Waldenström's macroglobulinaemia (C88.0)
E88.1Lipodystrophy, not elsewhere classified
Lipodystrophy NOS
Excludes:Whipple's disease (K90.8)
E88.2Lipomatosis, not elsewhere classified
Lipomatosis:
· NOS
· dolorosa [Dercum]
E88.8Other specified metabolic disorders
Launois-Bensaude adenolipomatosis
Trimethylaminuria
E88.9Metabolic disorder, unspecified

E89Postprocedural endocrine and metabolic disorders, not elsewhere classified
Latin: Disordines endocrini et disordines metabolici postprocedurales
E89.0Postprocedural hypothyroidism
Postirradiation hypothyroidism
Postsurgical hypothyroidism
E89.1Postprocedural hypoinsulinaemia
Postpancreatectomy hyperglycaemia
Postsurgical hypoinsulinaemia
E89.2Postprocedural hypoparathyroidism
Parathyroprival tetany
E89.3Postprocedural hypopituitarism
Postirradiation hypopituitarism
E89.4Postprocedural ovarian failure
E89.5Postprocedural testicular hypofunction
E89.6Postprocedural adrenocortical(-medullary) hypofunction
E89.8Other postprocedural endocrine and metabolic disorders
E89.9Postprocedural endocrine and metabolic disorder, unspecified

E90*Nutritional and metabolic disorders in diseases classified elsewhere
Latin: Disordines nutritionis et disordines metabolismi in morbis aliis


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