Excludes: | androgen resistance syndrome
congenital adrenal hyperplasia Ehlers-Danlos syndrome haemolytic anaemias due to enzyme disorders Marfan's syndrome 5-alpha-reductase deficiency |
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 | |||||||
Hyperleucine-isoleucinaemia
Hypervalinaemia Isovaleric acidaemia Methylmalonic acidaemia Propionic acidaemia |
||||||||
E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | |||||||
E71.3 | Disorders of fatty-acid metabolism | |||||||
Adrenoleukodystrophy [Addison-Schilder]
Muscle carnitine palmityltransferase deficiency |
||||||||
Excludes: | Schilder's disease
|
E72 | Other disorders of amino-acid metabolism | |||||||
Excludes: | abnormal findings without manifest disease
disorders of: · aromatic amino-acid metabolism · branched-chain amino-acid metabolism · fatty-acid metabolism · purine and pyrimidine metabolism gout |
|||||||
E72.0 | Disorders of amino-acid transport | |||||||
Cystinosis
Cystinuria Fanconi(-de Toni)(-Debré) syndrome Hartnup's disease Lowe's syndrome |
||||||||
Excludes: | disorders of tryptophan metabolism
|
|||||||
E72.1 | Disorders of sulfur-bearing amino-acid metabolism | |||||||
Cystathioninuria
Homocystinuria Methioninaemia Sulfite oxidase deficiency |
||||||||
Excludes: | transcobalamin II deficiency
|
|||||||
E72.2 | Disorders of urea cycle metabolism | |||||||
Argininaemia
Argininosuccinic aciduria Citrullinaemia Hyperammonaemia |
||||||||
Excludes: | disorders of ornithine metabolism
|
|||||||
E72.3 | Disorders of lysine and hydroxylysine metabolism | |||||||
Glutaric aciduria
Hydroxylysinaemia Hyperlysinaemia |
||||||||
Excludes: | Refsum's disease
Zellweger's syndrome |
|||||||
E72.4 | Disorders of ornithine metabolism | |||||||
Ornithinaemia (types I, II)
|
||||||||
E72.5 | Disorders of glycine metabolism | |||||||
Hyperhydroxyprolinaemia
Hyperprolinaemia (types I, II) Non-ketotic hyperglycinaemia Sarcosinaemia |
||||||||
E72.8 | Other specified disorders of amino-acid metabolism | |||||||
Disorders of:
· Beta-amino-acid metabolism · 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: | increased secretion of glucagon
diabetes mellitus hypoglycaemia NOS mucopolysaccharidosis |
|||||||
E74.0 | Glycogen storage disease | |||||||
Cardiac glycogenosis
Disease: · Andersen · Cori · Forbes · Hers · McArdle · Pompe · Tauri · von Gierke Liver phosphorylase deficiency |
||||||||
E74.1 | Disorders of fructose metabolism | |||||||
Essential fructosuria
Fructose-1,6-diphosphatase deficiency Hereditary fructose intolerance |
||||||||
E74.2 | Disorders of galactose metabolism | |||||||
Galactokinase deficiency
Galactosaemia |
||||||||
E74.3 | Other disorders of intestinal carbohydrate absorption | |||||||
Glucose-galactose malabsorption
Sucrase deficiency |
||||||||
Excludes: | lactose intolerance
|
|||||||
#E74.30 | Glucose malabsorption
Monosaccharide malabsorption
|
|||||||
#E74.38 | Other disorders of intestinal carbohydrate metabolism | |||||||
E74.4 | Disorders of pyruvate metabolism and gluconeogenesis | |||||||
Deficiency of:
· phosphoenolpyruvate carboxykinase · pyruvate: · carboxylase · dehydrogenase |
||||||||
Excludes: | with anaemia
|
|||||||
E74.8 | Other specified disorders of carbohydrate metabolism | |||||||
Essential pentosuria
Oxalosis Oxaluria Renal glycosuria |
||||||||
E74.9 | Disorder of carbohydrate metabolism, unspecified |
E75 | Disorders of sphingolipid metabolism and other lipid storage disorders | |||||||
Excludes: | mucolipidosis, types I-III
Refsum's disease |
|||||||
E75.0 | GM 2 gangliosidosis | |||||||
Disease:
· Sandhoff · Tay-Sachs GM 2 gangliosidosis: · NOS · adult · juvenile |
||||||||
E75.1 | Other gangliosidosis | |||||||
Gangliosidosis:
· NOS · GM 1 · GM 3 Mucolipidosis IV |
||||||||
E75.2 | Other sphingolipidosis | |||||||
Disease:
· Fabry(-Anderson) · Gaucher · Krabbe · Niemann-Pick Farber's syndrome Metachromatic leukodystrophy Sulfatase deficiency |
||||||||
Excludes: | adrenoleukodystrophy [Addison-Schilder]
|
|||||||
E75.3 | Sphingolipidosis, unspecified | |||||||
E75.4 | Neuronal ceroid lipofuscinosis | |||||||
Disease:
· Batten · Bielschowsky-Jansky · Kufs · Spielmeyer-Vogt |
||||||||
E75.5 | Other lipid storage disorders | |||||||
Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
Wolman's disease |
||||||||
E75.6 | Lipid storage disorder, unspecified |
E78 | Disorders of lipoprotein metabolism and other lipidaemias | |||||||
Excludes: | sphingolipidosis
|
|||||||
E78.0 | Pure hypercholesterolaemia | |||||||
Familial hypercholesterolaemia
Fredrickson's hyperlipoproteinaemia, type IIa Hyperbetalipoproteinaemia Hyperlipidaemia, group A Low-density-lipoprotein-type [LDL] hyperlipoproteinaemia |
||||||||
E78.1 | Pure hyperglyceridaemia | |||||||
Endogenous hyperglyceridaemia
Fredrickson's hyperlipoproteinaemia, type IV Hyperlipidaemia, group B Hyperprebetalipoproteinaemia Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinaemia |
||||||||
E78.2 | Mixed 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]
|
|||||||
E78.3 | Hyperchylomicronaemia | |||||||
Fredrickson's hyperlipoproteinaemia, type I or V
Hyperlipidaemia, group D Mixed hyperglyceridaemia |
||||||||
E78.4 | Other hyperlipidaemia | |||||||
Familial combined hyperlipidaemia
|
||||||||
E78.5 | Hyperlipidaemia, unspecified | |||||||
E78.6 | Lipoprotein deficiency | |||||||
Abetalipoproteinaemia
High-density lipoprotein deficiency Hypoalphalipoproteinaemia Hypobetalipoproteinaemia (familial) Lecithin cholesterol acyltransferase deficiency Tangier disease |
||||||||
E78.8 | Other disorders of lipoprotein metabolism | |||||||
E78.9 | Disorder of lipoprotein metabolism, unspecified |
E79 | Disorders of purine and pyrimidine metabolism | |||||||
Excludes: | calculus of kidney
combined immunodeficiency disorders gout orotaciduric anaemia xeroderma pigmentosum |
|||||||
E79.0 | Hyperuricaemia without signs of inflammatory arthritis and tophaceous disease | |||||||
Asymptomatic hyperuricaemia
|
||||||||
E79.1 | Lesch-Nyhan syndrome | |||||||
E79.8 | Other disorders of purine and pyrimidine metabolism | |||||||
Hereditary xanthinuria
|
||||||||
E79.9 | Disorder of purine and pyrimidine metabolism, unspecified |
E83 | Disorders of mineral metabolism | |||||||
Excludes: | dietary mineral deficiency
parathyroid disorders vitamin D deficiency |
|||||||
E83.0 | Disorders of copper metabolism | |||||||
Menkes' (kinky hair)(steely hair) disease
Wilson's disease |
||||||||
E83.1 | Disorders of iron metabolism | |||||||
Haemochromatosis
|
||||||||
Excludes: | anaemia:
· iron deficiency · sideroblastic |
|||||||
E83.2 | Disorders of zinc metabolism | |||||||
Acrodermatitis enteropathica
|
||||||||
E83.3 | Disorders of phosphorus metabolism | |||||||
Acid phosphatase deficiency
Familial hypophosphataemia Hypophosphatasia Vitamin-D-resistant: · osteomalacia · rickets |
||||||||
Excludes: | adult osteomalacia
osteoporosis |
|||||||
E83.4 | Disorders of magnesium metabolism | |||||||
Hypermagnesaemia
Hypomagnesaemia |
||||||||
E83.5 | Disorders of calcium metabolism | |||||||
# |
Familial hypocalciuric hypercalcaemia
Hypercalcaemia of malignancy Idiopathic hypercalciuria |
|||||||
Excludes: | chondrocalcinosis
hyperparathyroidism |
|||||||
E83.8 | Other disorders of mineral metabolism | |||||||
E83.9 | Disorder of mineral metabolism, unspecified |
E84 | Cystic fibrosis | |||||||
Includes: | mucoviscidosis
|
|||||||
E84.0 | Cystic fibrosis with pulmonary manifestations | |||||||
E84.1 | Cystic fibrosis with intestinal manifestations | |||||||
Meconium ileus+
|
||||||||
Excludes: | meconium obstruction in cases where cystic fibrosis is known not to be present
|
|||||||
E84.8 | Cystic fibrosis with other manifestations | |||||||
Cystic fibrosis with combined manifestations
|
||||||||
E84.9 | Cystic fibrosis, unspecified |
E85 | Amyloidosis | |||||||
Excludes: | Alzheimer's disease
|
|||||||
E85.0 | Non-neuropathic heredofamilial amyloidosis | |||||||
Familial Mediterranean fever
Hereditary amyloid nephropathy |
||||||||
E85.1 | Neuropathic heredofamilial amyloidosis | |||||||
Amyloid polyneuropathy (Portuguese)
|
||||||||
E85.2 | Heredofamilial amyloidosis, unspecified | |||||||
E85.3 | Secondary systemic amyloidosis | |||||||
Haemodialysis-associated amyloidosis
|
||||||||
E85.4 | Organ-limited amyloidosis | |||||||
Localized amyloidosis
|
||||||||
E85.8 | Other amyloidosis | |||||||
E85.9 | Amyloidosis, unspecified |
E86 | Volume depletion | |||||||
Dehydration
Depletion of volume of plasma or extracellular fluid Hypovolaemia |
||||||||
Excludes: | dehydration of newborn
hypovolaemic shock: · NOS · postoperative · traumatic |
E87 | Other disorders of fluid, electrolyte and acid-base balance | |||||||
E87.0 | Hyperosmolality and hypernatraemia | |||||||
Sodium [Na] excess
Sodium [Na] overload |
||||||||
E87.1 | Hypo-osmolality and hyponatraemia | |||||||
Sodium [Na] deficiency
|
||||||||
Excludes: | Syndrome of inappropriate secretion of antidiuretic hormone
|
|||||||
E87.2 | Acidosis | |||||||
Acidosis:
· NOS · lactic · metabolic · respiratory |
||||||||
Excludes: | diabetic acidosis
|
|||||||
E87.3 | Alkalosis | |||||||
Alkalosis:
· NOS · metabolic · respiratory |
||||||||
E87.4 | Mixed disorder of acid-base balance | |||||||
E87.5 | Hyperkalaemia | |||||||
Potassium [K] excess
Potassium [K] overload |
||||||||
E87.6 | Hypokalaemia | |||||||
Potassium [K] deficiency
|
||||||||
E87.7 | Fluid overload | |||||||
Excludes: | oedema
|
|||||||
E87.8 | Other disorders of electrolyte and fluid balance, not elsewhere classified | |||||||
Electrolyte imbalance NOS
Hyperchloraemia Hypochloraemia |
E88 | Other metabolic disorders | |||||||
Use additional external cause code (Chapter XX), if desired, to identify drug, if drug-induced. | ||||||||
Excludes: | histiocytosis X (chronic)
|
|||||||
E88.0 | Disorders of plasma-protein metabolism, not elsewhere classified | |||||||
Alpha-1-antitrypsin deficiency
Bisalbuminaemia |
||||||||
Excludes: | disorder of lipoprotein metabolism
monoclonal gammopathy polyclonal hypergammaglobulinaemia Waldenström's macroglobulinaemia |
|||||||
E88.1 | Lipodystrophy, not elsewhere classified | |||||||
Lipodystrophy NOS
|
||||||||
Excludes: | Whipple's disease
|
|||||||
E88.2 | Lipomatosis, not elsewhere classified | |||||||
Lipomatosis:
· NOS · dolorosa [Dercum] |
||||||||
E88.8 | Other specified metabolic disorders | |||||||
Launois-Bensaude adenolipomatosis
Trimethylaminuria |
||||||||
E88.9 | Metabolic disorder, unspecified |
E90* | Nutritional and metabolic disorders in diseases classified elsewhere |