ICD-10 Chapter E: Endocrine, nutritional and metabolic diseases

(E35.) Disorders of endocrine glands in diseases classified elsewhere
- (E 35.0) Disorders of thyroid gland in diseases classified elsewhere
- (E 35.1) Disorders of adrenal glands in diseases classified elsewhere
- (E 35.8) Disorders of other endocrine glands in diseases classified elsewhere

E40-68 - Nutritional diseases

(E40-E46) Malnutrition

(E 40.) Kwashiorkor
(E 41.) Nutritional marasmus
(E 42.) Marasmic kwashiorkor
(E 43.) Unspecified severe protein-energy malnutrition
(E 44.) Protein-energy malnutrition of moderate and mild degree
(E 45.) Retarded development following protein-energy malnutrition
(E 46.) Unspecified protein-energy malnutrition

(E50-E64) Other nutritional deficiencies

(E50.) Vitamin A deficiency
- (E 50.0) Vitamin A deficiency with conjunctival xerosis
- (E 50.1) Vitamin A deficiency with Bitot's spot and conjunctival xerosis
- (E 50.2) Vitamin A deficiency with corneal xerosis
- (E 50.3) Vitamin A deficiency with corneal ulceration and xerosis
- (E 50.4) Vitamin A deficiency with keratomalacia
- (E 50.5) Vitamin A deficiency with night blindness
- (E 50.6) Vitamin A deficiency with xerophthalmic scars of cornea
- (E50.7) Other ocular manifestations of vitamin A deficiency
  - Xerophthalmia NOS
(E51.) Thiamine deficiency
- (E 51.1) Beriberi
- (E 51.2) Wernicke's encephalopathy
(E 52.) Niacin deficiency (pellagra)
(E53.) Deficiency of other B group vitamins
- (E53.0) Riboflavin deficiency
  - Ariboflavinosis
- (E 53.1) Pyridoxine deficiency
- (E 53.8) Deficiency of other specified B group vitamins
(E54.) Ascorbic acid deficiency
- Scurvy
(E55.) Vitamin D deficiency
- (E 55.0) Rickets, active
(E56.) Other vitamin deficiencies
- (E 56.0) Deficiency of vitamin E
- (E 56.1) Deficiency of vitamin K
(E 58.) Dietary calcium deficiency
(E 59.) Dietary selenium deficiency
(E 60.) Dietary zinc deficiency
(E61.) Deficiency of other nutrient elements
- (E 61.1) Iron deficiency
- (E 61.2) Magnesium deficiency
- (E 61.3) Manganese deficiency
- (E 61.4) Chromium deficiency
- (E 61.5) Molybdenum deficiency
- (E 61.6) Vanadium deficiency
(E63.) Other nutritional deficiencies
- (E 63.0) Essential fatty acid (EFA) deficiency
(E 64.) Sequelae of malnutrition and other nutritional deficiencies

(E65-E68) Obesity and other hyperalimentation

(E65.) Localized adiposity
- Fat pad

(E66.) Obesity
- (E 66.0) Obesity due to excess calories
- (E 66.1) Drug-induced obesity
- (E66.2) Extreme obesity with alveolar hypoventilation
  - Pickwickian syndrome
- (E66.8) Other obesity
  - Morbid obesity
- (E 66.9) Obesity, unspecified

(E67.) Other hyperalimentation
- (E 67.0) Hypervitaminosis A
- (E 67.1) Hypercarotenaemia
- (E 67.2) Megavitamin-B 6 syndrome
- (E 67.3) Hypervitaminosis D

(E 68.) Sequelae of hyperalimentation

E70-E90 - Metabolic diseases

(E70-E79) Metabolic disorders of proteins, fats, and carbohydrates

(E70-E72) Amino-acids

(E70.) Disorders of aromatic amino-acid metabolism
- (E 70.0) Classical phenylketonuria
- (E 70.1) Other hyperphenylalaninaemias
- (E70.2) Disorders of tyrosine metabolism
  - Alkaptonuria (ILDS E70.210)
  - Ochronosis (ILDS E70.230)
  - Tyrosinaemia
- (E70.3) Albinism
- (E70.8) Other disorders of aromatic amino-acid metabolism
  - Disorders of histidine metabolism
  - Disorders of tryptophan metabolism
- (E 70.9) Disorder of aromatic amino-acid metabolism, unspecified

(E71.) Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
- (E 71.0) Maple-syrup-urine disease
- (E71.1) Other disorders of branched-chain amino-acid metabolism
- (E 71.2) Disorder of branched-chain amino-acid metabolism, unspecified
- (E71.3) Disorders of fatty-acid metabolism
  - Adrenoleukodystrophy (Addison-Schilder)
  - Muscle carnitine palmityltransferase deficiency

(E72.) Other disorders of amino-acid metabolism
- (E72.0) Disorders of amino-acid transport
- (E72.1) Disorders of sulfur-bearing amino-acid metabolism
- (E72.2) Disorders of urea cycle metabolism
- (E72.3) Disorders of lysine and hydroxylysine metabolism
- (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

(E73-E74) Carbohydrates

(E 73.) Lactose intolerance

(E74.) Other disorders of carbohydrate metabolism
- (E74.0) Glycogen storage disease
- (E74.1) Disorders of fructose metabolism
- (E74.2) Disorders of galactose metabolism
  - Galactosaemia
  - Galactokinase deficiency
- (E74.3) Other disorders of intestinal carbohydrate absorption
  - Glucose-galactose malabsorption
  - Sucrase deficiency
- (E74.4) Disorders of pyruvate metabolism and gluconeogenesis
- (E74.8) Other specified disorders of carbohydrate metabolism
- (E 74.9) Disorder of carbohydrate metabolism, unspecified

(E75) Lipids

(E75.) Disorders of sphingolipid metabolism and other lipid storage disorders
- (E75.0) GM 2 gangliosidosis
  - Sandhoff disease
  - Tay-Sachs disease
- (E75.1) Other gangliosidosis
- (E75.2) Other sphingolipidosis
  - Gaucher's disease (ILDS E75.220)
  - Niemann-Pick disease (ILDS E75.230)
  - Farber's disease (ILDS E75.240)
  - Fabry's disease (ILDS E75.250)
- (E 75.3) Sphingolipidosis, unspecified
- (E75.4) Neuronal ceroid lipofuscinosis
  - Batten disease (Type 3)
  - Bielschowsky-Jansky disease (Type 2)
  - Kufs disease (Type 4)
  - Spielmeyer-Vogt disease (Type 3)
- (E75.5) Other lipid storage disorders
  - Cerebrotendinous cholesterosis (van Bogaert-Scherer-Epstein)
  - Wolman's disease
- (E 75.6) Lipid storage disorder, unspecified

(E76-E78) Combinations

(E76.) Disorders of glycosaminoglycan metabolism
- (E76.0) Mucopolysaccharidosis, type I
  - Hurler syndrome
- (E76.1) Mucopolysaccharidosis, type II
  - Hunter syndrome
- (E76.2) Other mucopolysaccharidoses
  - Sanfilippo syndrome
  - Morquio syndrome

(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

(E78.) Disorders of lipoprotein metabolism and other lipidaemias
- (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
- (E78.3) Hyperchylomicronaemia
  - Fredrickson's hyperlipoproteinaemia, type I or V
  - Hyperlipidaemia, group D
  - Mixed hyperglyceridaemia
- (E78.4) Other hyperlipidaemia
  - Familial combined hyperlipidaemia
- (E 78.5) Hyperlipidaemia, unspecified
- (E78.6) Lipoprotein deficiency

(E79-E90) Other metabolic disorders

(E79.) Disorders of purine and pyrimidine metabolism
- (E 79.0) Hyperuricaemia without signs of inflammatory arthritis and tophaceous disease
- (E 79.1) Lesch-Nyhan syndrome
- (E79.8) Other disorders of purine and pyrimidine metabolism
  - Hereditary xanthinuria

(E80.) Disorders of porphyrin and bilirubin metabolism
- (E80.0) Hereditary erythropoietic porphyria
  - Erythropoietic protoporphyria (ILDS E80.010)
  - Erythropoietic porphyria, congenital (ILDS E80.020)
  - Gunther's disease (ILDS (ILDS E80.020)
  - Erythropoietic porphyria (ILDS (ILDS E80.030)
  - Erythropoietic coproporphyria (ILDS E80.040)
- (E80.1) Porphyria cutanea tarda
  - Sporadic porphyria cutanea tarda (ILDS E80.110)
  - Familial porphyria cutanea tarda (ILDS E80.120)
- (E80.2) Other porphyria
  - Acute intermittent porphyria (ILDS E80.210)
  - Hereditary coproporphyria (ILDS E80.222)
  - Variegate porphyria (ILDS E80.230)
  - Chester porphyria (ILDS E80.232)
  - Porphyria, hepatic (ILDS E80.240)
  - Pseudoporphyria (ILDS E80.250)
  - Toxic porphyria (ILDS E80.260)
  - Hepatoerythropoietic porphyria (ILDS E80.282)
  - Porphyria, NOS (ILDS E80.290)
- (E80.3) Defects of catalase and peroxidase
  - Acatalasia (Takahara)
- (E 80.4) Gilbert's syndrome
- (E 80.5) Crigler-Najjar syndrome
- (E80.6) Other disorders of bilirubin metabolism
  - Dubin-Johnson syndrome
  - Rotor's syndrome
- (E 80.7) Disorder of bilirubin metabolism, unspecified

(E83.) Disorders of mineral metabolism
- (E83.0) Disorders of copper metabolism
  - Wilson's disease
  - Menkes disease
- (E83.1) Disorders of iron metabolism
  - Aceruloplasminemia
  - Hemochromatosis
- (E83.2) Disorders of zinc metabolism
  - Acrodermatitis enteropathica
- (E83.3) Disorders of phosphorus metabolism
  - Acid phosphatase deficiency
  - Familial hypophosphataemia
  - Hypophosphatasia
  - Vitamin-D-resistant osteomalacia
  - Vitamin-D-resistant rickets
- (E83.4) Disorders of magnesium metabolism
  - Hypermagnesemia
  - Hypomagnesemia
- (E83.5) Disorders of calcium metabolism
  - Familial hypocalciuric hypercalcaemia
  - Idiopathic hypercalciuria

(E 84.) Cystic fibrosis

(E85.) Amyloidosis
- (E85.0) Non-neuropathic heredofamilial amyloidosis
  - Familial Mediterranean fever
  - Hereditary amyloid nephropathy
- (E85.1) Neuropathic heredofamilial amyloidosis
  - Amyloid polyneuropathy (Portuguese)
- (E 85.2) Heredofamilial amyloidosis, unspecified
- (E85.3) Secondary systemic amyloidosis
  - Haemodialysis-associated amyloidosis
- (E85.4) Organ-limited amyloidosis
  - Localized amyloidosis
- (E 85.8) Other amyloidosis
- (E 85.9) Amyloidosis, unspecified

(E86.) Volume depletion
- Dehydration
- Hypovolaemia

(E87.) Other disorders of fluid, electrolyte and acid-base balance
- (E 87.0) Hyperosmolality and hypernatraemia
- (E 87.1) Hypo-osmolality and hyponatraemia
- (E87.2) Acidosis
- (E 87.3) Alkalosis
- (E 87.4) Mixed disorder of acid-base balance
- (E 87.5) Hyperkalaemia
- (E 87.6) Hypokalaemia
- (E 87.7) Fluid overload
- (E87.8) Other disorders of electrolyte and fluid balance, not elsewhere classified

(E88.) Other metabolic disorders
- (E88.0) Disorders of plasma-protein metabolism, not elsewhere classified
  - Alpha-1-antitrypsin deficiency
- (E 88.1) Lipodystrophy, not elsewhere classified
- (E 88.2) Lipomatosis, not elsewhere classified

(E89.) Postprocedural endocrine and metabolic disorders, not elsewhere classified
- (E 89.0) Postprocedural hypothyroidism
- (E 89.1) Postprocedural hypoinsulinaemia
- (E 89.2) Postprocedural hypoparathyroidism
- (E 89.3) Postprocedural hypopituitarism
- (E 89.4) Postprocedural ovarian failure
- (E 89.5) Postprocedural testicular hypofunction
- (E 89.6) Postprocedural adrenocortical(-medullary) hypofunction

(E 90.) Nutritional and metabolic disorders in diseases classified elsewhere

v • d • e Endocrine pathology: endocrine diseases (E00-35, 240-259)
Thyroid	Hypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema) - Hyperthyroidism (Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii) - Thyroiditis (De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis) - Euthyroid sick syndrome
Pancreas	Diabetes mellitus (type 1, type 2, coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy) - Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor (Rabson-Mendenhall syndrome)
Parathyroid	Hypoparathyroidism (Pseudohypoparathyroidism) - Hyperparathyroidism (Primary, Secondary, Tertiary)
Pituitary	Hyperpituitarism (Acromegaly, Hyperprolactinaemia, SIADH) - Hypopituitarism (Simmonds' disease/Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus) - Adiposogenital dystrophy - Empty sella syndrome
Adrenal	Cushing's syndrome (Nelson's syndrome, Pseudo-Cushing's syndrome) - CAH (Lipoid, 3β, 11β, 17α, 21α) - Hyperaldosteronism (Conn syndrome, Bartter syndrome) - Adrenal insufficiency (Addison's disease) - Hypoaldosteronism
Gonads	ovarian dysfunction (Polycystic ovary syndrome, Premature ovarian failure) - testicular dysfunction (5-alpha-reductase deficiency) - testosterone biosynthesis (17-beta-hydroxysteroid dehydrogenase deficiency) - general (Hypogonadism, Delayed puberty, Precocious puberty)
Other	Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature (Laron syndrome, Psychogenic dwarfism) - Multiple endocrine neoplasia (1, 2) - Progeria - Woodhouse-Sakati syndrome

v • d • e Nutritional pathology (E40-68, 260-269)
Malnutrition	Kwashiorkor - Marasmus - Catabolysis
Avitaminosis	B vitamins: B1: Beriberi/Wernicke's encephalopathy, B2: Ariboflavinosis, B3: Pellagra, B6: Pyridoxine deficiency, B7: Biotin deficiency, B9: Folate deficiency, B12: Vitamin B12 deficiency other vitamins: A: Vitamin A deficiency/Bitot's spots, C: Scurvy, D: Rickets/Osteomalacia
Mineral deficiency	Zinc deficiency - Iron deficiency - Magnesium deficiency - Chromium deficiency
Hyperalimentation	Obesity - Vitamin poisoning (Hypervitaminosis A, Hypervitaminosis D, Hypervitaminosis E)

v • d • e Metabolic pathology / Inborn error of metabolism (E70-90, 270-279)
Amino acid	Aromatic (Phenylketonuria, Alkaptonuria, Ochronosis, Tyrosinemia, Albinism, Histidinemia) - Branched chain (Maple syrup urine disease, Propionic acidemia, Methylmalonic acidemia, Isovaleric acidemia, 3-Methylcrotonyl-CoA carboxylase deficiency) - Transport (Cystinuria, Cystinosis, Hartnup disease, Fanconi syndrome, Oculocerebrorenal syndrome) - Sulfur (Homocystinuria, Cystathioninuria) - Urea cycle disorder (N-Acetylglutamate synthase deficiency, Carbamoyl phosphate synthetase I deficiency, Ornithine transcarbamylase deficiency, Citrullinemia, Argininosuccinic aciduria, Hyperammonemia) - Glutaric acidemia type 1 - Sarcosinemia
Carbohydrate	Lactose intolerance - Glycogen storage disease (type I, type II, type III, type IV, type V, type VI, type VII) - fructose metabolism (Fructose intolerance, Fructose bisphosphatase deficiency, Essential fructosuria) - galactose metabolism (Galactosemia, Galactose-1-phosphate uridylyltransferase galactosemia, Galactokinase deficiency) - other intestinal carbohydrate absorption (Glucose-galactose malabsorption, Sucrose intolerance) - pyruvate metabolism and gluconeogenesis (PCD, PDHA) - Pentosuria - Renal glycosuria
Lipid storage	Sphingolipidoses/Gangliosidoses: GM2 gangliosidoses (Sandhoff disease, Tay-Sachs disease) - GM1 gangliosidoses - Mucolipidosis type IV - Gaucher's disease - Niemann-Pick disease - Farber disease - Fabry's disease - Metachromatic leukodystrophy - Krabbe disease Neuronal ceroid lipofuscinosis (Batten disease) - Cerebrotendineous xanthomatosis - Cholesteryl ester storage disease (Wolman disease)
Other lipid	Lipoprotein/lipidemias: Hyperlipidemia - Hypercholesterolemia - Familial hypercholesterolemia - Xanthoma - Combined hyperlipidemia - Lecithin cholesterol acyltransferase deficiency - Tangier disease - Abetalipoproteinemia Fatty acid: Adrenoleukodystrophy - Carnitine (Primary, I, II)
Mineral	Cu Wilson's disease/Menkes disease - Fe Haemochromatosis - Zn Acrodermatitis enteropathica - PO₄³⁻ Hypophosphatemia/Hypophosphatasia - Mg²⁺ Hypermagnesemia/Hypomagnesemia - Ca²⁺ Hypercalcaemia/Hypocalcaemia/Disorders of calcium metabolism
Fluid, electrolyte and acid-base balance	Electrolyte disturbance - Na⁺ Hypernatremia/Hyponatremia - Acidosis (Metabolic, Respiratory, Lactic) - Alkalosis (Metabolic, Respiratory) - Mixed disorder of acid-base balance - H₂O Dehydration/Hypervolemia - K⁺ Hypokalemia/Hyperkalemia - Cl⁻ Hyperchloremia/Hypochloremia
Purine and pyrimidine	Hyperuricemia - Lesch-Nyhan syndrome - Xanthinuria
Porphyrin	Acute intermittent, Gunther's, Cutanea tarda, Erythropoietic, Hepatoerythropoietic, Hereditary copro-, Variegate
Bilirubin	Unconjugated (Gilbert's syndrome, Crigler-Najjar syndrome) - Conjugated (Dubin-Johnson syndrome, Rotor syndrome)
Glycosaminoglycan	Mucopolysaccharidosis - 1:Hurler/Hunter - 3:Sanfilippo - 4:Morquio - 6:Maroteaux-Lamy - 7:Sly
Glycoprotein	Mucolipidosis - I-cell disease - Pseudo-Hurler polydystrophy - Aspartylglucosaminuria - Fucosidosis - Alpha-mannosidosis - Sialidosis
Other	Alpha 1-antitrypsin deficiency - Cystic fibrosis - Amyloidosis (Familial Mediterranean fever) - Acatalasia

ICD-10 Chapter E: Endocrine, nutritional and metabolic diseases

E00-E35 - Endocrine diseases

(E00-E07) Thyroid gland / Thyroid hormone

(E10-E16) Pancreas / Insulin, glucagon

(E10-E14) Diabetes mellitus

(E15-E16) Other disorders of glucose regulation and pancreatic internal secretion

(E20-E21) Parathyroid gland / PTH

(E22-E23) Pituitary gland / ADH, oxytocin, GH, ACTH, TSH, LH, FSH, prolactin

(E24-E27) Adrenal gland / Aldosterone, cortisol, epinephrine, norepinephrine

(E28-E30) Gonads / Estrogen, androgens, testosterone, etc.

(E31-E35) Other

E40-68 - Nutritional diseases

(E40-E46) Malnutrition

(E50-E64) Other nutritional deficiencies

(E65-E68) Obesity and other hyperalimentation

E70-E90 - Metabolic diseases

(E70-E79) Metabolic disorders of proteins, fats, and carbohydrates

(E70-E72) Amino-acids

(E73-E74) Carbohydrates

(E75) Lipids

(E76-E78) Combinations

(E79-E90) Other metabolic disorders

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