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

This is an overview about the chapter IV (also called chapter E) of the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10). This chapter is about Endocrine, nutritional and metabolic diseases.^[1]

The ICD-10 is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). The code set allows more than 155,000 different codes and permits tracking of many new diagnoses and procedures, a significant expansion on the 17,000 codes available in ICD-9.^[2]

The introduction to this article provides insufficient context for those unfamiliar with the subject. Please help improve the article with a good introductory style. (October 2009)

Contents 1 E00-E35 - Endocrine diseases 1.1 (E00-E07) Thyroid gland / Thyroid hormone 1.2 (E10-E16) Pancreas / Insulin, glucagon 1.2.1 (E10-E14) Diabetes mellitus 1.2.2 (E15-E16) Other disorders of glucose regulation and pancreatic internal secretion 1.3 (E20-E21) Parathyroid gland / PTH 1.4 (E22-E23) Pituitary gland / ADH, oxytocin, GH, ACTH, TSH, LH, FSH, prolactin 1.5 (E24-E27) Adrenal gland / Aldosterone, cortisol, epinephrine, norepinephrine 1.6 (E28-E30) Gonads / Estrogen, androgens, testosterone, etc. 1.7 (E31-E35) Other 2 E40-68 - Nutritional diseases 2.1 (E40-E46) Malnutrition 2.2 (E50-E64) Other nutritional deficiencies 2.3 (E65-E68) Obesity and other hyperalimentation 3 E70-E90 - Metabolic diseases 3.1 (E70-E79) Metabolic disorders of proteins, fats, and carbohydrates 3.1.1 (E70-E72) Amino-acids 3.1.2 (E73-E74) Carbohydrates 3.1.3 (E75) Lipids 3.1.4 (E76-E78) Combinations 3.2 (E79-E90) Other metabolic disorders 4 See also 5 References

E00-E35 - Endocrine diseases

(E00-E07) Thyroid gland / Thyroid hormone

• (E00.) Congenital iodine-deficiency syndrome

• (E01.) Iodine-deficiency-related thyroid disorders and allied conditions
  • (E01.0) Iodine-deficiency-related diffuse (endemic) goitre
  • (E01.1) Iodine-deficiency-related multinodular (endemic) goitre
  • (E01.2) Iodine-deficiency-related (endemic) goitre, unspecified
  • (E01.8) Other iodine-deficiency-related thyroid disorders and allied condition

• (E02.) Subclinical iodine-deficiency hypothyroidism
• (E03.) Other hypothyroidism
  • (E03.0) Congenital hypothyroidism with diffuse goitre
  • (E03.1) Congenital hypothyroidism without goitre
  • (E03.2) Hypothyroidism due to medicaments and other exogenous substances
  • (E03.3) Postinfectious hypothyroidism
  • (E03.4) Atrophy of thyroid (acquired)
  • (E03.5) Myxoedema coma
• (E04.) Other nontoxic goitre

• (E05.) Thyrotoxicosis (hyperthyroidism)
  • (E05.0) Thyrotoxicosis with diffuse goitre
    • Graves' disease
  • (E05.1) Thyrotoxicosis with toxic single thyroid nodule
  • (E05.2) Thyrotoxicosis with toxic multinodular goitre
  • (E05.3) Thyrotoxicosis from ectopic thyroid tissue
  • (E05.4) Thyrotoxicosis factitia
  • (E05.5) Thyroid crisis or storm
  • (E05.8) Other thyrotoxicosis
  • (E05.9) Thyrotoxicosis, unspecified

• (E06.) Thyroiditis
  • (E06.0) Acute thyroiditis
  • (E06.1) Subacute thyroiditis
    • De Quervain's thyroiditis
  • (E06.2) Chronic thyroiditis with transient thyrotoxicosis
  • (E06.3) Autoimmune thyroiditis
    • Hashimoto's thyroiditis
  • (E06.4) Drug-induced thyroiditis
  • (E06.5) Other chronic thyroiditis
    • Riedel's thyroiditis
  • (E06.9) Thyroiditis, unspecified

• (E07.) Other disorders of thyroid
  • (E07.0) Hypersecretion of calcitonin
  • (E07.1) Dyshormogenetic goitre
  • (E07.8) Other specified disorders of thyroid
    • Sick-euthyroid syndrome
  • (E07.9) Disorder of thyroid, unspecified

(E10-E16) Pancreas / Insulin, glucagon

(E10-E14) Diabetes mellitus

• Note: the following conditions are subtypes of each code from E10-14:
  • (E1x.0) Diabetic coma
  • (E1x.1) Diabetic ketoacidosis
  • (E1x.2) Diabetic nephropathy
  • (E1x.3) Diabetic retinopathy
  • (E1x.4) Diabetic neuropathy
  • (E1x.5) Diabetic angiopathy
  • (E1x.6) Diabetic arthropathy
• (E10.) Insulin-dependent diabetes mellitus
• (E11.) Non-insulin-dependent diabetes mellitus
• (E12.) Malnutrition-related diabetes mellitus
• (E13.) Other specified diabetes mellitus
• (E14.) Unspecified diabetes mellitus

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

• (E15.) Nondiabetic hypoglycaemic coma
  • • Drug-induced insulin coma in nondiabetic
    • Hyperinsulinism with hypoglycaemic coma
    • Hypoglycaemic coma NOS

• (E16.) Other disorders of pancreatic internal secretion
  • (E16.0) Drug-induced hypoglycaemia without coma
  • (E16.1) Other hypoglycaemia
    • Functional nonhyperinsulinaemic hypoglycaemia
    • Hyperinsulinism: NOS
    • Hyperinsulinism: functional
    • Hyperplasia of pancreatic islet beta cells NOS
    • Posthypoglycaemic coma encephalopathy
  • (E16.2) Hypoglycaemia, unspecified
  • (E16.3) Increased secretion of glucagon
  • (E16.4) Abnormal secretion of gastrin
    • Hypergastrinaemia
    • Zollinger-Ellison syndrome
  • (E16.8) Other specified disorders of pancreatic internal secretion
  • (E16.9) Disorder of pancreatic internal secretion, unspecified

(E20-E21) Parathyroid gland / PTH

• (E20.) Hypoparathyroidism
  • (E20.0) Idiopathic hypoparathyroidism
  • (E20.1) Pseudohypoparathyroidism

• (E21.) Hyperparathyroidism and other disorders of parathyroid gland
  • (E21.0) Primary hyperparathyroidism
  • (E21.1) Secondary hyperparathyroidism, not elsewhere classified

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

• (E22.) Hyperfunction of pituitary gland
  • (E22.0) Acromegaly and pituitary gigantism
  • (E22.1) Hyperprolactinaemia
  • (E22.2) Syndrome of inappropriate secretion of antidiuretic hormone
    • Central precocious puberty

• (E23.) Hypofunction and other disorders of pituitary gland
  • (E23.0) Hypopituitarism
    • Fertile eunuch syndrome
    • Hypogonadotropic hypogonadism
    • Idiopathic growth hormone deficiency
    • Isolated deficiency of gonadotropin
    • Isolated deficiency of growth hormone
    • Isolated deficiency of pituitary hormone
    • Kallmann's syndrome
    • Lorain-Levi short stature
    • Necrosis of pituitary gland (postpartum)
    • Panhypopituitarism
    • Pituitary cachexia
    • Pituitary insufficiency NOS
    • Pituitary short stature
    • Sheehan's syndrome
    • Simmonds' disease
  • (E23.1) Drug-induced hypopituitarism
  • (E23.2) Diabetes insipidus
  • (E23.3) Hypothalamic dysfunction, not elsewhere classified
  • (E23.6) Other disorders of pituitary gland
    • Abscess of pituitary
    • Adiposogenital dystrophy
  • (E23.7) Disorder of pituitary gland, unspecified

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

• (E24.) Cushing's syndrome
  • (E24.0) Pituitary-dependent Cushing's disease
  • (E24.1) Nelson's syndrome
  • (E24.2) Ectopic ACTH syndrome
  • (E24.3) Alcohol-induced pseudo-Cushing's syndrome
  • (E24.4) Other Cushing's syndrome
  • (E24.5) Cushing's syndrome, unspecified

• (E25.) Adrenogenital disorders
  • (E25.0) Congenital adrenogenital disorders associated with enzyme deficiency
    • Congenital adrenal hyperplasia
      • Congenital adrenal hyperplasia due to 21-hydroxylase deficiency

• (E26.) Hyperaldosteronism
  • (E26.0) Primary hyperaldosteronism
    • Conn's syndrome
    • Primary aldosteronism due to adrenal hyperplasia (bilateral)
  • (E26.1) Secondary hyperaldosteronism
  • (E26.8) Other hyperaldosteronism
    • Bartter's syndrome
  • (E26.9) Hyperaldosteronism, unspecified

• (E27.) Other disorders of adrenal gland
  • (E27.0) Other adrenocortical overactivity
  • (E27.1) Primary adrenocortical insufficiency
    • Addison's disease
  • (E27.2) Addisonian crisis
  • (E27.3) Drug-induced adrenocortical insufficiency
  • (E27.4) Other and unspecified adrenocortical insufficiency
    • Hypoaldosteronism
    • Adrenocortical insufficiency NOS
  • (E27.5) Adrenomedullary hyperfunction

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

• (E28.) Ovarian dysfunction
  • (E28.0) Estrogen excess
  • (E28.1) Androgen excess
  • (E28.2) Polycystic ovarian syndrome
  • (E28.3) Primary ovarian failure
    • Premature menopause NOS

• (E29.) Testicular dysfunction
  • (E29.0) Testicular hyperfunction
  • (E29.1) Testicular hypofunction
    • 5-Alpha-reductase deficiency (with male pseudohermaphroditism)
    • Testicular hypogonadism NOS

• (E30.) Disorders of puberty, not elsewhere classified
  • (E30.0) Delayed puberty
  • (E30.1) Precocious puberty
  • (E30.8) Other disorders of puberty
    • Premature thelarche

(E31-E35) Other

• (E31.) Polyglandular dysfunction
  • (E31.0) Autoimmune polyglandular failure
    • Schmidt's syndrome
  • (E31.1) Polyglandular hyperfunction

• (E32.) Diseases of thymus
  • (E32.0) Persistent hyperplasia of thymus
    • Hypertrophy of thymus
  • (E32.1) Abscess of thymus

• (E34.) Other endocrine disorders
  • (E34.0) Carcinoid syndrome
  • (E34.1) Other hypersecretion of intestinal hormones
  • (E34.2) Ectopic hormone secretion, not elsewhere classified
  • (E34.3) Short stature, not elsewhere classified
    • NOS
    • constitutional
    • Laron-type
    • psychosocial
  • (E34.4) Constitutional tall stature
    • Constitutional gigantism
  • (E34.5) Androgen resistance syndrome
    • Reifenstein's syndrome
  • (E34.8) Other specified endocrine disorders
    • Progeria

• (E35.) Disorders of endocrine glands in diseases classified elsewhere
  • (E35.0) Disorders of thyroid gland in diseases classified elsewhere
  • (E35.1) Disorders of adrenal glands in diseases classified elsewhere
  • (E35.8) Disorders of other endocrine glands in diseases classified elsewhere

E40-68 - Nutritional diseases

(E40-E46) Malnutrition

• (E40.) Kwashiorkor
• (E41.) Nutritional marasmus
• (E42.) Marasmic kwashiorkor
• (E43.) Unspecified severe protein-energy malnutrition
• (E44.) Protein-energy malnutrition of moderate and mild degree
• (E45.) Retarded development following protein-energy malnutrition
• (E46.) Unspecified protein-energy malnutrition

(E50-E64) Other nutritional deficiencies

• (E50.) Vitamin A deficiency
  • (E50.0) Vitamin A deficiency with conjunctival xerosis
  • (E50.1) Vitamin A deficiency with Bitot's spot and conjunctival xerosis
  • (E50.2) Vitamin A deficiency with corneal xerosis
  • (E50.3) Vitamin A deficiency with corneal ulceration and xerosis
  • (E50.4) Vitamin A deficiency with keratomalacia
  • (E50.5) Vitamin A deficiency with night blindness
  • (E50.6) Vitamin A deficiency with xerophthalmic scars of cornea
  • (E50.7) Other ocular manifestations of vitamin A deficiency
    • Xerophthalmia NOS
• (E51.) Thiamine deficiency
  • (E51.1) Beriberi
  • (E51.2) Wernicke's encephalopathy
• (E52.) Niacin deficiency (pellagra)
• (E53.) Deficiency of other B group vitamins
  • (E53.0) Riboflavin deficiency
    • Ariboflavinosis
  • (E53.1) Pyridoxine deficiency
  • (E53.8) Deficiency of other specified B group vitamins
• (E54.) Ascorbic acid deficiency
  • Scurvy
• (E55.) Vitamin D deficiency
  • (E55.0) Rickets, active
• (E56.) Other vitamin deficiencies
  • (E56.0) Deficiency of vitamin E
  • (E56.1) Deficiency of vitamin K
• (E58.) Dietary calcium deficiency
• (E59.) Dietary selenium deficiency
• (E60.) Dietary zinc deficiency
• (E61.) Deficiency of other nutrient elements
  • (E61.1) Iron deficiency
  • (E61.2) Magnesium deficiency
  • (E61.3) Manganese deficiency
  • (E61.4) Chromium deficiency
  • (E61.5) Molybdenum deficiency
  • (E61.6) Vanadium deficiency
• (E63.) Other nutritional deficiencies
  • (E63.0) Essential fatty acid (EFA) deficiency
• (E64.) Sequelae of malnutrition and other nutritional deficiencies

(E65-E68) Obesity and other hyperalimentation

• (E65.) Localized adiposity
  • Fat pad

• (E66.) Obesity
  • (E66.0) Obesity due to excess calories
  • (E66.1) Drug-induced obesity
  • (E66.2) Extreme obesity with alveolar hypoventilation
    • Pickwickian syndrome
  • (E66.8) Other obesity
    • Morbid obesity
  • (E66.9) Obesity, unspecified

• (E67.) Other hyperalimentation
  • (E67.0) Hypervitaminosis A
  • (E67.1) Hypercarotenaemia
  • (E67.2) Megavitamin-B 6 syndrome
  • (E67.3) Hypervitaminosis D

• (E68.) 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
  • (E70.0) Classical phenylketonuria
  • (E70.1) Other hyperphenylalaninaemias
  • (E70.2) Disorders of tyrosine metabolism
    • Alkaptonuria (ILDS E70.210)
    • Ochronosis (ILDS E70.230)
    • Tyrosinaemia
  • (E70.3) Albinism
    • Oculocutaneous albinism (ILDS E70.310)
    • Partial albinism (ILDS E70.312)
    • Oculocutaneous albinism type 1 (tyrosinase-negative) (ILDS E70.314)
    • Oculocutaneous albinism type 2 (tyrosinase positive) (ILDS E70.314)
    • Albinoidism (ILDS E70.318)
    • Waardenburg's syndrome (ILDS E70.320)
    • Chediak-Higashi syndrome (ILDS E70.340)
    • Piebaldism (ILDS E70.350)
    • Tietz's syndrome (ILDS E70.358)
    • Hermansky-Pudlak syndrome (ILDS E70.360)
    • Cross syndrome (ILDS E70.380)
  • (E70.8) Other disorders of aromatic amino-acid metabolism
    • Disorders of histidine metabolism
    • Disorders of tryptophan 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
    • Propionic acidemia
    • Methylmalonic acidemia
    • Isovaleric acidemia
  • (E71.2) 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
    • Cystinuria
    • Cystinosis
    • Hartnup disease
    • Lowe's syndrome
  • (E72.1) Disorders of sulfur-bearing amino-acid metabolism
    • Cystathioninuria
    • Homocystinuria
    • Methioninaemia
    • Sulfite oxidase deficiency
  • (E72.2) Disorders of urea cycle metabolism
    • Argininaemia
    • Argininosuccinic aciduria
    • Citrullinaemia
    • Hyperammonaemia
  • (E72.3) Disorders of lysine and hydroxylysine metabolism
    • Glutaric aciduria
    • Hydroxylysinaemia
    • Hyperlysinaemia
    • Lysinuric protein intolerance
  • (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

• (E73.) Lactose intolerance

• (E74.) Other disorders of carbohydrate metabolism
  • (E74.0) Glycogen storage disease
    • Glycogen storage disease type I (von Gierke's disease)
    • Glycogen storage disease type II (Pompe's disease)
    • Glycogen storage disease type III
    • Glycogen storage disease type IV
    • Glycogen storage disease type V (McArdle's disease)
  • (E74.1) Disorders of fructose metabolism
    • Essential fructosuria
    • Fructose-1,6-diphosphatase deficiency
    • Hereditary fructose intolerance
  • (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
    • Deficiency of phosphoenolpyruvate carboxykinase
    • Deficiency of pyruvate carboxylase
    • Deficiency of pyruvate dehydrogenase
  • (E74.8) Other specified disorders of carbohydrate metabolism
    • Essential pentosuria
    • Oxalosis
    • Oxaluria
    • Renal glycosuria
  • (E74.9) 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
    • GM 1
    • GM 3
    • Mucolipidosis IV
  • (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)
  • (E75.3) 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
  • (E75.6) 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
    • Aspartylglucosaminuria
    • Fucosidosis
    • Mannosidosis
    • Sialidosis (mucolipidosis I)

• (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
  • (E78.5) Hyperlipidaemia, unspecified
  • (E78.6) Lipoprotein deficiency
    • Abetalipoproteinaemia
    • High-density lipoprotein deficiency
    • Hypoalphalipoproteinaemia
    • Hypobetalipoproteinaemia (familial)
    • Lecithin cholesterol acyltransferase deficiency
    • Tangier disease

(E79-E90) Other metabolic disorders

• (E79.) Disorders of purine and pyrimidine metabolism
  • (E79.0) Hyperuricaemia without signs of inflammatory arthritis and tophaceous disease
  • (E79.1) 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)
  • (E80.4) Gilbert's 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
  • (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

• (E84.) Cystic fibrosis

• (E85.) Amyloidosis
  • (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
  • Hypovolaemia

• (E87.) Other disorders of fluid, electrolyte and acid-base balance
  • (E87.0) Hyperosmolality and hypernatraemia
  • (E87.1) Hypo-osmolality and hyponatraemia
  • (E87.2) Acidosis
    • Respiratory acidosis
    • Metabolic acidosis
    • Lactic acidosis
  • (E87.3) Alkalosis
  • (E87.4) Mixed disorder of acid-base balance
  • (E87.5) Hyperkalaemia
  • (E87.6) Hypokalaemia
  • (E87.7) Fluid overload
  • (E87.8) Other disorders of electrolyte and fluid balance, not elsewhere classified
    • Electrolyte imbalance NOS
    • Hyperchloraemia
    • Hypochloraemia

• (E88.) Other metabolic disorders
  • (E88.0) Disorders of plasma-protein metabolism, not elsewhere classified
    • Alpha-1-antitrypsin deficiency
    • Bisalbuminaemia
  • (E88.1) Lipodystrophy, not elsewhere classified
  • (E88.2) Lipomatosis, not elsewhere classified
  • (E88.8) Other specified metabolic disorders
    • Launois-Bensaude adenolipomatosis
    • Trimethylaminuria
  • (E88.9) Metabolic disorder, unspecified

• (E89.) Postprocedural endocrine and metabolic disorders, not elsewhere classified
  • (E89.0) Postprocedural hypothyroidism
  • (E89.1) Postprocedural hypoinsulinaemia
  • (E89.2) Postprocedural hypoparathyroidism
  • (E89.3) Postprocedural hypopituitarism
  • (E89.4) Postprocedural ovarian failure
  • (E89.5) Postprocedural testicular hypofunction
  • (E89.6) Postprocedural adrenocortical(-medullary) hypofunction

• (E90.) Nutritional and metabolic disorders in diseases classified elsewhere

See also

• List of ICD-10 codes
• International Statistical Classification of Diseases and Related Health Problems
• List of ICD-9 codes 240-279: Endocrine, nutritional and metabolic diseases, and immunity disorders

v • d • e Pathology: Medical conditions (Diseases/Disorders/Illness, Syndromes/Sequences, Symptoms/Signs, Injuries, etc.) and ICD codes (A/B, 001-139) Infectious disease/Infection: Bacterial disease (G+, G-) · Virus disease · Parasitic disease (Protozoan infection, Helminthiasis, Ectoparasitic infestation) · Mycosis · Zoonosis (C/D, 140-239 & 280-289) Cancer (C00-D48, 140-239) Tumor Myeloid hematologic (D50-D77, 280-289) Anemia · Coagulopathy Lymphoid immune (D80-D89, 279) Immunodeficiency · Immunoproliferative disorder · Hypersensitivity (E, 240-278) Endocrine disease · Nutrition disorder · Inborn error of metabolism (F, 290-319) Mental disorder (G, 320-359) Nervous system disease (CNS, PNS) · Neuromuscular disease (H, 360-389) Eye disease · Ear disease (I, 390-459) Cardiovascular disease (Heart disease, Vascular disease) (J, 460-519) Respiratory disease (Obstructive lung disease, Restrictive lung disease, Pneumonia) (K, 520-579) Stomatognathic disease (Tooth disease) · Digestive disease (Esophageal, Stomach, Enteropathy, Liver, Pancreatic) (L, 680-709) Skin disease · skin appendages (Nail disease, Hair disease, Sweat gland disease) (M, 710-739) Musculoskeletal disorders · Osteochondropathy (Osteopathy, Chondropathy) (N, 580-629) Urologic disease (Nephropathy, Urinary bladder disease) · Male genital disease · Breast disease · Female genital disease (O, 630-679) Complications of pregnancy · Obstetric labor complication · Puerperal disorder (P, 760-779) Fetal disease (Q, 740-759) Congenital disorder (R, 780-799) Syndromes · Medical signs (Eponymous) (S/T, 800-999) Bone fracture · Joint dislocation · Sprain · Strain · subluxation · Head injury · Chest trauma · Poisoning

v • d • e Endocrine pathology: endocrine diseases (E00-35, 240-259) Pancreas/ glucose metabolism Hypofunction Diabetes mellitus types: (type 1, type 2, MODY 1 2 3 4 5 6) · complications (coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy, cardiomyopathy) insulin receptor (Rabson-Mendenhall syndrome) · Insulin resistance Hyperfunction Hypoglycemia · beta cell (Hyperinsulinism) · G cell (Zollinger-Ellison syndrome) Hypothalamic/ pituitary axes Hypothalamus gonadotropin (Kallmann syndrome, Adiposogenital dystrophy) · CRH (Tertiary adrenal insufficiency) · vasopressin (Neurogenic diabetes insipidus) · general (Hypothalamic hamartoma) Pituitary Hyperpituitarism anterior (Acromegaly, Hyperprolactinaemia, Pituitary ACTH hypersecretion) · posterior (SIADH) · general (Nelson's syndrome) Hypopituitarism anterior (Kallmann syndrome, Growth hormone deficiency, ACTH deficiency/Secondary adrenal insufficiency) · posterior (Neurogenic diabetes insipidus) · general (Empty sella syndrome, Pituitary apoplexy, Sheehan's syndrome, Lymphocytic hypophysitis) Thyroid Hypothyroidism Iodine deficiency · Cretinism (Congenital hypothyroidism) · Myxedema · Euthyroid sick syndrome Hyperthyroidism Hyperthyroxinemia (Thyroid hormone resistance, Familial dysalbuminemic hyperthyroxinemia) · Hashitoxicosis · Thyrotoxicosis factitia · Graves' disease Thyroiditis Acute infectious · Subacute (De Quervain's, Subacute lymphocytic) · Autoimmune/chronic (Hashimoto's, Postpartum, Riedel's) Goitre Endemic goitre · Toxic nodular goitre · Toxic multinodular goitre Thyroid nodule Parathyroid Hypoparathyroidism Pseudohypoparathyroidism Hyperparathyroidism Primary · Secondary · Tertiary · Osteitis fibrosa cystica Adrenal Hyperfunction aldosterone: Hyperaldosteronism/Primary aldosteronism (Conn syndrome, Bartter syndrome, Glucocorticoid remediable aldosteronism) · AME · Liddle's syndrome · 17α CAH cortisol: Cushing's syndrome (Pseudo-Cushing's syndrome) sex hormones: 21α CAH · 11β CAH Hypofunction/ Adrenal insufficiency (Addison's, WF) aldosterone: Hypoaldosteronism (21α CAH, 11β CAH) cortisol: CAH (Lipoid, 3β, 11β, 17α, 21α) sex hormones: 17α CAH Gonads ovarian: Polycystic ovary syndrome · Premature ovarian failure testicular: enzymatic (5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency) · Androgen receptor (Androgen insensitivity syndrome) general: Hypogonadism (Delayed puberty) · Hypergonadism (Precocious puberty) Height Gigantism · Dwarfism/Short stature (Laron syndrome, Psychogenic dwarfism) Multiple Autoimmune polyendocrine syndrome (APS1, APS2) · Carcinoid syndrome · Multiple endocrine neoplasia (1, 2A, 2B) · Progeria · Woodhouse-Sakati syndrome endocrine navs: anat/physio/dev/hormones, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Nutrition disorders (E40-68, 260-269) Hypoalimentation/ malnutrition Protein-energy malnutrition Kwashiorkor · Marasmus · Catabolysis Avitaminosis B vitamins B1: Beriberi/Wernicke's encephalopathy(Thiamine deficiency) · B2: Ariboflavinosis · B3: Pellagra(Niacin deficiency) · 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: Hypovitaminosis D/Rickets/Osteomalacia · E: Vitamin E deficiency · K: Vitamin K deficiency Mineral deficiency Zinc · Iron · Magnesium · Chromium · Selenium (Keshan disease) · Manganese · Molybdenum · Copper · Calcium · Potassium Hyperalimentation Overweight · Obesity Childhood obesity · Obesity hypoventilation syndrome · Abdominal obesity Vitamin poisoning Hypervitaminosis A · Hypervitaminosis D · Hypervitaminosis E Mineral overload see inborn errors of metal metabolism, toxicity nutrition navs: disorders, symptoms+signs/eponymous

v • d • e Inborn error of amino acid metabolism (E70-72, 270) K→acetyl-CoA Lysine/straight chain Glutaric acidemia type 1 · type 2 · Hyperlysinemia · Pipecolic acidemia · Saccharopinuria Leucine Maple syrup urine disease · Isovaleric acidemia · 3-Methylcrotonyl-CoA carboxylase deficiency · 3-hydroxy-3-methylglutaryl-CoA lyase deficiency Tryptophan Hypertryptophanemia G G→pyruvate→citrate Glycine Sarcosinemia · D-Glyceric acidemia · Glutathione synthetase deficiency Glycine→Creatine: GAMT deficiency G→glutamate→ α-ketoglutarate Histidine Carnosinemia · Histidinemia · Urocanic aciduria Proline Hyperprolinemia · Prolidase deficiency Glutamate/glutamine SSADHD G→propionyl-CoA→ succinyl-CoA Valine Maple syrup urine disease · Hypervalinemia · Isobutyryl-CoA dehydrogenase deficiency Isoleucine Maple syrup urine disease · Beta-ketothiolase deficiency · 2-Methylbutyryl-CoA dehydrogenase deficiency Methionine Hypermethioninemia · Homocystinuria · Cystathioninuria General BC/OA Propionic acidemia · Methylmalonic acidemia · Methylmalonyl-CoA mutase deficiency G→fumarate Phenylalanine/tyrosine Phenylketonuria Tetrahydrobiopterin deficiency · 6-Pyruvoyltetrahydropterin synthase deficiency Tyrosinemia Type II tyrosinemia · Type III tyrosinemia/Hawkinsinuria · Alkaptonuria/Ochronosis · Type I tyrosinemia Tyrosine→Melanin Albinism: Ocular albinism (1) · Oculocutaneous albinism (Hermansky–Pudlak syndrome) · Waardenburg syndrome Tyrosine→Norepinephrine Dopamine beta hydroxylase deficiency G→oxaloacetate Urea cycle/Hyperammonemia (arginine, aspartate) N-Acetylglutamate synthase deficiency · Carbamoyl phosphate synthetase I deficiency · Ornithine transcarbamylase deficiency/translocase deficiency · Citrullinemia · Argininosuccinic aciduria · Argininemia Transport/ IE of RTT Solute carrier family: Cystinuria · Hartnup disease · Lysinuric protein intolerance · Iminoglycinuria other/general/unknown: Oculocerebrorenal syndrome · Fanconi syndrome · Cystinosis Other Trimethylaminuria · 2-Hydroxyglutaric aciduria · Fumarase deficiency see also Amino acid metabolism enzymes, intermediates see also Urea cycle enzymes, intermediates

v • d • e Inborn error of lipid metabolism: fatty-acid metabolism disorders (E71.3, 277.81-277.85) Synthesis Biotinidase deficiency Degradation Acyl transport Carnitine (Primary, I, II, -acylcarnitine) · Adrenoleukodystrophy Beta oxidation General Acyl CoA dehydrogenase (Short-chain, Medium-chain, Long-chain 3-hydroxy, Very long-chain) Mitochondrial trifunctional protein deficiency: Acute fatty liver of pregnancy Unsaturated 2,4 Dienoyl-CoA reductase deficiency Odd chain Propionic acidemia Other 3-hydroxyacyl-coenzyme A dehydrogenase deficiency To acetyl-CoA Malonic aciduria Aldehyde Sjögren-Larsson syndrome see also enzymes, intermediates

v • d • e Inborn error of carbohydrate metabolism: monosaccharide metabolism disorders (including glycogen storage diseases) (E73-74, 271) Sucrose, transport (extracellular) Disaccharide catabolism Lactose intolerance · Sucrose intolerance Monosaccharide transport Glucose-galactose malabsorption · Inborn errors of renal tubular transport (Renal glycosuria) · Fructose malabsorption Hexose → glucose Monosaccharide catabolism fructose: Essential fructosuria · Fructose intolerance galactose/galactosemia : GALK deficiency · GALT deficiency/GALE deficiency Glucose ⇄ glycogen Glycogenesis GSD type 0, glycogen synthase · GSD type IV, Andersen's, branching Glycogenolysis extralysosomal: GSD type V, McArdle, muscle glycogen phosphorylase/GSD type VI, Hers', liver glycogen phosphorylase · GSD type III, Cori's, debranching lysosomal/LSD: GSD type II, Pompe's, glucosidase Glucose ⇄ CAC Glycolysis MODY 2 · GSD type VII, Tarui's, phosphofructokinase · Triosephosphate isomerase deficiency · Pyruvate kinase deficiency Pyruvate catabolism PDHA · Fumarase deficiency Gluconeogenesis PCD · Fructose bisphosphatase deficiency · GSD type I, von Gierke, glucose 6-phosphatase Pentose phosphate pathway Glucose-6-phosphate dehydrogenase deficiency · Pentosuria Other Hyperoxaluria (Primary hyperoxaluria) see also fructose and galactose metabolism enzymes, intermediates; see also glycolysis enzymes, intermediates see also glycogenesis and glycogenolysis enzymes, intermediates; see also pentose phosphate pathway enzymes, intermediates

v • d • e (LSD) Inborn error of lipid metabolism: lipid storage disorders (E75, 272.7-272.8) Sphingolipidoses (to ceramide) From ganglioside (gangliosidoses) Ganglioside: GM1 gangliosidoses · GM2 gangliosidoses (Sandhoff disease, Tay-Sachs disease, AB variant) From globoside Globotriaosylceramide: Fabry's disease From sphingomyelin Sphingomyelin: phospholipid: Niemann-Pick disease (SMPD1-associated, type C) Glucocerebroside: Gaucher's disease From sulfatide (sulfatidoses, leukodystrophy) Sulfatide: Metachromatic leukodystrophy · Multiple sulfatase deficiency Galactocerebroside: Krabbe disease To sphingosine Ceramide: Farber disease NCL Infantile · Jansky-Bielschowsky disease · Batten disease Other Cerebrotendineous xanthomatosis · Cholesteryl ester storage disease (Wolman disease) · Sea-blue histiocyte syndrome see also enzymes, sphingolipids

v • d • e (LSD) Inborn error of carbohydrate metabolism: mucopolysaccharidosis (E76, 277.5) Anabolism Pentosuria Catabolism 1:Hurler/Scheie · 2:Hunter · 3:Sanfilippo ABCD · 4:Morquio · 6:Maroteaux-Lamy · 7:Sly see also enzymes, glycosaminoglycans

v • d • e (LSD) Inborn error of carbohydrate metabolism: glycoproteinosis/mucolipidosis (E77, 272.7) Post-translational modification of lysosomal enzymes I-cell disease/II · Pseudo-Hurler polydystrophy/III Glycoprotein degradation Aspartylglucosaminuria · Fucosidosis · mannosidosis (Alpha-mannosidosis, Beta-mannosidosis) - Sialidosis/I Other channelopathy (Mucolipidosis type IV) · solute carrier family (Salla disease) · Galactosialidosis see also enzymes, glycoproteins

v • d • e Inborn error of lipid metabolism: dyslipidemia (E78, 272.0-272.6) Hyperlipidemia Hypercholesterolemia/Hypertriglyceridemia (Type I, Familial hypercholesterolemia/Type IIa, Combined hyperlipidemia/Type IIb) · Xanthoma Hypolipoproteinemia Hypoalphalipoproteinemia/HDL Lecithin cholesterol acyltransferase deficiency · Tangier disease Hypobetalipoproteinemia/LDL Abetalipoproteinemia · Apolipoprotein B deficiency Lipodystrophy Barraquer-Simons syndrome Other Lipomatosis · Adiposis dolorosa · Lipoid proteinosis see also lipoprotein metabolism

v • d • e Inborn error of purine-pyrimidine metabolism (E79, 277.2) Purine metabolism Anabolism Adenylosuccinate lyase deficiency - Myoadenylate deaminase deficiency Nucleotide salvage Lesch-Nyhan syndrome/Hyperuricemia - Adenine phosphoribosyltransferase deficiency Catabolism Adenosine deaminase deficiency - Purine nucleoside phosphorylase deficiency - Xanthinuria - Gout Pyrimidine metabolism Anabolism Orotic aciduria Catabolism Dihydropyrimidine dehydrogenase deficiency see also nucleotide metabolism, intermediates

v • d • e Heme metabolism disorders (E80, 277.1, 277.4) Porphyria, hepatic and erythropoietic (porphyrin) early mitochondrial: ALAD porphyria · Acute intermittent porphyria cytoplasmic: Gunther disease/congenital erythropoietic porphyria · Porphyria cutanea tarda/Hepatoerythropoietic porphyria late mitochondrial: Hereditary coproporphyria · Variegate porphyria · Erythropoietic protoporphyria Hereditary hyperbilirubinemia (bilirubin) unconjugated: Gilbert's syndrome · Crigler-Najjar syndrome · Lucey-Driscoll syndrome conjugated: Dubin-Johnson syndrome · Rotor syndrome see also porphyrin metabolism enzymes, intermediates

v • d • e Inborn error of metal metabolism (E83, 275) Transition metal Cu high: Copper toxicity · Wilson's disease deficiency: Copper deficiency · Menkes disease Fe high: Primary iron overload disorder: Hemochromatosis/HFE1 · Juvenile/HFE2 · HFE3 · African iron overload/HFE4 · Aceruloplasminemia · Atransferrinemia · Hemosiderosis deficiency: Iron deficiency Zn high: Zinc toxicity deficiency: Acrodermatitis enteropathica Electrolyte PO43− high: Hyperphosphatemia deficiency: Hypophosphatemia · alkaline phosphatase (Hypophosphatasia) Mg2+ high: Hypermagnesemia deficiency: Hypomagnesemia Ca2+ high: Hypercalcaemia · Milk-alkali syndrome (Burnett's) · Calcinosis (Calciphylaxis, Calcinosis cutis) · Calcification (Metastatic calcification, Dystrophic calcification) deficiency: Hypocalcaemia · Osteomalacia · Pseudohypoparathyroidism (Albright's hereditary osteodystrophy) · Pseudopseudohypoparathyroidism see also metal metabolism

v • d • e Metabolic disease: amyloidosis (E85, 277.3) Common amyloid forming proteins Serum amyloid A  · Transthyretin  · Beta-2 microglobulin  · Bence Jones protein  · Amyloid precursor protein  · Beta amyloid  · Amylin Systemic amyloidosis AL amyloidosis  · AA amyloidosis  · Haemodialysis-associated amyloidosis  · Senile systemic amyloidosis  · Finnish type amyloidosis  · Familial Mediterranean fever Organ-limited amyloidosis Heart Cardiac amyloidosis Brain Familial amyloid neuropathy  · Cerebral amyloid angiopathy  · Alzheimer's disease Kidney Familial renal amyloidosis Cutaneous Primary cutaneous amyloidosis  · Amyloid purpura Thyroid Calcitonin Pituitary Prolactin Pancreas Amylin

v • d • e Water-electrolyte imbalance and acid-base imbalance (E86-E87, 276) Volume status Volume contraction (Dehydration/Hypovolemia)  · Hypervolemia Electrolyte Na+ Hypernatremia · Hyponatremia (Hypotonic, Isotonic) K+ Hyperkalemia · Hypokalemia Cl− Hyperchloremia · Hypochloremia Acid-base Acidosis Metabolic: High anion gap (Ketoacidosis/Diabetic ketoacidosis, Lactic) · Normal anion gap (Hyperchloremic, Renal tubular) Respiratory Alkalosis Metabolic: Contraction alkalosis Respiratory Both Mixed disorder of acid-base balance urinary system navs: anat/physio/dev, noncongen/acid+base/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Other metabolic pathology / Inborn error of metabolism (E70-90, 270-279) Other Aldolase A deficiency · Alpha 1-antitrypsin deficiency · Cystic fibrosis · Acatalasia

References

1. ^ http://apps.who.int/classifications/apps/icd/icd10online/?ge65.htm+e66
2. ^ CMS Office of Public Affairs (August 15, 2008). "HHS Proposes Adoption of ICD-10 Code Sets and Updated Electronic Transaction Standards" (web). News Release. U.S. Department of Health & Human Services. http://www.dhhs.gov/news/press/2008pres/08/20080815a.html. Retrieved 2008-10-22.