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

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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 com 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

(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
- (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
- (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

(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
  - Chediak-Higashi syndrome
  - Cross syndrome
  - Hermansky-Pudlak syndrome
- (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
- (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
- (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
- (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
- (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
  - 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

(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
  - 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

(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
- (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

(E88) Other metabolic disorders
- (E88.0) Disorders of plasma-protein metabolism, not elsewhere classified
  - Alpha-1-antitrypsin deficiency
- (E88.1) Lipodystrophy, not elsewhere classified
- (E88.2) Lipomatosis, not elsewhere classified

(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

v t 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 - Hypophysitis
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

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

Contents

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

See also

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