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

# 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
- (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
- (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
- (E05.0) Thyrotoxicosis with diffuse goitre
Graves' disease
- 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
- (E06.0) Acute thyroiditis
- (E06.1) Subacute thyroiditis
De Quervain's thyroiditis
- De Quervain's thyroiditis
- (E06.2) Chronic thyroiditis with transient thyrotoxicosis
- (E06.3) Autoimmune thyroiditis
Hashimoto's thyroiditis
- Hashimoto's thyroiditis
- (E06.4) Drug-induced thyroiditis
- (E06.5) Other chronic thyroiditis
Riedel's 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
- (E07.0) Hypersecretion of calcitonin
- (E07.1) Dyshormogenetic goitre
- (E07.8) Other specified disorders of thyroid
Sick-euthyroid syndrome
- 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
- (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
- Drug-induced insulin coma in nondiabetic
Hyperinsulinism with hypoglycaemic coma
Hypoglycaemic com NOS
- 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
- (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
- 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
- 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
- (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
- (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
- (E22.0) Acromegaly and pituitary gigantism
- (E22.1) Hyperprolactinaemia
- (E22.2) Syndrome of inappropriate secretion of antidiuretic hormone
Central precocious puberty
- 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
- (E23.0) 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
- 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
- (E23.1) 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
- (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
- (E25.0) Congenital adrenogenital disorders associated with enzyme deficiency
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
- Congenital adrenal hyperplasia
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
- 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
- (E26.0) Primary hyperaldosteronism
Conn's syndrome
Primary aldosteronism due to adrenal hyperplasia (bilateral)
- Conn's syndrome
- Primary aldosteronism due to adrenal hyperplasia (bilateral)
- (E26.1) Secondary hyperaldosteronism
- (E26.8) Other hyperaldosteronism
Bartter's syndrome
- 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
- (E27.0) Other adrenocortical overactivity
- (E27.1) Primary adrenocortical insufficiency
Addison's disease
- Addison's disease
- (E27.2) Addisonian crisis
- (E27.3) Drug-induced adrenocortical insufficiency
- (E27.4) Other and unspecified adrenocortical insufficiency
Hypoaldosteronism
Adrenocortical insufficiency NOS
- 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
- (E28.0) Estrogen excess
- (E28.1) Androgen excess
- (E28.2) Polycystic ovarian syndrome
- (E28.3) Primary ovarian failure
Premature menopause NOS
- Premature menopause NOS
- (E29) Testicular dysfunction
(E29.0) Testicular hyperfunction
(E29.1) Testicular hypofunction
5-Alpha-reductase deficiency (with male pseudohermaphroditism)
Testicular hypogonadism NOS
- (E29.0) Testicular hyperfunction
- (E29.1) Testicular hypofunction
5-Alpha-reductase deficiency (with male pseudohermaphroditism)
Testicular hypogonadism NOS
- 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
- (E30.0) Delayed puberty
- (E30.1) Precocious puberty
- (E30.8) Other disorders of puberty
Premature thelarche
- Premature thelarche

## (E31-E35) Other
- (E31) Polyglandular dysfunction
(E31.0) Autoimmune polyglandular failure
Schmidt's syndrome
(E31.1) Polyglandular hyperfunction
- (E31.0) Autoimmune polyglandular failure
Schmidt's syndrome
- Schmidt's syndrome
- (E31.1) Polyglandular hyperfunction
- (E32) Diseases of thymus
(E32.0) Persistent hyperplasia of thymus
(E32.1) Abscess 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
- (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
- NOS
- constitutional
- Laron-type
- psychosocial
- (E34.4) Constitutional tall stature
- (E34.5) Androgen resistance syndrome
Reifenstein's syndrome
- Reifenstein's syndrome
- (E34.8) Other specified endocrine disorders
Progeria
- 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
- (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
- (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
- Xerophthalmia NOS
- (E51) Thiamine deficiency
(E51.1) Beriberi
(E51.2) Wernicke's encephalopathy
- (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
- (E53.0) Riboflavin deficiency
Ariboflavinosis
- Ariboflavinosis
- (E53.1) Pyridoxine deficiency
- (E53.8) Deficiency of other specified B group vitamins
- (E54) Ascorbic acid deficiency
Scurvy
- Scurvy
- (E55) Vitamin D deficiency
(E55.0) Rickets, active
- (E55.0) Rickets, active
- (E56) Other vitamin deficiencies
(E56.0) Deficiency of vitamin E
(E56.1) Deficiency of vitamin K
- (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
- (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
- (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
- 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
- (E66.0) Obesity due to excess calories
- (E66.1) Drug-induced obesity
- (E66.2) Extreme obesity with alveolar hypoventilation
Pickwickian syndrome
- Pickwickian syndrome
- (E66.8) Other obesity
Morbid 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
- (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
- (E70.0) Classical phenylketonuria
- (E70.1) Other hyperphenylalaninaemias
- (E70.2) Disorders of tyrosine metabolism
Alkaptonuria (ILDS E70.210)
Ochronosis (ILDS E70.230)
Tyrosinaemia
- Alkaptonuria (ILDS E70.210)
- Ochronosis (ILDS E70.230)
- Tyrosinaemia
- (E70.3) Albinism
Chediak-Higashi syndrome
Cross syndrome
Hermansky-Pudlak syndrome
- 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
- 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
- (E71.0) Maple-syrup-urine disease
- (E71.1) Other disorders of branched-chain amino-acid metabolism
Propionic acidemia
Methylmalonic acidemia
Isovaleric acidemia
- 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
- 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
(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
- (E72.0) Disorders of amino-acid transport
Cystinuria
Cystinosis
Hartnup disease
Lowe's syndrome
- Cystinuria
- Cystinosis
- Hartnup disease
- Lowe's syndrome
- (E72.1) Disorders of sulfur-bearing amino-acid metabolism
Cystathioninuria
Homocystinuria
Methioninaemia
Sulfite oxidase deficiency
- Cystathioninuria
- Homocystinuria
- Methioninaemia
- Sulfite oxidase deficiency
- (E72.2) Disorders of urea cycle metabolism
Argininaemia
Argininosuccinic aciduria
Citrullinaemia
Hyperammonaemia
- Argininaemia
- Argininosuccinic aciduria
- Citrullinaemia
- Hyperammonaemia
- (E72.3) Disorders of lysine and hydroxylysine metabolism
Glutaric aciduria
Hydroxylysinaemia
Hyperlysinaemia
- Glutaric aciduria
- Hydroxylysinaemia
- Hyperlysinaemia
- (E72.4) Disorders of ornithine metabolism
Ornithinaemia (types I, II)
- Ornithinaemia (types I, II)
- (E72.5) Disorders of glycine metabolism
Hyperhydroxyprolinaemia
Hyperprolinaemia (types I, II)
Non-ketotic hyperglycinaemia
Sarcosinaemia
- 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
- (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)
- 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
- Essential fructosuria
- Fructose-1,6-diphosphatase deficiency
- Hereditary fructose intolerance
- (E74.2) Disorders of galactose metabolism
Galactosaemia
Galactokinase deficiency
- Galactosaemia
- Galactokinase deficiency
- (E74.3) Other disorders of intestinal carbohydrate absorption
Glucose-galactose malabsorption
Sucrase deficiency
- 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
- 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
- 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
- (E75.0) GM 2 gangliosidosis
Sandhoff disease
Tay-Sachs disease
- Sandhoff disease
- Tay-Sachs disease
- (E75.1) Other gangliosidosis
GM 1
GM 3
Mucolipidosis IV
- 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)
- 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)
- 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
- 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
- (E76.0) Mucopolysaccharidosis, type I
Hurler syndrome
- Hurler syndrome
- (E76.1) Mucopolysaccharidosis, type II
Hunter syndrome
- Hunter syndrome
- (E76.2) Other mucopolysaccharidoses
Sanfilippo syndrome
Morquio syndrome
- 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)
- (E77.0) Defects in post-translational modification of lysosomal enzymes
Mucolipidosis II (I-cell disease)
Mucolipidosis III (pseudo-Hurler polydystrophy)
- Mucolipidosis II (I-cell disease)
- Mucolipidosis III (pseudo-Hurler polydystrophy)
- (E77.1) Defects in glycoprotein degradation
Aspartylglucosaminuria
Fucosidosis
Mannosidosis
Sialidosis (mucolipidosis I)
- 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
- (E78.0) Pure hypercholesterolaemia
Familial hypercholesterolaemia
Fredrickson's hyperlipoproteinaemia, type IIa
Hyperbetalipoproteinaemia
Hyperlipidaemia, group A
Low-density-lipoprotein-type (LDL) hyperlipoproteinaemia
- 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
- 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
- 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
- Fredrickson's hyperlipoproteinaemia, type I or V
- Hyperlipidaemia, group D
- Mixed hyperglyceridaemia
- (E78.4) Other hyperlipidaemia
Familial combined 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
- 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
- (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
- 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
- (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)
- 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)
- 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)
- 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)
- 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
- 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
- (E83.0) Disorders of copper metabolism
Wilson's disease
Menkes disease
- Wilson's disease
- Menkes disease
- (E83.1) Disorders of iron metabolism
Hemochromatosis
- Hemochromatosis
- (E83.2) Disorders of zinc metabolism
Acrodermatitis enteropathica
- Acrodermatitis enteropathica
- (E83.3) Disorders of phosphorus metabolism
Acid phosphatase deficiency
Familial hypophosphataemia
Hypophosphatasia
Vitamin-D-resistant osteomalacia
Vitamin-D-resistant rickets
- Acid phosphatase deficiency
- Familial hypophosphataemia
- Hypophosphatasia
- Vitamin-D-resistant osteomalacia
- Vitamin-D-resistant rickets
- (E83.4) Disorders of magnesium metabolism
Hypermagnesemia
Hypomagnesemia
- Hypermagnesemia
- Hypomagnesemia
- (E83.5) Disorders of calcium metabolism
Familial hypocalciuric hypercalcaemia
Idiopathic hypercalciuria
- Familial hypocalciuric hypercalcaemia
- Idiopathic hypercalciuria
- (E84) Cystic fibrosis
- (E85) Amyloidosis
(E85.0) Non-neuropathic heredofamilial amyloidosis
Familial Mediterranean fever
- (E85.0) Non-neuropathic heredofamilial amyloidosis
Familial Mediterranean fever
- Familial Mediterranean fever
- (E86) Volume depletion
Dehydration
Hypovolaemia
- 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
- (E87.0) Hyperosmolality and hypernatraemia
- (E87.1) Hypo-osmolality and hyponatraemia
- (E87.2) Acidosis
Respiratory acidosis
Metabolic acidosis
Lactic 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
- Electrolyte imbalance NOS
- Hyperchloraemia
- Hypochloraemia
- (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
- (E88.0) Disorders of plasma-protein metabolism, not elsewhere classified
Alpha-1-antitrypsin deficiency
- 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
- (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