List of ICD-9 codes 240–279: endocrine, nutritional and metabolic diseases, and immunity disorders

3. Endocrine, nutritional and metabolic diseases, and immunity disorders (240–279) 1
- Disorders of thyroid gland (240–246) 1.1
- Diseases of other endocrine glands (249–259) 1.2
- Nutritional deficiencies (260–269) 1.3
- Other metabolic and immunity disorders (270–279) 1.4
  - Disorders of amino-acid transport and metabolism 1.4.1
  - Disorders of carbohydrate transport and metabolism 1.4.2
  - Disorders of lipoid metabolism 1.4.3
  - Disorders of plasma protein metabolism 1.4.4
  - Gout 1.4.5
  - Disorders of mineral metabolism 1.4.6
  - Disorders of fluid, electrolyte, and acid-base balance 1.4.7
  - Other and unspecified disorders of metabolism 1.4.8
  - Obesity and other hyperalimentation 1.4.9
  - Disorders involving the immune mechanism 1.4.10
See also 2

3. Endocrine, nutritional and metabolic diseases, and immunity disorders (240–279)

Disorders of thyroid gland (240–246)

(240) Simple and unspecified goiter
- (240.9) Goiter, unspec.
(241) Nontoxic nodular goiter
- (241.0) Thyroid nodule
- (241.9) Goiter, unspec. nontoxic nodular
(242) Thyrotoxicosis with or without goiter
- (242.0) Goiter toxic, diffuse
- (242.9) Hyperthyroidism, NOS
(243) Congenital hypothyroidism
(244) Acquired hypothyroidism
- (244.0) Hypothyroidism, post-surgical
- (244.1) Hypothyroidism, post-ablative
- (244.9) Hypothyroidism, unspec.
(245) Thyroiditis
- (245.0) Thyroiditis, acute
- (245.1) Thyroiditis, subacute
- (245.2) Thyroiditis, chronic, Hashimoto's
(246) Other disorders of thyroid
- (246.2) Thyroid cyst

Diseases of other endocrine glands (249–259)

Note: for 249–259, the following fifth digit can be added:
- (250.x0) Diabetes mellitus type 2
- (250.x1) Diabetes mellitus type 1
- (250.x2) Diabetes mellitus type 2, uncontrolled
- (250.x3) Diabetes mellitus type 1, uncontrolled
(249) Secondary diabetes mellitus
- (249.0) Secondary diabetes mellitus without mention of complication
- (249.1) Secondary diabetes mellitus with ketoacidosis
- (249.2) Secondary diabetes mellitus with hyperosmolarity
- (249.3) Secondary diabetes mellitus with other coma
- (249.4) Secondary diabetes mellitus with renal manifestations
- (249.5) Secondary diabetes mellitus with ophthalmic manifestations
- (249.6) Secondary diabetes mellitus with neurological manifestations
- (249.7) Secondary diabetes mellitus with peripheral circulatory disorders
- (249.8) Secondary diabetes mellitus with other specified manifestations
- (249.9) Secondary diabetes mellitus with unspecified complications
(250) Diabetes mellitus
- (250.0) Diabetes mellitus without mention of complication
- (250.1) Diabetes with ketoacidosis
- (250.2) Diabetes with hyperosmolarity
- (250.3) Diabetes with other coma
- (250.4) Diabetes with renal manifestations
- (250.5) Diabetes with ophthalmic manifestations
- (250.6) Diabetes with neurological manifestations
- (250.7) Diabetes with peripheral circulatory disorders
- (250.8) Diabetes with other specified manifestations
- (250.9) Diabetes with unspecified complication
(251) Other disorders of pancreatic internal secretion
- (251.2) Hypoglycemia, nondiabetic, unspec.
(252) Disorders of parathyroid gland
- (252.0) Hyperparathyroidism, unspec.
  - (252.01) Hyperparathyroidism, primary
- (252.1) Hypoparathyroidism
(253) Disorders of the pituitary gland and its hypothalamic control
- (253.3) Growth hormone deficiency
- (253.6) SIADH
(254) Diseases of thymus gland
(255) Disorders of adrenal glands
- (255.0) Cushing's syndrome
(256) Ovarian dysfunction
- (256.2) Ovarian failure, postablative
  - (256.39) Ovarian failure, other
- (256.4) Ovaries, polycystic
(257) Testicular dysfunction
- (257.2) Testicular hypofunction
(258) Polyglandular dysfunction and related disorders
(259) Other endocrine disorders
- (259.0) Puberty, delayed
- (259.1) Sexual precocity

Nutritional deficiencies (260–269)

(260) Kwashiorkor
(261) Nutritional marasmus
(262) Other severe protein-calorie malnutrition
(263) Other and unspecified protein-calorie malnutrition
(264) Vitamin A deficiency
- (264.0) With conjunctival xerosis
- (264.1) With conjunctival xerosis and Bitot's spot
- (264.2) With corneal xerosis
- (264.3) With corneal ulceration and xerosis
- (264.4) With keratomalacia
- (264.5) With night blindness
- (264.6) With xerophthalmic scars of cornea
- (264.7) Other ocular manifestations of vitamin A deficiency
- (264.8) Other manifestations of vitamin A deficiency
- (264.9) Unspecified vitamin A deficiency
(265) Thiamine and niacin deficiency states
- (265.0) Beriberi
- (265.1) Other and unspecified manifestations of thiamine deficiency
  - Wernicke's encephalopathy
- (265.2) Pellagra
(266) Deficiency of B-complex components
- (266.0) Ariboflavinosis
- (266.2) B12 deficiency w/o anemia
(267) Ascorbic acid deficiency
(268) Vitamin D deficiency
(269) Other nutritional deficiencies
- (269.0) Deficiency of vitamin K
- (269.1) Deficiency of other vitamins
- (269.2) Unspecified vitamin deficiency
- (269.3) Mineral deficiency, not elsewhere classified

Other metabolic and immunity disorders (270–279)

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 Hypoprolactinemia ACTH deficiency/Secondary adrenal insufficiency GnRH insensitivity FSH insensitivity LH/hCG insensitivity 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 goiter Thyroid nodule

Parathyroid

Hypoparathyroidism	Hypoparathyroidism Pseudohypoparathyroidism Pseudopseudohypoparathyroidism

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

testicular: enzymatic
Androgen receptor (Androgen insensitivity syndrome)

general: Hypogonadism (Delayed puberty)
Hypergonadism
- Precocious puberty
Hypoandrogenism
Hypoestrogenism
Hyperandrogenism
Hyperestrogenism
Postorgasmic illness syndrome

Height

Multiple

Index of hormones

Description	thyroid

Disease

Treatment	Drugs

Malnutrition or nutrition disorders (E40–E68, 260–269)

Malnutrition

Protein-energy
malnutrition

Avitaminosis

B vitamins	B₁: Beriberi / Wernicke–Korsakoff syndrome (Wernicke's encephalopathy Korsakoff's syndrome) 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

Overnutrition

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

Index of nutrition

Description	Fats Sugars

Disease

Treatment	Drugs

Inborn error of amino acid metabolism (E70–E72, 270)

K→acetyl-CoA

Lysine/straight chain	Glutaric acidemia type 1 type 2 Hyperlysinemia Pipecolic acidemia Saccharopinuria

Leucine	3-hydroxy-3-methylglutaryl-CoA lyase deficiency 3-Methylcrotonyl-CoA carboxylase deficiency 3-Methylglutaconic aciduria 1 Isovaleric acidemia Maple syrup urine disease

Tryptophan	Hypertryptophanemia

G→pyruvate→citrate

Glycine	D-Glyceric acidemia Glutathione synthetase deficiency Sarcosinemia Glycine→Creatine: GAMT deficiency Glycine encephalopathy

G→glutamate→
α-ketoglutarate

Histidine	Carnosinemia Histidinemia Urocanic aciduria

Proline	Hyperprolinemia Prolidase deficiency

Glutamate/glutamine	SSADHD

G→propionyl-CoA→
succinyl-CoA

Valine	Hypervalinemia Isobutyryl-CoA dehydrogenase deficiency Maple syrup urine disease

Isoleucine	2-Methylbutyryl-CoA dehydrogenase deficiency Beta-ketothiolase deficiency Maple syrup urine disease

Methionine	Cystathioninuria Homocystinuria Hypermethioninemia

General OA	Methylmalonic acidemia Methylmalonyl-CoA mutase deficiency Propionic acidemia

G→fumarate

Phenylalanine/tyrosine

Phenylketonuria	6-Pyruvoyltetrahydropterin synthase deficiency Tetrahydrobiopterin deficiency

Tyrosinemia	Alkaptonuria/Ochronosis Type I tyrosinemia Type II tyrosinemia Type III tyrosinemia/Hawkinsinuria

Tyrosine→Melanin	Albinism: Ocular albinism (1) Oculocutaneous albinism (Hermansky–Pudlak syndrome) Waardenburg syndrome

Tyrosine→Norepinephrine	Dopamine beta hydroxylase deficiency reverse: Brunner syndrome

G→oxaloacetate

Urea cycle/Hyperammonemia (arginine aspartate)	Argininemia Argininosuccinic aciduria Carbamoyl phosphate synthetase I deficiency Citrullinemia N-Acetylglutamate synthase deficiency Ornithine transcarbamylase deficiency/translocase deficiency

Transport/
IE of RTT

Other

Index of inborn errors of metabolism

Description	Enzymes and pathways:

Disorders

Treatment	Drugs

Inborn error of carbohydrate metabolism: monosaccharide metabolism disorders (including glycogen storage diseases) (E73–E74, 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

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/HHF3 GSD type VII, Tarui's, phosphofructokinase Triosephosphate isomerase deficiency Pyruvate kinase deficiency

Gluconeogenesis	PCD Fructose bisphosphatase deficiency GSD type I, von Gierke, glucose 6-phosphatase

Pentose phosphate pathway

Other

Index of inborn errors of metabolism

Description	Enzymes and pathways:

Disorders

Treatment	Drugs

(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

Other

Index of inborn errors of metabolism

Description	Enzymes and pathways:

Disorders

Treatment	Drugs

D89, 273)

PCDs/PP

Plasmacytoma
Multiple myeloma (Plasma cell leukemia)
MGUS
IgM (Macroglobulinemia/Waldenström's macroglobulinemia)
heavy chain (Heavy chain disease)
light chain (Primary amyloidosis)

Other hypergammaglobulinemia

Cryoglobulinemia

Index of the immune system

Description

Disease

Treatment	Drugs

Water-electrolyte imbalance and acid-base imbalance (E86–E87, 276)

Volume status

Electrolyte

Sodium	Hypernatremia Hyponatremia (Hypotonic, Isotonic)

Potassium	Hyperkalemia Hypokalemia

Chloride	Hyperchloremia Hypochloremia

Calcium	Hypercalcaemia Hypocalcaemia

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

Index of the urinary system

Description

Disease

Treatment

(LSD) Inborn error of carbohydrate metabolism: mucopolysaccharidosis (E76, 277.5)

Anabolism

Pentosuria

Heparan sulfate: EXT1 Hereditary multiple exostoses 1

Chondroitin sulfate: PAPSS2 Spondyloepimetaphyseal dysplasia, Pakistani type

Catabolism

IDUA
- 1:Hurler/Scheie
IDS
- 2:Hunter
SGSH/NAGLU/HGSNAT/GNS
- 3:Sanfilippo ABCD
GALNS/GLB1
- 4:Morquio
ARSB
- 6:Maroteaux-Lamy
GUSB
- 7:Sly

Index of inborn errors of metabolism

Description	Enzymes and pathways:

Disorders

Treatment	Drugs

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

Index of inborn errors of metabolism

Description	Enzymes and pathways:

Disorders

Treatment	Drugs

Peroxisomal disorders and lysosomal structural disorders (E80.3, 277.86)

Peroxisome biogenesis disorder

Enzyme-related

Transporter-related

X-linked adrenoleukodystrophy

Lysosomal

Danon disease

See also: ,

Index of cells

Description	Organelles

Disease	Structural Membrane

Non-Mendelian inheritance: Mitochondrial diseases (277.87)

Carbohydrate metabolism

PCD
PDHA

Primarily nervous system

Myopathies

Mitochondrial encephalomyopathy
- MELAS
- MERRF
KSS
PEO

No primary system

Chromosomal

see also

Index of cells

Description	Organelles

Disease	Structural Membrane

D76.0, 277.89)

WHO-I/Langerhans cell histiocytosis/
X-type histiocytosis

WHO-II/non-Langerhans cell histiocytosis/
Non-X histiocytosis

WHO-III/malignant histiocytosis

Ungrouped

Rosai–Dorfman disease

Index of cells from bone marrow

Description	heme and porphyrin

Disease

Treatment	Drugs

D80–D85, 279.0–4)

Primary

Antibody/humoral (B)

Hypogammaglobulinemia	X-linked agammaglobulinemia Transient hypogammaglobulinemia of infancy

Dysgammaglobulinemia	IgA deficiency IgG deficiency IgM deficiency Hyper IgM syndrome (1 2 3 4 5) Wiskott-Aldrich syndrome Hyper-IgE syndrome

Other	Common variable immunodeficiency ICF syndrome

T cell deficiency (T)

Nezelof syndrome
Ataxia telangiectasia)

peripheral: Purine nucleoside phosphorylase deficiency

Hyper IgM syndrome (1)

Severe combined (B+T)

x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
Omenn syndrome
ZAP70 deficiency
Bare lymphocyte syndrome

Acquired

AIDS

Leukopenia:
Lymphocytopenia

Idiopathic CD4+ lymphocytopenia

Complement deficiency

Index of the immune system

Description

Disease

Treatment	Drugs

List of ICD-9 codes 240–279: endocrine, nutritional and metabolic diseases, and immunity disorders