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(Top) 1 Disorders of thyroid gland (240–246) 2 Diseases of other endocrine glands (249–259) 3 Nutritional deficiencies (260–269) 4 Other metabolic and immunity disorders (270–279) 4.1 Disorders of amino-acid transport and metabolism 4.2 Disorders of carbohydrate transport and metabolism 4.3 Disorders of lipoid metabolism 4.4 Disorders of plasma protein metabolism 4.5 Gout 4.6 Disorders of mineral metabolism 4.7 Disorders of fluid, electrolyte, and acid-base balance 4.8 Other and unspecified disorders of metabolism 4.9 Obesity and other hyperalimentation 4.10 Disorders involving the immune mechanism

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

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ICD-9 chapters
Chapter	Block	Title
I	001–139	Infectious and Parasitic Diseases
II	140–239	Neoplasms
III	240–279	Endocrine, Nutritional and Metabolic Diseases, and Immunity Disorders
IV	280–289	Diseases of the Blood and Blood-forming Organs
V	290–319	Mental Disorders
VI	320–389	Diseases of the Nervous System and Sense Organs
VII	390–459	Diseases of the Circulatory System
VIII	460–519	Diseases of the Respiratory System
IX	520–579	Diseases of the Digestive System
X	580–629	Diseases of the Genitourinary System
XI	630–679	Complications of Pregnancy, Childbirth, and the Puerperium
XII	680–709	Diseases of the Skin and Subcutaneous Tissue
XIII	710–739	Diseases of the Musculoskeletal System and Connective Tissue
XIV	740–759	Congenital Anomalies
XV	760–779	Certain Conditions originating in the Perinatal Period
XVI	780–799	Symptoms, Signs and Ill-defined Conditions
XVII	800–999	Injury and Poisoning
	E800–E999	Supplementary Classification of External Causes of Injury and Poisoning
	V01–V82	Supplementary Classification of Factors influencing Health Status and Contact with Health Services
	M8000–M9970	Morphology of Neoplasms

This is a shortened version of the third chapter of the ICD-9: Endocrine, Nutritional and Metabolic Diseases, and Immunity Disorders. It covers ICD codes 240 to 279. The full chapter can be found on pages 145 to 165 of Volume 1, which contains all (sub)categories of the ICD-9. Volume 2 is an alphabetical index of Volume 1. Both volumes can be downloaded for free from the website of the World Health Organization.

Disorders of thyroid gland (240–246)[edit]

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)[edit]

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
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)[edit]

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)[edit]

Other

Mineral deficiency

Electrolyte imbalance
- Calcium
- Chloride
- Magnesium
- Phosphate
- Potassium
- Sodium
Iron
Zinc
Manganese
Copper
Iodine
Chromium
Molybdenum
Selenium
- Keshan disease
Fluorine

Growth

General

Inborn errorofamino acid metabolism

K→acetyl-CoA

Lysine/straight chain

Leucine

Tryptophan

Hypertryptophanemia

G→pyruvate→citrate

Glycine

G→glutamate→
α-ketoglutarate

Histidine

Proline

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 BC/OA	Methylmalonic acidemia Methylmalonyl-CoA mutase deficiency Propionic acidemia

G→fumarate

Phenylalanine/tyrosine

Phenylketonuria	6-Pyruvoyltetrahydropterin synthase deficiency Tetrahydrobiopterin deficiency
Tyrosinemia	Alkaptonuria/Ochronosis Tyrosinemia type I Tyrosinemia type II Tyrosinemia type III/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

Inborn errorofcarbohydrate metabolism: monosaccharide metabolism disorders
Including glycogen storage diseases (GSD)

Sucrose, transport
(extracellular)

Disaccharide catabolism

Monosaccharide transport

Glucose-galactose malabsorption
Inborn errors of renal tubular transport (Renal glycosuria)
Fructose malabsorption
De Vivo Disease (GLUT1 deficiency)
Fanconi-Bickel syndrome (GLUT2 deficiency)

Hexose → glucose

Monosaccharide catabolism

Fructose:

Galactose / galactosemia:

Glucose ⇄ glycogen

Glycogenesis

GSD type 0 (glycogen synthase deficiency)
GSD type IV (Andersen's disease, branching enzyme deficiency)
Adult polyglucosan body disease (APBD)
Lafora disease
GSD type XV (glycogenin deficiency)

Glycogenolysis

Extralysosomal:

GSD type III (Cori's disease, debranching enzyme deficiency)
GSD type VI (Hers' disease, liver glycogen phosphorylase deficiency)
GSD type V (McArdle's disease, myophosphorylase deficiency)
GSD type IX (phosphorylase kinase deficiency)
Phosphoglucomutase deficiency (PGM1-CDG, CDG1T, formerly GSD-XIV)

Lysosomal (LSD):

Glycogen storage disease type II (Pompe's disease, glucosidase deficiency, formerly GSD-IIa)
Danon disease (LAMP2 deficiency, formerly GSD-IIb)

Glucose ⇄ CAC

Glycolysis

MODY 2/HHF3
GSD type VII (Tarui's disease, phosphofructokinase deficiency)
Triosephosphate isomerase deficiency
Pyruvate kinase deficiency
Aldolase A deficiency
Phosphoglucose isomerase deficiency
Phosphoglycerate kinase deficiency
Mitochondrial pyruvate carrier deficiency (MPC1 deficiency)

Gluconeogenesis

Pyruvate carboxylase deficiency
Fructose bisphosphatase deficiency
GSD type I (von Gierke's disease, glucose 6-phosphatase deficiency)

Pentose phosphate pathway

Glucose-6-phosphate dehydrogenase deficiency
Transaldolase deficiency
SDDHD (Transketolase deficiency)
6-phosphogluconate dehydrogenase deficiency

Other

Hyperoxaluria
- Primary hyperoxaluria
Pentosuria
Fatal congenital nonlysosomal cardiac glycogenosis (AMP-activated protein kinase deficiency, PRKAG2)

Inborn erroroflipid metabolism: dyslipidemia

Hyperlipidemia

Hypolipoproteinemia

Hypoalphalipoproteinemia/HDL

Hypobetalipoproteinemia/LDL

Lipodystrophy

Barraquer–Simons syndrome

Other

v t e Lysosomal storage diseases: Inborn errorsofcarbohydrate metabolism (Glycoproteinoses)
Anabolism	Dolichol kinase deficiency Congenital disorder of glycosylation
Post-translational modification oflysosomal enzymes	Mucolipidosis: I-cell disease (ML II) Pseudo-Hurler polydystrophy (ML III)
Catabolism	Aspartylglucosaminuria Fucosidosis mannosidosis Alpha-mannosidosis Beta-mannosidosis Sialidosis Schindler disease
Other	solute carrier family (Salla disease) Galactosialidosis

Lysosomal storage diseases: Inborn errorsoflipid metabolism (Lipid storage disorders)

Sphingolipidoses
(toceramide)

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
Tosphingosine	Ceramide: Farber disease

NCL

Other

Immunoproliferative immunoglobulin disorders

PCDs/PP

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

Other hypergammaglobulinemia

Cryoglobulinemia

Metal deficiency and toxicity disorders

Iron

excess:

deficiency:

Iron deficiency

Copper

excess:

deficiency:

Zinc

excess:

Zinc toxicity

deficiency:

Acrodermatitis enteropathica

Other

Inborn errors of metabolism

Respiratory

Alkalosis

Other

Inborn errorofpurine–pyrimidine metabolism

Purine metabolism

Anabolism

Nucleotide salvage

Catabolism

Pyrimidine metabolism

Anabolism

Catabolism

Dihydropyrimidine dehydrogenase deficiency

Heme metabolism disorders

Porphyria,
hepatic and erythropoietic
(porphyrin)

early mitochondrial:

cytoplasmic:

late mitochondrial:

Hereditary hyperbilirubinemia
(bilirubin)

unconjugated:

conjugated:

Amyloidosis

Common amyloid forming proteins

Systemic amyloidosis

Organ-limited amyloidosis

Heart	AANF/Isolated atrial
Brain	Familial amyloid neuropathy ACys+ABri/Cerebral amyloid angiopathy Aβ/Alzheimer's disease
Kidney	AApoA1+AFib+ALys/Familial renal
Skin	Primary cutaneous amyloidosis Amyloid purpura
Endocrine	Thyroid ACal/Medullary thyroid cancer Pituitary APro/Prolactinoma Pancreas AIAPP/Insulinoma Type 2 diabetes

Lysosomal storage diseases: Inborn errorsofcarbohydrate metabolism (Mucopolysaccharidoses)

Catabolism

Inborn erroroflipid metabolism: fatty-acid metabolism disorders

Synthesis

Biotinidase deficiency (BTD)

Degradation

Acyl
transport

Carnitine
- CPT1
- CPT2
- CDSP
- CACTD
Adrenoleukodystrophy (ALD)

Beta
oxidation

General	Acyl CoA dehydrogenase Short-chain SCADD Medium-chain MCADD Long-chain 3-hydroxy LCHAD Very long-chain VLCADD Mitochondrial trifunctional protein deficiency (MTPD): Acute fatty liver of pregnancy
Unsaturated	2,4 Dienoyl-CoA reductase deficiency (DECRD)
Odd chain	Propionic acidemia (PCC deficiency)
Other	3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADHD) Glutaric acidemia type 2 (MADD)

To
acetyl-CoA

Malonic aciduria (MCD)

Aldehyde

Sjögren–Larsson syndrome (SLS)

v t e Genetic disorder, organelle: Peroxisomal disorders and lysosomal structural disorders
Peroxisome biogenesis disorder	Zellweger syndrome Neonatal adrenoleukodystrophy Infantile Refsum disease Adult Refsum disease-2 RCP 1
Enzyme-related	Acatalasia RCP 2&3 Mevalonate kinase deficiency D-bifunctional protein deficiency Adult Refsum disease-1
Transporter-related	X-linked adrenoleukodystrophy
Lysosomal	Danon disease
See also: proteins, intermediates

v t e Mitochondrial diseases
Carbohydrate metabolism	PCD PDHA
Primarily nervous system	Leigh disease LHON NARP
Myopathies	KSS Mitochondrial encephalomyopathy MELAS MERRF PEO
No primary system	DAD MNGIE Pearson syndrome
Chromosomal	OPA1 Kjer's optic neuropathy SARS2 HUPRA syndrome TIMM8A Mohr–Tranebjærg syndrome
see also mitochondrial proteins

v t e Histiocytosis
WHO-I/Langerhans cell histiocytosis/ X-type histiocytosis	Letterer–Siwe disease Hand–Schüller–Christian disease Eosinophilic granuloma Congenital self-healing reticulohistiocytosis
WHO-II/non-Langerhans cell histiocytosis/ Non-X histiocytosis	Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann–Pick disease Sea-blue histiocytosis Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis (Multicentric reticulohistiocytosis, Reticulohistiocytoma) Indeterminate cell histiocytosis
WHO-III/malignant histiocytosis	Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma
Ungrouped	Rosai–Dorfman disease