Excludes:	endocrine and metabolic disturbances specific
to the fetus and newborn (775.0-775.9)
Note:	All neoplasms, whether functionally active or not, are classified in Chapter 2.
 Codes in Chapter 3 (i.e., 242.8, 246.0, 251-253, 255-259) may be used to identify such
 functional activity associated with any neoplasm, or by ectopic endocrine tissue.

DISORDERS OF THYROID GLAND (240-246)

240	Simple and unspecified goiter
240.0	Goiter, specified as simple
Any condition classifiable to 240.9, specified as simple
240.9	Goiter, unspecified
Enlargement of thyroid
Goiter or struma:
NOS
diffuse colloid
endemic
hyperplastic
nontoxic (diffuse)
parenchymatous
sporadic
Excludes:	congenital (dyshormonogenic) goiter (246.1)
241	Nontoxic nodular goiter
Excludes:	adenoma of thyroid (226)
cystadenoma of thyroid (226)
241.0	Nontoxic uninodular goiter
Thyroid nodule
Uninodular goiter (nontoxic)
241.1	Nontoxic multinodular goiter
Multinodular goiter (nontoxic)
241.9	Unspecified nontoxic nodular goiter
Adenomatous goiter
Nodular goiter (nontoxic) NOS
Struma nodosa (simplex)
242	Thyrotoxicosis with or without goiter
Excludes:	neonatal thyrotoxicosis (775.3)
The following fifth-digit subclassification is for use with category 242:
0	without mention of thyrotoxic crisis or storm
1	with mention of thyrotoxic crisis or storm
242.0	Toxic diffuse goiter
Basedow's disease
Exophthalmic or toxic goiter NOS
Graves' disease
Primary thyroid hyperplasia
242.1	Toxic uninodular goiter
Thyroid nodule, toxic or with hyperthyroidism
Uninodular goiter, toxic or with hyperthyroidism
242.2	Toxic multinodular goiter
Secondary thyroid hyperplasia
242.3	Toxic nodular goiter, unspecified
Adenomatous goiter, toxic or with hyperthyroidism
Nodular goiter, toxic or with hyperthyroidism
Struma nodosa, toxic or with hyperthyroidism
Any condition classifiable to 241.9 specified as toxic or with hyperthyroidism
242.4	Thyrotoxicosis from ectopic thyroid nodule
242.8	Thyrotoxicosis of other specified origin
Overproduction of thyroid-stimulating hormone [TSH]
Thyrotoxicosis:
factitia from ingestion of excessive thyroid material
Use additional E code to identify cause, if drug-induced
242.9	Thyrotoxicosis without mention of goiter or other cause
Hyperthyroidism NOS
Thyrotoxicosis NOS
243	Congenital hypothyroidism
Congenital thyroid insufficiency
Cretinism (athyrotic) (endemic)
Use additional code to identify associated mental retardation
Excludes:	congenital (dyshormonogenic) goiter (246.1)
244	Acquired hypothyroidism
Includes:	athyroidism (acquired)
hypothyroidism (acquired)
myxedema (adult) (juvenile)
thyroid (gland) insufficiency (acquired)
244.0	Postsurgical hypothyroidism
244.1	Other postablative hypothyroidism
Hypothyroidism following therapy, such as irradiation
244.2	Iodine hypothyroidism
Hypothyroidism resulting from administration or ingestion of iodide
Use additional E to identify drug
244.3	Other iatrogenic hypothyroidism
Hypothyroidism resulting from:
P-aminosalicylic acid [PAS]
Phenylbutazone
Resorcinol
Iatrogenic hypothyroidism NOS
Use additional E to identify drug
244.8	Other specified acquired hypothyroidism
Secondary hypothyroidism NEC
244.9	Unspecified hypothyroidism
Hypothyroidism, primary or NOS
Myxedema, primary or NOS
245	Thyroiditis
245.0	Acute thyroiditis
Abscess of thyroid
Thyroiditis:
nonsuppurative, acute
pyogenic
suppurative
Use additional code to identify organism
245.1	Subacute thyroiditis
Thyroiditis:
de Quervain's
giant cell
granulomatous
viral
245.2	Chronic lymphocytic thyroiditis
Hashimoto's disease
Struma lymphomatosa
Thyroiditis:
autoimmune
lymphocytic (chronic)
245.3	Chronic fibrous thyroiditis
Struma fibrosa
Thyroiditis:
invasive (fibrous)
ligneous
Riedel's
245.4	Iatrogenic thyroiditis
Use additional E to identify cause
245.8	Other and unspecified chronic thyroiditis
Chronic thyroiditis:
NOS
nonspecific
245.9	Thyroiditis, unspecified
Thyroiditis NOS
246	Other disorders of thyroid
246.0	Disorders of thyrocalcitonin secretion
Hypersecretion of calcitonin or thyrocalcitonin
246.1	Dyshormonogenic goiter
Congenital (dyshormonogenic) goiter
Goiter due to enzyme defect in synthesis of thyroid hormone
Goitrous cretinism (sporadic)
246.2	Cyst of thyroid
Excludes:	cystadenoma of thyroid (226)
246.3	Hemorrhage and infarction of thyroid
246.8	Other specified disorders of thyroid
Abnormality of thyroid-binding globulin
Atrophy of thyroid
Hyper-TBG-nemia
Hypo-TBG-nemia
246.9	Unspecified disorder of thyroid
DISEASES OF OTHER ENDOCRINE GLANDS (250-259)
250	Diabetes mellitus
Excludes:	gestational diabetes (648.8)
hyperglycemia NOS (790.29)
neonatal diabetes mellitus (775.1)
nonclinical diabetes (790.29)
The following fifth-digit subclassification is for use with category 250:
0	type II or unspecified type, not stated as uncontrolled
Fifth-digit 0 is for use for type II patients, even if the patient requires insulin
Use additional code, if applicable, for associated long-term (current) insulin use V58.67
1	type I [juvenile type], not stated as uncontrolled
2	type II or unspecified type, uncontrolled
Use additional code, if applicable, for associated long-term (current) insulin use V58.67
Fifth-digit 2 is for use for type II, adult-onset, diabetic patients, even if the patient
 requires insulin
3	type I [juvenile type], uncontrolled
250.0	Diabetes mellitus without mention of complication
Diabetes mellitus without mention of complication or manifestation classifiable to 250.1-250.9
Diabetes (mellitus) NOS
250.1	Diabetes with ketoacidosis
Diabetic:
acidosis without mention of coma
ketosis without mention of coma
250.2	Diabetes with hyperosmolarity
Hyperosmolar (nonketotic) coma
250.3	Diabetes with other coma
Diabetic coma (with ketoacidosis)
Diabetic hypoglycemic coma
Insulin coma NOS
Excludes:	diabetes with hyperosmolar coma (250.2)
250.4	Diabetes with renal manifestations
Use additional code to identify manifestation, as:
chronic kidney disease (585.1-585.9)
diabetic:
nephropathy NOS (583.81)
nephrosis (581.81)
intercapillary glomerulosclerosis (581.81)
Kimmelstiel-Wilson syndrome (581.81)
250.5	Diabetes with ophthalmic manifestations
Use additional code to identify manifestation, as:
diabetic:
blindness (369.00-369.9)
cataract (366.41)
glaucoma (365.44)
macular edema (362.07)
retinal edema (362.07)
retinopathy (362.01-362.07)
250.6	Diabetes with neurological manifestations
Use additional code to identify manifestation, as:
diabetic:
amyotrophy (353.1)
gastroparalysis (536.3)
gastroparesis (536.3)
mononeuropathy (354.0-355.9)
neurogenic arthropathy (713.5)
peripheral autonomic neuropathy (337.1)
polyneuropathy (357.2)
250.7	Diabetes with peripheral circulatory disorders
Use additional code to identify manifestation, as:
diabetic:
gangrene (785.4)
peripheral angiopathy (443.81)
250.8	Diabetes with other specified manifestations
Diabetic hypoglycemia
Hypoglycemic shock
Use additional code to identify manifestation, as:
any associated ulceration (707.10-707.9)
diabetic bone changes (731.8)
Use additional E code to identify cause, if drug-induced
250.9	Diabetes with unspecified complication
251	Other disorders of pancreatic internal secretion
251.0	Hypoglycemic coma
Iatrogenic hyperinsulinism
Non-diabetic insulin coma
Use additional E code to identify cause, if drug-induced
Excludes:	hypoglycemic coma in diabetes mellitus (250.3)
251.1	Other specified hypoglycemia
Hyperinsulinism:
NOS
ectopic
functional
Hyperplasia of pancreatic islet beta cells NOS
Excludes:	hypoglycemia in diabetes mellitus (250.8)
hypoglycemia in infant of diabetic mother (775.0)
hypoglycemic coma (251.0)
neonatal hypoglycemia (775.6)
Use additional E code to identify cause, if drug-induced
251.2	Hypoglycemia, unspecified
Hypoglycemia:
NOS
reactive
spontaneous
Excludes:	hypoglycemia:
with coma (251.0)
in diabetes mellitus (250.8)
leucine-induced (270.3)
251.3	Postsurgical hypoinsulinemia
Hypoinsulinemia following complete or partial pancreatectomy
Postpancreatectomy hyperglycemia
251.4	Abnormality of secretion of glucagon
Hyperplasia of pancreatic islet alpha cells with glucagon excess
251.5	Abnormality of secretion of gastrin
Hyperplasia of pancreatic alpha cells with gastrin excess
Zollinger-Ellison syndrome
251.8	Other specified disorders of pancreatic internal secretion
251.9	Unspecified disorder of pancreatic internal secretion
Islet cell hyperplasia NOS
252	Disorders of parathyroid gland
252.0	Hyperparathyroidism
Excludes:	ectopic hyperparathyroidism (259.3)
252.00	Hyperparathyroidism, unspecified
252.01	Primary hyperparathyroidism
Hyperplasia of parathyroid
252.02	Secondary hyperparathyroidism, non-renal
Excludes:	secondary hyperparathyroidism (of renal origin) (588.81)
252.08	Other hyperparathyroidism
Tertiary hyperparathyroidism
252.1	Hypoparathyroidism
Parathyroiditis (autoimmune)
Tetany:
parathyroid
parathyroprival
Excludes:	pseudohypoparathyroidism (275.49)
pseudopseudohypoparathyroidism (275.49)
tetany NOS (781.7)
transitory neonatal hypoparathyroidism (775.4)
252.8	Other specified disorders of parathyroid gland
Cyst of parathyroid gland
Hemorrhage of parathyroid gland
252.9	Unspecified disorder of parathyroid gland
253	Disorders of the pituitary gland and its hypothalamic control
Includes:	the listed conditions whether the disorder is in the pituitary or the
 hypothalamus
Excludes:	Cushing's syndrome (255.0)
253.0	Acromegaly and gigantism
Overproduction of growth hormone
253.1	Other and unspecified anterior pituitary hyperfunction
Forbes-Albright syndrome
Excludes:	overproduction of:
ACTH (255.3)
thyroid-stimulating hormone [TSH] (242.8)
253.2	Panhypopituitarism
Cachexia, pituitary
Necrosis of pituitary (postpartum)
Pituitary insufficiency NOS
Sheehan's syndrome
Simmonds' disease
Excludes:	iatrogenic hypopituitarism (253.7)
253.3	Pituitary dwarfism
Isolated deficiency of (human) growth hormone [HGH]
Lorain-Levi dwarfism
253.4	Other anterior pituitary disorders
Isolated or partial deficiency of an anterior pituitary hormone, other than growth hormone
Prolactin deficiency
253.5	Diabetes insipidus
Vasopressin deficiency
Excludes:	nephrogenic diabetes insipidus (588.1)
253.6	Other disorders of neurohypophysis
Syndrome of inappropriate secretion of antidiuretic hormone [ADH]
Excludes:	ectopic antidiuretic hormone secretion (259.3)
253.7	Iatrogenic pituitary disorders
Hypopituitarism:
hormone-induced
hypophysectomy-induced
postablative
radiotherapy-induced
Use additional E code to identify cause
253.8	Other disorders of the pituitary and other syndromes of diencephalohypophyseal origin
Abscess of pituitary
Adiposogenital dystrophy
Cyst of Rathke's pouch
Fr�hlich's syndrome
Excludes:	craniopharyngioma (237.0)
253.9	Unspecified
Dyspituitarism
254	Diseases of thymus gland
Excludes:	aplasia or dysplasia with immunodeficiency (279.2)
hypoplasia with immunodeficiency (279.2)
myasthenia gravis (358.00-358.01)
254.0	Persistent hyperplasia of thymus
Hypertrophy of thymus
254.1	Abscess of thymus
254.8	Other specified diseases of thymus gland
Atrophy of thymus
Cyst of thymus
Excludes:	thymoma (212.6)
254.9	Unspecified disease of thymus gland
255	Disorders of adrenal glands
Includes:	the listed conditions whether the basic disorder is in the adrenals or is
 pituitary-induced
255.0	Cushing's syndrome
Adrenal hyperplasia due to excess ACTH
Cushing's syndrome:
NOS
iatrogenic
idiopathic
pituitary-dependent
Ectopic ACTH syndrome
Iatrogenic syndrome of excess cortisol
Overproduction of cortisol
Use additional E code to identify cause, if drug-induced
Excludes:	congenital adrenal hyperplasia (255.2)
255.1	Hyperaldosteronism
255.10	Hyperaldosteronism, unspecified
Aldosteronism NOS
Primary aldosteronism, unspecified
Excludes:	Conn's syndrome (255.12)
255.11	Glucocorticoid-remediable aldosteronism
Familial aldosteronism type I
Excludes:	Conn's syndrome (255.12)
255.12	Conn's syndrome
255.13	Bartter's syndrome
255.14	Other secondary aldosteronism
255.2	Adrenogenital disorders
Adrenogenital syndromes, virilizing or feminizing, whether acquired or associated with
 congenital adrenal hyperplasia consequent on inborn enzyme defects in hormone synthesis
Achard-Thiers syndrome
Congenital adrenal hyperplasia
Female adrenal pseudohermaphroditism
Male:
macrogenitosomia praecox
sexual precocity with adrenal hyperplasia
Virilization (female) (suprarenal)
Excludes:	adrenal hyperplasia due to excess ACTH (255.0)
isosexual virilization (256.4)
255.3	Other corticoadrenal overactivity
Acquired benign adrenal androgenic overactivity
Overproduction of ACTH
255.4	Corticoadrenal insufficiency
Excludes:	tuberculous Addison's disease (017.6)
255.41	Glucocorticoid deficiency
Addisonian crisis
Addison's disease NOS
Adrenal atrophy (autoimmune)
Adrenal calcification
Adrenal crisis
Adrenal hemorrhage
Adrenal infarction
Adrenal insufficiency NOS
Combined glucocorticoid and mineralocorticoid deficiency
Corticoadrenal insufficiency NOS
255.42	Mineralocorticoid deficiency
Hypoaldosteronism
Excludes:	combined glucocorticoid and mineralocorticoid deficiency (255.41)
255.5	Other adrenal hypofunction
Adrenal medullary insufficiency
Excludes:	Waterhouse-Friderichsen syndrome (meningococcal) (036.3)
255.6	Medulloadrenal hyperfunction
Catecholamine secretion by pheochromocytoma
255.8	Other specified disorders of adrenal glands
Abnormality of cortisol-binding globulin
255.9	Unspecified disorder of adrenal glands
256	Ovarian dysfunction
256.0	Hyperestrogenism
256.1	Other ovarian hyperfunction
Hypersecretion of ovarian androgens
256.2	Postablative ovarian failure
Ovarian failure:
iatrogenic
postirradiation
postsurgical
Use additional code for states associated with artificial menopause (627.4)
Excludes:	acquired absence of ovary (V45.77)
asymptomatic age-related (natural) postmenopausal status (V49.81)
256.3	Other ovarian failure
Use additional code for states associated with natural menopause (627.2)
Excludes:	asymptomatic age-related (natural) postmenopausal status (V49.81)
256.31	Premature menopause
256.39	Other ovarian failure
Delayed menarche
Ovarian hypofunction
Primary ovarian failure NOS
256.4	Polycystic ovaries
Isosexual virilization Stein-Leventhal syndrome
256.8	Other ovarian dysfunction
256.9	Unspecified ovarian dysfunction
257	Testicular dysfunction
257.0	Testicular hyperfunction
Hypersecretion of testicular hormones
257.1	Postablative testicular hypofunction
Testicular hypofunction:
iatrogenic
postirradiation
postsurgical
257.2	Other testicular hypofunction
Defective biosynthesis of testicular androgen
Eunuchoidism:
NOS
hypogonadotropic
Failure:
Leydig's cell, adult
seminiferous tubule, adult
Testicular hypogonadism
Excludes:	azoospermia (606.0)
257.8	Other testicular dysfunction
Excludes:	androgen insensitivity syndrome (259.5)
257.9	Unspecified testicular dysfunction
258	Polyglandular dysfunction and related disorders
258.0	Polyglandular activity in multiple endocrine adenomatosis
Multiple endocrine neoplasia [MEN] syndromes
Use additional codes to identify any malignancies and other conditions associated with the
 syndromes
258.01	Multiple endocrine neoplasia [MEN] type I
Wermer's syndrome
258.02	Multiple endocrine neoplasia [MEN] type IIA
Sipple's syndrome
258.03	Multiple endocrine neoplasia [MEN] type IIB
258.1	Other combinations of endocrine dysfunction
Lloyd's syndrome
Schmidt's syndrome
258.8	Other specified polyglandular dysfunction
258.9	Polyglandular dysfunction, unspecified
259	Other endocrine disorders
259.0	Delay in sexual development and puberty, not elsewhere classified
Delayed puberty
259.1	Precocious sexual development and puberty, not elsewhere classified
Sexual precocity:
NOS
constitutional
cryptogenic
idiopathic
259.2	Carcinoid syndrome
Hormone secretion by carcinoid tumors
259.3	Ectopic hormone secretion, not elsewhere classified
Ectopic:
antidiuretic hormone secretion [ADH]
hyperparathyroidism
Excludes:	ectopic ACTH syndrome (255.0)
259.4	Dwarfism, not elsewhere classified
Dwarfism:
NOS
constitutional
Excludes:	dwarfism:
achondroplastic (756.4)
intrauterine (759.7)
nutritional (263.2)
pituitary (253.3)
renal (588.0)
progeria (259.8)
259.5	Androgen insensitivity syndrome
Partial androgen insensitivity
Reifenstein syndrome
259.8	Other specified endocrine disorders
Pineal gland dysfunction
Progeria
Werner's syndrome
259.9	Unspecified endocrine disorder
Disturbance:
endocrine NOS
hormone NOS
Infantilism NOS
NUTRITIONAL DEFICIENCIES (260-269)
Excludes:	deficiency anemias (280.0-281.9)
260	Kwashiorkor
Nutritional edema with dyspigmentation of skin and hair
261	Nutritional marasmus
Nutritional atrophy
Severe calorie deficiency
Severe malnutrition NOS
262	Other severe protein-calorie malnutrition
Nutritional edema without mention of dyspigmentation of skin and hair
263	Other and unspecified protein-calorie malnutrition
263.0	Malnutrition of moderate degree
263.1	Malnutrition of mild degree
263.2	Arrested development following protein-calorie malnutrition
Nutritional dwarfism
Physical retardation due to malnutrition
263.8	Other protein-calorie malnutrition
263.9	Unspecified protein-calorie malnutrition
Dystrophy due to malnutrition
Malnutrition (calorie) NOS
Excludes:	nutritional deficiency NOS (269.9)
264	Vitamin A deficiency
264.0	With conjunctival xerosis
264.1	With conjunctival xerosis and Bitot's spot
Bitot's spot in the young child
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
Xerophthalmia due to vitamin A deficiency
264.8	Other manifestations of vitamin A deficiency
Follicular keratosis due to vitamin A deficiency
Xeroderma due to vitamin A deficiency
264.9	Unspecified vitamin A deficiency
Hypovitaminosis A NOS
265	Thiamine and niacin deficiency states
265.0	Beriberi
265.1	Other and unspecified manifestations of thiamine deficiency
Other vitamin B1 deficiency states
265.2	Pellagra
Deficiency:
niacin (-tryptophan)
nicotinamide
nicotinic acid
vitamin PP
Pellagra (alcoholic)
266	Deficiency of B-complex components
266.0	Ariboflavinosis
Riboflavin [vitamin B2] deficiency
266.1	Vitamin B6 deficiency
Deficiency:
pyridoxal
pyridoxamine
pyridoxine
Vitamin B6 deficiency syndrome
Excludes:	vitamin B6-responsive sideroblastic anemia (285.0)
266.2	Other B-complex deficiencies
Deficiency:
cyanocobalamin
folic acid
vitamin B12
Excludes:	combined system disease with anemia (281.0-281.1)
deficiency anemias (281.0-281.9)
subacute degeneration of spinal cord with anemia (281.0-281.1)
266.9	Unspecified vitamin B deficiency
267	Ascorbic acid deficiency
Deficiency of vitamin C
Scurvy
Excludes:	scorbutic anemia (281.8)
268	Vitamin D deficiency
Excludes:	vitamin D-resistant:
osteomalacia (275.3)
rickets (275.3)
268.0	Rickets, active
Excludes:	celiac rickets (579.0)
renal rickets (588.0)
268.1	Rickets, late effect
Any condition specified as due to rickets and stated to be a late effect or sequela of rickets
Code first the nature of late effect
268.2	Osteomalacia, unspecified
268.9	Unspecified vitamin D deficiency
Avitaminosis D
269	Other nutritional deficiencies
269.0	Deficiency of vitamin K
Excludes:	deficiency of coagulation factor due to vitamin K deficiency (286.7)
vitamin K deficiency of newborn (776.0)
269.1	Deficiency of other vitamins
Deficiency:
vitamin E
vitamin P
269.2	Unspecified vitamin deficiency
Multiple vitamin deficiency NOS
269.3	Mineral deficiency, not elsewhere classified
Deficiency:
calcium, dietary
iodine
Excludes:	deficiency:
calcium NOS (275.40)
potassium (276.8)
sodium (276.1)
269.8	Other nutritional deficiency
Excludes:	adult failure to thrive (783.7)
failure to thrive in childhood (783.41)
feeding problems (783.3)
newborn (779.3)
269.9	Unspecified nutritional deficiency
OTHER METABOLIC AND IMMUNITY DISORDERS (270-279)
Use additional code to identify any associated mental retardation
270	Disorders of amino-acid transport and metabolism
Excludes:	abnormal findings without manifest disease (790.0-796.9)
disorders of purine and pyrimidine metabolism (277.1-277.2)
gout (274.0-274.9)
270.0	Disturbances of amino-acid transport
Cystinosis
Cystinuria
Fanconi (-de Toni) (-Debr�) syndrome
Glycinuria (renal)
Hartnup disease
270.1	Phenylketonuria [PKU]
Hyperphenylalaninemia
270.2	Other disturbances of aromatic amino-acid metabolism
Albinism
Alkaptonuria
Alkaptonuric ochronosis
Disturbances of metabolism of tyrosine and tryptophan
Homogentisic acid defects
Hydroxykynureninuria
Hypertyrosinemia
Indicanuria
Kynureninase defects
Oasthouse urine disease
Ochronosis
Tyrosinosis
Tyrosinuria
Waardenburg syndrome
Excludes:	vitamin B6-deficiency syndrome (266.1)
270.3	Disturbances of branched-chain amino-acid metabolism
Disturbances of metabolism of leucine, isoleucine, and valine
Hypervalinemia
Intermittent branched-chain ketonuria
Leucine-induced hypoglycemia
Leucinosis
Maple syrup urine disease
270.4	Disturbances of sulphur-bearing amino-acid metabolism
Cystathioninemia
Cystathioninuria
Disturbances of metabolism of methionine, homocystine, and cystathionine
Homocystinuria
Hypermethioninemia
Methioninemia
270.5	Disturbances of histidine metabolism
Carnosinemia
Histidinemia
Hyperhistidinemia
Imidazole aminoaciduria
270.6	Disorders of urea cycle metabolism
Argininosuccinic aciduria
Citrullinemia
Disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine, and ammonia
Hyperammonemia
Hyperornithinemia
270.7	Other disturbances of straight-chain amino-acid metabolism
Glucoglycinuria
Glycinemia (with methylmalonic acidemia)
Hyperglycinemia
Hyperlysinemia
Pipecolic acidemia
Saccharopinuria
Other disturbances of metabolism of glycine, threonine, serine, glutamine, and lysine
270.8	Other specified disorders of amino-acid metabolism
Alaninemia
Ethanolaminuria
Glycoprolinuria
Hydroxyprolinemia
Hyperprolinemia
Iminoacidopathy
Prolinemia
Prolinuria
Sarcosinemia
270.9	Unspecified disorder of amino-acid metabolism
271	Disorders of carbohydrate transport and metabolism
Excludes:	abnormality of secretion of glucagon (251.4)
diabetes mellitus (250.0-250.9)
hypoglycemia NOS (251.2)
mucopolysaccharidosis (277.5)
271.0	Glycogenosis
Amylopectinosis
Glucose-6-phosphatase deficiency
Glycogen storage disease
McArdle's disease
Pompe's disease
von Gierke's disease
271.1	Galactosemia
Galactose-1-phosphate uridyl transferase deficiency
Galactosuria
271.2	Hereditary fructose intolerance
Essential benign fructosuria
Fructosemia
271.3	Intestinal disaccharidase deficiencies and disaccharide malabsorption
Intolerance or malabsorption (congenital) (of):
glucose-galactose
lactose
sucrose-isomaltose
271.4	Renal glycosuria
Renal diabetes
271.8	Other specified disorders of carbohydrate transport and metabolism
Essential benign pentosuria
Fucosidosis
Glycolic aciduria
Hyperoxaluria (primary)
Mannosidosis
Oxalosis
Xylosuria
Xylulosuria
271.9	Unspecified disorder of carbohydrate transport and metabolism
272	Disorders of lipoid metabolism
Excludes:	localized cerebral lipidoses (330.1)
272.0	Pure hypercholesterolemia
Familial hypercholesterolemia
Fredrickson Type IIa hyperlipoproteinemia
Hyperbetalipoproteinemia
Hyperlipidemia, Group A
Low-density-lipoid-type [LDL] hyperlipoproteinemia
272.1	Pure hyperglyceridemia
Endogenous hyperglyceridemia
Fredrickson Type IV hyperlipoproteinemia
Hyperlipidemia, Group B
Hyperprebetalipoproteinemia
Hypertriglyceridemia, essential
Very-low-density-lipoid-type [VLDL] hyperlipoproteinemia
272.2	Mixed hyperlipidemia
Broad- or floating-betalipoproteinemia
Fredrickson Type IIb or III hyperlipoproteinemia
Hypercholesterolemia with endogenous hyperglyceridemia
Hyperbetalipoproteinemia with prebetalipoproteinemia
Tubo-eruptive xanthoma
Xanthoma tuberosum
272.3	Hyperchylomicronemia
B�rger-Gr�tz syndrome
Fredrickson type I or V hyperlipoproteinemia
Hyperlipidemia, Group D
Mixed hyperglyceridemia
272.4	Other and unspecified hyperlipidemia
Alpha-lipoproteinemia
Combined hyperlipidemia
Hyperlipidemia NOS
Hyperlipoproteinemia NOS
272.5	Lipoprotein deficiencies
Abetalipoproteinemia
Bassen-Kornzweig syndrome
High-density lipoid deficiency
Hypoalphalipoproteinemia
Hypobetalipoproteinemia (familial)
272.6	Lipodystrophy
Barraquer-Simons disease
Progressive lipodystrophy
Use additional E code to identify cause, if iatrogenic
Excludes:	intestinal lipodystrophy (040.2)
272.7	Lipidoses
Chemically induced lipidosis
Disease:
Anderson's
Fabry's
Gaucher's
I cell [mucolipidosis I]
lipoid storage NOS
Niemann-Pick
pseudo-Hurler's or mucolipidosis III
triglyceride storage, Type I or II
Wolman's or triglyceride storage, Type III
Mucolipidosis II
Primary familial xanthomatosis
Excludes:	cerebral lipidoses (330.1)
Tay-Sachs disease (330.1)
272.8	Other disorders of lipoid metabolism
Hoffa's disease or liposynovitis prepatellaris
Launois-Bensaude's lipomatosis
Lipoid dermatoarthritis
272.9	Unspecified disorder of lipoid metabolism
273	Disorders of plasma protein metabolism
Excludes:	agammaglobulinemia and hypogammaglobulinemia (279.0-279.2)
coagulation defects (286.0-286.9)
hereditary hemolytic anemias (282.0-282.9)
273.0	Polyclonal hypergammaglobulinemia
Hypergammaglobulinemic purpura:
benign primary
Waldenstr�m's
273.1	Monoclonal paraproteinemia
Benign monoclonal hypergammaglobulinemia [BMH]
Monoclonal gammopathy:
NOS
associated with lymphoplasmacytic dyscrasias
benign
Paraproteinemia:
benign (familial)
secondary to malignant or inflammatory disease
273.2	Other paraproteinemias
Cryoglobulinemic:
purpura
vasculitis
Mixed cryoglobulinemia
273.3	Macroglobulinemia
Macroglobulinemia (idiopathic) (primary)
Waldenstr�m's macroglobulinemia
273.4	Alpha-1-antitrypsin deficiency
AAT deficiency
273.8	Other disorders of plasma protein metabolism
Abnormality of transport protein
Bisalbuminemia
273.9	Unspecified disorder of plasma protein metabolism
274	Gout
Excludes:	lead gout (984.0-984.9)
274.0	Gouty arthropathy
274.1	Gouty nephropathy
274.10	Gouty nephropathy, unspecified
274.11	Uric acid nephrolithiasis
274.19	Other
274.8	Gout with other specified manifestations
274.81	Gouty tophi of ear
274.82	Gouty tophi of other sites
Gouty tophi of heart
274.89	Other
Use additional code to identify manifestations, as:
gouty:
iritis (364.11)
neuritis (357.4)
274.9	Gout, unspecified
275	Disorders of mineral metabolism
Excludes:	abnormal findings without manifest disease (790.0-796.9)
275.0	Disorders of iron metabolism
Bronzed diabetes
Hemochromatosis
Pigmentary cirrhosis (of liver)
Excludes:	anemia:
iron deficiency (280.0-280.9)
sideroblastic (285.0)
275.1	Disorders of copper metabolism
Hepatolenticular degeneration
Wilson's disease
275.2	Disorders of magnesium metabolism
Hypermagnesemia
Hypomagnesemia
275.3	Disorders of phosphorus metabolism
Familial hypophosphatemia
Hypophosphatasia
Vitamin D-resistant:
osteomalacia
rickets
275.4	Disorders of calcium metabolism
Excludes:	parathyroid disorders (252.00-252.9)
vitamin D deficiency (268.0-268.9)
275.40	Unspecified disorder of calcium metabolism
275.41	Hypocalcemia
275.42	Hypercalcemia
275.49	Other disorders of calcium metabolism
Nephrocalcinosis
Pseudohypoparathyroidism
Pseudopseudohypoparathyroidism
275.8	Other specified disorders of mineral metabolism
275.9	Unspecified disorder of mineral metabolism
276	Disorders of fluid, electrolyte, and acid-base balance
Excludes:	diabetes insipidus (253.5)
familial periodic paralysis (359.3)
276.0	Hyperosmolality and/or hypernatremia
Sodium [Na] excess
Sodium [Na] overload
276.1	Hyposmolality and/or hyponatremia
Sodium [Na] deficiency
276.2	Acidosis
Acidosis:
NOS
lactic
metabolic
respiratory
Excludes:	diabetic acidosis (250.1)
276.3	Alkalosis
Alkalosis:
NOS
metabolic
respiratory
276.4	Mixed acid-base balance disorder
Hypercapnia with mixed acid-base disorder
276.5	Volume depletion
Excludes:	hypovolemic shock:
postoperative (998.0)
traumatic (958.4)
276.50	Volume depletion, unspecified
276.51	Dehydration
276.52	Hypovolemia
Depletion of volume of plasma
276.6	Fluid overload
Fluid retention
Excludes:	ascites (789.51-789.59)
localized edema (782.3)
276.7	Hyperpotassemia
Hyperkalemia
Potassium [K]:
excess
intoxication
overload
276.8	Hypopotassemia
Hypokalemia
Potassium [K] deficiency
276.9	Electrolyte and fluid disorders not elsewhere classified
Electrolyte imbalance
Hyperchloremia
Hypochloremia
Excludes:	electrolyte imbalance:
associated with hyperemesis gravidarum  (643.1)
complicating labor and delivery (669.0)
following abortion and ectopic or molar pregnancy (634-638 with .4, 639.4)
277	Other and unspecified disorders of metabolism
277.0	Cystic fibrosis
Fibrocystic disease of the pancreas
Mucoviscidosis
277.00	Without mention of meconium ileus
Cystic fibrosis NOS
277.01	With meconium ileus
Meconium:
ileus (of newborn)
obstruction of intestine in mucoviscidosis
277.02	With pulmonary manifestations
Cystic fibrosis with pulmonary exacerbation
Use additional code to identify any infectious organism present, such as:
pseudomonas (041.7)
277.03	With gastrointestinal manifestations
Excludes:	with meconium ileus (277.01)
277.09	With other manifestations
277.1	Disorders of porphyrin metabolism
Hematoporphyria
Hematoporphyrinuria
Hereditary coproporphyria
Porphyria
Porphyrinuria
Protocoproporphyria
Protoporphyria
Pyrroloporphyria
277.2	Other disorders of purine and pyrimidine metabolism
Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
Lesch-Nyhan syndrome
Xanthinuria
Excludes:	gout (274.0-274.9)
orotic aciduric anemia (281.4)
277.3	Amyloidosis
277.30	Amyloidosis, unspecified
Amyloidosis NOS
277.31	Familial Mediterranean fever
Benign paroxysmal peritonitis
Hereditary amyloid nephropathy
Periodic familial polyserositis
Recurrent polyserositis
277.39	Other amyloidosis
Hereditary cardiac amyloidosis
Inherited systemic amyloidosis
Neuropathic (Portuguese) (Swiss) amyloidosis
Secondary amyloidosis
277.4	Disorders of bilirubin excretion
Hyperbilirubinemia:
congenital
constitutional
Syndrome:
Crigler-Najjar
Dubin-Johnson
Gilbert's
Rotor's
Excludes:	hyperbilirubinemias specific to the perinatal period (774.0-774.7)
277.5	Mucopolysaccharidosis
Gargoylism
Hunter's syndrome
Hurler's syndrome
Lipochondrodystrophy
Maroteaux-Lamy syndrome
Morquio-Brailsford disease
Osteochondrodystrophy
Sanfilippo's syndrome
Scheie's syndrome
277.6	Other deficiencies of circulating enzymes
Hereditary angioedema
277.7	Dysmetabolic syndrome X
Use additional code for associated manifestation, such as:
cardiovascular disease (414.00-414.07)
obesity (278.00-278.01)
277.8	Other specified disorders of metabolism
277.81	Primary carnitine deficiency
277.82	Carnitine deficiency due to inborn errors of metabolism
277.83	Iatrogenic carnitine deficiency
Carnitine deficiency due to:
Hemodialysis
Valproic acid therapy
277.84	Other secondary carnitine deficiency
277.85	Disorders of fatty acid oxidation
Carnitine palmitoyltransferase deficiencies (CPT1, CPT2)
Glutaric aciduria type II (type IIA, IIB, IIC)
Long chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHAD)
Long chain/very long chain acyl CoA dehydrogenase deficiency (LCAD, VLCAD)
Medium chain acyl CoA dehydrogenase deficiency (MCAD)
Short chain acyl CoA dehydrogenase deficiency (SCAD)
Excludes:	primary carnitine deficiency (277.81)
277.86	Peroxisomal disorders
Adrenomyeloneuropathy
Neonatal adrenoleukodystrophy
Rhizomelic chrondrodysplasia punctata
X-linked adrenoleukodystrophy
Zellweger syndrome
Excludes: infantile Refsum disease (356.3)
277.87	Disorders of mitochondrial metabolism
Kearns-Sayre syndrome
Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes  (MELAS syndrome)
Mitochondrial Neurogastrointestinal Encephalopathy syndrome (MNGIE)
Myoclonus with Epilepsy and with Ragged Red Fibers  (MERRF syndrome)
Neuropathy, Ataxia and Retinitis Pigmentosa  (NARP syndrome)
Use additional code for associated conditions
Excludes:	disorders of pyruvate metabolism (271.8)
Leber's optic atrophy (377.16)
Leigh's subacute necrotizing encephalopathy (330.8)
Reye's syndrome (331.81)
277.89	Other specified disorders of metabolism
Hand-Sch�ller-Christian disease
Histiocytosis (acute) (chronic)
Histiocytosis X (chronic)
Excludes:	histiocytosis:
acute differentiated progressive (202.5)
X, acute (progressive) (202.5)
277.9	Unspecified disorder of metabolism
Enzymopathy NOS
278	Overweight, obesity and other hyperalimentation
Excludes:	hyperalimentation NOS (783.6)
poisoning by vitamins NOS (963.5)
polyphagia (783.6)
278.0	Overweight and obesity
Excludes:	adiposogenital dystrophy (253.8)
obesity of endocrine origin NOS (259.9)
Use additional code to identify Body Mass Index (BMI) if known (V85.0-V85.54)
278.00	Obesity, unspecified
Obesity NOS
278.01	Morbid obesity
Severe obesity
278.02	Overweight
278.1	Localized adiposity
Fat pad
278.2	Hypervitaminosis A
278.3	Hypercarotinemia
278.4	Hypervitaminosis D
278.8	Other hyperalimentation
279	Disorders involving the immune mechanism
279.0	Deficiency of humoral immunity
279.00	Hypogammaglobulinemia, unspecified
Agammaglobulinemia NOS
279.01	Selective IgA immunodeficiency
279.02	Selective IgM immunodeficiency
279.03	Other selective immunoglobulin deficiencies
Selective deficiency of IgG
279.04	Congenital hypogammaglobulinemia
Agammaglobulinemia:
Bruton's type
X-linked
279.05	Immunodeficiency with increased IgM
Immunodeficiency with hyper-IgM:
autosomal recessive
X-linked
279.06	Common variable immunodeficiency
Dysgammaglobulinemia (acquired) (congenital) (primary)
Hypogammaglobulinemia:
acquired primary
congenital non-sex-linked
sporadic
279.09	Other
Transient hypogammaglobulinemia of infancy
279.1	Deficiency of cell-mediated immunity
279.10	Immunodeficiency with predominant T-cell defect, unspecified
279.11	DiGeorge's syndrome
Pharyngeal pouch syndrome
Thymic hypoplasia
279.12	Wiskott-Aldrich syndrome
279.13	Nezelof's syndrome
Cellular immunodeficiency with abnormal immunoglobulin deficiency
279.19	Other
Excludes:	ataxia-telangiectasia (334.8)
279.2	Combined immunity deficiency
Agammaglobulinemia:
autosomal recessive
Swiss-type
X-linked recessive
Severe combined immunodeficiency [SCID]
Thymic:
alymphoplasia
aplasia or dysplasia with immunodeficiency
Excludes:	thymic hypoplasia (279.11)
279.3	Unspecified immunity deficiency
279.4	Autoimmune disease, not elsewhere classified
Autoimmune disease NOS
Excludes:	transplant failure or rejection (996.80-996.89)
279.8	Other specified disorders involving the immune mechanism
Single complement [C1-C9] deficiency or dysfunction
279.9	Unspecified disorder of immune mechanism
4.  DISEASES OF THE BLOOD AND BLOOD-FORMING ORGANS (