OTHER METABOLIC AND IMMUNITY DISORDERS (270-279)

Disorders of amino-acid transport and metabolism
Disorders of carbohydrate transport and metabolism
Disorders of lipoid metabolism
Disorders of plasma protein metabolism
Gout
Disorders of mineral metabolism
Disorders of fluid, electrolyte, and acid-base balance
Other and unspecified disorders of metabolism
Obesity and other hyperalimentation
Disorders involving the immune mechanism

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) (-Debré) 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

Bürger-Grütz 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
Waldenström'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)
Waldenström's macroglobulinemia

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 (252.0-252.9)
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

Dehydration
Depletion of volume of plasma or extracellular fluid
Hypovolemia
Excludes: hypovolemic shock:
postoperative (998.0)
traumatic (958.4)

276.6 Fluid overload

Fluid retention
Excludes: ascites (789.5)
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

Amyloidosis:
NOS
inherited systemic
nephropathic
neuropathic (Portuguese) (Swiss)
secondary
Benign paroxysmal peritonitis
Familial Mediterranean fever
Hereditary cardiac 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

Alpha 1-antitrypsin deficiency
Hereditary angioedema

277.7 Dysmetabolic syndrome X

Use additional code for associated manifestation, such as:
cardiovascular disease (414.00-414.06)
obesity (278.00-278.01)

277.8 Other specified disorders of metabolism

Hand-Schüller-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 Obesity and other hyperalimentation

Excludes: hyperalimentation NOS (783.6)
poisoning by vitamins NOS (963.5)
polyphagia (783.6)

278.0 Obesity

Excludes: adiposogenital dystrophy (253.8)
obesity of endocrine origin NOS (259.9)
278.00 Obesity, unspecified
Obesity NOS
278.01 Morbid obesity

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