Classification of diseases and injuries



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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 (779.31-779.34)

269.9 Unspecified nutritional deficiency

OTHER METABOLIC AND IMMUNITY DISORDERS (270-279)

Use additional code to identify any associated intellectual disabilities

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 (274.00-274.9)

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 (249.0-249.9, 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

Combined hyperlipidemia

Elevated cholesterol with elevated triglycerides NEC

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

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

273.4 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

274.00 Gouty arthropathy, unspecified

274.01 Acute gouty arthropathy

Acute gout

Gout attack

Gout flare

Podagra

274.02 Chronic gouty arthropathy without mention of tophus (tophi)



Chronic gout

274.03 Chronic gouty arthropathy with tophus (tophi)

Chronic tophaceous gout

Gout with tophi NOS

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

Excludes: gout with tophi NOS (274.03)

gouty arthropathy with tophi (274.03)

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

Excludes: anemia:

iron deficiency (280.0-280.9)

sideroblastic (285.0)

275.01 Hereditary hemochromatosis

Bronzed diabetes

Pigmentary cirrhosis (of liver)

Primary (hereditary) hemochromatosis

275.02 Hemochromatosis due to repeated red blood cell transfusions

Iron overload due to repeated red blood cell transfusions

Transfusion (red blood cell) associated hemochromatosis

275.03 Other hemochromatosis

Hemochromatosis NOS

275.09 Other disorders of iron metabolism

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: hungry bone syndrome (275.5)

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

275.5 Hungry bone syndrome

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 (249.1, 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 (998.09)

traumatic (958.4)

276.50 Volume depletion, unspecified

276.51 Dehydration

276.52 Hypovolemia

Depletion of volume of plasma

276.6 Fluid overload

Excludes: ascites (789.51-789.59)

localized edema (782.3)

276.61 Transfusion associated circulatory overload

Fluid overload due to transfusion (blood) (blood components)

TACO


276.69 Other fluid overload

Fluid retention

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 (274.00-274.9)

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 (278.00-278.03)

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)

277.88 Tumor lysis syndrome

Spontaneous tumor lysis syndrome

Tumor lysis syndrome following antineoplastic drug therapy

Use additional E code to identify cause, if drug-induced

277.89 Other specified disorders of metabolism

Hand-Schüller-Christian disease

Histiocytosis (acute) (chronic)

Histiocytosis X (chronic)

Excludes: histiocytosis:

acute differentiated progressive (202.5)

adult pulmonary Langerhans cell (516.5)

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

278.03 Obesity hypoventilation syndrome

Pickwickian syndrome

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

Use additional code for associated manifestations

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

Excludes: transplant failure or rejection (996.80-996.89)

279.41 Autoimmune lymphoproliferative syndrome

ALPS

279.49 Autoimmune disease, not elsewhere classified



Autoimmune disease NOS

279.5 Graft-versus-host disease

Code first underlying cause, such as:

complication of blood transfusion (999.89)

complication of transplanted organ (996.80-996.89)

Use additional code to identify associated manifestations, such as:

desquamative dermatitis (695.89)

diarrhea (787.91)

elevated bilirubin (782.4)

hair loss (704.09)

279.50 Graft-versus-host disease, unspecified

279.51 Acute graft-versus-host disease

279.52 Chronic graft-versus-host disease

279.53 Acute on chronic graft-versus-host disease

279.8 Other specified disorders involving the immune mechanism

Single complement [C1-C9] deficiency or dysfunction

279.9 Unspecified disorder of immune mechanism

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