OTHER METABOLIC AND IMMUNITY DISORDERS (ICD 9 CODES: 270-279)
Use additional code to identify any associated intellectual disabilities
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)
Cystinosis
Cystinuria
Fanconi (-de Toni) (-Debré) syndrome
Glycinuria (renal)
Hartnup disease
Hyperphenylalaninemia
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)
Disturbances of metabolism of leucine, isoleucine, and valine
Hypervalinemia
Intermittent branched-chain ketonuria
Leucine-induced hypoglycemia
Leucinosis
Maple syrup urine disease
Cystathioninemia
Cystathioninuria
Disturbances of metabolism of methionine, homocystine, and cystathionine
Homocystinuria
Hypermethioninemia
Methioninemia
Carnosinemia
Histidinemia
Hyperhistidinemia
Imidazole aminoaciduria
Argininosuccinic aciduria
Citrullinemia
Disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine, and ammonia
Hyperammonemia
Hyperornithinemia
Glucoglycinuria
Glycinemia (with methylmalonic acidemia)
Hyperglycinemia
Hyperlysinemia
Pipecolic acidemia
Saccharopinuria
Other disturbances of metabolism of glycine, threonine, serine, glutamine, and lysine
Alaninemia
Ethanolaminuria
Glycoprolinuria
Hydroxyprolinemia
Hyperprolinemia
Iminoacidopathy
Prolinemia
Prolinuria
Sarcosinemia
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)
Amylopectinosis
Glucose-6-phosphatase deficiency
Glycogen storage disease
McArdle's disease
Pompe's disease
von Gierke's disease
Galactose-1-phosphate uridyl transferase deficiency
Galactosuria
Essential benign fructosuria
Fructosemia
Intolerance or malabsorption (congenital) (of):
glucose-galactose
lactose
sucrose-isomaltose
Renal diabetes
Essential benign pentosuria
Fucosidosis
Glycolic aciduria
Hyperoxaluria (primary)
Mannosidosis
Oxalosis
Xylosuria
Xylulosuria
Excludes: localized cerebral lipidoses (330.1)
Familial hypercholesterolemia
Fredrickson Type IIa hyperlipoproteinemia
Hyperbetalipoproteinemia
Hyperlipidemia, Group A
Low-density-lipoid-type [LDL] hyperlipoproteinemia
Endogenous hyperglyceridemia
Fredrickson Type IV hyperlipoproteinemia
Hyperlipidemia, Group B
Hyperprebetalipoproteinemia
Hypertriglyceridemia, essential
Very-low-density-lipoid-type [VLDL] hyperlipoproteinemia
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
Bürger-Grütz syndrome
Fredrickson type I or V hyperlipoproteinemia
Hyperlipidemia, Group D
Mixed hyperglyceridemia
Alpha-lipoproteinemia
Hyperlipidemia NOS
Hyperlipoproteinemia NOS
Abetalipoproteinemia
Bassen-Kornzweig syndrome
High-density lipoid deficiency
Hypoalphalipoproteinemia
Hypobetalipoproteinemia (familial)
Barraquer-Simons disease
Progressive lipodystrophy
Use additional E code to identify cause, if iatrogenic
Excludes: intestinal lipodystrophy (040.2)
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)
Hoffa's disease or liposynovitis prepatellaris
Launois-Bensaude's lipomatosis
Lipoid dermatoarthritis
Excludes: agammaglobulinemia and hypogammaglobulinemia (279.0-279.2)
coagulation defects (286.0-286.9)
hereditary hemolytic anemias (282.0-282.9)
Hypergammaglobulinemic purpura:
benign primary
Waldenström's
Benign monoclonal hypergammaglobulinemia [BMH]
Monoclonal gammopathy:
NOS
associated with lymphoplasmacytic dyscrasias
benign
Paraproteinemia:
benign (familial)
secondary to malignant or inflammatory disease
Cryoglobulinemic:
purpura
vasculitis
Mixed cryoglobulinemia
Macroglobulinemia (idiopathic) (primary)
Waldenström's macroglobulinemia
AAT deficiency
Abnormality of transport protein
Bisalbuminemia
Excludes: lead gout (984.0-984.9)
Acute gout
Gout attack
Gout flare
Podagra
Chronic gout
Chronic tophaceous gout
Gout with tophi NOS
Gouty tophi of heart
Excludes: gout with tophi NOS (274.03)
gouty arthropathy with tophi (274.03)
Use additional code to identify manifestations, as:
gouty:
iritis (364.11)
neuritis (357.4)
Excludes: abnormal findings without manifest disease (790.0-796.9)
Excludes: anemia:
iron deficiency (280.0-280.9)
sideroblastic (285.0)
Bronzed diabetes
Pigmentary cirrhosis (of liver)
Primary (hereditary) hemochromatosis
Iron overload due to repeated red blood cell transfusions
Transfusion (red blood cell) associated hemochromatosis
Hemochromatosis NOS
Hepatolenticular degeneration
Wilson's disease
Hypermagnesemia
Hypomagnesemia
Familial hypophosphatemia
Hypophosphatasia
Vitamin D-resistant:
osteomalacia
rickets
Excludes: hungry bone syndrome (275.5)
parathyroid disorders (252.00-252.9)
vitamin D deficiency (268.0-268.9)
Nephrocalcinosis
Pseudohypoparathyroidism
Pseudopseudohypoparathyroidism
Excludes: diabetes insipidus (253.5)
familial periodic paralysis (359.3)
Sodium [Na] excess
Sodium [Na] overload
Sodium [Na] deficiency
Acidosis:
NOS
lactic
metabolic
respiratory
Excludes: diabetic acidosis (249.1, 250.1)
Alkalosis:
NOS
metabolic
respiratory
Hypercapnia with mixed acid-base disorder
Excludes: hypovolemic shock:
postoperative (998.09)
traumatic (958.4)
Depletion of volume of plasma
Excludes: ascites (789.51-789.59)
localized edema (782.3)
Fluid overload due to transfusion (blood) (blood components)
TACO
Fluid retention
Hyperkalemia
Potassium [K]:
excess
intoxication
overload
Hypokalemia
Potassium [K] deficiency
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)
Fibrocystic disease of the pancreas
Mucoviscidosis
Cystic fibrosis NOS
Meconium:
ileus (of newborn)
obstruction of intestine in mucoviscidosis
Cystic fibrosis with pulmonary exacerbation
Use additional code to identify any infectious organism present, such as:
pseudomonas (041.7)
Excludes: with meconium ileus (277.01)
Hematoporphyria
Hematoporphyrinuria
Hereditary coproporphyria
Porphyria
Porphyrinuria
Protocoproporphyria
Protoporphyria
Pyrroloporphyria
Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
Lesch-Nyhan syndrome
Xanthinuria
Excludes: gout (274.00-274.9)
orotic aciduric anemia (281.4)
Amyloidosis NOS
Benign paroxysmal peritonitis
Hereditary amyloid nephropathy
Periodic familial polyserositis
Recurrent polyserositis
Hereditary cardiac amyloidosis
Inherited systemic amyloidosis
Neuropathic (Portuguese) (Swiss) amyloidosis
Secondary amyloidosis
Hyperbilirubinemia:
congenital
constitutional
Syndrome:
Crigler-Najjar
Dubin-Johnson
Gilbert's
Rotor's
Excludes: hyperbilirubinemias specific to the perinatal period (774.0-774.7)
Gargoylism
Hunter's syndrome
Hurler's syndrome
Lipochondrodystrophy
Maroteaux-Lamy syndrome
Morquio-Brailsford disease
Osteochondrodystrophy
Sanfilippo's syndrome
Scheie's syndrome
Hereditary angioedema
Use additional code for associated manifestation, such as:
cardiovascular disease (414.00-414.07)
obesity (278.00-278.03)
Carnitine deficiency due to:
Hemodialysis
Valproic acid therapy
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)
Adrenomyeloneuropathy
Neonatal adrenoleukodystrophy
Rhizomelic chrondrodysplasia punctata
X-linked adrenoleukodystrophy
Zellweger syndrome
Excludes: infantile Refsum disease (356.3)
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)
Spontaneous tumor lysis syndrome
Tumor lysis syndrome following antineoplastic drug therapy
Use additional E code to identify cause, if drug-induced
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)
Enzymopathy NOS
Excludes: hyperalimentation NOS (783.6)
poisoning by vitamins NOS (963.5)
polyphagia (783.6)
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)
Obesity NOS
Severe obesity
Pickwickian syndrome
Fat pad
Use additional code for associated manifestations
Agammaglobulinemia NOS
Selective deficiency of IgG
Agammaglobulinemia:
Bruton's type
X-linked
Immunodeficiency with hyper-IgM:
autosomal recessive
X-linked
Dysgammaglobulinemia (acquired) (congenital) (primary)
Hypogammaglobulinemia:
acquired primary
congenital non-sex-linked
sporadic
Transient hypogammaglobulinemia of infancy
Pharyngeal pouch syndrome
Thymic hypoplasia
Cellular immunodeficiency with abnormal immunoglobulin deficiency
Excludes: ataxia-telangiectasia (334.8)
Agammaglobulinemia:
autosomal recessive
Swiss-type
X-linked recessive
Severe combined immunodeficiency [SCID]
Thymic:
alymphoplasia
aplasia or dysplasia with immunodeficiency
Excludes: thymic hypoplasia (279.11)
Excludes: transplant failure or rejection (996.80-996.89)
ALPS
Autoimmune disease NOS
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)
Single complement [C1-C9] deficiency or dysfunction
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