Metabolic disorders (E70-E88)
E70Disorders of aromatic amino-acid metabolism
E70.0Classical phenylketonuria
E70.1Other hyperphenylalaninemias
E70.2Disorders of tyrosine metabolism
Excludes 1: transitory tyrosinemia of newborn (P74.5)
E70.20Disorder of tyrosine metabolism, unspecified
E70.21Tyrosinemia
› Hypertyrosinemia
E70.29Other disorders of tyrosine metabolism
› Alkaptonuria
› Ochronosis
E70.3Albinism
E70.30Albinism, unspecified
E70.31Ocular albinism
E70.310X-linked ocular albinism
E70.311Autosomal recessive ocular albinism
E70.318Other ocular albinism
E70.319Ocular albinism, unspecified
E70.32Oculocutaneous albinism
Excludes 1: Chediak-Higashi syndrome (E70.330)
› Hermansky-Pudlak syndrome (E70.331)
E70.320Tyrosinase negative oculocutaneous albinism
› Albinism I
› Oculocutaneous albinism ty-neg
E70.321Tyrosinase positive oculocutaneous albinism
› Albinism II
› Oculocutaneous albinism ty-pos
E70.328Other oculocutaneous albinism
› Cross syndrome
E70.329Oculocutaneous albinism, unspecified
E70.33Albinism with hematologic abnormality
E70.330Chediak-Higashi syndrome
E70.331Hermansky-Pudlak syndrome
E70.338Other albinism with hematologic abnormality
E70.339Albinism with hematologic abnormality, unspecified
E70.39Other specified albinism
› Piebaldism
E70.4Disorders of histidine metabolism
E70.40Disorders of histidine metabolism, unspecified
E70.41Histidinemia
E70.49Other disorders of histidine metabolism
E70.5Disorders of tryptophan metabolism
E70.8Other disorders of aromatic amino-acid metabolism
E70.81Aromatic L-amino acid decarboxylase deficiency
› AADC deficiency
E70.89Other disorders of aromatic amino-acid metabolism
E70.9Disorder of aromatic amino-acid metabolism, unspecified
E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71.0Maple-syrup-urine disease
E71.1Other disorders of branched-chain amino-acid metabolism
E71.11Branched-chain organic acidurias
E71.110Isovaleric acidemia
E71.1113-methylglutaconic aciduria
E71.118Other branched-chain organic acidurias
E71.12Disorders of propionate metabolism
E71.120Methylmalonic acidemia
E71.121Propionic acidemia
E71.128Other disorders of propionate metabolism
E71.19Other disorders of branched-chain amino-acid metabolism
› Hyperleucine-isoleucinemia
› Hypervalinemia
E71.2Disorder of branched-chain amino-acid metabolism, unspecified
E71.3Disorders of fatty-acid metabolism
Excludes 1: peroxisomal disorders (E71.5)
› Refsum's disease (G60.1)
› Schilder's disease (G37.0)
Excludes 2: carnitine deficiency due to inborn error of metabolism (E71.42)
E71.30Disorder of fatty-acid metabolism, unspecified
E71.31Disorders of fatty-acid oxidation
E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
› LCAD deficiency
› VLCAD deficiency
E71.311Medium chain acyl CoA dehydrogenase deficiency
› MCAD deficiency
E71.312Short chain acyl CoA dehydrogenase deficiency
› SCAD deficiency
E71.313Glutaric aciduria type II
› Glutaric aciduria type II A
› Glutaric aciduria type II B
› Glutaric aciduria type II C
Excludes 1: glutaric aciduria (type 1) NOS (E72.3)
E71.314Muscle carnitine palmitoyltransferase deficiency
E71.318Other disorders of fatty-acid oxidation
E71.32Disorders of ketone metabolism
E71.39Other disorders of fatty-acid metabolism
E71.4Disorders of carnitine metabolism
Excludes 1: Muscle carnitine palmitoyltransferase deficiency (E71.314)
E71.40Disorder of carnitine metabolism, unspecified
E71.41Primary carnitine deficiency
E71.42Carnitine deficiency due to inborn errors of metabolism
Code Also: associated inborn error or metabolism
E71.43Iatrogenic carnitine deficiency
› Carnitine deficiency due to hemodialysis
› Carnitine deficiency due to Valproic acid therapy
E71.44Other secondary carnitine deficiency
E71.440Ruvalcaba-Myhre-Smith syndrome
E71.448Other secondary carnitine deficiency
E71.5Peroxisomal disorders
Excludes 1: Schilder's disease (G37.0)
E71.50Peroxisomal disorder, unspecified
E71.51Disorders of peroxisome biogenesis
› Group 1 peroxisomal disorders
Excludes 1: Refsum's disease (G60.1)
E71.510Zellweger syndrome
E71.511Neonatal adrenoleukodystrophy
Excludes 1: X-linked adrenoleukodystrophy (E71.42-)
E71.518Other disorders of peroxisome biogenesis
E71.52X-linked adrenoleukodystrophy
E71.520Childhood cerebral X-linked adrenoleukodystrophy
E71.521Adolescent X-linked adrenoleukodystrophy
E71.522Adrenomyeloneuropathy
E71.528Other X-linked adrenoleukodystrophy
› Addison only phenotype adrenoleukodystrophy
› Addison-Schilder adrenoleukodystrophy
E71.529X-linked adrenoleukodystrophy, unspecified type
E71.53Other group 2 peroxisomal disorders
E71.54Other peroxisomal disorders
E71.540Rhizomelic chondrodysplasia punctata
Excludes 1: chondrodysplasia punctata NOS (Q77.3)
E71.541Zellweger-like syndrome
E71.542Other group 3 peroxisomal disorders
E71.548Other peroxisomal disorders
E72Other disorders of amino-acid metabolism
Excludes 1: disorders of:
› aromatic amino-acid metabolism (E70.-)
› branched-chain amino-acid metabolism (E71.0-E71.2)
› fatty-acid metabolism (E71.3)
› purine and pyrimidine metabolism (E79.-)
› gout (M1A.-, M10.-)
E72.0Disorders of amino-acid transport
Excludes 1: disorders of tryptophan metabolism (E70.5)
E72.00Disorders of amino-acid transport, unspecified
E72.01Cystinuria
E72.02Hartnup's disease
E72.03Lowe's syndrome
Use Additional Code: code for associated glaucoma (H42)
E72.04Cystinosis
› Fanconi (-de Toni) (-Debré) syndrome with cystinosis
Excludes 1: Fanconi (-de Toni) (-Debré) syndrome without cystinosis (E72.09)
E72.09Other disorders of amino-acid transport
› Fanconi (-de Toni) (-Debré) syndrome, unspecified
E72.1Disorders of sulfur-bearing amino-acid metabolism
Excludes 1: cystinosis (E72.04)
› cystinuria (E72.01)
› transcobalamin II deficiency (D51.2)
E72.10Disorders of sulfur-bearing amino-acid metabolism, unspecified
E72.11Homocystinuria
› Cystathionine synthase deficiency
E72.12Methylenetetrahydrofolate reductase deficiency
E72.19Other disorders of sulfur-bearing amino-acid metabolism
› Cystathioninuria
› Methioninemia
› Sulfite oxidase deficiency
E72.2Disorders of urea cycle metabolism
Excludes 1: disorders of ornithine metabolism (E72.4)
E72.20Disorder of urea cycle metabolism, unspecified
› Hyperammonemia
Excludes 1: hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4
› transient hyperammonemia of newborn (P74.6)
E72.21Argininemia
E72.22Arginosuccinic aciduria
E72.23Citrullinemia
E72.29Other disorders of urea cycle metabolism
E72.3Disorders of lysine and hydroxylysine metabolism
› Glutaric aciduria NOS
› Glutaric aciduria (type I)
› Hydroxylysinemia
› Hyperlysinemia
Excludes 1: glutaric aciduria type II (E71.313)
› Refsum's disease (G60.1)
› Zellweger syndrome (E71.510)
E72.4Disorders of ornithine metabolism
› Hyperammonemia-Hyperornithinemia-Homocitrullinemia syndrome
› Ornithinemia (types I, II)
› Ornithine transcarbamylase deficiency
Excludes 1: hereditary choroidal dystrophy (H31.2-)
E72.5Disorders of glycine metabolism
E72.50Disorder of glycine metabolism, unspecified
E72.51Non-ketotic hyperglycinemia
E72.52Trimethylaminuria
E72.53Primary hyperoxaluria
› Oxalosis
› Oxaluria
E72.59Other disorders of glycine metabolism
› D-glycericacidemia
› Hyperhydroxyprolinemia
› Hyperprolinemia (types I, II)
› Sarcosinemia
E72.8Other specified disorders of amino-acid metabolism
E72.81Disorders of gamma aminobutyric acid metabolism
› 4-hydroxybutyric aciduria
› Disorders of GABA metabolism
› GABA metabolic defect
› GABA transaminase deficiency
› GABA-T deficiency
› Gamma-hydroxybutyric aciduria
› SSADHD
› Succinic semialdehyde dehydrogenase deficiency
E72.89Other specified disorders of amino-acid metabolism
› Disorders of beta-amino-acid metabolism
› Disorders of gamma-glutamyl cycle
E72.9Disorder of amino-acid metabolism, unspecified
E73Lactose intolerance
E73.0Congenital lactase deficiency
E73.1Secondary lactase deficiency
E73.8Other lactose intolerance
E73.9Lactose intolerance, unspecified
E74Other disorders of carbohydrate metabolism
Excludes 1: diabetes mellitus (E08-E13)
› hypoglycemia NOS (E16.2)
› increased secretion of glucagon (E16.3)
› mucopolysaccharidosis (E76.0-E76.3)
E74.0Glycogen storage disease
E74.00Glycogen storage disease, unspecified
E74.01von Gierke disease
› Type I glycogen storage disease
E74.02Pompe disease
› Cardiac glycogenosis
› Type II glycogen storage disease
E74.03Cori disease
› Forbes disease
› Type III glycogen storage disease
E74.04McArdle disease
› Type V glycogen storage disease
E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiency
› Danon disease
Code Also: , if applicable, associated manifestations such as:
› dilated cardiomyopathy (I42.0)
› obstructive hypertrophic cardiomyopathy (I42.1)
E74.09Other glycogen storage disease
› Andersen disease
› Glycogen storage disease, types 0, IV, VI-XI
› Hers disease
› Liver phosphorylase deficiency
› Muscle phosphofructokinase deficiency
› Tauri disease
E74.1Disorders of fructose metabolism
Excludes 1: muscle phosphofructokinase deficiency (E74.09)
E74.10Disorder of fructose metabolism, unspecified
E74.11Essential fructosuria
› Fructokinase deficiency
E74.12Hereditary fructose intolerance
› Fructosemia
E74.19Other disorders of fructose metabolism
› Fructose-1, 6-diphosphatase deficiency
E74.2Disorders of galactose metabolism
E74.20Disorders of galactose metabolism, unspecified
E74.21Galactosemia
E74.29Other disorders of galactose metabolism
› Galactokinase deficiency
E74.3Other disorders of intestinal carbohydrate absorption
Excludes 2: lactose intolerance (E73.-)
E74.31Sucrase-isomaltase deficiency
E74.39Other disorders of intestinal carbohydrate absorption
› Disorder of intestinal carbohydrate absorption NOS
› Glucose-galactose malabsorption
› Sucrase deficiency
E74.4Disorders of pyruvate metabolism and gluconeogenesis
› Deficiency of phosphoenolpyruvate carboxykinase
› Deficiency of pyruvate carboxylase
› Deficiency of pyruvate dehydrogenase
Excludes 1: disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
› Leigh's syndrome (G31.82)
E74.8Other specified disorders of carbohydrate metabolism
E74.81Disorders of glucose transport, not elsewhere classified
E74.810Glucose transporter protein type 1 deficiency
› De Vivo syndrome
› Glucose transport defect, blood-brain barrier
› Glut1 deficiency
› GLUT1 deficiency syndrome 1, infantile onset
› GLUT1 deficiency syndrome 2, childhood onset
E74.818Other disorders of glucose transport
› (Familial) renal glycosuria
E74.819Disorders of glucose transport, unspecified
E74.89Other specified disorders of carbohydrate metabolism
› Essential pentosuria
E74.9Disorder of carbohydrate metabolism, unspecified
E75Disorders of sphingolipid metabolism and other lipid storage disorders
Excludes 1: mucolipidosis, types I-III (E77.0-E77.1)
› Refsum's disease (G60.1)
E75.0GM2 gangliosidosis
E75.00GM2 gangliosidosis, unspecified
E75.01Sandhoff disease
E75.02Tay-Sachs disease
E75.09Other GM2 gangliosidosis
› Adult GM2 gangliosidosis
› Juvenile GM2 gangliosidosis
E75.1Other and unspecified gangliosidosis
E75.10Unspecified gangliosidosis
› Gangliosidosis NOS
E75.11Mucolipidosis IV
E75.19Other gangliosidosis
› GM1 gangliosidosis
› GM3 gangliosidosis
E75.2Other sphingolipidosis
Excludes 1: adrenoleukodystrophy [Addison-Schilder] (E71.528)
E75.21Fabry (-Anderson) disease
E75.22Gaucher disease
E75.23Krabbe disease
E75.24Niemann-Pick disease
› Acid sphingomyelinase deficiency (ASMD)
E75.240Niemann-Pick disease type A
› Acid sphingomyelinase deficiency type A (ASMD type A)
› Infantile neurovisceral acid sphingomyelinase deficiency
E75.241Niemann-Pick disease type B
› Acid sphingomyelinase deficiency type B (ASMD type B)
› Chronic visceral acid sphingomyelinase deficiency
E75.242Niemann-Pick disease type C
E75.243Niemann-Pick disease type D
E75.244Niemann-Pick disease type A/B
› Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
› Chronic neurovisceral acid sphingomyelinase deficiency
E75.248Other Niemann-Pick disease
E75.249Niemann-Pick disease, unspecified
› Acid sphingomyelinase deficiency (ASMD) NOS
E75.25Metachromatic leukodystrophy
E75.26Sulfatase deficiency
› Multiple sulfatase deficiency (MSD)
E75.27Pelizaeus-Merzbacher disease
E75.28Canavan disease
E75.29Other sphingolipidosis
› Farber's syndrome
› Sulfatide lipidosis
E75.3Sphingolipidosis, unspecified
E75.4Neuronal ceroid lipofuscinosis
› Batten disease
› Bielschowsky-Jansky disease
› Kufs disease
› Spielmeyer-Vogt disease
E75.5Other lipid storage disorders
› Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
› Wolman's disease
E75.6Lipid storage disorder, unspecified
E76Disorders of glycosaminoglycan metabolism
E76.0Mucopolysaccharidosis, type I
E76.01Hurler's syndrome
E76.02Hurler-Scheie syndrome
E76.03Scheie's syndrome
E76.1Mucopolysaccharidosis, type II
› Hunter's syndrome
E76.2Other mucopolysaccharidoses
E76.21Morquio mucopolysaccharidoses
E76.210Morquio A mucopolysaccharidoses
› Classic Morquio syndrome
› Morquio syndrome A
› Mucopolysaccharidosis, type IVA
E76.211Morquio B mucopolysaccharidoses
› Morquio-like mucopolysaccharidoses
› Morquio-like syndrome
› Morquio syndrome B
› Mucopolysaccharidosis, type IVB
E76.219Morquio mucopolysaccharidoses, unspecified
› Morquio syndrome
› Mucopolysaccharidosis, type IV
E76.22Sanfilippo mucopolysaccharidoses
› Mucopolysaccharidosis, type III (A) (B) (C) (D)
› Sanfilippo A syndrome
› Sanfilippo B syndrome
› Sanfilippo C syndrome
› Sanfilippo D syndrome
E76.29Other mucopolysaccharidoses
› beta-Glucuronidase deficiency
› Maroteaux-Lamy (mild) (severe) syndrome
› Mucopolysaccharidosis, types VI, VII
E76.3Mucopolysaccharidosis, unspecified
E76.8Other disorders of glucosaminoglycan metabolism
E76.9Glucosaminoglycan metabolism disorder, unspecified
E77Disorders of glycoprotein metabolism
E77.0Defects in post-translational modification of lysosomal enzymes
› Mucolipidosis II [I-cell disease]
› Mucolipidosis III [pseudo-Hurler polydystrophy]
E77.1Defects in glycoprotein degradation
› Aspartylglucosaminuria
› Fucosidosis
› Mannosidosis
› Sialidosis [mucolipidosis I]
E77.8Other disorders of glycoprotein metabolism
E77.9Disorder of glycoprotein metabolism, unspecified
E78Disorders of lipoprotein metabolism and other lipidemias
Excludes 1: sphingolipidosis (E75.0-E75.3)
E78.0Pure hypercholesterolemia
E78.00Pure hypercholesterolemia, unspecified
› Fredrickson's hyperlipoproteinemia, type IIa
› Hyperbetalipoproteinemia
› Low-density-lipoprotein-type [LDL] hyperlipoproteinemia
› (Pure) hypercholesterolemia NOS
E78.01Familial hypercholesterolemia
E78.1Pure hyperglyceridemia
› Elevated fasting triglycerides
› Endogenous hyperglyceridemia
› Fredrickson's hyperlipoproteinemia, type IV
› Hyperlipidemia, group B
› Hyperprebetalipoproteinemia
› Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinemia
E78.2Mixed hyperlipidemia
› Broad- or floating-betalipoproteinemia
› Combined hyperlipidemia NOS
› Elevated cholesterol with elevated triglycerides NEC
› Fredrickson's hyperlipoproteinemia, type IIb or III
› Hyperbetalipoproteinemia with prebetalipoproteinemia
› Hypercholesteremia with endogenous hyperglyceridemia
› Hyperlipidemia, group C
› Tubo-eruptive xanthoma
› Xanthoma tuberosum
Excludes 1: cerebrotendinous cholesterosis [van Bogaert-Scherer- Epstein] (E75.5)
› familial combined hyperlipidemia (E78.49)
E78.3Hyperchylomicronemia
› Chylomicron retention disease
› Fredrickson's hyperlipoproteinemia, type I or V
› Hyperlipidemia, group D
› Mixed hyperglyceridemia
E78.4Other hyperlipidemia
E78.41Elevated Lipoprotein(a)
› Elevated Lp(a)
E78.49Other hyperlipidemia
› Familial combined hyperlipidemia
E78.5Hyperlipidemia, unspecified
E78.6Lipoprotein deficiency
› Abetalipoproteinemia
› Depressed HDL cholesterol
› High-density lipoprotein deficiency
› Hypoalphalipoproteinemia
› Hypobetalipoproteinemia (familial)
› Lecithin cholesterol acyltransferase deficiency
› Tangier disease
E78.7Disorders of bile acid and cholesterol metabolism
Excludes 1: Niemann-Pick disease type C (E75.242)
E78.70Disorder of bile acid and cholesterol metabolism, unspecified
E78.71Barth syndrome
E78.72Smith-Lemli-Opitz syndrome
E78.79Other disorders of bile acid and cholesterol metabolism
E78.8Other disorders of lipoprotein metabolism
E78.81Lipoid dermatoarthritis
E78.89Other lipoprotein metabolism disorders
E78.9Disorder of lipoprotein metabolism, unspecified
E79Disorders of purine and pyrimidine metabolism
Excludes 1: Ataxia-telangiectasia (Q87.19)
› Bloom's syndrome (Q82.8)
› Cockayne's syndrome (Q87.19)
› calculus of kidney (N20.0)
› combined immunodeficiency disorders (D81.-)
› Fanconi's anemia (D61.09)
› gout (M1A.-, M10.-)
› orotaciduric anemia (D53.0)
› progeria (E34.8)
› Werner's syndrome (E34.8)
› xeroderma pigmentosum (Q82.1)
E79.0Hyperuricemia without signs of inflammatory arthritis and tophaceous disease
› Asymptomatic hyperuricemia
E79.1Lesch-Nyhan syndrome
› HGPRT deficiency
E79.2Myoadenylate deaminase deficiency
E79.8Other disorders of purine and pyrimidine metabolism
E79.81Aicardi-Goutières syndrome
E79.82Hereditary xanthinuria
E79.89Other specified disorders of purine and pyrimidine metabolism
E79.9Disorder of purine and pyrimidine metabolism, unspecified
E80Disorders of porphyrin and bilirubin metabolism
Includes: defects of catalase and peroxidase
E80.0Hereditary erythropoietic porphyria
› Congenital erythropoietic porphyria
› Erythropoietic protoporphyria
E80.1Porphyria cutanea tarda
E80.2Other and unspecified porphyria
E80.20Unspecified porphyria
› Porphyria NOS
E80.21Acute intermittent (hepatic) porphyria
E80.29Other porphyria
› Hereditary coproporphyria
E80.3Defects of catalase and peroxidase
› Acatalasia [Takahara]
E80.4Gilbert syndrome
E80.5Crigler-Najjar syndrome
E80.6Other disorders of bilirubin metabolism
› Dubin-Johnson syndrome
› Rotor's syndrome
E80.7Disorder of bilirubin metabolism, unspecified
E83Disorders of mineral metabolism
Excludes 1: dietary mineral deficiency (E58-E61)
› parathyroid disorders (E20-E21)
› vitamin D deficiency (E55.-)
E83.0Disorders of copper metabolism
E83.00Disorder of copper metabolism, unspecified
E83.01Wilson's disease
Code Also: associated Kayser Fleischer ring (H18.04-)
E83.09Other disorders of copper metabolism
› Menkes' (kinky hair) (steely hair) disease
E83.1Disorders of iron metabolism
Excludes 1: iron deficiency anemia (D50.-)
› sideroblastic anemia (D64.0-D64.3)
E83.10Disorder of iron metabolism, unspecified
E83.11Hemochromatosis
Excludes 1: GALD (P78.84)
› Gestational alloimmune liver disease (P78.84)
› Neonatal hemochromatosis (P78.84)
E83.110Hereditary hemochromatosis
› Bronzed diabetes
› Pigmentary cirrhosis (of liver)
› Primary (hereditary) hemochromatosis
E83.111Hemochromatosis due to repeated red blood cell transfusions
› Iron overload due to repeated red blood cell transfusions
› Transfusion (red blood cell) associated hemochromatosis
E83.118Other hemochromatosis
E83.119Hemochromatosis, unspecified
E83.19Other disorders of iron metabolism
Use Additional Code: code, if applicable, for idiopathic pulmonary hemosiderosis (J84.03)
E83.2Disorders of zinc metabolism
› Acrodermatitis enteropathica
E83.3Disorders of phosphorus metabolism and phosphatases
Excludes 1: adult osteomalacia (M83.-)
› osteoporosis (M80.-)
E83.30Disorder of phosphorus metabolism, unspecified
E83.31Familial hypophosphatemia
› Vitamin D-resistant osteomalacia
› Vitamin D-resistant rickets
Excludes 1: vitamin D-deficiency rickets (E55.0)
E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)
› 25-hydroxyvitamin D 1-alpha-hydroxylase deficiency
› Pseudovitamin D deficiency
› Vitamin D receptor defect
E83.39Other disorders of phosphorus metabolism
› Acid phosphatase deficiency
› Hypophosphatasia
E83.4Disorders of magnesium metabolism
E83.40Disorders of magnesium metabolism, unspecified
E83.41Hypermagnesemia
E83.42Hypomagnesemia
E83.49Other disorders of magnesium metabolism
E83.5Disorders of calcium metabolism
Excludes 1: autoimmune hypoparathyroidism (E20.812)
› autosomal dominant hypocalcemia (E20.810)
› chondrocalcinosis (M11.1-M11.2)
› hungry bone syndrome (E83.81)
› hyperparathyroidism (E21.0-E21.3)
› secondary hypoparathyroidism in diseases classified elsewhere (E20.811)
E83.50Unspecified disorder of calcium metabolism
E83.51Hypocalcemia
E83.52Hypercalcemia
› Familial hypocalciuric hypercalcemia
E83.59Other disorders of calcium metabolism
E83.8Other disorders of mineral metabolism
E83.81Hungry bone syndrome
E83.89Other disorders of mineral metabolism
E83.9Disorder of mineral metabolism, unspecified
E84Cystic fibrosis
Includes: mucoviscidosis
Code Also: exocrine pancreatic insufficiency (K86.81)
E84.0Cystic fibrosis with pulmonary manifestations
Use Additional Code: code to identify any infectious organism present, such as:
› Pseudomonas (B96.5)
E84.1Cystic fibrosis with intestinal manifestations
E84.11Meconium ileus in cystic fibrosis
Excludes 1: meconium ileus not due to cystic fibrosis (P76.0)
E84.19Cystic fibrosis with other intestinal manifestations
› Distal intestinal obstruction syndrome
E84.8Cystic fibrosis with other manifestations
E84.9Cystic fibrosis, unspecified
E85Amyloidosis
Excludes 2: Alzheimer's disease (G30.0-)
E85.0Non-neuropathic heredofamilial amyloidosis
› Hereditary amyloid nephropathy
Code Also: associated disorders, such as:
› autoinflammatory syndromes (M04.-)
Excludes 2: Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)
E85.1Neuropathic heredofamilial amyloidosis
› Amyloid polyneuropathy (Portuguese)
› Transthyretin-related (ATTR) familial amyloid polyneuropathy
E85.2Heredofamilial amyloidosis, unspecified
E85.3Secondary systemic amyloidosis
› Hemodialysis-associated amyloidosis
E85.4Organ-limited amyloidosis
› Localized amyloidosis
› Transthyretin-related (ATTR) familial amyloid cardiomyopathy
E85.8Other amyloidosis
E85.81Light chain (AL) amyloidosis
E85.82Wild-type transthyretin-related (ATTR) amyloidosis
› Senile systemic amyloidosis (SSA)
E85.89Other amyloidosis
E85.9Amyloidosis, unspecified
E86Volume depletion
Use Additional Code: code(s) for any associated disorders of electrolyte and acid-base balance (E87.-)
Excludes 1: dehydration of newborn (P74.1)
› postprocedural hypovolemic shock (T81.19)
› traumatic hypovolemic shock (T79.4)
Excludes 2: hypovolemic shock NOS (R57.1)
E86.0Dehydration
E86.1Hypovolemia
› Depletion of volume of plasma
E86.9Volume depletion, unspecified
E87Other disorders of fluid, electrolyte and acid-base balance
Excludes 1: diabetes insipidus (E23.2)
› electrolyte imbalance associated with hyperemesis gravidarum (O21.1)
› electrolyte imbalance following ectopic or molar pregnancy (O08.5)
› familial periodic paralysis (G72.3)
› metabolic acidemia in newborn, unspecified (P19.9)
E87.0Hyperosmolality and hypernatremia
› Sodium [Na] excess
› Sodium [Na] overload
Excludes 1: diabetes with hyperosmolarity (E08, E09, E11, E13 with final characters .00 or .01)
E87.1Hypo-osmolality and hyponatremia
› Sodium [Na] deficiency
Excludes 1: syndrome of inappropriate secretion of antidiuretic hormone (E22.2)
E87.2Acidosis
Excludes 1: diabetic acidosis - see categories E08-E10, E11, E13 with ketoacidosis
E87.20Acidosis, unspecified
› Lactic acidosis NOS
› Metabolic acidosis NOS
Code Also: , if applicable, respiratory failure with hypercapnia (J96. with 5th character 2)
E87.21Acute metabolic acidosis
› Acute lactic acidosis
E87.22Chronic metabolic acidosis
› Chronic lactic acidosis
Code First: underlying etiology, if applicable
E87.29Other acidosis
› Respiratory acidosis NOS
Excludes 2: acute respiratory acidosis (J96.02)
› chronic respiratory acidosis (J96.12)
E87.3Alkalosis
› Alkalosis NOS
› Metabolic alkalosis
› Respiratory alkalosis
E87.4Mixed disorder of acid-base balance
E87.5Hyperkalemia
› Potassium [K] excess
› Potassium [K] overload
E87.6Hypokalemia
› Potassium [K] deficiency
E87.7Fluid overload
Excludes 1: edema NOS (R60.9)
› fluid retention (R60.9)
E87.70Fluid overload, unspecified
E87.71Transfusion associated circulatory overload
› Fluid overload due to transfusion (blood) (blood components)
› TACO
E87.79Other fluid overload
E87.8Other disorders of electrolyte and fluid balance, not elsewhere classified
› Electrolyte imbalance NOS
› Hyperchloremia
› Hypochloremia
E88Other and unspecified metabolic disorders
Use Additional Code: codes for associated conditions
Excludes 1: histiocytosis X (chronic) (C96.6)
E88.0Disorders of plasma-protein metabolism, not elsewhere classified
Excludes 1: monoclonal gammopathy (of undetermined significance) (D47.2)
› polyclonal hypergammaglobulinemia (D89.0)
› Waldenström macroglobulinemia (C88.0)
Excludes 2: disorder of lipoprotein metabolism (E78.-)
E88.01Alpha-1-antitrypsin deficiency
› AAT deficiency
E88.02Plasminogen deficiency
› Dysplasminogenemia
› Hypoplasminogenemia
› Type 1 plasminogen deficiency
› Type 2 plasminogen deficiency
Code Also: , if applicable, ligneous conjunctivitis (H10.51)
Use Additional Code: code for associated findings, such as:
› hydrocephalus (G91.4)
› otitis media (H67.-)
› respiratory disorder related to plasminogen deficiency (J99)
E88.09Other disorders of plasma-protein metabolism, not elsewhere classified
› Bisalbuminemia
E88.1Lipodystrophy, not elsewhere classified
› Lipodystrophy NOS
Excludes 1: Whipple's disease (K90.81)
E88.2Lipomatosis, not elsewhere classified
› Lipomatosis NOS
› Lipomatosis (Check) dolorosa [Dercum]
E88.3Tumor lysis syndrome
› Tumor lysis syndrome (spontaneous)
› Tumor lysis syndrome following antineoplastic drug chemotherapy
Use Additional Code: code for adverse effect, if applicable, to identify drug (T45.1X5)
E88.4Mitochondrial metabolism disorders
Excludes 1: disorders of pyruvate metabolism (E74.4)
› Kearns-Sayre syndrome (H49.81)
› Leber's disease (H47.22)
› Leigh's encephalopathy (G31.82)
› Mitochondrial myopathy, NEC (G71.3)
› Reye's syndrome (G93.7)
E88.40Mitochondrial metabolism disorder, unspecified
E88.41MELAS syndrome
› Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
E88.42MERRF syndrome
› Myoclonic epilepsy associated with ragged-red fibers
Code Also: progressive myoclonic epilepsy (G40.3-)
E88.43Disorders of mitochondrial tRNA synthetases
E88.49Other mitochondrial metabolism disorders
E88.8Other specified metabolic disorders
E88.81Metabolic syndrome and other insulin resistance
Use Additional Code: codes for associated manifestations, such as:
› obesity (E66.-)
E88.810Metabolic syndrome
› Dysmetabolic syndrome
E88.811Insulin resistance syndrome, Type A
E88.818Other insulin resistance
› Insulin resistance syndrome, Type B
E88.819Insulin resistance, unspecified
E88.89Other specified metabolic disorders
› Launois-Bensaude adenolipomatosis
Excludes 1: adult pulmonary Langerhans cell histiocytosis (J84.82)
E88.9Metabolic disorder, unspecified
E88.AWasting disease (syndrome) due to underlying condition
› Cachexia due to underlying condition
Code First: underlying condition
Excludes 1: cachexia NOS (R64)
› nutritional marasmus (E41)
Excludes 2: failure to thrive (R62.51, R62.7)