ICD-10-CM Book 2024 Edition

Chapters  /  Chapter 4  /  E70-E88

Metabolic disorders (E70-E88)

Excludes1: androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)
Excludes2: Ehlers-Danlos syndromes (Q79.6-)
E70 Disorders of aromatic amino-acid metabolism
E70.0 Classical phenylketonuria
E70.1 Other hyperphenylalaninemias
E70.2 Disorders of tyrosine metabolism
Excludes1 transitory tyrosinemia of newborn (P74.5)
E70.20 Disorder of tyrosine metabolism, unspecified
E70.21 Tyrosinemia
Hypertyrosinemia
E70.29 Other disorders of tyrosine metabolism
Alkaptonuria
Ochronosis
E70.3 Albinism
E70.30 Albinism, unspecified
E70.31 Ocular albinism
E70.310 X-linked ocular albinism
E70.311 Autosomal recessive ocular albinism
E70.318 Other ocular albinism
E70.319 Ocular albinism, unspecified
E70.32 Oculocutaneous albinism
Excludes1 Chediak-Higashi syndrome (E70.330)
Hermansky-Pudlak syndrome (E70.331)
E70.320 Tyrosinase negative oculocutaneous albinism
Albinism I
Oculocutaneous albinism ty-neg
E70.321 Tyrosinase positive oculocutaneous albinism
Albinism II
Oculocutaneous albinism ty-pos
E70.328 Other oculocutaneous albinism
Cross syndrome
E70.329 Oculocutaneous albinism, unspecified
E70.33 Albinism with hematologic abnormality
E70.330 Chediak-Higashi syndrome
E70.331 Hermansky-Pudlak syndrome
E70.338 Other albinism with hematologic abnormality
E70.339 Albinism with hematologic abnormality, unspecified
E70.39 Other specified albinism
Piebaldism
E70.4 Disorders of histidine metabolism
E70.40 Disorders of histidine metabolism, unspecified
E70.41 Histidinemia
E70.49 Other disorders of histidine metabolism
E70.5 Disorders of tryptophan metabolism
E70.8 Other disorders of aromatic amino-acid metabolism
E70.81 Aromatic L-amino acid decarboxylase deficiency
AADC deficiency
E70.89 Other disorders of aromatic amino-acid metabolism
E70.9 Disorder of aromatic amino-acid metabolism, unspecified
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71.0 Maple-syrup-urine disease
E71.1 Other disorders of branched-chain amino-acid metabolism
E71.11 Branched-chain organic acidurias
E71.110 Isovaleric acidemia
E71.111 3-methylglutaconic aciduria
E71.118 Other branched-chain organic acidurias
E71.12 Disorders of propionate metabolism
E71.120 Methylmalonic acidemia
E71.121 Propionic acidemia
E71.128 Other disorders of propionate metabolism
E71.19 Other disorders of branched-chain amino-acid metabolism
Hyperleucine-isoleucinemia
Hypervalinemia
E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
E71.3 Disorders of fatty-acid metabolism
Excludes1 peroxisomal disorders (E71.5)
Refsum's disease (G60.1)
Schilder's disease (G37.0)
Excludes2 carnitine deficiency due to inborn error of metabolism (E71.42)
E71.30 Disorder of fatty-acid metabolism, unspecified
E71.31 Disorders of fatty-acid oxidation
E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
LCAD deficiency
VLCAD deficiency
E71.311 Medium chain acyl CoA dehydrogenase deficiency
MCAD deficiency
E71.312 Short chain acyl CoA dehydrogenase deficiency
SCAD deficiency
E71.313 Glutaric aciduria type II
Glutaric aciduria type II A
Glutaric aciduria type II B
Glutaric aciduria type II C
Excludes1 glutaric aciduria (type 1) NOS (E72.3)
E71.314 Muscle carnitine palmitoyltransferase deficiency
E71.318 Other disorders of fatty-acid oxidation
E71.32 Disorders of ketone metabolism
E71.39 Other disorders of fatty-acid metabolism
E71.4 Disorders of carnitine metabolism
Excludes1 Muscle carnitine palmitoyltransferase deficiency (E71.314)
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
CodeAlso associated inborn error or metabolism
E71.43 Iatrogenic carnitine deficiency
Carnitine deficiency due to hemodialysis
Carnitine deficiency due to Valproic acid therapy
E71.44 Other secondary carnitine deficiency
E71.440 Ruvalcaba-Myhre-Smith syndrome
E71.448 Other secondary carnitine deficiency
E71.5 Peroxisomal disorders
Excludes1 Schilder's disease (G37.0)
E71.50 Peroxisomal disorder, unspecified
E71.51 Disorders of peroxisome biogenesis
Group 1 peroxisomal disorders
Excludes1 Refsum's disease (G60.1)
E71.510 Zellweger syndrome
E71.511 Neonatal adrenoleukodystrophy
Excludes1 X-linked adrenoleukodystrophy (E71.42-)
E71.518 Other disorders of peroxisome biogenesis
E71.52 X-linked adrenoleukodystrophy
E71.520 Childhood cerebral X-linked adrenoleukodystrophy
E71.521 Adolescent X-linked adrenoleukodystrophy
E71.522 Adrenomyeloneuropathy
E71.528 Other X-linked adrenoleukodystrophy
Addison only phenotype adrenoleukodystrophy
Addison-Schilder adrenoleukodystrophy
E71.529 X-linked adrenoleukodystrophy, unspecified type
E71.53 Other group 2 peroxisomal disorders
E71.54 Other peroxisomal disorders
E71.540 Rhizomelic chondrodysplasia punctata
Excludes1 chondrodysplasia punctata NOS (Q77.3)
E71.541 Zellweger-like syndrome
E71.542 Other group 3 peroxisomal disorders
E71.548 Other peroxisomal disorders
E72 Other disorders of amino-acid metabolism
Excludes1 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.-)
E72.0 Disorders of amino-acid transport
Excludes1 disorders of tryptophan metabolism (E70.5)
E72.00 Disorders of amino-acid transport, unspecified
E72.01 Cystinuria
E72.02 Hartnup's disease
E72.03 Lowe's syndrome
UseAdditionalCode code for associated glaucoma (H42)
E72.04 Cystinosis
Fanconi (-de Toni) (-Debré) syndrome with cystinosis
Excludes1 Fanconi (-de Toni) (-Debré) syndrome without cystinosis (E72.09)
E72.09 Other disorders of amino-acid transport
Fanconi (-de Toni) (-Debré) syndrome, unspecified
E72.1 Disorders of sulfur-bearing amino-acid metabolism
Excludes1 cystinosis (E72.04)
cystinuria (E72.01)
transcobalamin II deficiency (D51.2)
E72.10 Disorders of sulfur-bearing amino-acid metabolism, unspecified
E72.11 Homocystinuria
Cystathionine synthase deficiency
E72.12 Methylenetetrahydrofolate reductase deficiency
E72.19 Other disorders of sulfur-bearing amino-acid metabolism
Cystathioninuria
Methioninemia
Sulfite oxidase deficiency
E72.2 Disorders of urea cycle metabolism
Excludes1 disorders of ornithine metabolism (E72.4)
E72.20 Disorder of urea cycle metabolism, unspecified
Hyperammonemia
Excludes1 hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4
transient hyperammonemia of newborn (P74.6)
E72.21 Argininemia
E72.22 Arginosuccinic aciduria
E72.23 Citrullinemia
E72.29 Other disorders of urea cycle metabolism
E72.3 Disorders of lysine and hydroxylysine metabolism
Glutaric aciduria NOS
Glutaric aciduria (type I)
Hydroxylysinemia
Hyperlysinemia
Excludes1 glutaric aciduria type II (E71.313)
Refsum's disease (G60.1)
Zellweger syndrome (E71.510)
E72.4 Disorders of ornithine metabolism
Hyperammonemia-Hyperornithinemia-Homocitrullinemia syndrome
Ornithinemia (types I, II)
Ornithine transcarbamylase deficiency
Excludes1 hereditary choroidal dystrophy (H31.2-)
E72.5 Disorders of glycine metabolism
E72.50 Disorder of glycine metabolism, unspecified
E72.51 Non-ketotic hyperglycinemia
E72.52 Trimethylaminuria
E72.53 Primary hyperoxaluria
Oxalosis
Oxaluria
E72.59 Other disorders of glycine metabolism
D-glycericacidemia
Hyperhydroxyprolinemia
Hyperprolinemia (types I, II)
Sarcosinemia
E72.8 Other specified disorders of amino-acid metabolism
E72.81 Disorders 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
E72.89 Other specified disorders of amino-acid metabolism
Disorders of beta-amino-acid metabolism
Disorders of gamma-glutamyl cycle
E72.9 Disorder of amino-acid metabolism, unspecified
E73 Lactose intolerance
E73.0 Congenital lactase deficiency
E73.1 Secondary lactase deficiency
E73.8 Other lactose intolerance
E73.9 Lactose intolerance, unspecified
E74 Other disorders of carbohydrate metabolism
Excludes1 diabetes mellitus (E08-E13)
hypoglycemia NOS (E16.2)
increased secretion of glucagon (E16.3)
mucopolysaccharidosis (E76.0-E76.3)
E74.0 Glycogen storage disease
E74.00 Glycogen storage disease, unspecified
E74.01 von Gierke disease
Type I glycogen storage disease
E74.02 Pompe disease
Cardiac glycogenosis
Type II glycogen storage disease
E74.03 Cori disease
Forbes disease
Type III glycogen storage disease
E74.04 McArdle disease
Type V glycogen storage disease
E74.05 Lysosome-associated membrane protein 2 [LAMP2] deficiency
Danon disease
CodeAlso , if applicable, associated manifestations such as:
dilated cardiomyopathy (I42.0)
obstructive hypertrophic cardiomyopathy (I42.1)
E74.09 Other glycogen storage disease
Andersen disease
Glycogen storage disease, types 0, IV, VI-XI
Hers disease
Liver phosphorylase deficiency
Muscle phosphofructokinase deficiency
Tauri disease
E74.1 Disorders of fructose metabolism
Excludes1 muscle phosphofructokinase deficiency (E74.09)
E74.10 Disorder of fructose metabolism, unspecified
E74.11 Essential fructosuria
Fructokinase deficiency
E74.12 Hereditary fructose intolerance
Fructosemia
E74.19 Other disorders of fructose metabolism
Fructose-1, 6-diphosphatase deficiency
E74.2 Disorders of galactose metabolism
E74.20 Disorders of galactose metabolism, unspecified
E74.21 Galactosemia
E74.29 Other disorders of galactose metabolism
Galactokinase deficiency
E74.3 Other disorders of intestinal carbohydrate absorption
Excludes2 lactose intolerance (E73.-)
E74.31 Sucrase-isomaltase deficiency
E74.39 Other disorders of intestinal carbohydrate absorption
Disorder of intestinal carbohydrate absorption NOS
Glucose-galactose malabsorption
Sucrase deficiency
E74.4 Disorders of pyruvate metabolism and gluconeogenesis
Deficiency of phosphoenolpyruvate carboxykinase
Deficiency of pyruvate carboxylase
Deficiency of pyruvate dehydrogenase
Excludes1 disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
Leigh's syndrome (G31.82)
E74.8 Other specified disorders of carbohydrate metabolism
E74.81 Disorders of glucose transport, not elsewhere classified
E74.810 Glucose 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
E74.818 Other disorders of glucose transport
(Familial) renal glycosuria
E74.819 Disorders of glucose transport, unspecified
E74.89 Other specified disorders of carbohydrate metabolism
Essential pentosuria
E74.9 Disorder of carbohydrate metabolism, unspecified
E75 Disorders of sphingolipid metabolism and other lipid storage disorders
Excludes1 mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)
E75.0 GM2 gangliosidosis
E75.00 GM2 gangliosidosis, unspecified
E75.01 Sandhoff disease
E75.02 Tay-Sachs disease
E75.09 Other GM2 gangliosidosis
Adult GM2 gangliosidosis
Juvenile GM2 gangliosidosis
E75.1 Other and unspecified gangliosidosis
E75.10 Unspecified gangliosidosis
Gangliosidosis NOS
E75.11 Mucolipidosis IV
E75.19 Other gangliosidosis
GM1 gangliosidosis
GM3 gangliosidosis
E75.2 Other sphingolipidosis
Excludes1 adrenoleukodystrophy [Addison-Schilder] (E71.528)
E75.21 Fabry (-Anderson) disease
E75.22 Gaucher disease
E75.23 Krabbe disease
E75.24 Niemann-Pick disease
Acid sphingomyelinase deficiency (ASMD)
E75.240 Niemann-Pick disease type A
Acid sphingomyelinase deficiency type A (ASMD type A)
Infantile neurovisceral acid sphingomyelinase deficiency
E75.241 Niemann-Pick disease type B
Acid sphingomyelinase deficiency type B (ASMD type B)
Chronic visceral acid sphingomyelinase deficiency
E75.242 Niemann-Pick disease type C
E75.243 Niemann-Pick disease type D
E75.244 Niemann-Pick disease type A/B
Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
Chronic neurovisceral acid sphingomyelinase deficiency
E75.248 Other Niemann-Pick disease
E75.249 Niemann-Pick disease, unspecified
Acid sphingomyelinase deficiency (ASMD) NOS
E75.25 Metachromatic leukodystrophy
E75.26 Sulfatase deficiency
Multiple sulfatase deficiency (MSD)
E75.27 Pelizaeus-Merzbacher disease
E75.28 Canavan disease
E75.29 Other sphingolipidosis
Farber's syndrome
Sulfatide lipidosis
E75.3 Sphingolipidosis, unspecified
E75.4 Neuronal ceroid lipofuscinosis
Batten disease
Bielschowsky-Jansky disease
Kufs disease
Spielmeyer-Vogt disease
E75.5 Other lipid storage disorders
Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
Wolman's disease
E75.6 Lipid storage disorder, unspecified
E76 Disorders of glycosaminoglycan metabolism
E76.0 Mucopolysaccharidosis, type I
E76.01 Hurler's syndrome
E76.02 Hurler-Scheie syndrome
E76.03 Scheie's syndrome
E76.1 Mucopolysaccharidosis, type II
Hunter's syndrome
E76.2 Other mucopolysaccharidoses
E76.21 Morquio mucopolysaccharidoses
E76.210 Morquio A mucopolysaccharidoses
Classic Morquio syndrome
Morquio syndrome A
Mucopolysaccharidosis, type IVA
E76.211 Morquio B mucopolysaccharidoses
Morquio-like mucopolysaccharidoses
Morquio-like syndrome
Morquio syndrome B
Mucopolysaccharidosis, type IVB
E76.219 Morquio mucopolysaccharidoses, unspecified
Morquio syndrome
Mucopolysaccharidosis, type IV
E76.22 Sanfilippo mucopolysaccharidoses
Mucopolysaccharidosis, type III (A) (B) (C) (D)
Sanfilippo A syndrome
Sanfilippo B syndrome
Sanfilippo C syndrome
Sanfilippo D syndrome
E76.29 Other mucopolysaccharidoses
beta-Glucuronidase deficiency
Maroteaux-Lamy (mild) (severe) syndrome
Mucopolysaccharidosis, types VI, VII
E76.3 Mucopolysaccharidosis, unspecified
E76.8 Other disorders of glucosaminoglycan metabolism
E76.9 Glucosaminoglycan metabolism disorder, unspecified
E77 Disorders of glycoprotein metabolism
E77.0 Defects in post-translational modification of lysosomal enzymes
Mucolipidosis II [I-cell disease]
Mucolipidosis III [pseudo-Hurler polydystrophy]
E77.1 Defects in glycoprotein degradation
Aspartylglucosaminuria
Fucosidosis
Mannosidosis
Sialidosis [mucolipidosis I]
E77.8 Other disorders of glycoprotein metabolism
E77.9 Disorder of glycoprotein metabolism, unspecified
E78 Disorders of lipoprotein metabolism and other lipidemias
Excludes1 sphingolipidosis (E75.0-E75.3)
E78.0 Pure hypercholesterolemia
E78.00 Pure hypercholesterolemia, unspecified
Fredrickson's hyperlipoproteinemia, type IIa
Hyperbetalipoproteinemia
Low-density-lipoprotein-type [LDL] hyperlipoproteinemia
(Pure) hypercholesterolemia NOS
E78.01 Familial hypercholesterolemia
E78.1 Pure hyperglyceridemia
Elevated fasting triglycerides
Endogenous hyperglyceridemia
Fredrickson's hyperlipoproteinemia, type IV
Hyperlipidemia, group B
Hyperprebetalipoproteinemia
Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinemia
E78.2 Mixed 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
Excludes1 cerebrotendinous cholesterosis [van Bogaert-Scherer- Epstein] (E75.5)
familial combined hyperlipidemia (E78.49)
E78.3 Hyperchylomicronemia
Chylomicron retention disease
Fredrickson's hyperlipoproteinemia, type I or V
Hyperlipidemia, group D
Mixed hyperglyceridemia
E78.4 Other hyperlipidemia
E78.41 Elevated Lipoprotein(a)
Elevated Lp(a)
E78.49 Other hyperlipidemia
Familial combined hyperlipidemia
E78.5 Hyperlipidemia, unspecified
E78.6 Lipoprotein deficiency
Abetalipoproteinemia
Depressed HDL cholesterol
High-density lipoprotein deficiency
Hypoalphalipoproteinemia
Hypobetalipoproteinemia (familial)
Lecithin cholesterol acyltransferase deficiency
Tangier disease
E78.7 Disorders of bile acid and cholesterol metabolism
Excludes1 Niemann-Pick disease type C (E75.242)
E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
E78.71 Barth syndrome
E78.72 Smith-Lemli-Opitz syndrome
E78.79 Other disorders of bile acid and cholesterol metabolism
E78.8 Other disorders of lipoprotein metabolism
E78.81 Lipoid dermatoarthritis
E78.89 Other lipoprotein metabolism disorders
E78.9 Disorder of lipoprotein metabolism, unspecified
E79 Disorders of purine and pyrimidine metabolism
Excludes1 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)
E79.0 Hyperuricemia without signs of inflammatory arthritis and tophaceous disease
Asymptomatic hyperuricemia
E79.1 Lesch-Nyhan syndrome
HGPRT deficiency
E79.2 Myoadenylate deaminase deficiency
E79.8 Other disorders of purine and pyrimidine metabolism
E79.81 Aicardi-Goutières syndrome
E79.82 Hereditary xanthinuria
E79.89 Other specified disorders of purine and pyrimidine metabolism
E79.9 Disorder of purine and pyrimidine metabolism, unspecified
E80 Disorders of porphyrin and bilirubin metabolism
Includes defects of catalase and peroxidase
E80.0 Hereditary erythropoietic porphyria
Congenital erythropoietic porphyria
Erythropoietic protoporphyria
E80.1 Porphyria cutanea tarda
E80.2 Other and unspecified porphyria
E80.20 Unspecified porphyria
Porphyria NOS
E80.21 Acute intermittent (hepatic) porphyria
E80.29 Other porphyria
Hereditary coproporphyria
E80.3 Defects of catalase and peroxidase
Acatalasia [Takahara]
E80.4 Gilbert syndrome
E80.5 Crigler-Najjar syndrome
E80.6 Other disorders of bilirubin metabolism
Dubin-Johnson syndrome
Rotor's syndrome
E80.7 Disorder of bilirubin metabolism, unspecified
E83 Disorders of mineral metabolism
Excludes1 dietary mineral deficiency (E58-E61)
parathyroid disorders (E20-E21)
vitamin D deficiency (E55.-)
E83.0 Disorders of copper metabolism
E83.00 Disorder of copper metabolism, unspecified
E83.01 Wilson's disease
CodeAlso associated Kayser Fleischer ring (H18.04-)
E83.09 Other disorders of copper metabolism
Menkes' (kinky hair) (steely hair) disease
E83.1 Disorders of iron metabolism
Excludes1 iron deficiency anemia (D50.-)
sideroblastic anemia (D64.0-D64.3)
E83.10 Disorder of iron metabolism, unspecified
E83.11 Hemochromatosis
Excludes1 GALD (P78.84)
Gestational alloimmune liver disease (P78.84)
Neonatal hemochromatosis (P78.84)
E83.110 Hereditary hemochromatosis
Bronzed diabetes
Pigmentary cirrhosis (of liver)
Primary (hereditary) hemochromatosis
E83.111 Hemochromatosis due to repeated red blood cell transfusions
Iron overload due to repeated red blood cell transfusions
Transfusion (red blood cell) associated hemochromatosis
E83.118 Other hemochromatosis
E83.119 Hemochromatosis, unspecified
E83.19 Other disorders of iron metabolism
UseAdditionalCode code, if applicable, for idiopathic pulmonary hemosiderosis (J84.03)
E83.2 Disorders of zinc metabolism
Acrodermatitis enteropathica
E83.3 Disorders of phosphorus metabolism and phosphatases
Excludes1 adult osteomalacia (M83.-)
osteoporosis (M80.-)
E83.30 Disorder of phosphorus metabolism, unspecified
E83.31 Familial hypophosphatemia
Vitamin D-resistant osteomalacia
Vitamin D-resistant rickets
Excludes1 vitamin D-deficiency rickets (E55.0)
E83.32 Hereditary vitamin D-dependent rickets (type 1) (type 2)
25-hydroxyvitamin D 1-alpha-hydroxylase deficiency
Pseudovitamin D deficiency
Vitamin D receptor defect
E83.39 Other disorders of phosphorus metabolism
Acid phosphatase deficiency
Hypophosphatasia
E83.4 Disorders of magnesium metabolism
E83.40 Disorders of magnesium metabolism, unspecified
E83.41 Hypermagnesemia
E83.42 Hypomagnesemia
E83.49 Other disorders of magnesium metabolism
E83.5 Disorders of calcium metabolism
Excludes1 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)
E83.50 Unspecified disorder of calcium metabolism
E83.51 Hypocalcemia
E83.52 Hypercalcemia
Familial hypocalciuric hypercalcemia
E83.59 Other disorders of calcium metabolism
E83.8 Other disorders of mineral metabolism
E83.81 Hungry bone syndrome
E83.89 Other disorders of mineral metabolism
E83.9 Disorder of mineral metabolism, unspecified
E84 Cystic fibrosis
Includes mucoviscidosis
CodeAlso exocrine pancreatic insufficiency (K86.81)
E84.0 Cystic fibrosis with pulmonary manifestations
UseAdditionalCode code to identify any infectious organism present, such as:
Pseudomonas (B96.5)
E84.1 Cystic fibrosis with intestinal manifestations
E84.11 Meconium ileus in cystic fibrosis
Excludes1 meconium ileus not due to cystic fibrosis (P76.0)
age Newborn: 0
E84.19 Cystic fibrosis with other intestinal manifestations
Distal intestinal obstruction syndrome
E84.8 Cystic fibrosis with other manifestations
E84.9 Cystic fibrosis, unspecified
E85 Amyloidosis
Excludes2 Alzheimer's disease (G30.0-)
E85.0 Non-neuropathic heredofamilial amyloidosis
Hereditary amyloid nephropathy
CodeAlso associated disorders, such as:
autoinflammatory syndromes (M04.-)
Excludes2 Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)
E85.1 Neuropathic heredofamilial amyloidosis
Amyloid polyneuropathy (Portuguese)
Transthyretin-related (ATTR) familial amyloid polyneuropathy
E85.2 Heredofamilial amyloidosis, unspecified
E85.3 Secondary systemic amyloidosis
Hemodialysis-associated amyloidosis
E85.4 Organ-limited amyloidosis
Localized amyloidosis
Transthyretin-related (ATTR) familial amyloid cardiomyopathy
E85.8 Other amyloidosis
E85.81 Light chain (AL) amyloidosis
E85.82 Wild-type transthyretin-related (ATTR) amyloidosis
Senile systemic amyloidosis (SSA)
E85.89 Other amyloidosis
E85.9 Amyloidosis, unspecified
E86 Volume depletion
UseAdditionalCode code(s) for any associated disorders of electrolyte and acid-base balance (E87.-)
Excludes1 dehydration of newborn (P74.1)
postprocedural hypovolemic shock (T81.19)
traumatic hypovolemic shock (T79.4)
Excludes2 hypovolemic shock NOS (R57.1)
E86.0 Dehydration
E86.1 Hypovolemia
Depletion of volume of plasma
E86.9 Volume depletion, unspecified
E87 Other disorders of fluid, electrolyte and acid-base balance
Excludes1 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)
E87.0 Hyperosmolality and hypernatremia
Sodium [Na] excess
Sodium [Na] overload
Excludes1 diabetes with hyperosmolarity (E08, E09, E11, E13 with final characters .00 or .01)
E87.1 Hypo-osmolality and hyponatremia
Sodium [Na] deficiency
Excludes1 syndrome of inappropriate secretion of antidiuretic hormone (E22.2)
E87.2 Acidosis
Excludes1 diabetic acidosis - see categories E08-E10, E11, E13 with ketoacidosis
E87.20 Acidosis, unspecified
Lactic acidosis NOS
Metabolic acidosis NOS
CodeAlso , if applicable, respiratory failure with hypercapnia (J96. with 5th character 2)
E87.21 Acute metabolic acidosis
Acute lactic acidosis
E87.22 Chronic metabolic acidosis
Chronic lactic acidosis
CodeFirst underlying etiology, if applicable
E87.29 Other acidosis
Respiratory acidosis NOS
Excludes2 acute respiratory acidosis (J96.02)
chronic respiratory acidosis (J96.12)
E87.3 Alkalosis
Alkalosis NOS
Metabolic alkalosis
Respiratory alkalosis
E87.4 Mixed disorder of acid-base balance
E87.5 Hyperkalemia
Potassium [K] excess
Potassium [K] overload
E87.6 Hypokalemia
Potassium [K] deficiency
E87.7 Fluid overload
Excludes1 edema NOS (R60.9)
fluid retention (R60.9)
E87.70 Fluid overload, unspecified
E87.71 Transfusion associated circulatory overload
Fluid overload due to transfusion (blood) (blood components)
TACO
E87.79 Other fluid overload
E87.8 Other disorders of electrolyte and fluid balance, not elsewhere classified
Electrolyte imbalance NOS
Hyperchloremia
Hypochloremia
E88 Other and unspecified metabolic disorders
UseAdditionalCode codes for associated conditions
Excludes1 histiocytosis X (chronic) (C96.6)
E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
Excludes1 monoclonal gammopathy (of undetermined significance) (D47.2)
polyclonal hypergammaglobulinemia (D89.0)
Waldenström macroglobulinemia (C88.0)
Excludes2 disorder of lipoprotein metabolism (E78.-)
E88.01 Alpha-1-antitrypsin deficiency
AAT deficiency
E88.02 Plasminogen deficiency
Dysplasminogenemia
Hypoplasminogenemia
Type 1 plasminogen deficiency
Type 2 plasminogen deficiency
CodeAlso , if applicable, ligneous conjunctivitis (H10.51)
UseAdditionalCode code for associated findings, such as:
hydrocephalus (G91.4)
otitis media (H67.-)
respiratory disorder related to plasminogen deficiency (J99)
E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
Bisalbuminemia
E88.1 Lipodystrophy, not elsewhere classified
Lipodystrophy NOS
Excludes1 Whipple's disease (K90.81)
E88.2 Lipomatosis, not elsewhere classified
Lipomatosis NOS
Lipomatosis (Check) dolorosa [Dercum]
E88.3 Tumor lysis syndrome
Tumor lysis syndrome (spontaneous)
Tumor lysis syndrome following antineoplastic drug chemotherapy
UseAdditionalCode code for adverse effect, if applicable, to identify drug (T45.1X5)
E88.4 Mitochondrial metabolism disorders
Excludes1 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)
E88.40 Mitochondrial metabolism disorder, unspecified
E88.41 MELAS syndrome
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
E88.42 MERRF syndrome
Myoclonic epilepsy associated with ragged-red fibers
CodeAlso progressive myoclonic epilepsy (G40.3-)
E88.43 Disorders of mitochondrial tRNA synthetases
E88.49 Other mitochondrial metabolism disorders
E88.8 Other specified metabolic disorders
E88.81 Metabolic syndrome and other insulin resistance
UseAdditionalCode codes for associated manifestations, such as:
obesity (E66.-)
E88.810 Metabolic syndrome
Dysmetabolic syndrome
E88.811 Insulin resistance syndrome, Type A
E88.818 Other insulin resistance
Insulin resistance syndrome, Type B
E88.819 Insulin resistance, unspecified
E88.89 Other specified metabolic disorders
Launois-Bensaude adenolipomatosis
Excludes1 adult pulmonary Langerhans cell histiocytosis (J84.82)
E88.9 Metabolic disorder, unspecified
E88.A Wasting disease (syndrome) due to underlying condition
Cachexia due to underlying condition
CodeFirst underlying condition
Excludes1 cachexia NOS (R64)
nutritional marasmus (E41)
Excludes2 failure to thrive (R62.51, R62.7)