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ICD-10-CM-2017 Diagnosis Codes Overview
ICD-10-CM-2017 > E00-E89 > E70-E88 > E71
E70-E88
Metabolic disorders
Excl1.:
androgen insensitivity syndrome (icd E34.5 Androgen insensitivity syndromeE34.5-)
congenital adrenal hyperplasia (icd E25.0 Congenital adrenogenital disorders associated with enzyme deficiencyE25.0)
Ehlers-Danlos syndrome (icd Q79.6 Ehlers-Danlos syndromeQ79.6)
hemolytic anemias attributable to enzyme disorders (icd D55 Anemia due to enzyme disordersD55.-)
Marfan's syndrome (icd Q87.4 Marfan's syndromeQ87.4)
5-alpha-reductase deficiency (icd E29.1 Testicular hypofunctionE29.1)
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
Info:
Hyperleucine-isoleucinemia
Hypervalinemia
E71.2
Disorder of branched-chain amino-acid metabolism, unspecified
E71.3

Disorders of fatty-acid metabolism

Excl1.:
peroxisomal disorders (icd E71.5 Peroxisomal disordersE71.5)
Refsum's disease (icd G60.1 Refsum's diseaseG60.1)
Schilder's disease (icd G37.0 Diffuse sclerosis of central nervous systemG37.0)
Excl2.:
carnitine deficiency due to inborn error of metabolism (icd E71.42 Carnitine deficiency due to inborn errors of metabolismE71.42)
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
Info:
LCAD
VLCAD
E71.311
Medium chain acyl CoA dehydrogenase deficiency
Info:
MCAD
E71.312
Short chain acyl CoA dehydrogenase deficiency
Info:
SCAD
E71.313
Glutaric aciduria type II
Info:
Glutaric aciduria type II A
Glutaric aciduria type II B
Glutaric aciduria type II C
Excl1.:
glutaric aciduria (type 1) NOS (icd E72.3 Disorders of lysine and hydroxylysine metabolismE72.3)
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

Excl1.:
Muscle carnitine palmitoyltransferase deficiency (icd E71.314 Muscle carnitine palmitoyltransferase deficiencyE71.314)
E71.40
Disorder of carnitine metabolism, unspecified
E71.41
Primary carnitine deficiency
E71.42
Carnitine deficiency due to inborn errors of metabolism
Use add'l
associated inborn error or metabolism
E71.43
Iatrogenic carnitine deficiency
Info:
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

Excl1.:
Schilder's disease (icd G37.0 Diffuse sclerosis of central nervous systemG37.0)
E71.50
Peroxisomal disorder, unspecified
E71.51
Disorders of peroxisome biogenesis
Info:
Group 1 peroxisomal disorders
Excl1.:
Refsum's disease (icd G60.1 Refsum's diseaseG60.1)
E71.510
Zellweger syndrome
E71.511
Neonatal adrenoleukodystrophy
Excl1.:
X-linked adrenoleukodystrophy (icd E71.42 Carnitine deficiency due to inborn errors of metabolismE71.42-)
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
Info:
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
Excl1.:
chondrodysplasia punctata NOS (icd Q77.3 Chondrodysplasia punctataQ77.3)
E71.541
Zellweger-like syndrome
E71.542
Other group 3 peroxisomal disorders
E71.548
Other peroxisomal disorders


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