2021 ICD-10-CM Diagnosis Code E71.510

Zellweger syndrome

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E71.510 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E71.510 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E71.510 - other international versions of ICD-10 E71.510 may differ.
The following code(s) above E71.510 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E71.510:
  • E00-E89
    2021 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E71.5
    ICD-10-CM Diagnosis Code E71.5

    Peroxisomal disorders

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • Schilder's disease (G37.0)
    Peroxisomal disorders
  • E71.51
    ICD-10-CM Diagnosis Code E71.51

    Disorders of peroxisome biogenesis

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Applicable To
    • Group 1 peroxisomal disorders
    Type 1 Excludes
    Disorders of peroxisome biogenesis
Clinical Information
  • A rare group of autosomal recessive inherited disorders characterized by the reduction or absence of peroxisomes in the tissues. Signs and symptoms include increased levels of iron and copper in the blood and tissues, hepatomegaly, facial abnormalities, mental retardation, seizures, and hypotonia.
  • An autosomal recessive disorder due to defects in peroxisome biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; muscle hypotonia; sensorineural hearing loss; visual compromise; seizures; progressive degeneration of the kidneys and the liver. Zellweger-like syndrome refers to phenotypes resembling the neonatal zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.
  • Autosomal recessive peroxisomal disorder, also known as zellweger syndrome, that typically presents in the neonatal period and is usually fatal; clinical features include hypotonia, dysmorphic skull and facial bones, visual compromise, multifocal seizures, hepatomegaly, biliary dysgenesis, and swallowing difficulties; pathologically, there are migration deficits of the neocortex and degeneration of white matter tracts; zellweger-like syndrome refers to conditions that phenotypically resemble neonatal zellweger syndrome, but occur in childhood or adulthood.
ICD-10-CM E71.510 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.510 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to E71.510:
  • Type 1 Excludes: Q87.8
    , E72.3
    ICD-10-CM Diagnosis Code Q87.8

    Other specified congenital malformation syndromes, not elsewhere classified

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    ICD-10-CM Diagnosis Code E72.3

    Disorders of lysine and hydroxylysine metabolism

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Glutaric aciduria NOS
    • Glutaric aciduria (type I)
    • Hydroxylysinemia
    • Hyperlysinemia
    Type 1 Excludes

Diagnosis Index entries containing back-references to E71.510:
  • Disorder (of) - see also Disease
    • peroxisomal E71.50
      ICD-10-CM Diagnosis Code E71.50

      Peroxisomal disorder, unspecified

        2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • Syndrome - see also Disease
    • Zellweger syndrome E71.510
  • Zellweger's syndrome E71.510

ICD-10-CM Codes Adjacent To E71.510
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
E71.43 Iatrogenic carnitine deficiency
E71.44 Other secondary carnitine deficiency
E71.440 Ruvalcaba-Myhre-Smith syndrome
E71.448 Other secondary carnitine deficiency
E71.5 Peroxisomal disorders
E71.50 Peroxisomal disorder, unspecified
E71.51 Disorders of peroxisome biogenesis
E71.510 Zellweger syndrome
E71.511 Neonatal adrenoleukodystrophy
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
E71.529 …… unspecified type
E71.53 Other group 2 peroxisomal disorders
E71.54 Other peroxisomal disorders

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.