2019 ICD-10-CM Diagnosis Code E25.0

Congenital adrenogenital disorders associated with enzyme deficiency

    2016 2017 2018 2019 Billable/Specific Code
  • E25.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • Short description: Congenital adrenogenital disorders assoc w enzyme deficiency
  • The 2019 edition of ICD-10-CM E25.0 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of E25.0 - other international versions of ICD-10 E25.0 may differ.
Applicable To
  • Congenital adrenal hyperplasia
  • 21-Hydroxylase deficiency
  • Salt-losing congenital adrenal hyperplasia
The following code(s) above E25.0 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 E25.0:
  • E00-E89
    2019 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
  • E20-E35
    2019 ICD-10-CM Range E20-E35

    Disorders of other endocrine glands

    Type 1 Excludes
    Disorders of other endocrine glands
  • E25
    ICD-10-CM Diagnosis Code E25

    Adrenogenital disorders

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Includes
    • adrenogenital syndromes, virilizing or feminizing, whether acquired or due to adrenal hyperplasia consequent on inborn enzyme defects in hormone synthesis
    • Female adrenal pseudohermaphroditism
    • Female heterosexual precocious pseudopuberty
    • Male isosexual precocious pseudopuberty
    • Male macrogenitosomia praecox
    • Male sexual precocity with adrenal hyperplasia
    • Male virilization (female)
    Type 1 Excludes
    • indeterminate sex and pseudohermaphroditism (Q56)
    • chromosomal abnormalities (Q90-Q99)
    Adrenogenital disorders
Approximate Synonyms
  • 3 beta-hydroxysteroid dehydrogenase deficiency
  • Adrenal hyperplasia, congenital
  • Adrenal hyperplasia, congenital, simple virilizing
  • Adrenal hyperplasia, congenital, virilizing, non salt
  • Congenital adrenal hyperplasia
  • Congenital adrenal hyperplasia, undervirilized xy due to 17-alpha hydroxylase deficiency
  • Congenital adrenal hyperplasia, virilizing, non-salt-losing
  • Congenital adrenal hyperplasia, virilizing, salt-losing
  • Late onset congenital adrenal hyperplasia
  • Steroid 21-monooxygenase deficiency, simple virilizing type
  • Undervirilized xy, 3-beta hydroxysteroid deficiency
  • Undervirilized xy, congenital adrenal hyperplasis,
Clinical Information
  • A genetic disorder characterized by defects in the synthesis of cortisol and/or aldosterone, resulting in hyperplasia of the adrenal cortical cells.
  • A group of inherited disorders of the adrenal glands, caused by enzyme defects in the synthesis of cortisol (hydrocortisone) and/or aldosterone leading to accumulation of precursors for androgens. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in steroid 21-hydroxylase; steroid 11-beta-hydroxylase; steroid 17-alpha-hydroxylase; 3-beta-hydroxysteroid dehydrogenase (3-hydroxysteroid dehydrogenases); testosterone 5-alpha-reductase; or steroidogenic acute regulatory protein; among others, underlie these disorders.
  • Group of syndromes caused by inherited defects in cortisol and/or aldosterone biosynthesis; acth elevation is caused by low levels of cortisol, whose production is impaired by absence or deficiency of one of the five enzymes necessary for its synthesis from cholesterol.
ICD-10-CM E25.0 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 643 Endocrine disorders with mcc
  • 644 Endocrine disorders with cc
  • 645 Endocrine disorders without cc/mcc

Convert E25.0 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
Code annotations containing back-references to E25.0:
  • Type 1 Excludes: Q89.1
    , E24
    , E30.1
    ICD-10-CM Diagnosis Code Q89.1

    Congenital malformations of adrenal gland

      2016 2017 2018 2019 Billable/Specific Code POA Exempt
    Type 1 Excludes
    • adrenogenital disorders (E25.-)
    • congenital adrenal hyperplasia (E25.0)
    ICD-10-CM Diagnosis Code E24

    Cushing's syndrome

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • congenital adrenal hyperplasia (E25.0)
    ICD-10-CM Diagnosis Code E30.1

    Precocious puberty

      2016 2017 2018 2019 Billable/Specific Code Pediatric Dx (0-17 years)
    Applicable To
    • Precocious menstruation
    Type 1 Excludes
    • Albright (-McCune) (-Sternberg) syndrome (Q78.1)
    • central precocious puberty (E22.8)
    • congenital adrenal hyperplasia (E25.0)
    • female heterosexual precocious pseudopuberty (E25.-)
    • male isosexual precocious pseudopuberty (E25.-)

Diagnosis Index entries containing back-references to E25.0:
  • Adrenogenital syndrome E25.9
    ICD-10-CM Diagnosis Code E25.9

    Adrenogenital disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Adrenogenital syndrome NOS
    • congenital E25.0
    • salt loss E25.0
  • Adrenogenitalism, congenital E25.0
  • Defect, defective Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • 3-beta-hydroxysteroid dehydrogenase E25.0
    • 11-hydroxylase E25.0
    • 21-hydroxylase E25.0
  • Deficiency, deficient
    • 3-beta hydroxysteroid dehydrogenase E25.0
    • 11-hydroxylase E25.0
    • 21-hydroxylase E25.0
  • Hyperadrenocorticism E24.9
    ICD-10-CM Diagnosis Code E24.9

    Cushing's syndrome, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    • congenital E25.0
  • Hyperfunction
    • adrenal cortex, not associated with Cushing's syndrome E27.0
      ICD-10-CM Diagnosis Code E27.0

      Other adrenocortical overactivity

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Overproduction of ACTH, not associated with Cushing's disease
      • Premature adrenarche
      Type 1 Excludes
      • Cushing's syndrome (E24.-)
      • virilism E25.9
        ICD-10-CM Diagnosis Code E25.9

        Adrenogenital disorder, unspecified

          2016 2017 2018 2019 Billable/Specific Code
        Applicable To
        • Adrenogenital syndrome NOS
        • congenital E25.0
  • Hyperplasia, hyperplastic
    • adrenal (capsule) (cortex) (gland) E27.8
      ICD-10-CM Diagnosis Code E27.8

      Other specified disorders of adrenal gland

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Abnormality of cortisol-binding globulin
      • with
        • sexual precocity (male) E25.9
          ICD-10-CM Diagnosis Code E25.9

          Adrenogenital disorder, unspecified

            2016 2017 2018 2019 Billable/Specific Code
          Applicable To
          • Adrenogenital syndrome NOS
          • congenital E25.0
        • virilism, adrenal E25.9
          ICD-10-CM Diagnosis Code E25.9

          Adrenogenital disorder, unspecified

            2016 2017 2018 2019 Billable/Specific Code
          Applicable To
          • Adrenogenital syndrome NOS
          • congenital E25.0
        • virilization (female) E25.9
          ICD-10-CM Diagnosis Code E25.9

          Adrenogenital disorder, unspecified

            2016 2017 2018 2019 Billable/Specific Code
          Applicable To
          • Adrenogenital syndrome NOS
          • congenital E25.0
      • congenital E25.0
        • salt-losing E25.0
  • Macrogenitosomia (adrenal) (male) (praecox) E25.9
    ICD-10-CM Diagnosis Code E25.9

    Adrenogenital disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Adrenogenital syndrome NOS
    • congenital E25.0
  • Masculinization (female) E25.9
    ICD-10-CM Diagnosis Code E25.9

    Adrenogenital disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Adrenogenital syndrome NOS
    • congenital E25.0
  • Precocity, sexual (constitutional) (cryptogenic) (female) (idiopathic) (male) E30.1
    ICD-10-CM Diagnosis Code E30.1

    Precocious puberty

      2016 2017 2018 2019 Billable/Specific Code Pediatric Dx (0-17 years)
    Applicable To
    • Precocious menstruation
    Type 1 Excludes
    • Albright (-McCune) (-Sternberg) syndrome (Q78.1)
    • central precocious puberty (E22.8)
    • congenital adrenal hyperplasia (E25.0)
    • female heterosexual precocious pseudopuberty (E25.-)
    • male isosexual precocious pseudopuberty (E25.-)
    • with adrenal hyperplasia E25.9
      ICD-10-CM Diagnosis Code E25.9

      Adrenogenital disorder, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Adrenogenital syndrome NOS
      • congenital E25.0
  • Pseudohermaphroditism Q56.3
    ICD-10-CM Diagnosis Code Q56.3

    Pseudohermaphroditism, unspecified

      2016 2017 2018 2019 Billable/Specific Code POA Exempt
    • female Q56.2
      ICD-10-CM Diagnosis Code Q56.2

      Female pseudohermaphroditism, not elsewhere classified

        2016 2017 2018 2019 Billable/Specific Code Female Dx POA Exempt
      Applicable To
      • Female pseudohermaphroditism NOS
      • adrenal E25.0 (congenital)
  • Syndrome - see also Disease
    • adrenogenital E25.9
      ICD-10-CM Diagnosis Code E25.9

      Adrenogenital disorder, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Adrenogenital syndrome NOS
      • congenital, associated with enzyme deficiency E25.0
  • Virilism (adrenal) E25.9
    ICD-10-CM Diagnosis Code E25.9

    Adrenogenital disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Adrenogenital syndrome NOS
    • congenital E25.0
  • Virilization (female) (suprarenal) E25.9
    ICD-10-CM Diagnosis Code E25.9

    Adrenogenital disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Adrenogenital syndrome NOS
    • congenital E25.0

ICD-10-CM Codes Adjacent To E25.0
E23.7 Disorder of pituitary gland, unspecified
E24 Cushing's syndrome
E24.0 Pituitary-dependent Cushing's disease
E24.1 Nelson's syndrome
E24.2 Drug-induced Cushing's syndrome
E24.3 Ectopic ACTH syndrome
E24.4 Alcohol-induced pseudo-Cushing's syndrome
E24.8 Other Cushing's syndrome
E24.9 Cushing's syndrome, unspecified
E25 Adrenogenital disorders
E25.0 Congenital adrenogenital disorders associated with enzyme deficiency
E25.8 Other adrenogenital disorders
E25.9 Adrenogenital disorder, unspecified
E26 Hyperaldosteronism
E26.0 Primary hyperaldosteronism
E26.01 Conn's syndrome
E26.02 Glucocorticoid-remediable aldosteronism
E26.09 Other primary hyperaldosteronism
E26.1 Secondary hyperaldosteronism
E26.8 Other hyperaldosteronism
E26.81 Bartter's syndrome

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