1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E20-E35 Disorders of other endocrine glands
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4. E25- Adrenogenital disorders
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2018 ICD-10-CM Diagnosis Code E25.0

Congenital adrenogenital disorders associated with enzyme deficiency

2016 2017 2018 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 2018 edition of ICD-10-CM E25.0 became effective on October 1, 2017.
• This is the American ICD-10-CM version of E25.0 - other international versions of ICD-10 E25.0 may differ.

• 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,

• 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.

• 643 Endocrine disorders with mcc
• 644 Endocrine disorders with cc
• 645 Endocrine disorders without cc/mcc

Convert E25.0 to ICD-9-CM

• 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

• Type 1 Excludes: E24
  , E30.1
  , Q89.1
  ICD-10-CM Diagnosis Code E24

  Cushing's syndrome

  2016 2017 2018 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 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.-)
  ICD-10-CM Diagnosis Code Q89.1

  Congenital malformations of adrenal gland

  2016 2017 2018 Billable/Specific Code POA Exempt

  Type 1 Excludes

  • adrenogenital disorders (E25.-)
  • congenital adrenal hyperplasia (E25.0)

• Adrenogenital syndrome E25.9
  ICD-10-CM Diagnosis Code E25.9

  Adrenogenital disorder, unspecified

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

    Female pseudohermaphroditism, not elsewhere classified

    2016 2017 2018 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 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 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 Billable/Specific Code

  Applicable To

  • Adrenogenital syndrome NOS
  • congenital E25.0

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