2020 ICD-10-CM Diagnosis Code E74.01

von Gierke disease

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E74.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E74.01 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E74.01 - other international versions of ICD-10 E74.01 may differ.
Applicable To
  • Type I glycogen storage disease
The following code(s) above E74.01 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 E74.01:
  • E00-E89
    2020 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
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E74
    ICD-10-CM Diagnosis Code E74

    Other disorders of carbohydrate metabolism

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Other disorders of carbohydrate metabolism
Approximate Synonyms
  • Deficiency of glucose-6-phosphatase
  • Deficiency of glucose-6-phosphate dehydrogenase
  • Glucose 6 phosphatase deficiency
  • Glycogen storage disease, type 1
  • Glycogen storage disease, type i
Clinical Information
  • An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
  • An autosomal recessive inherited type of glycogen storage disease. It is characterized by a deficiency of the enzyme glucose-6-phosphatase, resulting in the inability of the liver to produce free glucose causing severe hypoglycemia. There is abnormal accumulation of glycogen in the liver and kidneys.
  • Autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production; accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly; increased concentrations of lactic acid and hyperlipidemia appear in the plasma; clinical gout often appears in early childhood.
ICD-10-CM E74.01 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E74.01 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

Diagnosis Index entries containing back-references to E74.01:
  • Deficiency, deficient
    • glucose-6-phosphatase E74.01
  • Disease, diseased - see also Syndrome
    • glycogen storage E74.00
      ICD-10-CM Diagnosis Code E74.00

      Glycogen storage disease, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • glucose-6-phosphatase deficiency E74.01
      • type I E74.01
      • Von Gierke's E74.01
    • Gierke's E74.01 (glycogenosis I)
    • van Creveld-von Gierke E74.01 (glycogenosis I)
    • von Gierke's E74.01 (glycogenosis I)
  • Gierke's disease E74.01 (glycogenosis I)
  • Van Creveld-von Gierke disease E74.01
  • Von Gierke's disease E74.01

ICD-10-CM Codes Adjacent To E74.01
E72.89 Other specified disorders of amino-acid metabolism
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
E74.0 Glycogen storage disease
E74.00 …… unspecified
E74.01 von Gierke disease
E74.02 Pompe disease
E74.03 Cori disease
E74.04 McArdle disease
E74.09 Other glycogen storage disease
E74.1 Disorders of fructose metabolism
E74.10 Disorder of fructose metabolism, unspecified
E74.11 Essential fructosuria
E74.12 Hereditary fructose intolerance
E74.19 Other disorders of fructose metabolism
E74.2 Disorders of galactose metabolism

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