2024 ICD-10-CM Diagnosis Code E25.0

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• 17 alpha-Hydroxyprogesterone aldolase deficiency
• 3 beta-Hydroxysteroid dehydrogenase deficiency
• 3-Beta-hydroxy-delta-5-C27-steroid dehydrogenase deficiency
• 46,XY disorder of sex development
• 46,XY disorder of sex development due to isolated 17,20-lyase deficiency
• Adrenal virilism
• Adrenal virilism
• Adrenal virilism
• Adrenal virilism
• Adrenal virilism
• Aldosterone deficiency
• CAH - desmolase deficiency
• Cholesterol monooxygenase deficiency
• Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency
• Congenital adrenal hyperplasia
• Congenital adrenal hyperplasia due to 21-hydroxylase deficiency non-classic form
• Congenital adrenal hyperplasia due to cytochrome P450 oxidoreductase deficiency
• Congenital adrenal hypoplasia, X-linked
• Congenital hypoplasia of adrenal gland
• Congenital lipoid adrenal hyperplasia due to STAR deficiency
• Congenital lipoid adrenal hyperplasia due to steroidogenic acute regulatory protein deficiency classic form
• Congenital lipoid adrenal hyperplasia due to steroidogenic acute regulatory protein deficiency non classic form
• Corticosterone 18-monooxygenase deficiency
• Deficiency of 3alpha-hydroxysteroid dehydrogenase
• Deficiency of 3beta-hydroxysteroid dehydrogenase
• Deficiency of steroid 11-beta-monooxygenase
• Deficiency of steroid 21-monooxygenase
• Disorder of cholesterol catabolism
• Disorder of cholesterol metabolism
• Female pseudohermaphroditism
• Female pseudohermaphroditism due to congenital adrenal hyperplasia
• Fetal endocrine disorder
• Fetal virilism
• Hyperandrogenism due to non-classic 21-hydroxylase deficiency
• Hypertension due to congenital adrenal hyperplasia
• Late onset congenital adrenal hyperplasia
• Male pseudohermaphroditism
• Male pseudohermaphroditism due to congenital adrenal hyperplasia
• Mild steroid 21-hydroxylase deficiency
• Moderate steroid 21-hydroxylase deficiency
• Pseudohermaphrodite, female with adrenocortical disorder
• Pseudohermaphroditism due to congenital adrenal hyperplasia
• Salt-losing congenital adrenal hyperplasia
• Salt-losing congenital adrenal hyperplasia with virilism
• Severe steroid 21-hydroxylase deficiency
• Steroid 21-monooxygenase deficiency, salt wasting type
• Steroid 21-monooxygenase deficiency, simple virilizing type
• Synthetic defect of bile acids
• Virilization-adrenogenital syndrome
• Virilizing syndrome of adrenal origin
• Virilizing syndrome of adrenal origin

E25.0 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
643	ENDOCRINE DISORDERS WITH MCC	10	1.6451
644	ENDOCRINE DISORDERS WITH CC	10	1.0617
645	ENDOCRINE DISORDERS WITHOUT CC/MCC	10	0.7609

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert E25.0 to ICD-9-CM

• ICD-9-CM Code: 255.2 - Adrenogenital disorders
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Adrenal Gland Disorders

What are adrenal glands?

Your adrenal glands are two small organs that sit on top of each kidney. The adrenal glands make different types of hormones you need to stay alive and healthy. Hormones are chemicals that travel in your bloodstream and control how different parts of your body work.

The adrenal glands make the hormones cortisol, aldosterone, adrenaline, and noradrenaline. They also make hormones that your body uses to make sex hormones (estrogen and testosterone). All of these hormones do many important jobs, including:

• Turning food into energy and managing blood sugar levels
• Balancing salt and water
• Keeping blood pressure normal
• Responding to illness and stress (your "fight or flight" response)
• Timing when and how fast a child develops sexually
• Supporting pregnancy

What are adrenal gland disorders?

When you have an adrenal gland disorder, your body makes too much or too little of one or more hormones. The symptoms depend on the type of problem you have and how much it affects the hormone levels in your body.

There are many types of adrenal gland disorders, including:

• Addison's Disease - a condition in which the adrenal glands don't make enough cortisol
• Cushing's Syndrome - a condition caused by too much cortisol in the body, often from taking steroid medicines for a long time
• Aldosterone-producing adenoma - a benign tumor (not cancer) that makes too much aldosterone and may cause serious high blood pressure
• Hereditary paraganglioma-pheochromocytoma - an inherited condition causing different types of tumors that make adrenaline and other hormones. Some tumors may become cancerous.
• Adrenal gland cancer - cancerous tumors, including adrenocortical carcinoma and neuroblastoma
• Congenital Adrenal Hyperplasia (CAH) - a group of inherited disorders in which the adrenal glands don't make enough cortisol. The most common type is 21-hydroxylase deficiency (also called CAH1). In the United States, newborn babies get a blood test to see if they have CAH. People born with CAH may not have symptoms until childhood or later in life.

What causes adrenal gland disorders?

The cause of adrenal gland disorders depends on the type of disorder you have. Causes can include:

• Medicines such as steroids
• A problem in another gland, such as the pituitary gland. The pituitary gland releases hormones that affect how the adrenal glands work.
• Changes in genes (mutations). These changes can cause the adrenal glands to make too much or too little of one or more hormones.
• Infections

In many cases the cause of the problem isn't clear.

How are adrenal gland disorders diagnosed?

Health care providers use different tests to check for adrenal disorders depending on your symptoms and health history. For example, you may have tests of your blood, urine (pee), or saliva (spit). These tests check your hormone levels. Your provider may order x-rays, CT scans, or MRI scans to look for tumors.

What are the treatments for adrenal gland disorders?

Different types of adrenal gland disorders have different treatments. They include medicines and surgery. Radiation therapy is sometimes a treatment for tumors. There are treatments to cure certain adrenal gland disorders. For other disorders, treatments can manage your symptoms.

NIH: National Institute of Child Health and Human Development

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21-hydroxylase deficiency

21-hydroxylase deficiency is an inherited disorder that affects the adrenal glands. The adrenal glands are located on top of the kidneys and produce a variety of hormones that regulate many essential functions in the body. In people with 21-hydroxylase deficiency, the adrenal glands produce excess androgens, which are male sex hormones.

There are three types of 21-hydroxylase deficiency. Two types are classic forms, known as the salt-wasting and simple virilizing types. The third type is called the non-classic type. The salt-wasting type is the most severe, the simple virilizing type is less severe, and the non-classic type is the least severe form.

Males and females with either classic form of 21-hydroxylase deficiency tend to have an early growth spurt, but their final adult height is usually shorter than others in their family. Additionally, affected individuals may have a reduced ability to have biological children (decreased fertility). Females may also develop excessive body hair growth (hirsutism), male pattern baldness, and irregular menstruation.

Approximately 75 percent of individuals with classic 21-hydroxylase deficiency have the salt-wasting type. Hormone production is extremely low in this form of the disorder. Affected individuals lose large amounts of sodium in their urine, which can be life-threatening in early infancy. Babies with the salt-wasting type can experience poor feeding, weight loss, dehydration, and vomiting. Individuals with the simple virilizing form do not experience salt loss.

In both the salt-wasting and simple virilizing forms of this disorder, females typically have external genitalia that do not look clearly male or female (ambiguous genitalia). Males usually have normal genitalia, but the testes may be small.

Females with the non-classic type of 21-hydroxylase deficiency have normal female genitalia. As affected females get older, they may experience hirsutism, male pattern baldness, irregular menstruation, and decreased fertility. Males with the non-classic type may have early beard growth and small testes. Some individuals with this type of 21-hydroxylase deficiency have no symptoms of the disorder.

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3-beta-hydroxysteroid dehydrogenase deficiency

3-beta (β)-hydroxysteroid dehydrogenase (HSD) deficiency is an inherited disorder that affects hormone-producing glands including the gonads (ovaries in females and testes in males) and the adrenal glands. The gonads direct sexual development before birth and during puberty. The adrenal glands, which are located on top of the kidneys, regulate the production of certain hormones and control salt levels in the body. People with 3β-HSD deficiency lack many of the hormones that are made in these glands. 3β-HSD deficiency is one of a group of disorders known as congenital adrenal hyperplasias that impair hormone production and disrupt sexual development and maturation.

There are three types of 3β-HSD deficiency: the salt-wasting, non-salt-wasting, and non-classic types. In the salt-wasting type, hormone production is extremely low. Individuals with this type lose large amounts of sodium in their urine, which can be life-threatening. Individuals affected with the salt-wasting type are usually diagnosed soon after birth due to complications related to a lack of salt reabsorption, including dehydration, poor feeding, and vomiting. People with the non-salt-wasting type of 3β-HSD deficiency produce enough hormone to allow sodium reabsorption in the kidneys. Individuals with the non-classic type have the mildest symptoms and do not experience salt wasting.

In males with any type of 3β-HSD deficiency, problems with male sex hormones lead to abnormalities of the external genitalia. These abnormalities range from having the opening of the urethra on the underside of the penis (hypospadias) to having external genitalia that do not look clearly male or female (ambiguous genitalia). The severity of the genital abnormality does not consistently depend on the type of the condition. Because of the hormone dysfunction in the testes, males with 3β-HSD deficiency are frequently unable to have biological children (infertile).

Females with 3β-HSD deficiency may have slight abnormalities of the external genitalia at birth. Females affected with the non-salt-wasting or non-classic types are typically not diagnosed until mid-childhood or puberty, when they may experience irregular menstruation, premature pubic hair growth, and excessive body hair growth (hirsutism). Females with 3β-HSD deficiency have difficulty conceiving a child (impaired fertility).

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Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

Congenital adrenal hyperplasia (CAH) due to 11-beta-hydroxylase deficiency is one of a group of disorders (collectively called congenital adrenal hyperplasia) that affect the adrenal glands. The adrenal glands are located on top of the kidneys and produce a variety of hormones that regulate many essential functions in the body. In people with CAH due to 11-beta-hydroxylase deficiency, the adrenal glands produce excess androgens, which are male sex hormones.

There are two types of CAH due to 11-beta-hydroxylase deficiency, the classic form and the non-classic form. The classic form is the more severe of the two types.

Females with the classic form of CAH due to 11-beta-hydroxylase deficiency have external genitalia that do not look clearly male or female (atypical genitalia). However, the internal reproductive organs develop normally. Males and females with the classic form of this condition have early development of their secondary sexual characteristics such as growth of facial and pubic hair, deepening of the voice, appearance of acne, and onset of a growth spurt. The early growth spurt can prevent growth later in adolescence and lead to short stature in adulthood. In addition, approximately two-thirds of individuals with the classic form of CAH due to 11-beta-hydroxylase deficiency have high blood pressure (hypertension). Hypertension typically develops within the first year of life.

Females with the non-classic form of CAH due to 11-beta-hydroxylase deficiency have normal female genitalia. As affected females get older, they may develop excessive body hair growth (hirsutism) and irregular menstruation. Males with the non-classic form of this condition do not typically have any signs or symptoms except for short stature. Hypertension is not a feature of the non-classic form of CAH due to 11-beta-hydroxylase deficiency.

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Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.