ICD-10 Diagnosis Code E25.0

Congenital adrenogenital disorders assoc w enzyme deficiency

Diagnosis Code E25.0

ICD-10: E25.0
Short Description: Congenital adrenogenital disorders assoc w enzyme deficiency
Long Description: Congenital adrenogenital disorders associated with enzyme deficiency
This is the 2018 version of the ICD-10-CM diagnosis code E25.0

Valid for Submission
The code E25.0 is valid for submission for HIPAA-covered transactions.

Code Classification
  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Disorders of other endocrine glands (E20-E35)
      • Adrenogenital disorders (E25)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code E25.0 is grouped in the following Diagnostic Related Group(s) (MS-DRG V34.0)

  • 643 - ENDOCRINE DISORDERS WITH MCC
  • 644 - ENDOCRINE DISORDERS WITH CC
  • 645 - ENDOCRINE DISORDERS WITHOUT CC/MCC

Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

Synonyms
  • 17 alpha-Hydroxyprogesterone aldolase deficiency
  • 21-hydroxylase deficiency
  • 3 beta-Hydroxysteroid dehydrogenase deficiency
  • 3-Beta-hydroxy-delta-5-C27-steroid dehydrogenase deficiency
  • Adrenal virilism
  • Adrenal virilism
  • Aldosterone deficiency
  • Cholesterol monooxygenase
  • Congenital adrenal hyperplasia
  • Congenital adrenal hyperplasia - desmolase deficiency
  • Congenital adrenal hypoplasia, X-linked
  • Congenital hypoplasia of adrenal gland
  • 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
  • Disorder of lipid metabolism
  • Fetal endocrine disorder
  • Fetal virilism
  • Hyperandrogenism due to non-classic type of 21-hydroxylase deficiency
  • Late onset congenital adrenal hyperplasia
  • Mild steroid 21-hydroxylase deficiency
  • Moderate steroid 21-hydroxylase deficiency
  • Perinatal endocrine and metabolic disorders
  • Pseudohermaphrodite, female with adrenocortical disorder
  • 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

Index of Diseases and Injuries
References found for the code E25.0 in the Index of Diseases and Injuries:


Information for Patients


Adrenal Gland Disorders

The adrenal glands are small glands located on top of each kidney. They produce hormones that you can't live without, including sex hormones and cortisol. Cortisol helps you respond to stress and has many other important functions.

With adrenal gland disorders, your glands make too much or not enough hormones. In Cushing's syndrome, there's too much cortisol, while with Addison's disease, there is too little. Some people are born unable to make enough cortisol.

Causes of adrenal gland disorders include

  • Genetic mutations
  • Tumors including pheochromocytomas
  • Infections
  • A problem in another gland, such as the pituitary, which helps to regulate the adrenal gland
  • Certain medicines

Treatment depends on which problem you have. Surgery or medicines can treat many adrenal gland disorders.

NIH: National Institute of Child Health and Human Development

  • 17-hydroxycorticosteroids (Medical Encyclopedia)
  • 17-OH progesterone (Medical Encyclopedia)
  • 24-hour urinary aldosterone excretion rate (Medical Encyclopedia)
  • ACTH (cosyntropin) stimulation test (Medical Encyclopedia)
  • ACTH blood test (Medical Encyclopedia)
  • Acute adrenal crisis (Medical Encyclopedia)
  • Adrenal glands (Medical Encyclopedia)
  • Adrenalectomy (Medical Encyclopedia)
  • Aldosterone blood test (Medical Encyclopedia)
  • Congenital adrenal hyperplasia (Medical Encyclopedia)
  • Hyperaldosteronism - primary and secondary (Medical Encyclopedia)


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Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

A birth defect may affect how the body looks, works or both. Some birth defects like cleft lip or neural tube defects are structural problems that can be easy to see. To find others, like heart defects, doctors use special tests. Birth defects can vary from mild to severe. Some result from exposures to medicines or chemicals. For example, alcohol abuse can cause fetal alcohol syndrome. Infections during pregnancy can also result in birth defects. For most birth defects, the cause is unknown.

Some birth defects can be prevented. Taking folic acid can help prevent some birth defects. Talk to your doctor about any medicines you take. Some medicines can cause serious birth defects.

Babies with birth defects may need surgery or other medical treatments. Today, doctors can diagnose many birth defects in the womb. This enables them to treat or even correct some problems before the baby is born.

Centers for Disease Control and Prevention

  • Intersex (Medical Encyclopedia)


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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 (ambiguous 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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