Bilateral adrenal hyperplasia (BAH) and congenital adrenal hyperplasia (CAH) sound similar enough. While CAH is a specific type of BAH, there are other key differences that set them apart. Each condition causes specific sets of symptoms and develops in different age groups.

This article will explore how BAH and CAH differ from one another. Along with the causes and symptoms, we’ll also discuss how doctors treat these conditions. To learn more about BAH and CAH, talk to your endocrinologist (hormone specialist).

What Is Adrenal Hyperplasia?

Your adrenal glands are small, triangle-shaped glands that sit on top of your kidneys. Each kidney has one adrenal gland. They belong to your endocrine system, which helps control your hormone levels. According to Cleveland Clinic, the adrenal glands play an important role in controlling:

• Development of sexual organs and characteristics, like genitalia, ovaries, and testes
• Metabolism or energy production
• Blood pressure levels
• Reactions to stress

Your adrenal glands are responsible for making key hormones. These include:

• Androgens — Sex hormones that affect your normal development
• Cortisol — Your body’s “stress hormone” that controls blood pressure, blood sugar, and energy levels
• Aldosterone — A hormone that regulates your blood pressure and salt levels

The term “hyperplasia” means that a tissue grows larger than it normally would. The cells in the adrenal glands divide more, making the glands bigger. Enlarged adrenal glands throw off your hormone balance, leading to medical conditions like Cushing syndrome.

What Are BAH and CAH?

Most cases of adrenal hyperplasia are bilateral, meaning both glands on top of each kidney are affected.

The majority of research focuses on two forms of BAH, depending on how they affect the adrenal glands. Macronodular BAH creates large growths or nodules on the adrenal glands. On the other hand, the micronodular form is responsible for making smaller nodules.

There are several types of BAH, including:

• Primary bilateral macronodular adrenal hyperplasia (PBMAH)
• Isolated micronodular adrenal hyperplasia (iMAD)
• Primary pigmented micronodular adrenal hyperplasia (PPNAD)

CAH describes specific types of BAH caused by several different genetic disorders. People with CAH have certain gene mutations (changes) that affect their ability to make hormones. The most common types of CAH are classic and nonclassic.

What Causes BAH and CAH?

Both BAH and CAH develop from genetic mutations. The exact mutations affect which condition you develop.

How Does BAH Develop?

BAH can develop on its own or as part of a genetic syndrome. People with certain mutations are more likely to have BAH.

The most common mutation associated with BAH affects a protein called PKA. This enzyme is important for controlling how quickly cells grow and divide to make copies of themselves. The PRKAR1A gene provides instructions for making a specific piece of the PKA enzyme. Certain mutations tell cells to destroy this piece before it can be used. Without enough normal PKA proteins, adrenal gland cells grow out of control.

Up to 60 percent of adrenal hyperplasias are caused by mutations in the instructions for PKA proteins. Studies show that PKA mutations lead to a condition called Carney complex. Tumors or growths on the adrenal glands are one sign of Carney complex. This condition can lead to a type of BAH known as PPNAD.

PBMAH and iMAD are less common types of BAH. Some cases develop from different mutations that affect PKA proteins. Others come from mutations in different genes that control when cells grow and divide.

How Does CAH Develop?

CAH develops from mutations in the instructions for making the enzyme 21-hydroxylase. Researchers refer to CAH as an autosomal recessive disorder. This means you need to inherit two faulty genes — or one from each of your parents.

A 21-hydroxylase deficiency stops your body from making enough cortisol. When you don’t have enough cortisol, your adrenal glands try to compensate by making more androgens. This creates a hormone imbalance that leads to CAH.

There are two main types of CAH — classic and nonclassic. Within classic CAH, you can have salt-wasting (more severe) or non-salt-wasting forms.

How Do BAH and CAH Symptoms Differ?

While BAH and CAH both have genetic causes, they create different sets of symptoms. People with BAH usually develop Cushing syndrome. On the other hand, CAH causes abnormal sexual development due to high androgen levels. Other, more severe symptoms occur in those with salt-wasting CAH.

BAH Symptoms

Your specific BAH symptoms depend on what type of disease you have. Most people develop Cushing syndrome, a condition caused by too much cortisol. You may hear your doctor call this “hypercortisolism.” Symptoms of Cushing syndrome to look out for include:

• Hypertension (high blood pressure)
• Having a round “moon face”
• Abnormally fast weight gain in the chest, belly area, and back of the neck
• Diabetes
• Excess hair on your chest, neck, breasts, face, and thighs
• Easy bruising and wounds that heal slower than normal

BAH symptoms tend to develop later in life compared to CAH. For example, research shows that the median age to develop PPNAD is 25. This means that at least half of people with PPNAD are diagnosed when they’re 25 years old. Most people develop PPNAD in their 20s and 30s — but some children develop it by the time they’re 3 years old. PBMAH takes years to develop. Most people diagnosed with PBMAH are between 40 and 60 years old.

CAH Symptoms

CAH is a congenital condition, meaning it starts at birth. Infants born with CAH can have symptoms from the beginning of their lives onward. On the other hand, BAH usually develops in adults.

Depending on the type of CAH you have, you’ll experience different symptoms. People with classic CAH have abnormally high androgen levels. This affects sexual development and can lead to:

• Changes in genitalia in infants, such as an enlarged penis in males
• Signs of early puberty in younger children, like acne and hair growth
• Infertility or difficulty having children
• Abnormal menstrual cycles (periods) in females

Classic CAH is typically diagnosed at birth. Hospitals run newborn screenings for certain genetic conditions, including CAH. If you know CAH runs in your family, you may also choose to have early genetic testing.

Nonclassic CAH is milder than classic CAH. Because the symptoms aren’t always obvious, you may be diagnosed later in life. Signs of the nonclassic form include:

• Early puberty, including acne and growth spurts
• Abnormal periods
• Male-pattern baldness
• Too much body or facial hair in females

Salt-wasting classic CAH is a severe form that develops from extremely low aldosterone levels. Without enough of this hormone, you may experience:

• Low blood pressure and blood sugar levels
• Low sodium levels
• Irregular heart rhythms
• Dehydration or loss of too much water
• Weight loss
• Vomiting and diarrhea

How Do Doctors Treat BAH and CAH?

Your treatment options differ depending on whether you have BAH or CAH. Studies show that most BAH cases can be treated with surgery. Your endocrinologist will likely recommend removing one or both of your adrenal glands. Surgery also helps treat Cushing syndrome.

CAH treatments focus on managing hormone imbalances. If you have salt-wasting syndrome, you may take salt supplements to bring your sodium levels back up to normal. Other medications replace hormones you can’t make on your own. Mineralocorticoids treat low aldosterone levels, and glucocorticoids (steroids) replace cortisol.

Examples of medications for CAH include:

• Prednisone
• Dexamethasone
• Hydrocortisone
• Fludrocortisone

Find Your Team

On CAHteam, the social network for people with congenital adrenal hyperplasia and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with CAH.

Are you or a loved one living with BAH or CAH? Do you have other questions about the differences? Share them in the comments below.