Familial Hyperaldosteronism—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)

Familial hyperaldosteronism is a group of inherited conditions in which the adrenal glands, which are small glands located on top of each kidney, produce too much of the hormone aldosterone. Aldosterone helps control the amount of salt retained by the kidneys. Excess aldosterone causes the kidneys to retain more salt than normal, which in turn increases the body’s fluid levels and blood pressure. People with familial hyperaldosteronism may develop severe high blood pressure (hypertension), often early in life. Without treatment, hypertension increases the risk of strokes, heart attacks, and kidney failure.Familial hyperaldosteronism is categorized into three types, distinguished by their clinical features and genetic causes. In familial hyperaldosteronism type I, hypertension generally appears in childhood to early adulthood and can range from mild to severe. This type can be treated with steroid medications called glucocorticoids, so it is also known as glucocorticoid-remediable aldosteronism (GRA). In familial hyperaldosteronism type II, hypertension usually appears in early to middle adulthood and does not improve with glucocorticoid treatment. In most individuals with familial hyperaldosteronism type III, the adrenal glands are enlarged up to six times their normal size. These affected individuals have severe hypertension that starts in childhood. The hypertension is difficult to treat and often results in damage to organs such as the heart and kidneys. Rarely, individuals with type III have milder symptoms with treatable hypertension and no adrenal gland enlargement.There are other forms of hyperaldosteronism that are not familial. These conditions are caused by various problems in the adrenal glands or kidneys. In some cases, a cause for the increase in aldosterone levels cannot be found.