The adrenal insufficiency , also briefly adrenal insufficiency , is characterized by insufficient production of hormones underactive adrenal cortex (NNR), which is to be treated well.
Adrenal insufficiency is found in around 40 out of 100,000 people.
The adrenal gland is a hormonal gland that is attached to the upper pole of the kidneys . It consists of the adrenal medulla , which produces catecholamines , and the adrenal cortex, which synthesizes the steroid hormones cortisol ( glucocorticoids ) and aldosterone ( mineralocorticoids ) as well as sex hormones (androgens) and g-strophanthin . The production of cortisol is controlled by the hormone ACTH , which is produced in the anterior pituitary gland of the pituitary gland . This in turn is controlled by the corticotropin-releasing hormone (CRH), a hormone of the hypothalamus , a superordinate brain region. Aldosterone as an important hormone regulating the water and electrolyte balance is regulated within the framework of the renin-angiotensin-aldosterone system .
There are three types of hypofunction of the adrenal cortex depending on the origin.
Primary adrenal insufficiency
In the primary form (synonyms: Addison's disease and Addison's disease , named after the English doctor Thomas Addison , who first described the disease in its symptoms and as starting from the adrenal glands in 1855 , before Charles-Édouard Brown-Séquard between 1855 and 1870 the Function of the adrenal glands has investigated in more detail) - about 80 percent of all cases - the disorder (insufficient hormone production) lies in the adrenal gland itself
- the autoimmunological form (around 70%) in which antibodies against the corticosteroid-producing cells in the adrenal cortex are formed and destroy them
- the storage disease amyloidosis
- Functional impairment due to metastases (for example from a malignant melanoma )
- the Waterhouse-Friderichsen syndrome (hemorrhagic infarction of the adrenal region in the context of disseminated intravascular coagulation )
- Infectious diseases (e.g. tuberculosis , meningococcal infections , cytomegaly , AIDS)
Tuberculosis, which affects the adrenal glands, used to be the most common cause of Addison's disease. At that time the number of men predominated. Today autoimmune disease is the predominant cause in developed countries and women are more often affected than men.
Due to the low cortisol level in the blood, more ACTH is formed in the pituitary gland, which is supposed to stimulate the formation of cortisol. ACTH is produced by splitting proopiomelanocortin , whereby melanocyte-stimulating hormone is also produced, which together with ACTH stimulates the melanocytes in the skin to accumulate more pigment. The skin appears browner as a result, which is why Addison's disease is also known as "bronze disease".
Secondary adrenal insufficiency
The secondary form is caused by an underactive pituitary gland ( pituitary insufficiency ). The lack of ACTH means that the adrenal cortex is not sufficiently stimulated to produce cortisol. A “relative adrenal insufficiency” was previously suspected and called this hypadrenia and addisonism . Causes can be:
- Injuries ( traumatic brain injury , birth trauma )
- Circulatory disorders, special form: Sheehan syndrome
- Autoimmune reactions
Tertiary adrenal insufficiency
An underactive hypothalamus causes the tertiary form. Too little CRH is formed, which means that the pituitary gland is not sufficiently stimulated to produce ACTH and, as in the secondary form, there is a relative lack of ACTH. Hypothalamic disorders are rare. Longer-term, higher-dose treatment with corticosteroids is more common. This suppresses the formation of CRH in the long term; abrupt withdrawal can lead to an Addison's crisis.
Patients who have been taking cortisone preparations above the Cushing threshold dose for more than five days must be given additional cortisol during operations, as the body cannot generate the cortisol surge required during operations. A single dose of cortisol is sufficient for minor operations. In the case of major operations, additional cortisol should be administered continuously for the first 24 hours in order to return to the continuous dose before the operation afterwards.
The leading complaint is initially mainly stress-related weakness and weakness ( asthenia ). Hyperpigmentation, which is also very common (an increasing brown discoloration of areas exposed to light, as already described by Addison in addition to weakness, muscle fatigue and digestive disorders) of the skin can be completely absent, so normal pigmentation does not rule out the diagnosis.
|Anorexia , nausea, vomiting||90|
|Location-dependent low blood pressure (<110/70 mmHg)||87|
|Pigmentation of the mucous membranes||82|
|Cravings for salty foods ("hunger for salt")||22nd|
- Addison crisis
Due to special stress situations, such as B. operations and illnesses, especially a not yet treated adrenal insufficiency can suddenly worsen within a few hours. This potentially life-threatening condition, known as the Addison's crisis , is characterized by clouding of consciousness through to coma, drop in blood pressure, fever, massive dehydration of the organism, hypoglycaemia and abdominal discomfort ( pseudoperitonitis ). Prodromes are restlessness, anxiety and paleness.
If adrenal insufficiency is suspected, laboratory diagnostics are carried out first. With imaging methods (ultrasound, computer, magnetic resonance tomography) changes in the adrenal glands, the pituitary gland or the hypothalamus can be shown.
|shape||sodium||potassium||Cortisol||ACTH||Aldosterone||ACTH test||CRH test||CNR antibodies|
|primary||reduced||elevated||reduced||elevated||reduced||Cortisol doesn't rise||Cortisol does not
increase ACTH increases
|secondary||normal||normal||reduced||reduced||normal||Cortisol increases||Cortisol does not
rise ACTH does not rise
|tertiary||normal||normal||reduced||reduced||normal||Cortisol increases||Cortisol increases,
Treatment and healing prospects
Treatment for Addison's disease consists in supplying the body with insufficiently produced hormones in the form of
- Hydrocortisone to compensate for the cortisol deficiency and
- Fludrocortisone to replace the missing aldosterone.
If the body is particularly stressed, such as during operations, the dose of hydrocortisone must be temporarily increased. Any infections that are the cause of Addison's disease are treated with antibiotics, and tumors are operated on. An Addison's crisis requires intensive medical monitoring and treatment with infusions containing sugar and cortisol. With the exception of the tertiary form based on cortisol therapy, the disease cannot be cured, but it can be treated through lifelong administration of the missing hormones in the form of medication. It is important that affected patients have an emergency kit with hydrocortisone and an emergency passport with them so that they can react quickly in the event of a life-threatening Addison crisis.
Replacement therapy with dehydroepiandrosterone (DHEA), which is also reduced in primary and secondary adrenal insufficiency , has shown in studies a positive influence on mood, sexuality and health-related quality of life, but has not been clinically evaluated and is therefore not covered by health insurance companies.
In the now rare tuberculous form of primary adrenal insufficiency, the administration of corticosteroids alone leads to an exacerbation of the tuberculosis. The tuberculosis must first be cured, and only then can the hormone deficiency be permanently replaced.
Adrenal insufficiency in animals
Adrenal insufficiency or hypoadrenocorticism is occasionally seen in domestic dogs. In 95% of the cases it is an immune-mediated primary adrenal insufficiency (Addison's disease), occasionally it can also occur with overdose of mitotane or trilostane in the treatment of Cushing's syndrome . This leads to a deficiency of the hormones cortisol (glucocorticoid) and usually also aldosterone (mineralocorticoid) formed in the adrenal cortex. The disease often develops insidiously and often only manifests itself initially in a changing general condition and deterioration with increased stress. Fatigue, loss of appetite, weakness, decreased performance, weight loss and vomiting are the most common. Chronic diarrhea, increased drinking and urination, and abdominal pain can also occur. An Addison Crisis, on the other hand, is dramatic with profound weakness, dehydration, breakdown and sudden death.
Typical laboratory findings are hyponatremia and hyperkalemia (in about 80 to 90% of dogs), (prerenal) azotemia , non-regenerative anemia and lymphocytosis . The basal cortisol concentration is usually below 2 µg / dl and remains below this threshold in the ACTH stimulation test . Stimulation values of 2.1 to 5 µg / dl cortisol are considered a gray area. The acute treatment of a dog in Addison's crisis is primarily based on the intravenous administration of physiological saline solution and the administration of a glucocorticoid. For long-term treatment since 2016 in the EU Desoxycortonpivalat (trade name Zycortal allowed). It only has a mineralocorticoid effect and must be supplemented with prednisolone. Previously, dogs were usually treated with fludrocortisone with or without the addition of prednisolone, but this did not control the disease in some of the dogs.
- Adrenogenital Syndrome
- Conn syndrome
- Cushing's Syndrome
- Triple-A syndrome , a very rare hereditary disease with a. Addison's disease
- Pernicious anemia
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