Definition
Addison's disease, also known as chronic adrenal insufficiency, is a pathological condition that occurs as a result of a malfunction of the adrenal glands; Put simply, Addison's disease occurs when the adrenal glands lose the ability to synthesize corticosteroids.
The outermost portion of the adrenal glands is used for the synthesis of androgens (e.g. testosterone), mineralocorticoids (e.g. aldosterone) and glucocorticoids (e.g. cortisol)
Causes
The hormonal alteration that distinguishes Addison's disease can result from several etiological elements: anomalies in the development of the adrenal glands, alterations in the synthesis of adrenal cortical hormones (starting from cholesterol), damage to the adrenal cortical cells, reduced ACTH activity Addison's disease can also be caused by anticoagulant abuse, severe bleeding, autoimmune disease, tuberculosis, cancer.
Symptoms
Initially vague, the symptoms associated with Addison's disease can become more and more accentuated, until they become highly disabling: mood alteration, anorexia, diarrhea, weight loss, dehydration, pain in the joints, inappetence, hyperpigmentation of the skin, hypoglycemia, hypotension , nausea, confusion, vomiting.
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Medicines
Being a chronic disease, Addison's disease requires periodic administration of drugs; it should be remembered, however, that adrenal damage is irreversible, consequently drug therapy is aimed at replenishing the absent hormones, while not acting directly on the triggering cause.
In general, treatment for Addison's disease involves the administration of glucocorticoids (cortisone-hydrocortisone), mineralocorticoids (fludrocortisone) and, in women, estrogen. It is recommended to take medications regularly and not to forget doses; in fact, failure to administer these drugs can trigger effects that are sometimes dangerous for the patient's life.
In the event of an adrenal crisis, it is essential to inject hydrocortisone as soon as possible from the onset of symptoms.
When a period of particularly accentuated stress is associated with Addison's disease, the dose of drugs is generally increased, to escape the Addisonian crisis; analogous speech for traumatic episodes and persistent and high fever.
The following are the classes of drugs most used in the therapy against Addison's disease, and some examples of pharmacological specialties; it is up to the doctor to choose the most suitable active ingredient and dosage for the patient, based on the severity of the disease, the state of health of the patient and his response to treatment:
- Fludrocortisone or 9α-fluorohydrocortisone (eg. Florinef): the drug is a mineralocorticoid indicated to replace aldosterone in the context of Addison's disease: for this purpose, for adults, it is recommended to take the drug at a dose of 0.05- 0.1 mg, orally, over 24 hours; in general, the dose can vary from 0.1 mg, three times a week, up to 0.2 mg per day. For newborns with Addison's disease, it is recommended to administer a dose ranging from 0.1 to 0.2 mg orally per day; for children, the dose can vary from 0.05 to 0.1 mg per day. Consult your doctor. The drug is widely used in therapy also to treat pathological hypotension.
- Prednisone acetate (eg Deltacortene, Lodotra): indicated to replace cortisol. The dosage of prednisone must be established by the doctor based on the severity of the disease. The drug can be taken either in a single daily dose or in several doses. Consult your doctor.
- Dexamethasone (eg. Decadron, Dexameta, Etacortilen): glucocorticoid indicated for hormone replacement treatment in Addison's disease. The drug can be taken orally, or intramuscularly and intravenously at a dosage of 0.03-0.15 mg / kg per day; alternatively, take 0.6-0.75 mg / m2 / day, divided into several doses, every 6-12 hours. The posology just described is indicated for both adults and children with Addison's disease.
Given that in a healthy person, the synthesis of cortisol is influenced by circadian rhythms synchronized with the sleep-wake alternation, it is understandable how, in a patient with Addison's disease, the administration of glucocorticoids is preferably divided into several doses during 24 hours, in order to mimic the circadian trend that regulates the production of hormones in a healthy person; however, some glucocorticoids, such as prednisone and dexamethasone just described, can have a long duration of action, so they can only be administered once a day.
- Cortisone (eg. Cortone, Cortis Acet FN): indicated to replace cortisol in the context of Addison's disease. Indicatively, take orally or intramuscularly a dose ranging from 25 to 300 mg per day, in 1-2 doses. For affected children, it is recommended to take 0.5-0.75 mg / kg per day, orally, divided into 3 doses over 24 hours (every 8 hours); alternatively, administer 0.25-0.35 mg / kg of drug, intramuscularly per day.
- Hydrocortisone (eg Proctosedyl, Hydrocortone, Plenadren): glucocorticoid drug. Available in 5-20 mg modified-release capsules, the drug is often used in therapy to treat Addison's disease. It is recommended to administer the medicine in the morning, before breakfast, with plenty of water. Do not exceed 20-30 mg per day, in full compliance with what is established by the attending physician.
- Dehydroepiandrosterone or DHEA (eg Gynodian DEPOT): hormone useful as a substitute for androgens. Indicated for women with Addison's disease. It seems that the administration of this hormone, in Addisonian patients, can improve the general perception of well-being, promote libido and sexual satisfaction. DHEA is used by the body for the synthesis of sex hormones and cortisol. The dosage should be established by the doctor based on the severity of the disease.
Note:
- Addisonian crises must be promptly treated by administering hydrocortisone, saline and glucose; in fact, these crises can cause a marked reduction in blood pressure (hypotension) and blood sugar (hypoglycemia), as well as increase the levels of potassium in the blood.
- The dosage of a given drug must always be established in relation to the severity of the disease and the condition of the subject.
- Corticosteroid injections are indicated when the patient with Addison's disease cannot tolerate oral medications or is suffering from vomiting.
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