Description of Addison’s disease
Addison’s disease is infrequent but serious endocrine disorder that is characterized by reduced levels of hormones of the adrenal cortex with all its negative consequences for the body. Without adequate treatment can be fatal, thankfully, we are now wide-quality possibilities of coping with the disease.Its course is usually chronic bouts of so-called acute addisonských crisis. Disease may be primary, originating in adrenal damage or secondary when the causes are outside the adrenal glands, most often in the pituitary gland. Primary Addison’s disease affects one to four people out of a hundred thousand, and it can occur at any age, but most often between thirty and fifty years ago. Disease prefer sex, men and women about equally affected.
Risk factors
We can say that again risk factor prevalence of secondary illness in the family, or pituitary disorders. Also at risk are people with congenital adrenal n edostatečným development, which then even with their small lesions may be insufficient in their functions. Risk is also a previous attack of meningitis and inflammation of the adrenal gland, treatment of blood clotting and certain metabolic diseases such as amyloidosis or hemochromatosis.
Prevention Addison’s disease
Options are given to prevent the type of Addison’s disease and the main reason of its origin. It is important to pay attention to any difficulties and timely consult a doctor, especially when suspected inflammation of the adrenal, who could seriously damage and even lead to manifestations of the syndrome. Furthermore, judicious dosage of various drugs which may also contribute to the tissue destruction of the adrenal gland, as well as when it is necessary to think of the so-called meningitis Waterhouse- Fridrichsen syndrome, again damaging this tissue.
Signs and symptoms of Addison’s disease
Symptoms of the disease are of course given smaller amounts of the vital hormones of the adrenal cortex. These hormones include the mineralocorticoid aldosterone with the main agent, which is mainly involved in the management of the organism with sodium and potassium, to maintain the volume of body fluids and thereby participates in the maintenance of blood pressure. The second group of hormones glucocorticoids are necessary for the maintenance of blood glucoseas an antagonist of insulin stimulate the formation of glycogen degradation of lipids, ie fats, and proteins (protein) and have a great influence on the immune system- suppresses its activity and anti-inflammatory effects. And finally, the third group sou androgens whose main part is produced, although the sexual organs, but also adrenal body part is important.
Manifestations of Addison’s disease are manifested when the cortex is dysfunctional, at least ninety percent. The causes of the disease are varied and can accordingly syndrome divided into two groups: primary and secondary, which is slightly different in their speeches. Primary Addison’s disease is attributable directly to the adrenal cortex. The reason for its outbreak can be especially damaging adrenal proběhnutým inflammation, less common reason is the presence of tumor metastases (lungs, bronchi, breast cancer), also possible is an autoimmune basis, bleeding into the bark failing treatment of meningitis, and in the treatment of excessive blood coagulation. The adrenal glands may be pathologically deposited amyloid or iron and reduce its function, and finally, perhaps congenital disorder of the enzyme in the adrenal glands or their total lack of development, called hypoplasia.
Features of this type of disease is usually chronic, with occasional bursts especially in stressful situations where there is not enough glucocorticoids that are for managing stress necessary (included in the so-called stress axis). Lack of aldosterone causes potassium retention and conversely the lack of sodium, which is reflected as dehydration and low blood pressure, which can manifest as so-called orthostatic hypotension, a difficulty in changing the position from lying down to standing, in the worst cases can result in a shock state. Increased amount of potassium is also dangerous for the heart, which may produce minor necrosis, and arrhythmia. Sodium deficiency also leads to extremely strong craving for salt.
Lack of glucocorticoids is reflected in the changes in blood glucose levels especially among children, which have reduced. Reduced amounts of androgens then causes emaciation.
Since the damaged cortex is unable to recapture the hormone ACTH (adrenocorticotropic hormone), which arises from a common base together with the hormone MSH (melanocyte-stimulating hormone), which is involved in the formation of melanin, is a typical finding of increased pigmentation throughout the body, especially at the point where already normally is more pigment.This includes the area around the genitals and anus, around the breast in hands and places more exposed to solar radiation- face, arms, typical is the emergence of so-called graphite spots on the oral mucosa in approximately the first upper molars.
Secondary Addisonův syndrome is due to insufficient ACTH, which stimulates the production of glucocorticoids in the adrenal gland, the pituitary. This may be because of the prolonged administration of glucocorticoids or with congenital deficiency of the pituitary gland. Because ACTH has no significant impact on the production of mineralocorticoids (here has a greater importance in the kidneys produced hormone renin), their level is normal, and therefore, this species lackshypotension, dehydration and damage to the heart, and also the lack of increased pigmentation(ACTH is not in the blood more as the primary syndrome, but rather less).
Other unspecific symptoms are fatigue, loss of weight and appetite, muscle weakness and pain. Not infrequently, patients exhibit depression and irritability. Diarrhea, nausea or vomiting are also not exceptional.
Illness do not always have a chronic course only: sometimes appears as a so-called Addisonian crisis. This includes a sudden pain in the lower back, abdominal and leg, sudden vomiting and diarrhea, which can lead to dehydration. Further reductions in blood pressure, which resulted in the afflicted may fall into unconsciousness.
Screening & Diagnosis
After taking medical history, interviewing the patient, assessment of the obvious symptoms and physical examination (including for example, blood pressure measurement) doctor on suspicion of the disease proceed first to the blood testing. Here we concentrate mainly on changes in the levelsof potassium, sodium, cortisol and adrenocorticotropic hormone (ACTH), it is possible to detect autoantibodies as Addison’s disease was an autoimmune basis.
Another test is the ACTH stimulation test. The patient is injected with synthetic ACTH. It stimulates the production of cortisol by the adrenal glands. If there are functional, increases cortisol naturally, but if they are damaged, cortisol increases insufficiently or not at all. Similarly, insulin can be given in case of suspected damage to the pituitary gland. After its submission to measure levels of cortisol (with the disease is not increased, so a healthy person) and glucose (decreases).
The doctor may also use imaging such as central tomography (CT) of the abdominal cavity to evaluate the appearance of the adrenal gland, or CT or MRI pituitary suspected secondary Addison’s disease.
Treatment for Addison’s disease
If the patient is an outbreak of acute Addisonian crisis, it is necessary to act quickly, since this condition is life threatening. Necessitates immediate intervention of a doctor who injected hydrocortisone (synthetic glucocorticoid), saline and sugar (dextrose). By quickly dispels crisis manifested as extremely low blood pressure, high potassium levels and hypoglycemia.
In chronic diseases they are often prescribed mainly corticosteroids which lacks body and for its correct functioning should be artificially replaced. Naturally, the body forming cortisol is replaced by synthetic hydrocortisone, prednisone and cortisone acetate. Aldosterone missing in the primary syndrome is replaced pharmacologically fludrocortisone. These drugs are usually administered orally in the form of powders (orally) every day in corresponding doses attempting to approximately imitate quantity as would naturally formed in the body. This minimizes the possibility of the development of side effects, which have glucocorticoid unfortunately large quantities. Prior to stressful situations, operations, while infectious or less severe disease, your doctor may increase in the short term. When illness with vomiting should orally administered drugs have been vomiting, must therefore proceed to their administration by injection. Side effects of glucocorticoids are numerous: in terms of damage to the stomach (must not be given to patients such as ulcer chodobou), the formation of stretch marks on the skin, its overall weakening, water retention and the formation of central obesity, facial image měsícovitého, osteoporosis due to release of calcium from bones or hypertension and heart damage.
In some cases, your doctor may recommend compensation for androgens adrenal cortex using dehydroepiandrosterone. This therapy has a positive effect on women who are administering these hormones improve their sexual life.
What can I do for myself
Above all, follow medication. Affected would carry still had to have a card or bracelet with information that is suffering from Addison’s disease. During the outbreak of the crisis Addisonian then paramedics immediately know what kind of care patients are often unconscious needs.
It will be appreciated that any shortfall in the dosage of drugs can have serious consequences, because the patient should always have extra dose of medication at various locations against forgetfulness, and also from the doctor require corticosteroid injection solution against onset of the crisis.
Complication
Patient very complicated life through the illness and the need for lifelong taking corticosteroids.Serious complications are very Addisonian outbreak of the crisis. It is difficult to distinguish between what can still be described as symptoms of the disease and what is its complications. Often their only formal division, largely cut.
It is necessary to take the disease seriously and responsibly, because untreated can be fatal.
Other names: hypocorticalism, Addison’s disease, Addisonův syndrome, chronic panhypokortikalismus, adrenal insufficiency, insufficiency of the adrenal cortex, hypokortizolismus