condition associated with a decrease in the production of hormones, it is produced in the adrenal cortex, called "adrenal insufficiency" or "gipokortitsizm".Sometimes it is acute or chronic.
With not too noticeable symptoms, chronic adrenal insufficiency is often not recognized for a long time and is not treated (one of the symptoms - the love of salty foods, which is rarely alarming), and this leads to serious complications.Acute
the failure of the cortex does not remain unnoticed: it develops a shock with a drop in blood pressure (read about the blood pressure rate), shortness of breath, sometimes - seizures and loss of consciousness.Such a crisis can occur for no apparent reason and remind abdominal disease, but more often it causes a sharp decrease in dosage or withdrawal of hormones that have been designated for the treatment of chronic hypocorticoidism.
Arise insufficiency adrenal hormones can be a consequence of the defeat of the adrenal glands themselves, and as a result of a pathology of the central bodies that regulate the activity of the endocrine organs.
Knowing the symptoms of adrenal insufficiency and its timely detection in the modern world, where you can completely replace the lack of its own hormones with synthetic analogues, can significantly improve the prognosis.
About the adrenal glands and their management
Adrenal glands - paired organ is a small triangular shaped sizes 4 * 0.3 * 2 cm, located just above the kidneys.The mass of each of them about 5 grams.
adrenal glands consist of two layers.Wide outer called cortical (cortex, hence the name - "gipokortitsizm"), while the central, which accounts for 20% of body weight - the brain.In the latter are synthesized hormones that regulate blood pressure, adrenaline, dopamine, norepinephrine.Lack of hormones that part of the body are not called adrenal insufficiency, therefore we treat them will not.
In cortical layer synthesized several kinds of hormones:
- Glucocorticoids: korizol.
- mineralocorticoid: aldosterone.
- male sex hormone - in both sexes: androstenedione, dehydroepiandrosterone.
- Female sex hormones (min): estrogen and progesterone (progesterone read about the rules for women).
Supervise the elaboration of these bioactive substances central endocrine organs, localized in the cranial cavity and anatomically inseparable from the brain - the hypothalamus and the pituitary gland.The hypothalamus produces corticotropin, the latter reaching the pituitary, commanding generation of adrenocorticotropic hormone (ACTH) and have it directly affects the adrenal glands.
adrenal insufficiency is a condition when the body is formed deficiency of two hormones:
what they are responsible?
|hormone name and its metabolites||Glucocorticosteroids||mineralocorticoid|
|Influence on carbohydrate metabolism||raise blood glucose levels by accelerating the formation of glucose from proteins in the liver,while reducing waste it in muscle tissue||not affect|
|Effect on exchange||electrolytes only affects calcium, accelerating its release into the blood and reducing the absorption of bone|| Maintain the balance of sodium, potassium, chloride and bicarbonate: |
|Protein metabolism||Increase the breakdown of proteins into amino acids, decrease the rate of formation of the amino acids of the protein||not affect|
|Exchange lipid||Strengthen fat deposits under the skin of the trunk and face.Simultaneously, the breakdown of fat in the limbs||not affect|
|Effect on immunity||As anti-inflammatory agents to suppress the immune system in large volumes.Because of this, synthetic glucocorticoids are used to treat autoimmune diseases, for the prevention of rejection of the transplanted organ||not affect|
|Side effects||An increase above the required level inhibit the synthesis of new skin cells, leaving it thinner.Also reduces muscle mass, suppressing the production of these new proteins||in large quantities due to sodium retention, which draws water into the blood vessels, increases blood pressure|
Developing both glucose and mineralocorticoid,as already mentioned, it has a triple regulation: hypothalamus - pituitary gland - the adrenal glands.That is, the decrease hormone levels may be due to the violation of their synthesis in one of these organs.Depending on the localization of the disease adrenal insufficiency can be:
- primary , when there is a failure of the adrenal glands themselves;
- secondary .It is related to the pituitary disease which synthesizes a little or not at all synthesizes ACTH hormone;
- tertiary , when the center, the hypothalamus produces corticotropin little.
gipokortitsizm can also be caused by a condition when the hormone is synthesized by a sufficient amount, but the receptors are occupied or substances that block the receptors for cortisol or aldosterone, or insensitive.
Primary adrenal insufficiency is more severe secondary and tertiary forms.
Depending on the nature of the process of developing adrenal insufficiency is:
- acute (this is also called addisonicheskim crisis, and if it caused bleeding in the adrenal tissue - the syndrome Waterhouse-Friderichsen) - a deadly disease that requires urgent correction;
- chronic - this state if competent timely correction can be kept under control for many years.Deficiency may be in the stage of compensation, and decompensation subcompensation.
Adrenal glands - paired organ, besides having a large compensatory capacity.Thus, its symptoms of chronic lesions develop only when 90% of affected cortex.Primary adrenal insufficiency develops when the adrenal pathology themselves.This can be:
- autoimmune disease (ie, attack its own tissue as antibodies) - the cause of almost 98% of cases hypocorticoidism.The cause of this condition is unknown, it is most often combined with other autoimmune endocrine organs lesions;
- congenital hypoplasia body crust;
- Algrouva syndrome;
- tubercular process - it first develops in the lungs (see how TB is transmitted.);
- Amyloidosis - deposition of abnormal protein in the tissue of the adrenal glands, resulting in the presence of long-term chronic illness;
- adrenoleukodystrophy - genetic, transmitted through the X-chromosome pathology accumulation in the body fatty acid, which leads to progressive destruction of certain parts of the brain and adrenal glands;
- tumor metastases in the adrenal tissue;
- bleeding in the adrenal tissue.Basically, when developing serious infections involving the release of large amounts of bacteria in the blood: meningococcal infection, sepsis, scarlet fever, diphtheria, severe;
- thrombosis, feed the adrenal glands;
- tumor of the adrenal glands themselves;
- necrosis of tissue in the body of HIV infection;
- newborn similar condition can develop if it is to labor for some time remained without oxygen;
- Kearns syndrome consisting in ocular tissue and muscles;
- Smith-Opitz syndrome: a combination of several pathologies: a small cranial volume, mental retardation, as well as anomalies of genitalia.
the case of secondary failure, its causes - pituitary pathology:
- infectious nature (often - caused by a viral infection);
- arisen due to hemorrhage due to injury;
- provoked by a tumor;
- connected with the destruction of the body as a result of irradiation of it surgery or long-term treatment with synthetic glucocorticoids;
- congenital reducing body weight (this may be a lack of ACTH once and all pituitary hormones);
- pituitary lesion own antibodies.
Tertiary adrenal insufficiency occurs due to congenital or acquired (tumor, gamma rays, infection, hemorrhage) hypothalamic disease.
With a shortage of aldosterone in the body develops dehydration (read about the symptoms of dehydration), which progresses, as continued to lose sodium (and with it the water).With this potassium contrary, it accumulates.It is a violation of the digestive tract function, heart rhythm disturbances.If the potassium concentration exceeds 7 mmol / l, may develop heart failure.
Reducing cortisol causes a decrease in the formation of glycogen - a kind of storage of glucose in the liver.In response to a deficiency of this hormone, the pituitary gland begins to produce ACTH in large amounts, but the latter, having a wide range of action, trying to "force" be synthesized not only cortisol, but also melanotropin - a substance that increases the production of melanin pigment cells of the skin, retina and hair.This leads to darkening of the skin.
Symptoms of adrenal insufficiency will depend on how quickly the affected tissue of the adrenal glands.If you die at once a large number of body cells develop acute failure, having some symptoms.If the process of adrenal cell death occurs gradually, that is, it develops chronic gipokortitsizm, to the fore are the other symptoms.
This condition has several key symptoms.They develop most frequently after stressful situations which can act as trauma, childbirth, the flu or other infection, psycho-emotional stress, aggravation of chronic disease.
Increased pigmentation of the skin and mucous
for primary hypocorticoidism characteristic staining of the skin in a brownish color, the mucous membranes while also darken.On this basis gipokortitsizm also called "bronze disease."
beginning darken the parts of the body that are not protected by clothing: the face, palms of the hands, neck.In parallel, a brown are the places, which are normally darker: scrotum, nipple areola, armpits, crotch.It appears pigmentation on areas of skin that are in contact with the clothes (collar region or zone), which is especially noticeable on the background of typically colored skin.
intensity of the color depends on the duration of the disease.She may have a shade of light tan, paint the skin of bronze, obscure "haze", which creates the effect of dirty skin, and even painted in a dark color.
It stains and mucous, which is much in the language, is attached to the teeth surface of the cheek, gums, palate (painted as the vagina and rectum).
If it is a primary autoimmune gipokortitsizm, against the background of dark areas often appear depigmented areas - spots of vitiligo.
Secondary and tertiary adrenal insufficiency occur without staining of the skin and mucous membranes.
Due to severe lack of nutrients a person's weight is reduced from moderate (3-5 kg) to a condition called malnutrition (deficiency of 15 kg or more).
for chronic primary adrenal insufficiency is characterized by:
- severe muscle weakness - to a complete loss of working capacity;
- depressive disorders.
for primary chronic hypocorticoidism characteristic:
- decreased appetite;
- abdominal pain, which has no clear position, "wanders";
- vomiting without a headache or nausea the previous;
- constipation and diarrhea alternate.
chronic primary adrenal insufficiency may be suspected already by the fact that the elevated or normal pressure is gradually decreased before.Now the man - at a relatively normal state of health - measures at the numbers 5-10 mmHgbelow that previously recorded.
Other symptoms characteristic of addiction to salty foods to chronic destruction of the adrenal cortex.A fasting occur weakness, muscle tremors that take place after a meal - a sign of decrease in blood glucose levels.
In secondary gipokortitsizm addiction to salty not, but after eating a few hours later develop bouts of weakness and trembling.
Symptoms addisonicheskogo crisis
acute adrenal insufficiency in children up to 3 years can be caused by even a seemingly minor external factors: SARS, intestinal infections, stress, vaccinations.It develops immediately after birth, if they were to fetal hypoxia, or if the baby was born from the breech.This is due to the fact that adrenal tissue in these kids is not enough mature.
Acute hypercortisolism occurs when:
- meningococcal infection (it occurs more frequently in children, while adults are often just carriers of meningococcal bacteria nasopharynx);
- adrenal vein thrombosis;
- infections: Listeriosis, cytomegalovirus infection, toxoplasmosis, diphtheria;
- removal of the adrenal gland;
- abrupt cancellation of synthetic glucocorticoids.
Acute gipokortitsizm may occur without any previous symptoms.His appearance suggests:
- sudden pallor;
- weakness - to the state of prostration;
- nonlocalized abdominal pain;
- loose stools;
- decrease in amount of urine;
- skin becomes "marble", the fingertips turn blue;
- are convulsions;
- if the cause - meningococcal infection, skin rash appears brownish-black color, no fade when pressing glass or clear glass.
If time does not help, coma, which can be fatal.
How is it diagnosed
To determine what kind of adrenal insufficiency in question - primary, secondary or tertiary, perform:
- ultrasound adrenal gland, which may not show the body, even if it contains TB or cancer.In this case, additional wires adrenal CT;
- brain MRI examination with the impact area of the hypothalamus and pituitary.
But the basic diagnosis of the disease - laboratory.To find out, the lack of any hormones originated, and how it is expressed, are studied:
- cortisol levels in the blood: it reduced in any type of failure;
- 17-ACS and 17 CS in urine collected over night: the metabolites of cortisol and lowered at the primary and secondary type hypocorticoidism;
- ACTH in the blood: it decreases when the secondary-tertiary insufficiency increases - at disease napochechnikov themselves;
- determination of aldosterone in the blood: as far as he is lowered;
- hidden forms hypocorticoidism reveals stimulus test with ACTH: cortisol determined by the source, and in half an hour and an hour after injection of synthetic ACTH.If cortisol levels rose less than 550 nmol / L - it means there is adrenal insufficiency.Normally, cortisol increases 4-6 times.The test is carried out at a certain time - 8:00, when there is a natural hormone peak activity studied.
diagnosis is established.