Addisonian crisis is a severe endocrine disorder that develops as a result of a sudden pronounced decrease or absolute cessation of hormone synthesis by the adrenal cortex. It is manifested by adynamia, decreased blood pressure, loss of appetite, nausea, abdominal pain, vomiting, diarrhea, acetone smell from the mouth, acetonuria, convulsions. In severe cases, dehydration, collapse, confusion, coma develops. Diagnostics includes a survey and examination of the patient, blood and urine tests, ECG. Medical treatment is aimed at restoring normal hormone levels, eliminating dehydration and its consequences.
ICD 10
E27.2 Addison’s Crisis
Meaning
Synonyms of Addisonian crisis are acute adrenal insufficiency, hypoadrenal crisis, adrenal crisis, adrenocortical crisis, acute hypocorticism, acute insufficiency of the adrenal cortex. The crisis failure of the adrenal cortical layer is named after the British therapist Thomas Addison, who described the clinic of this condition in ser. XIX century . In the International Classification of Diseases of the 10th revision, the hypoadrenal crisis is classified as “Other adrenal disorders” (code – E27.2). The prevalence of this condition in clinical endocrinology is 50 cases per 1 million people, or 0.005%. It is more often diagnosed in women, the gender ratio is 2:1. The peak period of morbidity is the age from 55 to 63 years.
Causes of addisonian crisis
The crisis condition most often develops in patients with primary and tertiary adrenal insufficiency, Addisonian disease, Schmidt syndrome. In these categories of patients, the causes of exacerbation of chronic diseases are various stresses, shock, intense stress, taking medications. The most common provoking factors are:
- Intoxication. A crisis occurs with alcohol and acute food poisoning. Vomiting and diarrhea contribute to the loss of electrolytes and dehydration, the work of target organs is disrupted, chronic diseases worsen.
- Pregnancy. During the period of carrying a child, there is a restructuring in the work of the endocrine system. The activity of the glands changes, so the probability of deficiency of certain hormones increases.
- Infectious diseases. A high risk of Addisonian crisis exists in severe acute infections, generalized inflammatory processes. It is often diagnosed with meningitis, diphtheria, toxic forms of influenza.
- Incorrect use of medicines. A decrease in hormone production can be associated with improper use of insulin, diuretics and sedatives, narcotic analgesics. Another reason is the patient’s independent replacement of one hormonal drug with another, a decrease in the dosage of glucocorticoids or the complete cessation of replacement therapy.
- Stress, shock states. Deficiency of hormones produced by the cortical layer of the adrenal glands can be caused by injuries to the lower back or abdomen, large blood loss during surgical operations, burns, childbirth. Also, the crisis is provoked by prolonged psychoemotional and physical exertion.
In individuals who do not have chronic adrenal pathology, Addisonian crisis is the result of autoimmune lesions of the cortical substance, congenital disorders of enzyme activity, bilateral acute infarction of the adrenal cortical tissues or hemorrhages in them, surgical removal of one or two adrenal glands. Sometimes a sharp decrease in hormone secretion becomes the first diagnosed sign of a latent form of chronic hypocorticism, thyroadrenocortical insufficiency.
Pathogenesis
The adrenal glands are an endocrine secretion gland that produces several types of hormones. The adrenal cortex produces corticosteroids such as cortisone, cortisol, aldosterone, corticosterone, deoxycorticosterone. The activity of these compounds ensures the normal functioning of the nervous and cardiovascular system, the course of metabolic processes and digestion, the production of connective tissue, maintains sugar levels.
With an addisonic crisis, the production of corticosteroids decreases very quickly. The body’s systems do not have time to adapt to the sudden shortage of hormones. As a result, many types of metabolic processes are disrupted, dehydration occurs, and the amount of circulating blood decreases. The change in potassium metabolism affects the work of the heart muscle: it begins to contract worse, and blood pressure decreases. Kidney failure develops, the concentration of blood sugar drops. The state of the crisis becomes life-threatening.
Classification
The adissonic crisis unfolds over a period of several hours to several days. There is a pre-crisis stage in which patients experience general weakness, muscle pain, decreased appetite, and a stage of a detailed clinical picture characterized by progressive deterioration of well-being. Another classification is based on differences in the clinical picture. According to it , there are five forms of addison ‘s crisis:
- Gastrointestinal. Acute dyspeptic disorders come to the fore. Vomiting, nausea, diarrhea, spastic abdominal pains prevail, there is no appetite.
- Pseudoperitoneal. This form of crisis is clinically similar to an acute abdomen. It is characterized by sharp pains, tension of the abdominal muscles.
- Myocardial. The leading symptoms are associated with a violation of the functions of the heart and blood vessels. Circulatory insufficiency is manifested by cyanosis of the mucous membranes and skin, hypotension, slowing of the pulse.
- Meningoencephalic. Neuropsychiatric disorders dominate. Focal and hallucinatory-delusional symptoms develop, convulsions, depression of consciousness, delirium.
- Respiratory. It is manifested by respiratory insufficiency. Shortness of breath increases, general weakness, dizziness, there is a feeling of lack of air.
Symptoms of Addisonian crisis
In patients with chronic forms of adrenal insufficiency, the symptoms of a crisis increase for several days, the rapid development of a serious condition in 3-6 hours is possible with infectious and shock conditions. At first, patients note a decrease in working capacity, lethargy, adynamia. Muscle weakness is so pronounced that they prefer to stay in bed most of the time, often need care (feeding, changing clothes, toilet). When trying to get up and walk around the room, dizziness occurs, sometimes fainting. A sharp drop in blood pressure provokes a collapse.
The characteristic “bronze”, golden-brown pigmentation of the skin and mucous membranes increases. Appetite is noticeably reduced to the point of complete intolerance to the sight and smell of food. Patients complain of nausea, which, with the progression of symptoms, develops into indomitable vomiting. After another attack, the state of health does not improve. Pain in the abdominal area increases: at first it concentrates in the epigastrium, then spreads throughout the abdominal area. Acute pains are accompanied by the release of vomit with blood, black, tar-like stools or diarrhea. Less often, the pain is localized in the lower back. Diarrhea and vomiting provoke dehydration of the body, loss of electrolytes.
A decrease in sodium levels and a reduction in blood volume leads to a sharp decrease in systolic and diastolic pressure. Organs don’t get enough oxygen. Hypoxia of the brain is manifested by increased drowsiness and dizziness, confusion of consciousness. Due to impaired blood flow in the kidneys, glomerular filtration worsens, urine volumes decrease, oliguria develops, then anuria. Urea and nitrogen accumulate in the blood, acidosis is formed, the main sign of which is the acetone smell from the oral cavity and from the patient’s skin. On the part of the nervous system, symptoms such as seizures, pseudomeningeal syndrome, psychotic states with paranoid delusions and / or hallucinations, delirium are possible.
Complications
Without providing the patient with emergency medical care, the clinical manifestations of Addisonian crisis progress. The risk of acute heart failure with loss of consciousness, dehydration of brain tissues, acute renal failure increases. Consciousness becomes clouded, the skin becomes cold to the touch. The pulse is barely palpable, threadlike, blood pressure cannot be determined, heart tones are sharply weakened. Coma develops, and then death. The prevalence of death among women is 0.52%, among men – 0.89%.
Diagnostics
Addisonian crisis is an acute condition that requires urgent medical care, therefore, express diagnostic methods are more common – clinical data collection and several laboratory tests that can detect metabolic disorders, differentiate the crisis with peritonitis, heart failure, celiac disease, myopathy, hyperparathyroidism and some other diseases. As a rule, the complex of examinations of the patient includes:
Survey, inspection. The endocrinologist collects anamnesis: in most cases, chronic hypocorticism is determined, as well as factors that can provoke the development of a crisis (infections, injuries, childbirth, stress, physical exertion). Complaints describe the clinical picture of acute adrenal insufficiency. Upon examination of the patient, slowness of speech and movements, apathy, characteristic pigmentation of the skin, weak slow pulse are noted.
Urine and blood tests. The complete laboratory examination includes a general and biochemical blood test, a clinical urine test, a study of glucose in the blood, adrenal hormones in urine and blood. Elevated indicators of leukocytes, erythrocytes and hemoglobin, increased ESR, hypoglycemia, hyperkalemia and hyponatremia are characteristic. According to the urine test, the appearance of protein, erythrocytes, an increase in sodium is determined, acetone may be detected.
ECG. Electrocardiography is performed to detect hyperkalemia. Changes in indicators are observed at a concentration of potassium in plasma from 7 mmol / l. There is a high pointed T-wave with a normal QT interval, a reduced amplitude of the P-wave with an elongated PQ interval. Increased hyperkalemia is accompanied by the expansion of QRS complexes, atrial asystole.
Treatment of Addisonian crisis
Assistance to patients is provided in the conditions of the Department of endocrinology or intensive care. The main set of measures is carried out on the first day of the patient’s stay in the hospital, at the end of this period it is possible to evaluate the effectiveness of therapy and make a prognosis. The stable recovery period lasts 4-6 days. Treatment is carried out in four directions:
- Replacement therapy with corticosteroids. Water-soluble hydrocortisone preparations have been widely used. Patients are prescribed intravenous injections, less often intramuscular.
- Elimination of dehydration, hypoglycemia. Patients are prescribed intravenous drip administration of glucose solution, saline solution, Ringer’s solution. The volume of drugs is calculated individually, gradually reduced by the end of treatment.
- Restoring electrolyte balance. To compensate for hypochloremia and hyponatremia, infusions of sodium chloride solution, drinking brackish water are indicated. To eliminate hyperkalemia, a solution of glucose and calcium gluconate is administered.
- Symptomatic therapy. If necessary, measures are taken to detoxify, restore the work of the heart, blood vessels, respiratory system, fight against collapse, infections, shock conditions, protein metabolism disorders. Specialists from other areas are involved in the treatment.
Forecast and forecast
The outcome of an addisonic crisis directly depends on the timeliness of treatment: the earlier therapeutic measures are initiated, the more favorable the prognosis. Patients with concomitant autoimmune and infectious diseases are at an increased risk of mortality. To prevent a crisis, people with chronic hypocorticism need systematic diagnosis of adrenal function, medical monitoring of the effectiveness of supportive treatment. Self-cancellation of medications, replacement of prescribed medications with analogues is unacceptable. With increased emotional or physical exertion, acute diseases, pregnancy, you need to consult with an endocrinologist about increasing the dosage of drugs.
Literature
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