Common causes of hypoadrenocorticism.
(1) Primary adrenocortical hypofunction (referring to cortical hypofunction caused by lesions of the adrenal glands themselves).
(i) idiopathic adrenocortical atrophy.
② bilateral adrenal tuberculosis.
③ bilateral inflammation of the adrenal glands.
④ tumor invasion (lung cancer, leukemia, lymphoma, breast cancer, etc.).
⑤ bilateral adrenalectomy.
(6) Hereditary diseases: congenital adrenal hypoplasia, lipid-type congenital adrenocortical hyperplasia, adrenal-leukoencephalopathy, congenital unresponsiveness to adrenocorticotropic hormone syndrome, etc.
(2) Secondary adrenocortical hypofunction.
(i) Hypothalamic lesions: craniopharyngioma and ectopic pineal tumor affecting the synthesis of adrenocorticotropin-releasing hormone.
(ii) Pituitary lesions: tumors, inflammation, hemorrhage and atrophy cause damage to pituitary pro-adrenocorticotropic hormone cells.
(iii) long-term use of glucocorticoids inhibits its own adrenocortical function, and sudden discontinuation of the drug can cause hyperalgesia.
(iv) Removal of pituitary adrenocorticotropic hormone adenoma and ectopic secreting adrenocorticotropic hormone adenoma at the initial stage.
What are the main manifestations of hypoadrenocorticism?
(1) Hypotension and heart shrinkage. It is caused by insufficient glucocorticoid secretion, which weakens the heart’s sensitivity to catecholamines and decreases the contractility of the heart muscle; coupled with sodium and water loss and reduced blood volume. Blood pressure is usually at 12/8 kPa, and ECG shows low voltage and sinus bradycardia. (b) Glucocorticoid deficiency decreases peripheral vascular tone, slowing the vasoconstrictor response to postural changes and resulting in upright hypotension.
(2) Hypoglycemia. Weakens the glucagon effect caused by corticosteroids and reduces the resistance to insulin, resulting in fasting blood glucose at the low limit of normal. Hypoglycemia can occur in the presence of hunger, infection and exertion, manifested as palpitations, cold sweat, blurred vision, or even coma. Little change in blood glucose level in oral glucose tolerance test.
(3) Low blood sodium, low blood chloride and high blood potassium. Loss of the sodium retention effect of salicorticoids, increased urinary sodium excretion with subsequent chloride ion excretion and suppressed potassium ion excretion.
(4) Gastrointestinal symptoms. About 80% of patients have loss of appetite, nausea and vomiting; 10% have diarrhea. Gastrointestinal dysfunction can aggravate water and sodium loss and induce adrenal cortical crisis.
(5) Weight loss. Associated with dehydration and malnutrition.
(6) Mental abnormalities. About 70% of patients show various neurological and psychiatric symptoms, with mental depression being the most common, which is related to the lack of corticosteroids reducing the excitatory effect on the cerebral cortex. Hypoglycemia and hyponatremia may be accompanied by different manifestations of psychiatric symptoms, such as excitement, agitation, depression and dullness.
(7) Skin pigmentation. Appears in patients with lesions of the adrenal glands themselves.
What are the clinical features of acute hypoadrenocorticism?
Acute hypoadrenocorticism mostly has certain causes. It is characterized by the sudden onset (or aggravation) of nausea, vomiting, diarrhea, high fever, severe headache, drowsiness, coma or convulsions, as well as critical symptoms such as decreased blood pressure, dehydration and shock, on top of the aggravation of the existing disease. Laboratory tests may reveal the presence of hypoglycemia, low blood sodium, increased blood urea nitrogen and decreased blood cortisol levels.
In addition to the above symptoms, there are also manifestations of diseases that promote or cause acute adrenocortical insufficiency.
What are the clinical features of chronic hypoadrenocorticism?
(1) Skin and mucous membrane pigmentation. This is the characteristic symptom of the disease and the majority of patients have this change.
(2) Weakness. It is an important symptom in the early stage of the disease, and the degree of weakness is directly proportional to the severity of the disease.
(3) Preference for salty food. Patients tend to like salty food, which is a substitute for excessive sodium loss in the body.
This is a compensatory phenomenon of excessive sodium loss in the body, and some patients have adrenocortical crisis due to the lack of access to salty food.
(4) Weight loss. This is a common phenomenon, the degree of weight loss is related to the length of the disease
The degree of weight loss is related to the duration of the disease and the severity of the disease. Weight loss often coexists with loss of appetite and gastrointestinal dysfunction.
(5) Reduced resistance. The patient lacks stress capacity to various stimuli (cold, exertion, mental stimulation, pain, trauma, infection and surgery, etc.); he is very sensitive to anesthetics and sedatives, and a small amount can cause drowsiness and even coma.
(6) symptoms related to hypoglycemia, hyponatremia, dehydration, hypotension and other water-electrolyte balance disorders.
(7) Neurological symptoms. Almost all patients have neurological symptoms of varying degrees, including mental inactivity, indifference, memory loss in mild cases; dizziness and drowsiness in severe cases, as well as insomnia, irritability, and even mental disorders.
(8) Sexual function disorders. Male patients may have impotence, and women may have menstrual disorders, amenorrhea, loss of axillary and pubic hair.
What is the diagnosis of chronic hypoadrenocorticism based on?
The diagnosis of this disease mainly includes 2 aspects, one is to clarify the presence of hypoadrenocorticism, and the other is to find the possible causative factors. The main bases of diagnosis are as follows.
(1) The presence of skin pigmentation, weakness, wasting, loss of appetite, preference for salty food, mental depression and hypotension.
(2) Biochemical examination reveals hypoglycemia, hyponatremia and hypochlorhydria.
(3) Hormone measurements show that blood cortisol, urinary free cortisol or 17-hydroxycorticosteroids are lower than normal, and plasma adrenocorticotropic hormone is significantly elevated. Blood and urinary aldosterone levels may be lower or near normal.
(4) The patient’s blood cortisol secretion is not significantly elevated during the adrenocorticotropic hormone excitation test.
After the presence of hypoadrenocorticism is clearly established, attention is paid to check for the presence of tuberculosis and whether the patient is taking drugs that affect cortisol metabolism, etc. It is also important to differentiate the disease from those with hyperpigmentation and weakness, such as hepatic sclerosis and ectopic tumors that secrete adrenocorticotropic hormone.
How to treat chronic hypoadrenocorticism?
The treatment of this disease can be divided into.
(1) Basic treatment (conventional replacement therapy): daily supplementation with physiological doses of adrenocorticotropic hormone, commonly hydrocortisone 20-30 mg/day or prednisone 5- 7.5 mg/day, which can be adjusted according to the patient’s needs.
(2) Treatment during stress: In stressful situations such as infection, trauma and surgery, the human body’s need for glucocorticoids increases, and in addition to the daily basal dose of corticosteroids, it should be increased by 1-2 times, and hydrocortisone can be increased by 50 mg/day or dexamethasone 5 mg/day. Corticosteroids can be premedicated before major surgery. If intravenous supplementation is needed, the dose should be increased to a total of 200-400 mg of hydrocortisone or 10-20 mg of dexamethasone per day, with appropriate water and electrolyte supplementation. Gradually reduce the dose until the condition stabilizes, and finally return to the maintenance dose.
(3) Treatment in case of acute adrenocortical hypofunction.
(1) Immediate supplementation with adequate amount of adrenocorticotropic hormone.
(2) Replenish fluids and correct electrolyte imbalance.
(3) Remove the cause of hypoadrenocorticism as soon as possible.
Initially, adrenocorticotropic hormone should be given by intravenous infusion, mostly hydrocortisone, which can be 400-2000 mg on the first day according to the condition, and gradually reduce the amount or change to oral preparation after the symptoms stabilize. At the same time to strengthen the anti-shock, anti-infection and other measures. As long as the treatment is timely, most cases can be relieved, except for the original chronic hyperalgesia, most do not remain permanent hypofunction.
(4) Etiological treatment: Those with uncontrolled tuberculosis infection continue anti-tuberculosis treatment.
The disease is difficult to cure, so patients need lifelong application of glucocorticoid replacement therapy. A few patients have more concerns about long-term hormone use and often stop the drug on their own, sometimes even inducing acute adrenocortical hypofunction and causing unnecessary damage to themselves. The nature of taking hormones for this disease is different from treating other diseases with hormones, but it is only supplemented according to the patient’s needs, and the dose is appropriate, so there will be no side effects, so do not interrupt the treatment at will.