Adrenal cortical hormone deficiency

Introduction A rare endocrine disease caused by insufficient secretion of adrenocortical hormone due to the adrenal gland itself. Adrenocortical hormones are a general term for hormones secreted by the adrenal cortex and belong to the steroidal compound. Can be divided into three categories: 1 mineralocorticoids (mineralocorticoids), secreted by the globular band, there are aldosterone (desostercortone, desoxycortone, desoxycorticosterone). 2 glucocorticoids, synthesized and secreted by the fascicle, hydrocortisone and cortisone, etc., whose secretion and production are regulated by corticotropin (ACTH). 3 sex hormones, secreted by the reticular band, usually referred to as adrenocortical hormone, excluding the latter. Clinically used corticosteroids are glucocorticoids.

Cause

(A) chronic adrenal insufficiency (Addison disease): due to infection, trauma and surgery and other stress conditions, or stop taking hormones to induce acute hypocortical function.

(B) long-term large amount of adrenocortical hormone treatment: inhibition of hypothalamic-pituitary-adrenal axis function, even if the drug is discontinued for 1 year, its function is still in a low state, especially the response to stress is poor. Oyama studied long-term treatment with steroids in 14 patients before the induction of anesthesia, 30 minutes after induction and 1 hour after surgery, blood cortisol was 107 ± 18g / L (10.7 ± 1.8g / dl), 108 ± 15g / L (10.8 ± 1.5 g/dl) and 148±25 g/L (14.8±2.5 g/dl). In the control group, the blood cortisol in these three times was 108±14g/L (10.8±1.4g/dl), 175±16g/L (17.5±1.6g/dl) and 263±18g/L. 26.3 ± 1.8 g / dl). Therefore, patients who have been treated with corticosteroids for a long time will experience acute adrenal insufficiency if they are stressed and do not replenish or increase the dose of hormones.

(3) After adrenal surgery: adrenalectomy due to adrenal hyperplasia or extra-adrenal gland hyperplasia (such as metastatic breast cancer) depending on the hypothalamic pituitary; or adrenal gland atrophy often remains after adrenal gland ablation - The function of the adrenal axis, due to the long-term secretion of a large amount of cortisol, is inhibited, and its function recovery requires at least 9 months or more, if no hormone is added or the hormone dose is not increased under stress conditions. It can also cause acute adrenal insufficiency.

(D) acute adrenal hemorrhage: common is severe sepsis, mainly meningococcal septicemia, causing adrenal hemorrhage, and diffuse intravascular coagulation. Other bacteria-induced sepsis, epidemic hemorrhagic fever, etc. may also be complicated by adrenal hemorrhage.

(5) Congenital adrenal hyperplasia: There are nine enzyme defects known to date, including 21 hydroxylase, 11 hydroxylase, 17 hydroxylase, 18 hydroxylase, 18 oxidase, 5-3 steroid sterol Hydrogenase, 22 carbon chain enzyme, 17 hydroxysteroid dehydrogenase and 17, 20 lyase enzymes. All are required for cortisol synthesis. Defects such as 5-3 hydroxysteroid dehydrogenase, 22 carbon chain enzyme, 18 hydroxylase and 18 oxidase can also affect the synthesis of sputum hormones.

an examination

Related inspection

Adrenaline test urinary adrenaline adrenal medullary imaging

A, blood, urinary cortisol levels were determined. In most patients, blood, urinary cortisol and urinary 17-hydroxycorticosteroids were measured below normal, but also at normal low limits, so multiple measurements were needed and plasma cortex disappeared.

B. Determination of plasma adrenocorticotropic hormone and its related peptide N-POMC. The level of plasma adrenocorticotropic hormone and related peptide N-POMC can be measured by radioimmunoassay 5 to 50 times higher than that of normal people, while the secondary adrenal insufficiency is generally lower than normal or at normal low limit. It is of great significance to the diagnosis of Edison's illness.

C, adrenocorticotropic hormone stimulation test: is an important indicator of the diagnosis of Edison's disease, can determine the reserve function of cortisol secretion from the adrenal cortex. Practice: add adrenocorticotropic hormone 25u to 5% glucose solution 500ml every day for 8 hours, a total of 3 days, on the control day and the first day of stimulation, on the third day, respectively, 24 hours urine to determine urinary free cortisol or 17 - hydroxycorticosteroid levels. The basic control value of patients with Edison was lower than normal and the adrenocortical hormone stimulation showed no significant increase after 3 days, while the normal human adrenocorticotropic hormone stimulation was 1 to 2 times higher than the control day after 1 day. If the condition is severe, dexamethasone should be treated at the same time to prevent adrenal crisis.

D, other. Such as abdominal plain film and adrenal CT scan showed calcification shadow in the adrenal gland, it can be confirmed the diagnosis of Addison's disease caused by adrenal tuberculosis. In addition, if the adrenal CT scan reveals atrophy of the adrenal glands, it may also contribute to the diagnosis of autoimmune adrenalitis. If you can measure anti-adrenal antibodies in the blood, it is a good indicator for autoimmune adrenalitis.

Differential diagnosis

Need to be identified with the following symptoms:

Elevated corticosteroids: a class of steroidal compounds synthesized and secreted by the adrenal cortex whose primary function is to regulate water-salt metabolism and glucose metabolism in animals. Corticosteroids have too many serious adverse reactions. The adverse reactions of corticosteroids include: cortical hyperfunction, steroid diabetes, muscle atrophy and osteoporosis, induce and aggravate infection, induce and aggravate ulcers, induce mental symptoms, and develop eye diseases. Teratogenicity, inappropriate withdrawal of the drug may also have symptoms such as insufficient cortical function, hormone withdrawal syndrome and symptom rebound.

The secretion of salt corticosteroids is too small: it mainly acts on sodium, potassium, chloride and water metabolism, so it is called mineralocorticoid. Among these hormones are aldosterone and deoxycorticosterone, among which aldosterone is the strongest. Mineralocorticoids have the effect of preserving sodium and potassium, promoting the reabsorption of sodium by the renal tubules and the effect of potassium release, thereby maintaining the proper concentration of sodium and potassium in the plasma. When the adrenal cortex hyperfunction (such as Cushing's syndrome), 11-deoxycorticosterone secretion, due to retention of sodium and water in the body beyond the limits of edema, increased blood volume, elevated blood pressure, high blood sugar, hypokalemia Wait. Conversely, if the adrenal cortical function is insufficient, the metabolism of sugar and minerals is disordered, causing a bronze disease, or "Addison's disease", which shows muscle weakness, decreased blood pressure, skin pigmentation and hypoglycemia, blood. Symptoms such as decreased sodium and increased potassium, while blood loses water and concentrates, and are life-threatening in severe cases.

Too little secretion of glucocorticoids: too few glucocorticoids are commonly seen in patients with secondary adrenal insufficiency.

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