Pregnancy with hypercortisolism
Introduction
Introduction to pregnancy with hypercortisolism Cortisol syndrome, also known as Cushingssyndrome, is the most common form of adrenal hyperfunction, which is caused by excessive secretion of cortisol from the adrenal cortex, but often secretes other corticosteroids. Pregnancy with hypercortisolism is extremely rare, and most patients end with abortion, premature delivery or stillbirth. basic knowledge Sickness ratio: 0.0001% Susceptible population: pregnant women Mode of infection: non-infectious Complications: pregnancy-induced hypertension, pulmonary edema, pregnancy-induced hypertension, abortion, premature delivery
Cause
Pregnancy with the cause of hypercortisolism
(1) Causes of the disease
1. Pituitary secretion of adrenocorticotropic hormone (ACTH) is the most common type of this disease, accounting for about 70%, more common (80%) of the lesions are pituitary ACTH microadenomas, 10% of the anterior pituitary gland About 10% of the tumors are hypothalamic dysfunction, and the secretion of corticotropin releasing hormone (CRH) is excessive. The regulation of ACTH secretion is increased. Excessive ACTH stimulates diffuse hyperplasia of bilateral adrenal cortex, and the bundled cells proliferate and hypertrophy. It can secrete a large amount of glucocorticoids, and the androgen of the reticular zone can also be secreted. In patients with chronic disease or older age, the adrenal cortex may proliferate with nodules and have certain autonomic functions.
2. Adrenal cortical tumors account for 25% of the incidence of hypercortisolism (20% are adenomas, 5% are adenocarcinomas). The growth and secretion functions of these tumors are autonomic, not controlled by pituitary ACTH, and the tumors secrete a large amount. Cortisol can feedback inhibition of the release of pituitary ACTH, ACTH in patients with blood decreased, ipsilateral and contralateral adrenal cortex atrophy, children with cortical adenocarcinoma are more common.
3. Ectopic ACTH syndrome Lung cancer, thymic cancer and pancreatic cancer, medullary thyroid carcinoma can produce ACTH, stimulate excessive secretion of glucocorticoids in the adrenal cortex.
(two) pathogenesis
In patients with Cushing's syndrome, basal cells of the anterior pituitary gland often undergo hyaline degeneration and vacuolization. This change is often the result of increased secretion of adrenocortical hormone. In this case, the secretion of gonadotropin will undoubtedly be affected. The ovary is correspondingly changed, the follicular development is blocked, and a large number of atresia follicles are formed, leading to amenorrhea. Although amenorrhea is one of the prominent symptoms of the syndrome, 20% of patients have normal menstruation and can be pregnant, often during pregnancy or pregnancy. After the end of the first clinical symptoms, it is speculated that before a pregnancy is a mild patient, the condition worsened after pregnancy and childbirth.
Prevention
Pregnancy with prevention of hypercortisolism
Cortisol syndrome, also known as Cushing's syndrome, also known as Cushing's syndrome, according to the general care routine of internal medicine and systemic diseases.
[condition observation]
1. Obesity, high blood pressure.
2. Dry skin, subcutaneous hemorrhage, hemorrhoids, traumatic purulence, peripheral limbs purpura, edema, hairy, low muscle strength, fatigue, fatigue, osteoporosis and pathological fractures.
3. Urine volume, urine urinary hematuria, proteinuria, urine sugar.
4. Psychiatric symptoms, insomnia, restlessness, depression, and excitement.
5. The symptoms of infection are feverish.
6. Female patients have abnormal menstruation.
[symptomatic care]
1. Prevent infection, keep skin clean, bathe, change clothes, keep the bed unit clean and well, do oral and perineal care.
2. Observing mental symptoms and preventing accidents, patients with irritability, abnormal excitement or depression, pay attention to strict care, prevent falling, use bed files or use restraint belt to protect patients, it is not appropriate to place dangerous goods around patients to avoid irritation Words and deeds, patience and care, should pay more attention to care.
3. Patients with adrenal cancer chemotherapy were observed for nausea, vomiting, lethargy, motor disorders, and memory loss.
4. Height, weight, and sudden compression fractures of the spine were measured weekly.
5. Do all the tests correctly and promptly.
[General care]
1. Rest in bed, lighter can be appropriate activities.
2. Diet should be given high protein, high vitamin, low fat, low sodium, high potassium food, each meal should not be too much or too little, to eat evenly.
[health guidance]
1. Instruct patients in daily life, pay attention to prevent infection, keep the skin clean, prevent trauma, and fracture.
2. Instruct patients to properly take a balanced diet and give low sodium, high potassium, high protein foods.
3. Take the medicine as directed by your doctor, and do not take the medicine or stop the medicine.
4. Regular outpatient follow-up.
Complication
Pregnancy with complications of hypercortisolism Complications, pregnancy, hypertension, pulmonary edema, pregnancy-induced hypertension, abortion, premature delivery
Pregnancy-induced hypertension, pre-eclampsia is almost impossible to avoid, pulmonary edema is also more common, pregnancy-induced hypertension often occurs early, more than 24 weeks before pregnancy, may be related to adrenal hyperfunction, increased aldosterone secretion and caused by sodium retention.
Abortion, premature delivery, stillbirth can account for 65% of the total number of pregnancies, may be associated with severe pre-eclampsia and corticosteroids have a strong anti-insulin effect on blood sugar.
Symptom
Pregnancy with symptoms of hypercortisolism Common symptoms Weight gain Cortisol Increase blood sugar High blood pressure Acne-like rash Amenorrhea Lack of osteoporosis Full moon face
The clinical manifestations of hypercortisolism are due to excessive cortisol in the body, causing sugar, protein, fat, electrolyte metabolism disorder and various organ dysfunction. The main clinical manifestations are full moon face, centripetal obesity, purple pattern, hemorrhoids, Diabetes tendency, high blood pressure, osteoporosis, amenorrhea, fatigue, etc.
Examine
Examination of pregnancy with hypercortisolism
First, the general examination of red blood cell count and hemoglobin content is high, the total number of white blood cells and neutrophils increased, the absolute value of lymphocytes and eosinophils decreased.
Second, the 24-hour content of urinary 17-hydroxycorticosteroid increased significantly over 38.4mol/24h (13.9mg/24h) (male) and 30.9mol/24h (11.2mg/24h (female), 17 ketosteroids often exceeded 40.3 mol/24h (11.6mg/24h) (male) and 37.3mol/24h (8.5mg/24h) (female).
3. The blood cortisol concentration increased by more than 0.28 mol/L (10.1 mg/dl) at midnight, and the quiet state exceeded 0.69 mol/L (24.9 mg/dl) at 8 am.
Fourth, the blood cortisol is disappearing day and night, that is, the midnight cortisol concentration exceeds the morning 8 o'clock level, and the circadian rhythm disappears in the early stage of the disease.
5. Low-dose dexamethasone inhibition test Cortisol is not inhibited, and other reactive or functional cortisols can reduce blood cortisol concentration or 24-hour urinary 17-hydroxycorticosteroid content by more than 50% of the baseline value. It is mainly used for identification of simple obesity. Method: Simplified method; blood cortisol concentration is taken as the basic value at 8 o'clock on the first day, dexamethasone is taken at midnight: 1.5 mg, and blood sterol is retested at 8 o'clock the next morning. , the formal method: the first day measured 8 o'clock morning blood cortisol concentration or 24 hours urine 17 hydroxycorticosteroid content, the next day began to take dexamethasone 0.75mg three times a day for 4 days, after reviewing blood cortisol concentration or urine 17 The content of hydroxycorticosteroids was compared with that before administration.
Six, high-dose dexamethasone inhibition test adrenal hyperplasia more than 50% inhibition, adrenal adenoma or adenocarcinoma is not significantly inhibited, method, the day before the test blood sterol or urine 17-hydroxycorticosteroid content, after Dexamethasone 2mg, once every 6 hours, a total of 5 days, after review of blood cortisol concentration or urine 17-hydroxycorticosteroid content, and compared with before taking the drug.
7. ACTH excitatory test in normal people, simple obesity and adrenal hyperplasia can increase blood cortisol concentration or urinary 17-hydroxycorticosteroid content by more than 2 times after injection of ACTH. Adrenal adenoma or adenocarcinoma has no significant increase. High, method: Simplified method, immediately after 8 hours of blood cortisol concentration, intramuscular or intravenous injection of ACTH25mg, at 8:30 and 9 o'clock respectively, blood was measured cortisol concentration, regular method, the first, 2 days stay 24 The hourly urine test measures the content of 17-hydroxycorticosteroids. On the 3rd and 4th day, 8 hours of intravenous infusion of ACTH 24mg (with 5% glucose solution in 500ml) is continued for 8 hours, and the urine 17-hydroxycorticosteroid content is measured 24 hours a day.
Eight, imaging examination is a localization examination, including B-mode ultrasound, CT scan, adrenal angiography, magnetic resonance examination, venous catheter segmentation blood sampling determination of cortisol, etc., when conditions are also used for 131 isotope-labeled cholesterol isotope scanning Or gamma photography to aid diagnosis.
Diagnosis
Diagnosis and differential diagnosis of pregnancy complicated with cortisol
diagnosis
During the pregnancy, the metabolism of glucocorticoids and salt corticosteroids changes, and the production of cortisol and aldosterone increases with the increase of pregnancy. Therefore, the diagnosis of hypercortisolism during pregnancy is difficult. Patients with pregnancy complicated with hypercortisolism Typical people have a full moon face, purple lines on the inside of the abdomen and limbs, hemorrhoids, weight gain, and elevated blood sugar; laboratory tests for 24-hour urine 17-hydroxycorticosteroids (17-OHCS) and free cortisol are significantly elevated; adrenal gland B ultrasound Examination and perirenal inflation imaging can be helpful in diagnosis.
Differential diagnosis
Careful analysis of laboratory results and other test results, diagnosis is not difficult, mainly should be distinguished from simple obesity, ectopic ACTH-like tumors, diabetes, other adrenal cortical secondary lesions, the differential diagnosis of various types of cortisol Schedule.
Differential diagnosis of bilateral adrenal hyperplasia (hypothalamic-pituitary dysfunction) adrenal adenoma adrenal cortical carcinoma ectopic ACTH syndrome urinary 17-hydroxyl (mg/24 hours) in different pathologies and pathological cortisols ) Generally moderately increased, about 30mg with the same proliferation increased significantly, up to 50mg or more with the same cancer 17-ketone (mg / 24 hours) moderate increase, about 20mg can be normal or increased significantly, up to 50mg or more significantly increased, More than 50mg of bloody skin alcohol (g / dl) 8:30 am 35 35 50 4:25 pm 25 ~ 35 35 50 high-dose dexamethasone inhibition test + most can be inhibited, a few can not be inhibited can not be inhibited can not be inhibited Can not be inhibited, a few ectopic CRF can be inhibited ACTH stimulation test ++ response, more than half of the normal no response, half of the majority of reactions have no response, a small number of ectopic ACTH secretion is particularly large, no response Pyridone test ++ + reaction, often higher than normal, generally no reaction, a small number of reactions, no reaction, some reactions, some of the non-reactive hypokalemia alkalosis can be abnormal, there are often sella photo In a small number of patients, the enlargement of the sella does not enlarge and does not enlarge the posterior peritoneal angiography. The adrenal gland on both sides of the adrenal gland enlarges the side of the adenoma. The diameter is generally 2~6cm. The diameter of the tumor is often greater than 6cm, but often due to adhesion. The gas can not enter the adrenal gland adrenal gland to increase the radioactive iodinated cholesterol adrenal gland scan or photographic bilateral adrenal gland imaging, increase the tumor side imaging, increase the cancer side imaging, or do not develop bilateral imaging, increase Adrenal ultrasonography and CT scan on both sides of the adrenal gland showed that the tumor showed that the adrenal gland enlargement on both sides of the tumor plasma ACTH was slightly higher than normal in the morning, and the night did not decrease as normal and decreased significantly.
Note: + 2mg each time, once every 6 hours, for 2 consecutive days, the second day of urine 17-hydroxyl decreased to less than 50% of the control value, indicating inhibition.
++ ACTH25u, dissolved in 5% glucose water 500ml, intravenous infusion for 8 hours, a total of 2 days, the normal 17-hydroxyl base value of urine drops more than 2 times.
+++ Mepyridone daily 2~3g, divided orally, for 2 consecutive days, on the second day or on the first day after drug withdrawal, the 17-hydroxy or 17-ketogenic steroids more than doubled compared with the control value, indicating a reaction.
