库欣综合症（cushing´s syndrome）

库欣综合症（cushing´s syndrome） 库欣综合症(cushing's syndrome) cushing's syndrome Cushing syndrome is by a variety of causes of excessive secretion of adrenal glucocorticoids (mainly cortisol). The disease caused, most of them see as adrenocorticotropic hormone (ACTH) secretion caused by clinical types, known as Cushing's disease (Cushing disease). Take the full moon surface, the multi blood appearance, the centripetal obesity, the skin purple stripe, the hypertension, the osteoporosis and so on the main manifestation. (I) pathophysiology and clinical presentation The clinical manifestations are mainly caused by excessive secretion of cortisol, resulting in metabolic disturbances and decreased resistance to infection. 1., fat metabolism disorders, fat accumulation in the face and trunk (central obesity). Patients with Mianrumanyue, chest, neck and back fat very thick. To the later stage of the disease, the muscles of the limbs are relatively thin due to muscle consumption and fat transfer, which is clearly contrasted with the obesity of the trunk and the face. Centripetal obesity students due to a fat mobilization mechanism of cortisol, triglycerides into glycerol and fatty acids, while preventing glucose into fat cells, inhibit the synthesis of fat; on the other hand, promote gluconeogenesis, glucose stimulated insulin secretion increased, and promote fat synthesis, therefore, hypercortisolism fat mobilization and Chengdu is promoting, the redistribution of fat formation, central obesity. 2., protein metabolism disorders, a large number of cortisol, promote protein decomposition, inhibit protein synthesis. An amino acid produced by the decomposition of Pr into the liver and deamination to provide gluconeogenesis. The body is in a state of negative nitrogen balance. The phenomenon of excessive consumption of protein in clinic; thin skin, increased capillary fragility, damage can be caused by slight bruises. In the lower abdominal, arm, thigh, due to fat deposition, skin elastic fiber fracture, through the thin skin microvascular see red, forming a typical purple lines. (very distinctive). The course is long, muscle atrophy, osteoporosis, vertebral compression deformity can occur, stature is shorter, sometimes is bent, fracture. The growth and development of children were inhibited. 3. glucose metabolism disorder excess cortisol promote hepatic gluconeogenesis, and antagonize the effect of insulin and reduce the utilization of glucose in tissues, hepatic glucose output increased due to impaired glucose tolerance, some patients with steroid diabetes. 4. a lot of cortisol have disorder of electrolyte of sodium retention and excretion of potassium effect. Low potash poisoning was particularly prevalent in adrenocortical carcinoma and ectopic ACTH syndrome. 5. major vascular diseases, hypertension are common, large amounts of cortisol, increase in corticosterone may be the cause. The elevated renin concentration in the blood plasma is responsible for the increased production of angiotensin II, which results in elevated blood pressure. Atherosclerosis and hardening of the renal arteries are often associated with atherosclerosis. Patients are prone to arteriovenous thrombosis, which increases the incidence of cardiovascular complications. 6., the resistance to infection weakened, long-term cortisol secretion increased, so that weakened immune function, infection after the inflammatory response is often not significant. Fungal infection is common in the skin, and purulent bacterial infection is difficult to localize. 7. hematopoietic system and blood change cortisol stimulate bone marrow, make red blood cell count and hemoglobin content is high, facial expression is sanguine. A lot of cortisol to WBC and neutrophils increased, but the lymphoid tissue atrophy, lymphocyte and eosinophil percentage distribution, the absolute value of the two kinds of cells and white blood cell classification were reduced. 8. female sexual dysfunction in patients with adrenal androgen excess and testosterone and cortisol on pituitary gonadotropin inhibition, mostly reduce menstruation, irregular or menopause, common mild alopecia, acne, obvious male breast atrophy, should, laryngeal node hypertrophy, hypertrophy of the clitoris, male patients with sexual desire drops: penis narrow, testicular soft, erectile dysfunction. This is associated with a large amount of cortisol inhibiting pituitary gonadotropin. 9., nervous and mental disorders, emotional instability, irritability, insomnia, severe mental disorders, individual paranoia may occur. 10. skin pigmentation, ectopic ACTH patients, because the tumor produces a large number of ACTH, skin pigment significantly deepened. (two) the etiology and clinical features of various types One Cushing disease, which relies on pituitary ACTH, is the most common, accounting for about 76% of Cushing syndrome, more common in adults, and more in women than in men. The most common pituitary lesions are ACTH micro adenoma, and the rest is hypothalamic dysfunction. ACTH micro adenoma is not completely autonomous, but can still be inhibited by large dose of exogenous glucocorticoid, and can also be excited by CRH and vasopressin. In Cushing's disease, because of excessive ACTH stimulation, bilateral adrenal cortex diffuse proliferation, mainly producing glucocorticoid band hyperplasia and hypertrophy. Sometimes the reticular cell cells also proliferated. 2. ectopic ACTH syndrome is caused by malignant tumors outside the pituitary gland, producing ACTH, which stimulates adrenal hyperplasia and secretes excessive amounts of corticosteroids. The most common causes of ectopic ACTH syndrome are lung cancer (small cell or oat cell), bronchial carcinoid, thymic carcinoma, and pancreatic cancer. Clinically, it is divided into two types: slow progression and rapid progression. 3. the growth and secretion function of the adrenal cortical adenoma is independent and controlled by pituitary ACTH. Approximately 15%~20% of Cushing syndrome is more common in adults than in males. 4. adrenal cortical carcinoma accounts for 5% of Cushing syndrome, the condition is serious, and progress is fast. Clinically, there are severe Cushing syndrome, high blood pressure, low blood potassium, female hair, acne, clitoral hypertrophy, abdominal pain, back pain and other manifestations. 5. bilateral small nodular hyperplasia (dysplasia) that does not depend on ACTH is also called Meador syndrome or primary nodular pigmented adrenal disease. The majority of patients are children or young people. Some of the patients presented with general Cushing syndrome and another with familial dominant inheritance syndrome. ACTH in the blood was low or could not be measured. There are two factors of pathogenesis: heredity and immunity. 6. adrenal nodular hyperplasia that does not depend on ACTH. This syndrome is Cushing syndrome and can be seen in men or women of all ages. Plasma ACTH was low and high-dose dexamethasone test was not effective. Bilateral hyperplasia of adrenal glands with benign nodules above 5mm, non pigmented. (three) diagnosis and differential diagnosis 1. diagnostic criteria (1) the clinical manifestations of typical symptoms and signs, from the appearance of the diagnosis can be made. (2) all types of Cushing syndrome have abnormal glucocorticoid secretion, increase in cortisol secretion, loss of circadian rhythm and inhibition by small doses of dexamethasone. The urine 17 hydroxycorticosteroid (17 - hydroxy) in 55mmol / 24h or more, especially in the more than 70 mol / 24h, the greater significance of diagnosis; the urinary free cortisol in 304nmol / 24h above, can reflect the free cortisol levels in the blood, and less affected by other pigment interference. Low dose dexamethasone suppression test. The normal circadian rhythm of plasma cortisol disappeared, and the cortisol concentration was higher in the morning than at normal in the evening and lower than that in the morning. Etiological diagnosis of 2.Cushing syndrome should be familiar with the clinical features of various types of master, with imaging examination, blood and urine cortisol level, serum ACTH level and dynamic test results often can make the correct diagnosis and treatment. 3. differential diagnosis Most of the obese patients have high cortisol and 17 hydroxy, which can be suppressed by small dose of dexamethasone, and the circadian rhythm of cortisol remains normal, which can help to differentiate. The clinical manifestations of type 2 diabetes mellitus without Cushing syndrome, and the circadian rhythm of cortisol maintained normal. Alcoholism and liver damage, the biochemical abnormality disappeared after one week of abstinence. Clinical manifestations of Cushing syndrome in patients with depression. (four) treatment; Should be treated according to different causes 1.Cushing disease (1) transsphenoidal resection of pituitary micro adenoma is the first choice for the treatment of this disease. (2) complete adrenalectomy of one side and subtotal or subtotal adrenalectomy of the other side. Postoperative radiotherapy of pituitary gland was performed with radiotherapy. (3) pituitary adenoma, craniotomy, radiotherapy, radiotherapy. (4) drug therapy. 2. adenomas of the adrenal gland were surgically removed and treated with hydrocortisone or cortisone for an extended period of time. Three As early as possible, adrenal adenocarcinoma reduces the production of adrenal cortical hormone and is combined with medication. 4. do not rely on ACTH micronodular macronodular adrenal hyperplasia or bilateral adrenalectomy, postoperative hormone replacement therapy. 5. ectopic ACTH syndrome for primary tumor, depending on the condition for surgery or radiotherapy and chemotherapy. Take a synthetic block of the adrenal cortex hormone.