cortisol deficiency
Introduction
Introduction Psilotype anterior hypofunction (Simon-Siehan syndrome) is a clinical syndrome of multiple pituitary hormone secretion caused by multiple causes, secondary hypogonadism, thyroid, and adrenal insufficiency. Shehan syndrome is a common pituitary disease that occurs in women of reproductive age and has a history of postpartum hemorrhage and a history of shock. Conreux and Kan reported that Xihan syndrome can occur not only in vaginal delivery, but also after cesarean section. Today, when modern cesarean section rate rises, it should be highly valued by obstetricians. Pituitary ischemia caused by postpartum hemorrhage, infarct pituitary is more sensitive to hypoxia, vasospasm occurs easily in the high estrogen state, and postpartum hemorrhage causes hypotension, so the pituitary ischemic infarction. In recent years, with the improvement of childbirth and nursing techniques, the severe Xihan syndrome has been significantly reduced, but mild symptoms often appear in the years to ten years after delivery, or may not be diagnosed in the life without severe stress. In patients with Han syndrome, ischemic necrosis can occur in the pituitary gland (anterior pituitary), and then fibrosis forms scars. The remaining normal tissue is a non-secretory chromophobe. The pituitary gland (posterior pituitary) is often normal. . In Xihan syndrome, the gonadal function is reduced and the female ovary is significantly reduced. The uterus and breast are atrophied. The secretion of various hormones caused by pituitary lesions is drastically reduced, and thyroid hormones, adrenocortical hormones, and sex hormones are insufficiently secreted.
Cause
Cause
Due to postpartum hemorrhage, especially with long-term hemorrhagic shock, the pituitary tissue is hypoxic, degeneration and necrosis, followed by fibrosis, and finally the syndrome of hypopituitarism, the incidence of postpartum hemorrhage and hemorrhagic shock patients About 25%. Studies in recent years have shown that the occurrence of Sheehan's syndrome is not only related to hypopituitarism, Otsuka reported that 40% of patients showed signs of hypofunction of the whole pituitary gland, 50% of which showed neuropituitary function There are also varying degrees of anomalies. Pituitary tumors, hypothalamic lesions, pituitary avascular necrosis, surgical radiotherapy and trauma in the sellar region, infection and inflammation, long-term treatment of glucocorticoids, moderate pituitary apoplexy.
Hypertrophy of the pituitary gland during pregnancy, increased oxygen demand, peak during childbirth, so it is particularly sensitive to hypoxia. After delivery, the pituitary gland quickly relapses, blood flow is reduced, and the corresponding hormones secreted by it are also rapidly decreasing. Such as massive bleeding during childbirth, causing hemorrhagic shock, reduced blood supply or severance of the body, degeneration and necrosis of pituitary tissue cells, causing the hormones secreted by the pituitary gland and its target organs to be drastically reduced, resulting in the action of various hormones The function of the target organ degenerates prematurely and causes a series of syndromes. The pathological changes of pituitary dysfunction vary with the extent of postpartum hemorrhage and the length of the disease. Early lesions are hemorrhagic, degeneration, advanced fibrosis, or a small number of cystic epithelial cells. The thyroid gland, adrenal cortex, ovary, etc. are obviously atrophied. Less thyroid follicles, epithelial cells, flat adrenal cortical bundles and reticular band thinning ovary only a few primary follicles and white body, no ovulation uterus atrophy, thin muscle wall, thin endometrium, less glandular, interstitial fibrosis The remaining organs can also have different degrees of atrophy.
The pituitary gland is located in the sella, which is adjacent to the bottom of the third ventricle, the hypothalamus, and the optic chiasm. The lower part is the sphenoid sinus, and there are cavernous sinus, internal jugular vein and the 3rd, 4th, 5th and 6th pairs of cranial nerves on both sides. The pituitary gland mainly consists of two parts: the pituitary gland and the pituitary gland. Both have endocrine functions. Known hormones secreted by the pituitary are: thyroid stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), growth hormone (GH), luteinizing hormone (LH), follicle-stimulating hormone (FSH), prolactin (PRL), Human melanocyte stimulating hormone (MSH) and a variety of active peptides. Adult pituitary weight 0.6 ~ 0.8g, hypertrophy of pituitary hyperplasia during pregnancy, volume can be increased by 20% ~ 40%, weight can reach 1.0 ~ 1.2g, hormones secreted by the pituitary gland such as prolactin, thyrotropin, adrenocorticotropic hormone, The content of melanocyte stimulating hormone in the blood increased the oxytocin and vasopressin produced by the pituitary gland without change. Due to the increase of pituitary during pregnancy, the oxygen demand of the pituitary is increased, and the oxygen demand reaches a peak during childbirth, so it is particularly sensitive to hypoxia.
After delivery, the fetal placenta is excreted, and the concentration of various hormones secreted by the placenta in the blood suddenly drops. The pituitary gland rapidly relapses and blood flow decreases. At this time, if there is major bleeding, hemorrhagic shock, circulatory failure, due to the direct supply of arteries in the pituitary gland, and the pituitary gland supplies blood through the pituitary portal system, so in the case of major bleeding, the blood flow of the pituitary gland is sharply reduced, extremely easy Ischemic necrosis occurs. When hemorrhage occurs after childbirth, when shock occurs, the sympathetic reflex is happy to cause arterial spasm to contract or even occlude, so that the blood supply to the pituitary is significantly reduced, or even severed, thrombosis, resulting in a significant reduction in the supply of pituitary blood and hypothalamic hormones. Long can not restore blood supply in time, then the pituitary ischemic necrosis, loss of function.
Examine
an examination
Related inspection
Dynamic electrocardiogram (Holter monitoring)
Symptoms and signs
The pituitary gland has a strong compensatory function. There is no clinical symptom when the death range is <50%. The clinical symptoms are very small when the temperature is >75%. Only when the tissue necrosis exceeds 90%, there are obvious symptoms. If the damage area is 95%, then Symptoms are severe, and when the pituitary dysfunction is reduced, the most sensitive is the reduction of gonadotropin secretion, which in turn affects the secretion of thyroid stimulating hormone and adrenal hormone. The age of onset is mostly in the growth period of 20 to 40 years old, and amenorrhea can occur in 3 months to 32 years after delivery. The mother is more than the primipara, and the rural area is more than the city.
The levels of various hormones caused by pituitary lesions are reduced, the degree of which varies, and the corresponding clinical manifestations of target organ dysfunction are not completely parallel. The onset is different, the symptoms are different, the early symptoms are slightly, and the diagnosis is not easy. . Typical patients are: after the postpartum hemorrhagic shock puerperium, long-term aging fatigue, the first is no milk secretion, and then secondary amenorrhea, even if menstruation recovery, often rare, and secondary infertility. Loss of libido, vaginal dryness, difficulty in escaping. Pubic hair, hair loss, hair, eyebrows sparse, breast, genital atrophy, mental apathy, lethargy, dislike activity, slow response, chills, no sweat, dry and rough skin, loss of appetite, less food, constipation, low body temperature, pulse Slow, low blood pressure, pale, anemia. Most patients have edema, weight loss, and a few have weight loss.
With a history of postpartum hemorrhage or a history of shock, the above-mentioned typical clinical manifestations and signs can be diagnosed in combination with laboratory tests.
Laboratory inspection:
1. Pituitary hormone test: GH, FSH, LH, ACTH, PRL decreased.
2. Thyroid hormone detection: TT3, TT4, T3, T4, TSH are reduced.
3. Adrenal hormone test: Cortisol, urine 17-hydroxyl, 17-ketone decreased, fasting blood glucose decreased.
4. Sex hormone detection: estrogen, progesterone, testosterone propionate are reduced.
5. Blood routine: Hemoglobin, red blood cells, and hematocrit decrease.
6. Immunological test: It has not been confirmed that the occurrence of Xihan syndrome is related to autoimmunity. The immunological test indicates that the blood test is negative for pituitary antibody negative and pituitary peroxidase antibody negative.
7. Determination of pituitary reserve function
(1) Thyroid stimulating hormone releasing hormone (TRH) happy test: the principle is that TRH can stimulate the production of TSH and PRL in the anterior pituitary, and the TRH l00200g is dissolved in 2ml of normal saline intravenously, respectively, before and after injection. At 30 and 60 min, 3 ml of blood was drawn, and the base values of TSH and PRL and the change of the value after administration were measured. RESULTS: TSH peaked at 20 to 30 minutes after injection, and the peak value was 6.5 to 20.5 min/L. If there was no significant increase after injection of TRH, it indicated that the pituitary reserve function was insufficient. The PRL basal level was <25g/L, which increased to 40g/L 30min after injection of TRH. If there is no obvious increase or increase, it is not obvious, suggesting that the pituitary function is insufficient.
(2) luteal production of releasing hormone (LH) happy test: using LHRH 50 ~ 100g dissolved in 5ml saline intravenously, respectively, before injection and 15, 30, 60, 90min after injection, 3ml, using radioimmunoassay The method measures FSH and LH. FSH and LH increased by 2 to 4 times 30 min after normal injection. If there is no response, the function of pituitary gland is poor.
8. The remaining blood tests: lower blood sugar, lower hemoglobin, and higher eosinophils.
The remaining auxiliary inspections:
1. Imaging examination: Ultrasound examination shows uterine atrophy, ovarian small, no follicular development, and no ovulation. The X-ray of the cranial area showed no significant changes in the sella. The CT and MRI of the brain showed that the pituitary atrophy became smaller. MRI showed that 83% of the patients had a markedly smaller pituitary image, but their density was significantly reduced, even showing a cavity echo in the sellar region. It is called "empty saddle".
2. The rest: the basal body temperature drops, manifested as a single phase. Electrocardiogram, echocardiography, and cardiac function tests may have manifestations of myocardial ischemia.
3. Vaginal smear: shows low levels of estrogen.
4. Urine examination: 17-KS, 17-OH in urine was significantly reduced in 24 hours.
Diagnosis
Differential diagnosis
1. Primary hypogonadism: the common cause of primary hypogonadism is the vas deferens dysplasia associated with the 47,XXY karyotype, which is non-separatively reduced by the mother (a few are the father) The number splits to get an extra X chromosome. Clinical features are discussed in Section 261 Sex Chromosomal Abnormalities. Before the development of puberty, the diagnosis of most patients is still unclear, until the sexual development is abnormal or inspected due to infertility. When normal puberty is reached, gonadotropins increase, but testosterone is still below normal or at normal low levels.
2. Primary hypothyroidism: Thyroid hormone deficiency caused by thyroid disease is a common disease in daily life. This deficiency of thyroxine is different from edema caused by heart disease and kidney disease, and is characterized by the use of a finger to press the skin without leaving a depression.
3. Primary adrenal insufficiency: called Addison's disease, Chinese medicine belongs to the category of black and black. It is a systemic disease caused by hypofunction of the adrenal cortex, which is characterized by low blood pressure, malaise, skin and mucous membrane pigmentation.
4. Anorexia nervosa: also known as nervous appetite loss, characterized by long-term anorexia, significant weight loss. The age of onset is over 10 years old. Female adolescents are more common. If left untreated, it can lead to severe malnutrition and extreme exhaustion, affecting the physical and mental health and development of adolescents. The reason for the mental disorder that has not been fully ascertained is that in order to keep the body thin and long-term excessive dieting or refusal to eat, resulting in weight loss, nutritional deficiencies, metabolic and endocrine dysfunction, and serious life-threatening. More common in adolescents, the prevalence of women is much higher than that of men. Rehabilitation can be achieved with appropriate treatment.
5. Intracranial infection: Intracranial infections are mainly characterized by headache and meningeal irritation, including encephalitis, meningitis, toxic encephalopathy, and brain arachnoiditis. Dizziness is not necessarily a symptom. General vertigo is most common in purulent encephalitis, especially cerebellar abscess, posterior fossa arachnoiditis, and cerebellar cerebral horn arachnoiditis.
