Sheehan syndrome

Introduction to Sheen syndrome Multiple lesions of the pituitary or hypothalamus may involve the endocrine function of the pituitary. When all or most of the pituitary is destroyed, a series of manifestations of endocrine gland dysfunction may occur, mainly involving the gonads, thyroid gland and Adrenal cortex, clinically known as ateliortpituitaryhypofunction, also known as Sheehansyndrome, the most common cause is postpartum pituitary avascular necrosis and pituitary adenoma. Pituitary hyperplasia during pregnancy, increased oxygen demand, which 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. If hemorrhage occurs during childbirth, causing hemorrhagic shock, or even DIC, sympathetic reflex excitability causes arterial spasm and even occlusion, reducing the blood supply to the pituitary artery or severing, degeneration and necrosis of the anterior pituitary tissue, and the anterior pituitary and its The various hormones secreted by the target organs are drastically reduced, leading to premature degeneration of the target organs of various hormones and causing a series of syndromes. basic knowledge Sickness ratio: 0.1% Susceptible people: no special people Mode of infection: non-infectious Complications: Diabetes Diabetes insipidus Retinopathy

The cause of Sheen syndrome

(1) Causes of the disease

Pituitary hypofunction is caused by insufficient secretion of multiple or single pituitary hormones after the pituitary gland is damaged. It occurs in the latter case, which is called Sheehan syndrome. If the patient has insufficient neurohypophyseal hormone, it is called For total hypopituitarism (panhypopituitarism).

The etiology of this disease is complicated, and various diseases such as the hypothalamus, pituitary and its adjacent tissues, such as the pituitary gland, can cause this disease.

According to the location of the primary lesion, the disease can be divided into two categories: secondary hypopituitarism caused by hypothalamic release of hormone deficiency; primary pituitary hypofunction caused by pituitary disease itself According to the condition of hormone involvement, it can be divided into multi-hormone deficiency type and mono-hormone deficiency type. The former is more common. The latter includes simple GH deficiency, simple ACTH deficiency, simple LH/FSH deficiency and simple TSH deficiency, and simple GH. Lack of more common, polyhormone-deficient hypopituitarism is also known as combined pituitary hormone deficiency (CPHD), mono-steroid-deficient hypopituitarism is also known as solitary pituitary hormone deficiency (Table 1).

Primary hypogland hypofunction

(1) Congenital: Some congenital malformations can cause pituitary dysplasia, resulting in a variety of pituitary hormone deficiency. These diseases include: anencephaly, noropodence-phaly, de Morsier synthesis Signs, Hall-Pallister syndrome and Reiger syndrome, forebrain non-cracking malformations include cyclopia, cebocephaly, orbital hypotelorism, and de Morsier syndrome - Septo-optic dysplasia, the patient's septum pellucidum is absent, in the neonatal period, there is apnea, hypotonia, convulsions, persistent jaundice without hypoglycemia with hyperinsulinemia, Small penis (male), de Morsie I syndrome is caused by inactivation mutation of Hesx-1 gene, de Gauss syndrome patients with hypothalamic dysplasia, resulting in hypopituitarism, of which GH deficiency is the most common, but other Lack of pituitary hormones, some patients may also have diabetes insipidus, Hall-Pallister syndrome also has pituitary hypoplasia or even pituitary abscess, and may be associated with hypothalamic miscellaneous embryos Hamartoblastoma, Hall-Pallister syndrome also often has multiple finger (toe) malformations, nail dysplasia, epiglottis dysplasia, anal atresia, and abnormalities of the heart, lungs, kidneys, etc., Reiger syndrome except pituitary development In addition, there is a defect in the iris, which is prone to glaucoma and may be associated with abnormal development of the kidney, gastrointestinal tract and umbilicus. This syndrome is caused by a mutation in the Ptx-2 gene. In addition, cleft lip and cleft palate may also be combined with GH deficiency. According to foreign data, about 4% of cleft lip and cleft palate have GH deficiency, and mutations of transcription factors Pit-1 and Prop-1 can also cause pituitary dysplasia, which causes a variety of pituitary hormones.

In addition, mutations in some pituitary hormone genes can cause a deficiency in the corresponding hormone, for example, mutations in the GH-1 gene can cause a deficiency in GH.

(2) pituitary tumors: pituitary tumors are the most common cause of this disease. Pituitary adenomas are the most common pituitary tumors. Various pituitary adenomas can cause hypopituitarism by compressing normal pituitary tissues and pituitary stalks. Non-functioning adenoma hormones have high secretion symptoms, and the onset is late, which is most likely to cause hypopituitarism. Other pituitary tumors and tumors adjacent to the pituitary gland can also cause hypopituitarism. These tumors include craniopharyngioma. , Rathke cyst, dermoid cyst, ganglion cell tumor, paraganglioma, nasal glioma (esthesioneuroblastoma), sarcoma, lipoma, hemangiopericytoma, embryonic cell tumor and so on.

Pituitary dysfunction caused by pituitary tumors is the earliest and most common symptom of secondary hypogonadism caused by gonadotropin deficiency. Secondly, secondary hypothyroidism caused by insufficient TSH; secondary adrenal cortex caused by insufficient ACTH Symptoms of hypofunction are generally milder, and less common, GH can also be reduced, but the lack of specificity in adult performance is often overlooked.

(3) pituitary apoplexy: pituitary apoplexy refers to ischemic necrosis or hemorrhage of pituitary tissue. Pituitary tumor and postpartum hemorrhage are the most common causes of pituitary apoplexy. Arteriosclerosis, especially diabetes, combined with arteriosclerosis is also prone to pituitary apoplexy. Such as radiation exposure, trauma and other less caused by pituitary apoplexy, the secretion function of the pituitary after the pituitary apoplexy decreased, thereby causing the disease.

(4) Infection: bacterial (pituitary tuberculosis, pituitary abscess, etc.), fungal, viral (encephalitis, epidemic hemorrhagic fever, etc.) and spirochete (syphilis, etc.) infection can cause hypopituitarism.

(5) invasive lesions: some invasive lesions such as hemochromatosis, sarcoidosis, Wegener granulomatosis can affect the pituitary gland, resulting in hypopituitarism, lymphocytic pituitary inflammation is also an invasive lesion.

(6) External injury: The injury of the pituitary can damage the pituitary tissue and cause its function to decline.

(7) pituitary surgery: excessive pituitary tissue or pituitary injury during pituitary surgery can cause hypopituitarism.

(8) Radiation injury: If the pituitary tumor is treated with radiation, if the dose is large, it will easily cause the function of the pituitary gland, and the incidence will increase year by year. The radiation therapy of other intracranial or extracranial tumors can also be produced. Glandular hypofunction.

(9) Other diseases: empty saddle syndrome, internal carotid aneurysm, cavernous sinus thrombosis, etc. can also cause hypopituitarism.

(10) idiopathic: idiopathic hypopituitarism etiology is unknown, some patients have perinatal abnormalities, such as breech production, transverse production, forceps, midwifery, etc., may be due to these Abnormal factors cause fetal pituitary damage, and MRI shows that the pituitary gland and pituitary stalk become smaller.

2. Secondary hypogonadal hypofunction The hypothalamic or other parts of the disease, such as causing hypothalamic pituitary release hormone secretion or ineffectively acting on the pituitary gland can also produce hypopituitarism, is secondary to hypogonadal hypofunction.

(1) pituitary stalk lesions: external injury, surgery can damage the pituitary stalk; pituitary and its adjacent tumors can compress the pituitary stalk, both of which can cause dysfunction of the pituitary portal system, making the hypothalamic pituitary release hormone not effective Acts on the pituitary gland, causing hypofunction of the pituitary gland.

(2) Hypothalamus and its adjacent lesions: various lesions of the hypothalamus, such as tumors, infections, invasive lesions, radiation damage, trauma, surgery, etc., can cause hypothalamic secretion of pituitary hormones, resulting in pituitary function. Decrease.

Kallmann syndrome is also a hypothalamic pituitary hypofunction. The disease-causing gene of this disease has been cloned and located in the Xp22.3 region of the X chromosome. The encoded product is a neuron migration protein, and the gene is deleted or Mutations can cause migration disorders in GnRH neurons, resulting in insufficient secretion of LH/FSH.

(3) Functionality: malnutrition, hyperkinesia and anorexia nervosa can cause hypothalamic dysfunction, resulting in insufficient secretion of GnRH in the hypothalamus, resulting in insufficient LH/FSH. Mental stress can cause hypothalamic dysfunction in children, GHRH Repressed, thus causing insufficient secretion of GH, various critical illnesses can reduce the production of TRH in the hypothalamus, and the secretion of TSH in the pituitary gland decreases. The long-term use of glucocorticoids inhibits CRH in the hypothalamus and decreases the secretion of ACTH.

(two) pathogenesis

The occurrence of hypopituitarism can be caused by:

1 pituitary lesions cause decreased secretion of pituitary hormones, which is a primary pituitary dysfunction,

2 hypothalamic lesions, hinder the secretion of hormones (or factors) of the hypothalamic pituitary hormones,

3 The hypothalamic-pituitary connection (pituitary portal system) is interrupted, and the hypothalamic pituitary hormone releasing hormone cannot reach the pituitary gland, and the pituitary cells are dysfunctional due to lack of excitement; the latter two are collectively referred to as Secondary hypogonadal hypofunction.

The pituitary gland of a normal person weighs about 0.5 g. The pituitary gland has an independent blood supply. The pituitary gland is mainly supplied by the superior pituitary artery of the internal carotid artery. The superior pituitary artery forms an arterial ring at the root of the pituitary stalk. Branches enter the hypothalamus and median carina, and also constitute the first microvascular plexus of the pituitary portal system, so the portal vein is connected to the hypothalamic median carina, the lower pituitary gland, pregnancy due to placental prolactin, estrogen Stimulation, prolactin secretion of prolactin (PRL), pituitary hyperplasia hypertrophy, volume increased 2 to 3 times before pregnancy; hypertrophic pituitary body is limited by bone, in the acute ischemic swelling is very easy to damage, plus There is no overlapping of the pituitary portal vessels, and it is not easy to establish collateral circulation during ischemia. Therefore, the blood supply to the pituitary, the integrity of the neuroendocrine center and pituitary stalk of the hypothalamus, and the lesions occupying the space of the pituitary fossa can all cause this. disease.

1. Postpartum pituitary necrosis and atrophy, hemorrhage occurs during childbirth, such as placenta retention, placenta previa, etc., easy to cause blood flow of the pituitary gland and pituitary vasospasm, resulting in pituitary necrosis or atrophy, occurs in postpartum pituitary dysfunction Known as Sheehan syndrome, the annual incidence rate in the United States is about 1 in 1 million. The incidence rate in developing countries is high. It can also occur in eclampsia, amniotic fluid embolism, septic shock, etc. during childbirth. In the case of disseminated intravascular coagulation, in addition, due to vascular disease and hypercoagulable state, diabetic patients are more prone to pituitary ischemic infarction (called Houssay phenomenon), patients need less insulin, other vascular lesions such as Rheumatic diseases, sickle cell anemia, cavernous sinus embolism and carotid aneurysm can also cause this disease.

In recent years, it has been reported that pregnancy or postpartum can be complicated by autoimmune lymphocytic pituitary inflammation. The lesions are often found on CT scans. The biopsy shows that the lesion is composed of lymphocytic infiltration. Lymphocytic pituitary inflammation is caused by autoimmune pituitary damage. Often accompanied by other autoimmune diseases such as Hashimoto (autoimmune) thyroiditis and gastric mucosal atrophy, anti-prolactin cell antibodies in some patients have also been discovered, although only 30 cases of lymphocytic pituitary have been reported. Inflammation, but about 7% of patients with other autoimmune diseases, there are prolactin antibodies in the serum, it is not clear whether autoimmune pituititis, is a common cause of adult "idiopathic" hypopituitarism.

2. Pituitary and hypothalamic tumors can cause hypopituitarism, the most common in adults are chromophobe adenomas, the most common in children are craniopharyngioma, in addition to other tumors such as meningioma, ectopic pine Glandular tumors, gliomas, etc., pituitary large adenomas can cause pituitary hypoxia by destroying the pituitary gland, hypothalamic nucleus, nerve bundle or portal vein, while pituitary microadenomas generally cause hyperfunction.

3. Infection or invasive disease infection can damage the pituitary gland in different ways, for example: pituitary abscess, syphilis, tuberculosis can directly destroy the pituitary gland, encephalitis, meningitis can affect hypothalamic pituitary hormone production or release pituitary, Leukemia, lymphoma, xanthoma, sarcoidosis, etc., can also be complicated by hypopituitarism.

4. Surgery, radiotherapy, craniocerebral traumatic pituitary hypofunction, may also be the consequences of therapeutic pituitary resection, pituitary tumors or diabetic retinopathy, metastatic breast cancer, etc., after therapeutic pituitary resection, pituitary can occur Low function.

In addition, when radiotherapy is performed on tumors of the head and neck, if the hypothalamus and pituitary are in the field, the pituitary dysfunction may occur several years later. After the usual amount of radiotherapy for pituitary tumors, many patients may be found after long-term follow-up. Hypothalamic and pituitary function is low.

In patients with severe traumatic brain injury, the fracture of the pituitary stalk or the interruption of the pituitary portal vein due to the skull base fracture may be accompanied by dysfunction of the anterior and posterior pituitary. For those who have obvious polyuria after the traumatic brain injury and persistent coma, consider it. The possibility of concurrent pituitary necrosis, a small number of patients surviving after trauma, can also occur after traumatic hypopituitarism.

Sheen syndrome prevention

Suspected pituitary crisis cases, banned or used narcotics such as morphine, barbital sleeping pills, chlorpromazine and other central nervous system inhibitors and various hypoglycemic drugs to prevent coma, for mental disorders or unconscious, should be strengthened Safety precautions to prevent accidents, provide a reasonable diet, and guarantee nutrition.

Prevention of pituitary dysfunction caused by certain causes can be prevented from getting sick by strengthening preventive measures. For example, improving the health level of pregnant women can reduce the hypogland hypofunction caused by postpartum pituitary necrosis; improving the level of brain surgery and radiation therapy Helps reduce the hypopituitary dysfunction caused by these factors.

Sheen syndrome complications Complications Diabetes diabetes insipidus retinopathy

1. The etiology of diabetic pituitary dysfunction, mostly pituitary necrosis caused by postpartum hemorrhage, or pituitary migratory abscess necrosis, sarcoidosis involving pituitary and cancer infiltrating pituitary, the etiology and systemic arteries of many cases Hardening involves the pituitary, causing thrombosis, vascular damage in diabetes may be the basis of the disease, clinical manifestations according to the urgency of hypopituitarism, can be divided into two types:

(1) Chronic type: It is manifested that diabetic patients become particularly sensitive to insulin during treatment, prone to hypoglycemia, reduce the need for insulin, and gradually appear various manifestations of pituitary hypofunction.

(2) acute type: the onset of pituitary hypofunction is rapid, due to pituitary hemorrhage or pituitary abscess and acute necrosis, etc., in addition to the symptoms caused by local pituitary lesions, often manifested as sudden disappearance of diabetes, hypoglycemia and coma .

Retinal vasculopathy can be improved after diabetes patients with pituitary dysfunction, which is related to the elimination of growth hormone. Growth hormone can aggravate diabetic vascular disease, so pituitary resection can be used to treat retinopathy of diabetes.

2. Surgery, tumor, inflammation, etc. in the hypothalamus or pituitary area of diabetes insipidus can cause both diabetes insipidus and pituitary dysfunction, vascular lesions that cause postpartum pituitary necrosis, and sometimes damage the supraoptic nucleus pituitary Combined with diabetes insipidus, pituitary dysfunction has a significant effect on the condition of diabetes insipidus. After the disease, polyuria is relieved and urine osmotic pressure is also higher; the patient's glomerular filtration rate is reduced, the kidney is concentrated and Dilution function is limited, the ability to drain water load is weakened; urine volume depends on the amount of solute excretion, the main reason for this phenomenon is glucocorticoid deficiency, glucocorticoids and vasopressin antagonism, but the mode of action It is not clear that some people think that glucocorticoids can inhibit the secretion of vasopressin. Others believe that glucocorticoids may act on the kidneys and prevent the return of water in the collecting duct. Therefore, when glucocorticoids are deficient, vasopressin deficiency The phenomenon is also alleviated. In addition, when glucocorticoids and thyroxine are reduced, the excretion of solute is reduced, which is also a cause of reducing polyuria. It should be noted that when these two conditions are combined, the symptoms of diabetes insipidus are aggravated after supplementation with glucocorticoids, and the need for vasopressin is increased. For patients with diabetes insipidus, if the condition is relieved, consider The possibility of hypopituitarism.

3. Patients with mild hypopituitarism in pregnancy, especially after hormonal supplementation of the thyroid, adrenal cortex and gonads, may occasionally be pregnant, which may be due to hormone supplementation treatment, the body's metabolic status is improved, residual The pituitary tissue has recovered some functions. During pregnancy, because the placenta can produce a variety of hormones, the pituitary tissue can also proliferate, so the performance of the pituitary hypofunction can be alleviated. At the time of delivery, close attention should be paid to avoid the occurrence of crisis. Adrenal cortex hormone should be supplemented when necessary. After delivery, the pituitary hypofunction can be improved, but it may return to its original condition or even worse.

4. Can be complicated by infection, pituitary crisis and coma, pituitary crisis, coma treatment: hypoglycemic patients use 50% glucose solution, intravenous injection, followed by 10% glucose solution 500ml hydrogenated cortisone 100 ~ 200mg intravenously Infection, circulatory failure, hypotension, supplementation of the required amount of glucose saline hydrocortisone in shock, antibiotics, booster drug intravenous infusion, if necessary, blood transfusion, hypothermia should be kept warm, application of triiodothyronine ammonia Acid, 25ml each time, intravenously, once every 6 hours, while using hydrocortisone 50 ~ 100mg intravenous drip, hydrochloric acid application of hydrocortisone 100 ~ 150mg intravenous drip.

Sheen syndrome symptoms Common symptoms Postpartum extreme weakness, pubic hair loss, chills, fatigue, postpartum pituitary necrosis, madness, unexplained, fever, convulsions, head, dizziness, confusion

1. Postpartum pituitary necrosis cases of hemorrhage due to dystocia during childbirth, fainting, history of shock, or infection during childbirth, patients are extremely weak after childbirth, breasts are not swelled, no milk secretion, may have hypoglycemia symptoms, pulse speed Less urine, blood urea nitrogen can be elevated, can be complicated by pneumonia and other infections, postpartum systemic conditions can not be restored, menstruation no longer, and gradually appear sexual dysfunction and thyroid, adrenal insufficiency symptoms.

Pituitary tumors can cause headaches, visual impairment, and sometimes intracranial hypertension syndrome. The following symptoms can occur when the lesions involve the hypothalamus:

(1) Anorexia nervosa or polyphagia, or alternating between the two.

(2) increased drinking water (due to diabetes insipidus or neurological polydipsia); may also have a thirst or no thirst (thirst center in the anterior hypothalamus near the supraoptic nucleus).

(3) often sleepy during the day, insomnia at night.

(4) Unexplained fever or low temperature.

(5) Loss of libido or hyperactivity.

(6) sphincter dysfunction (constipation).

(7) Mental metamorphosis.

(8) Inter-cerebral epilepsy, convulsions.

(9) Excessive sweat or no sweat.

(10) Hand and foot cyanosis (due to vasomotor dysfunction).

(11) tachycardia, arrhythmia or insufficient blood supply to the coronary arteries (also vasomotor dysfunction).

(12) The activity ability is low, so that they do not want to be active. Others caused by surgery, trauma, inflammation, etc., each have their own special medical history.

2. The performance of pituitary dysfunction The severity of pituitary dysfunction is related to the degree of pituitary destruction. In general, pituitary tissue loss is 95%, clinical manifestations are severe, loss of 75% is moderate, loss of 60% is Mild, loss of 50% or less will not cause symptoms of dysfunction, however, the above relationship is not absolute, sometimes the pituitary gland is nearly completely destroyed, and the patient's endocrine function decline is not very serious, or even pregnant again, or survive 30 ~ 40 years old.

The phenomenon of insufficient secretion of various hormones in the pituitary gland gradually appears. Generally, symptoms of prolactin, gonadotropin, and growth hormone deficiency occur first, followed by thyroid stimulating hormone, and finally adrenocorticotropic hormone, sometimes the symptoms of adrenal insufficiency may appear early. In hypothyroidism.

(1) Insufficient secretion of prolactin: After delivery, the breasts are not inflated and no milk is secreted.

(2) Insufficient growth hormone secretion: In adults, it is mainly prone to hypoglycemia, because growth hormone has a blood sugar effect.

(3) Insufficient secretion of gonadotropin: in female patients, manifested as amenorrhea, loss of libido or disappearance, breast and genital atrophy, loss of fertility, the difference between amenorrhea and general menopausal women in this disease is no vasomotor disorder For example, paroxysmal facial flushing, male patients showed secondary sexual deterioration, such as pubic hair sparse, soft voice, muscle underdevelopment, increased subcutaneous fat, and testicular atrophy, sperm development stopped, scrotal hypopigmentation, external genitalia , prostate shrinkage, loss of libido, impotence and so on.

(4) Insufficient thyroid-stimulating hormone secretion: pale complexion, facial aging, sparse eyebrows, pubic hair, pubic hair loss, dry skin, thin and atrophy, or edema, but less mucinous edema; indifferent expression, unresponsive , low pitch, mental decline, chills, sometimes illusion delusions, mental disorders, even madness, slow heart rate, low voltage of ECG, can appear T wave flat, inverted, the heart does not expand, often narrowed, can be Identification of primary hypothyroidism.

(5) Insufficient secretion of adrenocorticotropic hormone: mainly affects the secretion of glucocorticoids, cortisol reduction, patient weakness, fatigue, loss of appetite, nausea and vomiting, upper abdominal pain, weight loss, weak heart sound, slow heart rate, lower blood pressure, intolerance Hunger, prone to hypoglycemia, poor body resistance, prone to infection, prone to shock after infection, coma, adrenocortical hormone deficiency, glucocorticoids are most affected, secretion is significantly reduced, and mineralocorticoid aldosterone The effects are not as severe as glucocorticoids. Under the basal state, there is still a certain amount (although less than normal) aldosterone secretion, and can also preserve sodium; when the sodium intake is reduced, the adrenal cortex can still increase aldosterone secretion. The response, although not as fast as normal, does not reach normal levels, the effect of sodium sputum is slightly worse than normal, but there is still a certain ability of sodium sputum, so patients with hypopituitarism, unlike primary adrenal function As a result of hypotension, severe sodium loss is likely to occur. Due to the lack of cortisol, the patient's ability to drain water is reduced. This is related to the fact that patients generally do not like drinking more water. Patients often have hyponatremia, especially when the condition is aggravated or ingested. After injecting too much water, the cause is mainly due to renal drainage disorder, water retention, body fluid dilution, and blood. If the sodium is too low, if the intake of sodium is reduced and/or lost, the hyponatremia can be aggravated and dehydration can be caused.

(6) Insufficient secretion of melanocyte stimulating hormone: Both melanocyte stimulating hormone and adrenocorticotropic hormone have the effect of promoting skin pigmentation. Due to the lack of these two hormones, the skin color is light, even if exposed to sunlight. The skin pigmentation will not be deepened, and the darker parts of the normal pigments, such as the areola, the color of the midline of the abdomen will become more pronounced. A few patients may have dark brown spots, irregular edges, no characteristic features, and chronic adrenal glands. There is a significant difference in the pigmentation of cortical hypofunction. Sometimes there is yellow pigmentation at the fingertips, which may be related to carotene deposition.

3. Pituitary crisis If the patient is not diagnosed and treated in time, and develops to the later stage, the crisis may occur due to various inducements, and there are many types of clinical symptoms: pituitary crisis.

(1) hypoglycemic coma: the cause may be spontaneous, that is, due to eating too little or not eating, especially when there is infection; or insulin induced (for islet tolerance test or insulin treatment of appetite) Insufficient); or due to high glucose diet or injection of large amounts of glucose, causing hypoglycemia caused by endogenous insulin secretion, patients with this disease due to lack of cortisol, reduced glycogen storage, decreased growth hormone, increased sensitivity to insulin, In addition, the thyroid function is reduced, the absorption of glucose in the intestine is reduced, so the fasting blood glucose is low in normal times. Once the above situation occurs, it is easy to cause hypoglycemia and coma. This type of coma is the most common, and the patient is weak when hypoglycemia occurs. Dizziness, dizziness, sweating, palpitation, pale, can have headache, vomiting, nausea, blood pressure is generally low, severe can not be measured, can be irritated or unresponsive, pupils reflect light, reflexes disappear after initial hyperactivity The stroke test can be positive, and there may be an increase in muscle tone or paralysis, convulsions, and a coma in severe cases.

(2) infection-induced coma: patients with this disease due to the lack of a variety of hormones, mainly lack of adrenocorticotropic hormone and cortisol, so the body's resistance is low, prone to infection, in the concurrent infection, high fever, prone to unconsciousness Coma, low blood pressure and shock, most of the loss of consciousness caused by infection is gradually appearing, the body temperature can be as high as 39 ~ 40 ° C, the pulse often does not increase correspondingly, blood pressure is reduced, systolic blood pressure is often below 80 ~ 90mmHg, when severe Shock occurred.

(3) sedation, coma caused by anesthesia: the patient is very sensitive to sedation and anesthesia. The commonly used dose can cause the patient to fall into a long period of slumber and even coma, pentobarbital sodium or thiopental, morphine, Phenobarbital and meperidine can cause coma, and long-term lethargy can also occur after receiving a general therapeutic dose of chlorpromazine (oral or intramuscular).

(4) sodium loss coma: sodium loss caused by gastrointestinal disorders, surgery, infection, etc., can promote a crisis like primary adrenal insufficiency, this type of crisis coma is particularly significant in peripheral circulatory failure It is worth noting that patients with this disease may have increased excretion of sodium in the first few days after starting the application of corticosteroids, probably because the glomerular filtration rate is very low, and it has been improved after treatment. Less than 1 week after treatment with cortisol, the patient entered a coma with a significant negative balance of sodium. In addition, when the thyroid preparation was used alone, especially when the dosage was too large, the body's need for adrenocortical hormone increased due to an increase in metabolic rate. The increase in the amount, the lack of adrenocortical hormone is more serious, on the other hand, thyroid preparations in the hypothyroidism have promoted solute excretion, resulting in loss of water, loss of sodium.

(5) Toxic coma in water: The patient has drainage disorder. When the water is too much, water retention can occur, and the extracellular fluid is diluted to cause hypotonic state. Then the water enters the cell, and the introduced water contains too much water and the cells are swollen. Cell metabolism and dysfunction, excessive water in nerve cells, can cause a series of neurological symptoms, the occurrence of this situation can be spontaneous, can also be caused by water diuretic test, especially the original patient blood sodium concentration has been It is more likely to occur when it is very low. Therefore, blood sodium should be measured before the water test. Those with low blood sodium should not be tested. The clinical manifestations of water poisoning are weak, lethargy, loss of appetite, vomiting, mental disorder, convulsions, and finally fall into a coma. This type of coma is different from the crisis caused by salt loss. The patient has no signs of dehydration. Instead, there may be edema and weight gain. If there is no obvious loss of sodium, the blood circulation remains normal, the blood volume is reduced, the serum sodium concentration is lowered, and the potassium is lowered. Normal or reduced, generally no acidosis or azotemia.

(6) hypothermia coma: some patients feel ambiguous in the winter, when exposed to cold, can induce coma, or prolong the coma that has occurred, such crisis often occurs in winter, slow onset, gradually enter Coma, body temperature is very low, using ordinary thermometer to measure temperature does not rise, you must use the thermometer used in the laboratory to measure the rectal temperature, only to know its low temperature, can be as low as 30 °C.

(7) coma after pituitary resection: after pituitary resection for pituitary tumor or metastatic breast cancer, severe diabetic retinopathy, etc., patients may develop coma, patients with pituitary dysfunction before surgery, more prone to pituitary resection Postoperative coma can cause disturbance of consciousness due to local injury, or due to hypofunction of endocrine glands, especially pre-operative adrenal insufficiency, can not tolerate severe stimulation caused by surgery, or water and electrolyte metabolism before and after surgery Disorder, the patient can not recover after surgery, is in a state of lethargy or coma, can last for several days or even months, incontinence, may still respond to painful stimulation, sometimes temporarily awaken, holding reflection and sucking reflex disappears, The pulse rate and blood pressure can be normal or slightly lower, the body temperature can be high or low, or normal, and the blood sugar and blood sodium can be normal or slightly lower.

(8) pituitary apoplexy: rapid onset, headache, dizziness, vomiting, and then into coma, due to acute hemorrhage in the pituitary tumor, hypothalamic and other life centers are compressed, the occurrence of pituitary hypofunction coma, The main reason is the metabolic disorder caused by the lack of various hormones. The body's ability to resist various stimuli is weak. The maintenance of consciousness depends on the integrity of some nerve centers in the cerebral cortex, thalamus, hypothalamus and midbrain reticular structure. If there is a disorder in the metabolism of nerve cells in these centers of consciousness, confusion or loss of consciousness occurs, and the maintenance of normal neuronal metabolism depends mainly on the oxidation of glucose and glutamate under the catalysis of some specific enzymes. Patients with hypopituitarism have biochemical changes, such as hypoglycemia, hyponatremia, and sometimes due to trauma, compression or edema, which impede the delivery of oxygen and nutrients to the above-mentioned nerve centers. Adrenal cortex hormones and thyroxine deficiency can cause Neuronal cell metabolism has a disorder, and coma is more common in severe peripheral endocrine gland function reduction. In patients with adrenal insufficiency, adrenal cortical function can only cope with the needs of patients with very low metabolic status. Once severe stimulation occurs, adrenal insufficiency occurs. Adrenal cortex hormones are most effective in treating patients with coma. Good, after the patient was treated with adrenal cortical hormone replacement therapy, the incidence of coma was significantly reduced.

Sheen syndrome check

Laboratory examination

(1) Pituitary hormone detection decreased GH, FSH, LH, ACTH, PRL.

(2) Thyroid hormone test TT3, TT4, T3, T4, TSH decreased.

(3) Adrenal hormone test blood cortisol, urinary cortisol decreased, fasting blood glucose decreased.

(4) Sex hormone detection decreased estrogen, progesterone and testosterone.

(5) Blood routine often has hemoglobin, red blood cell reduction, and hematocrit decline.

(6) Immunological tests have not confirmed that the occurrence of Sheehan syndrome is related to autoimmunity. Immunological tests indicate that the patient's blood test is negative for pituitary antibodies and negative for pituitary peroxidase antibodies.

2. Imaging examination

Ultrasound examination showed 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 the density was significantly reduced, even in the sellar region. It is called "empty saddle".

Sheen syndrome diagnosis and identification

Diagnostic criteria

Clinical features

(1) There are multiple endocrine target hypogonadism syndromes, each of which may exist alone or simultaneously (depending on the extent and extent of pituitary damage).

1FSH, LH and PRL insufficient secretion syndrome: postpartum no milk, breast atrophy, amenorrhea, the first appearance of the disease, hair often shedding, male with impotence, loss of libido or disappear, female genital atrophy, male testicular soft Zoom out.

2TSH secretion deficiency syndrome: as the clinical manifestations of primary hypothyroidism, but generally lighter, serum TSH levels decreased as its main identification point.

3ACTH secretion deficiency syndrome: like primary adrenal insufficiency, often fatigue, anorexia, weight loss, but skin tone is light, serum ACTH level is normal or reduced as its identification point.

(2) Ask about the medical history related to the cause, pay attention to related signs, such as pituitary tumors, often have vision, visual field changes.

(3) Such patients may be classified into hypoglycemia, circulatory failure, low temperature, water due to infection, trauma, surgery, excessive drinking water, malnutrition, sedatives or insulin, cold, vomiting, diarrhea, etc. Poisoning type.

2. Check

(1) blood routine, blood electrolytes, blood glucose measurement: pituitary dysfunction coma can be misdiagnosed as cerebral thrombosis due to the gradual emergence of coma, misdiagnosed as meningitis due to neck stiffness, misdiagnosed as epilepsy due to convulsions, due to The pulse is slow and misdiagnosed as cardiogenic cerebral ischemic syndrome (A-S syndrome), which is misdiagnosed as diabetic coma due to hunger-induced ketonuria. It has been misdiagnosed as anesthetic poisoning because of taking an anesthetic. Patients who are not clear about the cause of coma should be vigilant, taking into account the possibility of hypopituitarism, and make detailed medical history and comprehensive examination.

(2) Determination of pituitary hormones and target glands.

(3) If necessary, perform pituitary reserve function tests, such as stimulating growth hormone (GH), prolactin (PRL), insulin hypoglycemia test (there is a certain risk, careful monitoring of blood glucose), luteinizing hormone releasing hormone (LHRH) Excitatory test, TRH excitement test.

(4) suspected lesions, can be used for fundus examination, visual field examination, head positive, lateral position, CT, magnetic resonance examination.

Differential diagnosis

1. Anorexia nervosa Anorexia patients have weight loss, amenorrhea, due to neurological disorders and malnutrition can affect pituitary function, some symptoms similar to pituitary dysfunction, but the characteristics of this disease are mostly around 20 years old, have mental stimulation History, its weight loss is heavier than the pituitary dysfunction, while the mane, pubic hair often does not fall off, urine 17-ketosteroids and urine 17-hydroxycorticosteroids are normal or only slightly reduced.

2. Primary hypothyroidism Primary hypothyroidism, in addition to thyroid function, other endocrine gland function may also be low, so can be mistaken for pituitary hypofunction, the identification of the two is primary Visceral edema of hypothyroidism is more obvious, blood cholesterol concentration is more obvious, the heart tends to expand, TSH stimulation test: primary hypothyroidism TSH overreaction, hypogland hypofunction can be no TSH elevated response, under Thalamic patients have a delayed response. The most valuable value is the determination of thyroid stimulating hormone in plasma, which is elevated in primary hypothyroidism and undetectable in hypopituitarism.

3. Chronic adrenal insufficiency The identification of chronic adrenal insufficiency and pituitary hypofunction is: the former has typical skin, mucosal pigmentation, and sexual atrophy and hypothyroidism are not obvious, on the adrenal gland Corticosteroids do not respond, and sodium loss is more serious.

4. Autoimmune multiple endocrine glands in this patient, there are a variety of endocrine gland dysfunction, but the cause is not due to pituitary dysfunction, but due to multiple primary endocrine gland dysfunction, and pituitary gland The identification of dysfunction is mainly based on the adrenocorticotropic hormone and thyroid stimulating hormone excitatory test. In this group, there is no response, and in the pituitary hypofunction, there is often a delayed response.

5. Chronic wasting disease may be associated with weight loss, fatigue, sexual dysfunction, low urinary 17-ketosteroids, severe malnutrition, and even secondary hypofunction of the pituitary gland. Gradually recover.

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