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Internal Medicine - Endocrinology - Hypothalamic and Pituitary Diseases
Hypothalamic pituitary disease is a common endocrine disorder, the main symptoms of which are disorders of pituitary hormone secretion. It’s full of useful information, friends in need should quickly collect it!
Modificato alle 2024-03-09 13:29:11
Internal Medicine - Endocrinology - Hypothalamic and Pituitary Diseases
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hypothalamic pituitary disease
Review relevant basic knowledge
Pituitary gland: approximately 1.2cm x 1.0cm x 0.5cm in size, with an average weight of 750mg (males 350-700mg, females 450-900mg), located in the sella turcica at the base of the skull. It is connected to the hypothalamus above through the pituitary stalk. Crossing the sella septum anteriorly and superiorly is the optic chiasm diagram. The cavernous sinuses on both sides have the passages of the III, IV, V (2, 4) cranial nerves and the internal carotid artery. The sellar floor is the top of the sphenoid sinus
blood supply to pituitary gland
Anterior pituitary - superior hypophyseal artery First microvascular plexus (median eminence) pituitary portal vein Second microvascular plexus (anterior pituitary) Posterior pituitary - inferior hypophyseal artery
pituitary hormone
Front lobe: ACTH (adrenocorticotropic hormone) TSH (thyroid stimulating hormone) FSH (follicle-stimulating hormone) LH (luteinizing hormone) PRL (prolactin) GH (Growth Hormone) MSH (Melanin) Posterior lobe: ADH (antidiuretic hormone OXT (Oxytocin)
Hypothalamic disease (hypothalamus)
Hypothalamus anatomy and function
Hormones secreted by the hypothalamus and their regulation
Growth hormone-releasing hormone (GNRH)—stimulates growth hormone (GH) Somatostatin (SS)—inhibits GH, insulin, and glucagon Prolactin release inhibitory factor (PIF)—inhibits PRL Thyrotropin-releasing hormone (TRH)—stimulates TSH, PRL Corticotropin-releasing hormone (CRH)—stimulates ACTH Gonadotropin-releasing hormone (GnRH)—stimulates LH and FSH Antidiuretic hormone (ADH) regulates water metabolism Oxytocin (OXT) promotes uterine smooth muscle contraction, breast lactation, etc. ...
Hypothalamic diseases and clinical manifestations
manifestations of endocrine dysfunction Nervous system manifestations: eating, drinking, sleeping, warmth, nervousness, sweating
Hypothalamic related diseases
Pituitary tumor
1. Definition
Pituitary tumors: are a group of tumors originating from the adenohypophysis, Neurohypophysis and embryonic craniopharyngeal duct remnants Tumors of squamous epithelium.
2. Causes and pathogenesis
1. Genetic factors: MEN1, gene mutation, prop-1 overactivation, etc. 2. Hypothalamic factors: such as excess GHRH, excess CRH, certain hypothalamic hormones Activating mutations of receptors, etc. 3. Pituitary factors: such as signal transduction molecule mutations and excessive growth factors. Oncogenes activate tumor suppressor genes and inactivate them. 4. Environmental factors: radiotherapy. 5. Target gland failure: thyroid, gonads, adrenal glands.
Initial stage: The pituitary cells themselves are defective. Promotion stage: Hypothalamic regulation disorders and other factors play an important role, that is, a certain pituitary cell mutates, leading to the activation of oncogenes and/or inactivation of tumor suppressor genes. Under the promotion of internal and external factors, monoclonal mutant cells continue to proliferate, and gradually Develop pituitary tumors.
3. Classification of pituitary tumors
1. Function classification
functional pituitary tumor
Functional pituitary tumors: prolactinoma, growth hormone tumor, adrenocorticotroph tumor, thyroid-stimulating tumor, luteinizing hormone/follicle-stimulating hormone tumor and mixed tumor, etc.
non-functioning pituitary tumor
No synthetic or secreted hormones
Secretion of biologically inactive glycoprotein hormone α subunit or very weakly biologically active glycoprotein hormone β subunit (quiescent ACTH tumor)
2. Morphological classification
Diameter size
macroadenoma
≥10mm
microadenoma
<10mm
Growth type: expansion, infiltration
3. Histopathological classification
Immunohistochemistry: distinguish what hormones are contained in tumor tissue
Light microscopy: acidophilia, basophilia, Chromophobe tumor, mixed tumor
3. Clinical manifestations
1. Symptoms caused by the compression of surrounding tissues by the tumor’s mass effect.
1. Symptoms caused by the compression of surrounding tissues by the tumor’s mass effect. 2. Hypothalamic syndrome related to hypothalamic compression. 3. Pituitary apoplexy.
1. Headache: increased pressure in the dural sac, The tumor breaks through the diaphragm sellae, the headache is relieved, and the pituitary gland is apoplexy. 2. Symptoms of optic nerve pathway compression: optic chiasm, optic nerve, optic tract. 3. Other symptoms: cavernous sinus syndrome, hypothalamic syndrome, cerebrospinal fluid rhinorrhea, diplopia, and loss of smell.
2. Functional pituitary tumors cause symptoms of increased hormone secretion.
4. Functional pituitary tumors cause symptoms of increased hormone secretion. 5. Direct compression of other pituitary cells secondary to pituitary tumor and/or compression of the pituitary stalk causing abnormal hormone secretion function.
1. Increased hormone secretion 2. Reduced hormone secretion: Tumor compresses normal pituitary tissue or pituitary stalk, with mild symptoms and slow progression
3. Direct compression of other pituitary cells secondary to pituitary tumor and/or pituitary gland
Abnormal hormone secretion function caused by pressure on the stalk.
4. Hypothalamic syndrome related to hypothalamic compression.
5. Pituitary apoplexy.
4. Diagnosis and differential diagnosis
(1) Early diagnostic clues 1. Chronic headache, or headache accompanied by abnormal vision/field of vision or hemianopia. 2. Cavernous sinus syndrome. 3. Cranial nerve damage. 4. Hydrocephalus and increased intracranial pressure. 5. Hypothalamic dysfunction, hypophyseal function or hormone hyperactivity clinical manifestations 6. Amenorrhea-galactorrhea or hypogonadism 7. The sella turcica is enlarged or the sella turcica has abnormal shape.
Diagnose based on
1. Medical history and physical examination (nervous system, vision, visual field examination) 2. Head CT Brain MRI can detect 3mm microadenoma and show the hypothalamus Head X-ray lacks specificity and sensitivity 3. Pituitary hormone measurement 4. Final diagnosis: pathological examination
Reasons for Diagnosis Difficulties
1. The increase in hormone secreted by microadenomas is not significant or is secreted intermittently. 2. The clinical manifestations of increased hormones are obvious, but pituitary tumors cannot be found. 3. Double pituitary tumor, mixed pituitary tumor 4. When the tumor compresses the normal pituitary tissue, it is accompanied by symptoms of hypopituitarism. 5. Multiple endocrine neoplasia syndrome with pituitary tumor as the first manifestation (MEN1)
Differential diagnosis
1. Craniopharyngioma: It commonly occurs in children and adolescents. Hypothalamic and pituitary hormones are often reduced. Among them, intracranial pressure is increased, hemianopsia and visual field defects are difficult to distinguish from pituitary tumors. The diagnosis relies on MRI and endocrine function tests. 2. Rathke's cyst: When the cyst is large, it can produce symptoms of sella and suprasellar compression, causing hypopituitarism. Identification method: MRI.
3. Lymphocytic hypophysitis: common in pregnant and postpartum women autoimmune diseases, Symptoms: (i) headache and mass pressing symptoms; (ii) Symptoms of adenohypophysialism (ACTH) (iii) hyperprolactinemia; (iv) symptoms of neurohypophysial involvement, Confirming histopathology: Examination revealed a large number of lymphocytes and plasma cells infiltrating the pituitary gland.
4. Optic nerve glioma: It is common in children and is a primary tumor of the optic nerve and optic chiasm. It manifests as progressive vision loss and proptosis without pituitary endocrine dysfunction. 5. Ectopic pineal tumor: more common in children and adolescents. Diabetes insipidus and adenohypophysialism may occur. Identification: MRI 6. Internal carotid artery aneurysm: often causes hemianopia and hypophysialism. Identification: MRI.
7. Retrobulbar optic neuritis: Unilateral visual impairment is common, with acute onset and improvement within a few weeks. It is often accompanied by eyeball pain and has no symptoms of endocrine disorders. Identification: MRI. 8. Meningioma: HyperPRLemia caused only by compression of the pituitary stalk, which is easily misdiagnosed as a non-functioning pituitary tumor. 9. Enlarged sella turcica: empty sella turcica, suprasellar germ cell tumor, etc. Identification: MRI.
6. Treatment
Make overall arrangements based on: patient condition, tumor nature, previous treatment history, impact on growth and development, and the experience of the therapist. Three methods: surgical treatment, drug treatment, and radiotherapy
Treatment goals: 1. Remove tumor tissue, inhibit growth, and alleviate mass effect 2. Correct the state of autonomous hypersecretion and alleviate clinical manifestations 3. Restore and preserve the inherent function of the pituitary gland, restore the secretion of affected hormones, and restore the normal regulation of the pituitary gland-target gland. 4. Prevent and treat tumor recurrence, or recurrence at clinical and biochemical levels 5. Reduce local and systemic complications caused by treatment
treatment method: 1. Surgical treatment: Except for prolactinoma, surgical removal is the first choice. Surgical treatment should be considered when there are clinical symptoms of increased pituitary hormone secretion and/or compression of the cranial nerves and tissue structures around the sella turcica. Pituitary apoplexy must be treated immediately or surgically as soon as possible.
Surgical treatment efficacy: 1. Physician’s experience and level 2. The size of the tumor 3. Whether the tumor invades the bone or dura mater 4. Previous treatment status
medical treatement: Dopamine receptor agonist (bromocriptine): prolactinoma, somatotrophoma, ACTH tumor, GnH tumor Growth hormone analogs: somatostoma, TSH tumor, GnH tumor Radiation Therapy: Adjuvant treatment for surgery: Indications: 1. The residual amount of tumor after surgery is large and cannot be effectively controlled by drugs; 2. The tumor relapses after surgery; 3. Suprasellar lesions, and the patient refuses transfrontal surgery; 4. The affected area is negative, but the biochemical changes and Those with obvious clinical symptoms
Hypopituitarism
Overview
A clinical syndrome caused by insufficient secretion of one or more adenohypophyseal hormones due to various causes of damage to the hypothalamus, hypothalamic-pituitary pathway, and pituitary gland. Adult: Simmond disease Postpartum women: Sheehan syndrome Childhood: pituitary dwarfism
Primary: Pituitary disease Secondary: hypothalamic or other central nervous system lesions: Pituitary portal system disorders Panadenohypopituitarism, pay attention to the difference from panhypopituitarism Partial adenohypophysialism single adenohypophyseal hormone deficiency
Etiology and pathogenesis (primary)
1. Congenital adenohypophysis hypoplasia Kallmann syndrome, Prader Willi syndrome
2. Pituitary tumors and tumors near the pituitary gland and hypothalamus (the most common) Divided into functional and non-functional, mainly tumors can compress normal pituitary tissue, causing hypofunction, hyperfunction, or both. Adults mostly have pituitary tumors, while children often have craniopharyngioma. A few causes are various metastases.
The most common, accounting for about 50% of this disease. 1) Patients with high hormone secretion state: large functional tumors, such as PRL tumors and GH tumors. Tumor diameter is generally >10mm. 2) Those without hormone hypersecretion: most common in non-functioning tumors and craniopharyngioma; metastatic tumors are rare.
3. Ischemic necrosis of pituitary gland Caused by postpartum hemorrhage, American doctor Sheehan first described it in 1935. The adenohypophysis of pregnant women undergoes physiological hyperplasia and hypertrophy, and the weight can increase from 0.5g to 1.0g. The blood supply is extremely rich. At this time, the adenohypophysis is particularly sensitive to ischemia, which becomes more obvious during delivery. Hypopituitarism generally does not occur in women who experience hemorrhagic shock during non-pregnancy or childbirth. When the mother goes into shock and coma due to blood loss during the delivery of the placenta after delivery, ischemic necrosis and atrophy of the adenohypophysis occurs, which is Sheehan syndrome. Rare causes: diabetic vascular disease, surgical bleeding
4. Infection and infiltrative lesions 5. Radiation damage 6. Craniocerebral trauma or pituitary gland surgery 7. Vacuolar sella syndrome 8. Autoimmunity 9. Pituitary apoplexy
Etiology and pathogenesis (secondary)
1. Pituitary stalk damage Surgery, trauma, tumors, etc. 2. Hypothalamic lesions and central nervous system diseases Such as tumors, inflammation, infiltrative lesions, granulomas, etc., can directly destroy hypothalamic neurosecretory cells, reduce the secretion of released hormones, thereby reducing the secretion of adenohypophyseal hormones and leading to target gland hypofunction.
pathology
Pituitary necrosis and shrinkage target organ atrophy
clinical manifestations
Symptoms will only appear when the pituitary tissue is destroyed >50%. >75% have obvious symptoms >95% have severe symptoms Severe postpartum hemorrhage and acute pituitary apoplexy: patients can die quickly from shock and pituitary coma. People with mild pituitary necrosis can survive. Patients with pituitary tumors have a slow onset of disease. Those with less damage have no obvious symptoms and only show deficiencies in stress states and when doing pituitary reserve function tests.
(1) Symptoms and signs related to the cause:
1. Sheehan syndrome: Panhypopituitarism caused by peripartum hemorrhage shock, all pituitary hormones are deficient, but there are no space-occupying clinical manifestations. 2. Pituitary tumors: The onset is slow, and the first signs of intracranial space-occupying lesions are: headache, changes in vision, appearance of increased corresponding hormone secretion, etc., followed by symptoms of tumor compression and infiltration, followed by functional decline.
(2) Manifestations of frontal lobe dysfunction
1. Symptoms of decreased secretion of LH and FSH Females: Postpartum no breasts, degeneration and softness of mammary glands, discoloration of areola, amenorrhea, infertility, loss of hair, loss of pubic and armpit hair, atrophy of vulva and genitals. Males: secondary sexual characteristics degeneration, testicular atrophy, external genitalia and prostate shrinkage, and sexual dysfunction.
GH deficiency syndrome childhood dwarfism Muscle loss, poor work tolerance, and central obesity in adulthood
2. Symptoms of reduced TSH secretion Pale complexion, aging face, indifferent expression, slow reaction, low tone, decreased intelligence, fear of cold, dry skin, less sweating, loss of eyebrows, loss of appetite, constipation, mental depression, and even mental disorder. The heart rate slowed down, the electrocardiogram showed low voltage, and the thyroid gland was not enlarged.
3. Symptoms of decreased ACTH secretion Weakness, loss of appetite, intolerance to hunger, prone to fasting hypoglycemia (coordinated with GH deficiency), skin hypopigmentation, weight loss, low blood pressure, poor resistance, can easily prolong the course of the disease and easily lead to pituitary crisis.
4. Pituitary crisis
inducement On the basis of panhypopituitarism, various stresses such as infection, sepsis, diarrhea, vomiting, dehydration, hunger, cold, acute myocardial infarction, cerebrovascular accident, surgery, trauma, anesthesia and the use of sedatives, hypnotics, Antidiabetic drugs, etc. clinical manifestations High fever type: T>40℃, but P does not increase accordingly Hypothermic type: T<30℃, TSH deficiency is the main factor, more common in cold seasons Hypoglycemic type: the most common, with obvious mental symptoms Hypotension, circulatory collapse type: Water poisoning type: Na can be lower than 110mmol/L Hybrid:
laboratory tests
Reflected by target gland functional status 1. Determination of adenohypophysis target gland hormone: Can better determine whether target gland hypofunction is primary or secondary 2. Blood biochemical tests, electrolytes, blood sugar 3. CT or MRI
Diagnosis and differential diagnosis
(1) Diagnosis basis:
Those with a history of massive hemorrhage and shock during delivery, and amenorrhea with no milk secretion after delivery There are headaches, vision loss, hemianopia, nausea, vomiting, papilledema and other intracranial hypertension clusters. X-ray, CT and MRI show the expansion and deformation of the sella turcica and bone erosion. Symptoms and signs of hypofunction of two or more target glands laboratory tests Those who meet two or more of the above criteria can be diagnosed clearly
Differential diagnosis
1. Anorexia nervosa: more common in women, with psychiatric symptoms and cachexia
Adenohypophysialism Women of childbearing age between 20 and 40 years old Most have a history of postpartum hemorrhage and shock Armpit and pubic hair fall off, premature aging sexual organ atrophy Decreased cortisol secretion There may be changes in the sella turcica
anorexia nervosa Girl around 20 years old Many have a history of mental trauma rare none normal none
2. Primary hypothyroidism
primary hypothyroidism Myxedema appears obvious Heavy or irregular menstruation Significant increase in blood cholesterol No response to TSH stimulation test TSH measurement increased significantly TH preparation alone is effective
secondary hypothyroidism Not significant Amenorrhea and genital atrophy Not obvious delayed response Undetectable or extremely low Inducing pituitary crisis
3. Primary adrenal insufficiency (Addison’s)
Addison’s Pigmentation of skin and mucous membranes Sexual function and thyroid function have not changed significantly Electrolyte imbalance, sodium loss, halophilia No response to ACTH stimulation test
Simmonds-Sheehan’s Light skin pigment, light areola obvious Obvious hypoglycemia delayed response
4. Multiple endocrine hypofunction Primary hypofunction of multiple endocrine glands, identification Depends on unresponsiveness to stimulation test 5. Maternal love syndrome Changes in pituitary function are temporary, changing environment and relationships Aike restores growth
treat
First, the cause should be treated Tumor: surgery, radiotherapy, chemotherapy Sheehan’s: Strengthen perinatal monitoring and correct obstetric pathological conditions
hormone replacement therapy Treatment principle: Make up for what is missing, and make up for what is missing. Depending on the condition of hypopituitarism, corresponding hormone replacement therapy will be given. The dosage is based on physiological secretion. Alternative treatment can achieve satisfactory results, improve mental and physical activities, improve systemic metabolism and sexual function, and prevent osteoporosis. Long-term, lifelong maintenance treatment is required.
Glucocorticoid supplementation first, then thyroid hormone supplementation Start with a small amount of thyroid hormone and slowly increase it Generally do not supplement mineralocorticoids Long-term or even lifelong maintenance treatment The dose of glucocorticoids needs to be increased under stress conditions Suitable for oral administration
1. Adrenocortical hormone Due to insufficient secretion of ACTH, the function of the zona glomerulosa is minimally affected, so it is more appropriate to use prednisone instead. Usage: 6~8Am 5mg. Po. 2~4Pm 2.5mg. Po. Mild disease: 6~8Am 5mg. Po. is enough
2. Thyroid hormone Start with a low dose and increase it every 3 to 4 weeks until the blood FT3 and FT4 levels return to normal. The dose is the maintenance dose. TSH during treatment does not accurately reflect thyroid function.
Note: GC should be supplemented first, and then TH or both should be supplemented at the same time, but the latter must start with a small amount. TH alone can induce pituitary crisis.
3. Sex hormones and restoration of fertility Female patients under the age of 40 can be given female hormones and progesterone periodically, and those after the age of 40 can be treated as postmenopausal. Although this kind of artificial cyclic hormone withdrawal bleeding cannot restore ovulation and fertility, it is beneficial to improving the quality of life and reducing and delaying osteoporosis. Sheehan’s patients who want to have children can try HMG and HCG treatments to promote follicle development and maturation for pregnancy. At the same time, they should provide perinatal care to avoid recurrence of heavy bleeding during delivery.
General treatment
Patients should pay attention to nutrition and regular life. Prevent infection, fatigue and other stress. If you feel stressed, adjust the dosage of medication or seek medical advice promptly. Anesthetics, sedatives, and hypoglycemic drugs are prohibited or used with caution.
pituitary crisispituitary crisis
definition
On the basis of anterior pituitary hypofunction, the condition undergoes a sharp and fatal exacerbation.
inducement
1) Stress: infection, trauma, surgery, dehydration, cold, etc. 2) Sudden discontinuation of HRT. 3) Excessive use of sedatives and anesthetics.
clinical manifestations
Early stage of crisis: Mainly gastrointestinal and psychiatric symptoms such as anorexia, nausea, and vomiting. Crisis period: Severe hypoglycemia, coma, and shock occur. Various types of clinical manifestations: Circulatory failure type, hypoglycemia type, hyperpyrexia type, hypothermia type, Water poisoning type, etc. The root cause is insufficient adrenocortical hormones, followed by insufficient thyroid hormones.
Hunger, hypoglycemic drugs, etc.: May occur slowly - mental changes (drowsiness or restlessness, gradual coma) trigger hypoglycemia It can also happen quickly—sweating, trembling, rapid heart rate, epilepticus-like seizures, and brief coma.
Infection:
High fever, anorexia, vomiting, confusion, and easy coma when combined with hypoglycemia
Sedative-hypnotics:
Hypoglycemia may occur during sleep or the original hypoglycemia may worsen and gradually lead to coma.
Excessive fluid infusion - water intoxication:
Vomiting, apathy, lethargy or delirium, grand mal seizures, hyponatremia
Cold in winter:
Dry and cold skin, low body temperature, bradycardia, shallow and slow breathing, and impaired consciousness
Crisis management: In a crisis, intravenous glucocorticoids and stress doses are required Day 1~3 Hydrocortisone 200~300 mg/d Change to oral administration after 3 to 8 days depending on the condition. Reduce gradually to the maintenance dose in 2 to 3 weeks (large doses can easily cause mental symptoms) Correct hypoglycemia and water and electrolyte disorders correct shock Anti-infective
Summarize
Hypopituitarism definition The etiology and pathogenesis of hypopituitarism Diagnosis and treatment of hypopituitarism Diagnosis and treatment of pituitary crisis
central diabetes insipidus
definition
Severe or partial deficiency of arginine vasopressin (AVP), also known as antidiuretic hormone (ADH) (central diabetes insipidus), or kidney insensitivity to AVP (nephrogenic diabetes insipidus), causing tubular reabsorption water dysfunction Polyuria, polydipsia, polydipsia, low specific gravity urine and hypotonic urine More common among teenagers Male to female ratio 2:1
Etiology and pathogenesis
Acquired (secondary) about 50% of hypothalamic neuropituitary and nearby tumors, 10% of head trauma, a few brain infections, granulomatous lesions, and vascular lesions Heredity: often dominant, often recessive, X-linked recessive inheritance, Wolfram syndrome Idiopathic: about 30%, no cause can be found
clinical manifestations
According to the degree of AVP deficiency, it is divided into complete and partial Polyuria, polydipsia, polydipsia, and the patient likes cold drinks Low urine specific gravity and urine osmotic pressure Failure to replenish water in time may lead to severe dehydration
Diagnosis and Differential Diagnosis
(1) Diagnostic basis
Excessive urine output 4-10L/d Hypotonic urine, urine osmosis < blood osmosis, generally lower than 200mOsm/(kg.H2O), urine specific gravity <1.005 The water deprivation test cannot significantly increase urine osmotic pressure, but after vasopressin injection, urine output decreases and urine osmotic pressure increases by more than 9% compared with before injection. DDAVP or AVP treatment has obvious effects
(2) Diagnostic methods
water deprivation vasopressin test Plasma arginine vasopressin measurement Etiological diagnosis of central diabetes insipidus
water deprivation vasopressin test
Compare the changes in urine osmolality before and after water deprivation and after using vasopressin Method: 6-16 hours, record blood pressure, weight, urine output, urine osmotic pressure in 1-2 hours, inject 5U of vasopressin Results: DI patients still had excessive urine output after water deprivation, and after injection of vasopressin, the urine osmotic pressure further increased.
(3) Differential diagnosis
primary polydipsia nephrogenic diabetes insipidus gestational diabetes insipidus other illnesses
treat
DDAVP, desmopressin acetate tablets, nasal spray, intramuscular injection Tannic acid vasopressin injection Pituitaryin aqueous solution Others hydrochlorothiazide, chlorpropamide Cause treatment