Tumors that secrete growth hormone in the anterior pituitary gland in childhood will increase bone length and lead to gigantism. After puberty, because the bone cuboid is closed, growth hormone can't make the long bone grow again, and the parts with cartilage in the body, such as fingers (toes), mandible, orbit and nasal bone, continue to proliferate and become metaplasia, which makes the patient appear the typical signs of acromegaly. The patient's appearance is ugly: eyebrow guide, cheekbones, protruding chin, big nose, thick lips, thick tongue, big head, and hands and feet as generous as shovels. At the early stage of the disease, the patient's internal organs and muscles proliferated and hypertrophy, and the extubation was too fierce. In the later stage, they developed secondary diabetes, hypertension, decreased visceral function, backache, muscle weakness and so on. Symptoms such as headache, decreased vision and loss of vision may also occur. Patients with typical signs can be diagnosed by measuring plasma growth hormone concentration and shooting sella turcica. Treatment generally adopts deep X-ray or 60-drill long-distance radiotherapy, or embeds radionuclide 90 Yb or 198 gold in pituitary fossa for internal irradiation to destroy tumors. If the vision changes, pituitary adenoma can be surgically removed and then radiotherapy can be performed. If there are complications such as pituitary dysfunction after treatment, corresponding hormone replacement should be given. If there is recurrence, the dose of bromocriptine can be 30mg/ day, and the patients with secondary diabetes can be treated with insulin.

symptom

Symptoms: Acromegaly is mostly pituitary growth hormone cell adenoma, and a few are hyperplasia or canceration; Giant disease is mostly hyperplasia. In addition to growth hormone cells, adenomas can also contain prolactin cells, which are called mixed cell adenomas. In other patients, although adenoma cells have a single morphology, they can simultaneously produce gh and PRL. This adenoma is called stem cell adenoma. The hypothalamus secretes growth hormone releasing inhibitory hormone (SS), or the hypothalamus secretes growth hormone releasing hormone (GHIH) too much, which stimulates the pituitary gland for a long time, making it proliferate or form adenoma. Diffuse growth hormone cell proliferation is rare. Excessive secretion of growth hormone may also be related to excessive secretion of ectopic growth hormone, such as bronchial adenoma, islet cell tumor and carcinoid tumor. Some patients with hypothalamic hamartoma may have acromegaly or gigantism. Most patients with ectopic secretion of growth hormone have enlarged sella turcica and are often mistaken for pituitary adenoma or hyperplasia. Growth hormone cells in adenomas often have abnormal responses to pituitary stimulating hormones, such as insensitivity to growth hormone releasing inhibitory hormone, suggesting that they have no response to hypothalamic control.

Detailed description of symptoms

Acromegaly is recessive and usually occurs between the ages of 30 and 50. There is no gender or racial difference. Only a few patients are familial, showing familial multiple endocrine diseases (MEN I). If the hypersecretory state of GH begins in childhood, its main clinical manifestations are accelerated growth, prolonged puberty, or hypogonadism, and they often coexist and show the proportion of testicular limbs. When the epiphysis is closed, GH secretion increases, and the early manifestations are gradually ugly face, long hands and feet, rough skin and wrinkles, especially on the forehead. The lips are thick and prominent, and the tongue is big. Too many tongues can lead to poor airway. The changes of skin are mainly due to the proliferation of connective tissue and the increase of intercellular substance. Due to the deposition of hyaluronic acid, interstitial edema can also be caused. Body hair thickens, sebaceous glands and sweat glands increase, function is enhanced, sweating is increased, and there is a sense of self. The skin becomes darker, and small skin fibromas or skin papillomas may appear. Bone hyperplasia, through X-ray photos, can show bone cortex thickening and osteoporosis. The mandible becomes longer and thicker, which leads to reverse occlusion and sparse teeth. Skull thickening and osteoporosis. Frontal sinus, mastoid sinus and ethmoid sinus are enlarged. The ribs become longer and finally form a barrel chest. The posterior concavity of vertebral body increases, trabecula decreases, and humpback can be formed in severe cases. Acromegaly patients may have joint symptoms, such as back pain or joint pain, and even degenerative arthritis. Articular cartilage hyperplasia, osteophyte formation, X-ray photos can show that the joint cavity widened. Thyroid cartilage hyperplasia and wide throat cavity lead to low pronunciation. About15 patients have paresthesia, which may be related to overgrowth of bone or connective tissue. The median nerve is compressed at the carpal tunnel, which may lead to hand weakness and abnormal sensation. Fibrosis inside and outside the nerve can thicken the peripheral nerve, which may also be one of the factors of nerve injury. Foot drop, muscular atrophy and neuropathic arthropathy are rare. In addition, neuropathy can also lead to proximal muscle weakness. Hypertension will appear in the circulatory system. Even without cardiac insufficiency, B-ultrasound can also show ventricular wall and interventricular septum hypertrophy. Middle-aged and elderly acromegaly patients may also have progressive congestive heart failure. Physical examination showed that the liver was enlarged, and the thyroid gland, parathyroid gland, spleen, pancreas and kidney were larger than normal. The reabsorption of glucose and the maximum excretion of chlorohippuric acid by renal tubules can reach twice that of normal people. Growth hormone increases the reabsorption of phosphorus by renal tubules, leading to mild hyperphosphatemia. About half of acromegaly patients have impaired glucose tolerance or diabetes. Diabetic retinopathy is common in acromegaly diabetic patients, but diabetic glomerulosclerosis is rare. About14 patients can see goiter, and the basal metabolic rate of patients is slightly increased, but the determination of thyroid function is generally political. The increase of basal metabolic rate of acromegaly may be due to the direct effect of GH increase. Plasma prolactin level increased, and about 40% of female patients and 27% of male patients showed an increase in prolactin concentration. 1/5 female patients have galactorrhea. In patients without prolactin elevation, galactorrhea is attributed to the secretion of human growth hormone. ACTH secretion is generally normal, and urine 17- hydroxycorticosteroid alcohol excretion is also normal. Decreased gonadotropin secretion. Immature gonads are common in patients with gigantism and acromegaly. Men may be impotent, and women may have irregular menstruation or amenorrhea.

diagnose

The main basis for diagnosis of this disease is as follows: 1. Typical symptoms and signs; 2. skull x-ray, CT scan and magnetic vibration; 3. Determination of plasma growth hormone level; The patient's GH value is more than 65438 00μ g/L (65438 00μ g/) or even as high as hundreds of μ g/L. Oral glucose tolerance test can not inhibit the patient's GH level. In TRH or gonadotropin-releasing hormone (GnRH) test, GH in normal people did not respond, but GH in patients increased significantly. Differential diagnosis 1. Thickening of periosteum of hand and foot skin is characterized by thick and wrinkled skin of hand, foot, face and neck, sebum overflow and facial sweating. Thickening of periosteum of tibia and distal radius leads to hypertrophy of condylar wrist joint. The photo of the sella turcica is normal. Plasma growth hormone levels are normal. This disease is rare, mostly for young men. Second, whether acromegaly is constitutional or familial. From infancy, facial changes and figure are similar to acromegaly, but to a lesser extent, sella turcica is normal and blood GH is normal.

treat cordially

First, drug treatment for patients who can't be completely cured or have only temporary curative effect during surgery, because radiotherapy is slow to take effect, drug treatment is often needed. Drug treatment is the only way out for patients with serious failure. (1) bromocriptine can stimulate dopamine receptors in the central nervous system and inhibit the secretion of GH and PRL. The dosage is relatively large, often 30mg/d, but some of them are as low as10 ~ 20 mg/d. At first, the dosage is small and gradually increases to avoid side effects such as nausea, vomiting and dizziness. Taking large doses may cause constipation, and it may also cause finger vasospasm when it is cold. After using bromocriptine, plasma growth hormone level decreased, soft tissues, especially face, hands and feet subsided, headache and hyperhidrosis were alleviated, and glucose tolerance, hypertension and dermatolipomatosis were improved. About half of patients' visual field can be improved. About half of patients' visual field can be improved. It takes 3~4 years for the bones to change. Bromocriptine can be used in combination with surgery or radiotherapy. (2) Adjuvant treatment When the GH level is restored or close to normal and the clinical situation is stable, facial plastic surgery, especially nose and eyelid plastic surgery, can be considered when necessary, and the mandible can be partially removed to restore teeth and improve the face. Second, surgical treatment and tumor resection can quickly control and cure the disease. At present, the best operation is to enter the pituitary fossa through the sphenoid sinus from the back of the upper lip or the midline of the nasal cavity, and to remove pituitary adenoma by microsurgery. However, when it invades the sellar region, craniotomy should be performed to enter the operating area. Visual field injury is the indication of the original operation, and the operation can effectively reduce the compression of tumor optic chiasma. If the tumor is small, with a diameter of < < 1.0cm, most tumors can be completely removed with proper operation, and good therapeutic effect can be achieved. If the tumor is large, the cure rate is very low. The level of circulating GH should be monitored after operation, and if it is still maintained at a high level, it is best to supplement radiotherapy or drug treatment in pituitary region. Third, the total course of radiotherapy is 4500rad, which can improve the clinical symptoms, but the effect is slow. Radiotherapy must be carried out slowly, each irradiation should be less than 200rad, and attention should be paid to avoid damaging normal tissues, such as optic chiasma and hypothalamus. High-energy heavy particle beam, X-ray beam and radiotherapy are effective quickly, but the risk of complications is also great. 60-drill irradiation treatment can also be used.