Acromegaly

　　Behind the human eyes, at the top of the nasal cavity, there is a gland called the pituitary gland, which can secrete growth hormone. When a tumor appears in the pituitary gland, it will cause excessive secretion of growth hormone, forming gigantism. The function of growth hormone is to promote the development and growth of body tissues. It can promote the increase and enlargement of the number of cells in the body, making the tissues and organs of various parts of the body larger. It is an important factor in the growth of everyone. In the secondary sexual development process during the growth process, if the secretion of growth hormone is excessive or insufficient, it will cause 'gigantism' or 'dwarfism'.

　　The causes of excessive growth hormone secretion are mainly pituitary and extrapituitary reasons:

　　Pituitary: dense granule or sparse granule GH cell adenomas or hyperplasia, GH and PRL mixed cell adenomas, lactotropin cell adenomas, polyhormone-secreting cell adenomas, which may occasionally be part of multiple endocrine adenopathy type.

　　Extrahypothalamic: ectopic GH-secreting tumors (such as pancreatic cancer lung tumors) GHRH-secreting tumors (hypothalamic hamartoma, insulinoma, bronchial and intestinal carcinoids, etc.) are mostly caused by excessive secretion of GH from pituitary adenomas and are collectively referred to as somatotropin-secreting tumors.

　　: The prognosis of patients with this disease is poor, with high disability and mortality rates, which is obviously related to the increase in complications such as cardiovascular diseases, diabetes, pulmonary diseases, and malignant lesions, resulting in a reduction of average life expectancy by 10 years.

　　1. Disordered sugar metabolism: Patients may have excessive secretion of GH and IGF-1, manifesting insulin resistance, reduced glucose tolerance (29％～45％), and secondary diabetes (10％～20％), increased insulin secretion causing hyperinsulinemia, which may be accompanied by hypertriglyceridemia and decreased lipoprotein lipase activity.

　   2. Lung diseases: The incidence of lung diseases increases, lung function abnormalities, reduced vital capacity, and increased total lung volume; there may be narrowing of the upper respiratory tract and small airways, thus increasing respiratory tract infections, wheezing, and dyspnea; there may be sleep apnea syndrome, which is related to tongue posterior prolapse and inspiratory collapse of the lower pharynx, thus increasing the mortality rate of patients.

　　3. Cardiovascular diseases: The main manifestations are myocardial hypertrophy, interstitial fibrosis, cardiac enlargement, left ventricular dysfunction, heart failure, coronary heart disease, and atherosclerosis. Hypertension is related to hyperinsulinemia, increased reabsorption of sodium in the renal tubules, sodium retention, increased extracellular volume, increased activity of the renin-angiotensin-aldosterone system, and increased excitability of the sympathetic nervous system. Cardiovascular lesions are related to the increase in GH and IGF-1 and the long course of the disease.

　　4. Other complications: Patients with this disease may have increased levels of 1,25-(OH)2D3, with increased intestinal calcium absorption and increased hypercalcemia, hypercalciuria, and increased urinary calculi. If there is hypercalcemia, consider hyperparathyroidism (multiple endocrine adenomas) in conjunction. Hyperphosphatemia is related to increased reabsorption of phosphorus in the renal tubules. In addition, increased bone turnover contributes to the occurrence of osteoporosis. The incidence of colon polyps is high, and the incidence of colon and rectal cancer increases, which is related to the increase in skin tags (skin tags).

　　Patients with acromegaly have an insidious onset and a slow progression. Half of the patients have a course of disease of more than 5 years at the time of diagnosis, and the longest can exceed 30 years. The clinical manifestations of patients are mainly determined by the size, growth speed, secretion status of growth hormone, and the impact on normal pituitary tissue of the pituitary tumor itself. The main manifestations include local compression of the pituitary tumor and excessive secretion of GH.

      1. Symptoms of pituitary tumor compression

　　Due to the slow onset of the disease, diagnosis is often delayed by 5-10 years, and when discovered, the pituitary tumor is often a large adenoma, which can compress surrounding structures. (1) Headache; (2) Blurred vision, field defects, ophthalmoplegia, diplopia; (3) Large GH tumors compressing normal pituitary tissue can cause hypopituitarism in patients; (4) Hyperprolactinemia is more common; (5) Tumor growth affects the hypothalamus, and symptoms of hypothalamic dysfunction may occur, such as increased appetite, obesity, sleep disorders, abnormal thermoregulation, diabetes insipidus, and increased intracranial pressure.

　　2. Manifestations of GH over-secretion

　　Excess secretion of GH can cause excessive growth of bones, cartilage, and soft tissues. Acromegaly is characterized by thick skin, hyperactive sebaceous gland secretion (oily texture), and hyperactive sweat gland secretion (polydactyly). The facial manifestations are particularly prominent, with thick lips, prominent nasolabial grooves, thickened scalp skin呈脑回状, thickened forehead wrinkles, wide nose, and large tongue. The head circumference increases, the mandible increases and protrudes forward, the interdental spaces widen, there is difficulty in occlusion, and there may be temporomandibular joint arthritis. The brow ridges and cheekbones are elongated, the sinusitis is enlarged, the vocal cords become thick and thick, and the voice becomes deep. The hands and feet are large, thick, and the fingers become thick, and they cannot perform fine movements. The shoes, hats, and gloves they prepare are too small, and they still need to increase the size after adulthood. There may be skin hyperpigmentation, acanthosis nigricans, and hirsutism. The incidence of osteoarthritis and joint pain is high, affecting the shoulder, hip, knee joints, lumbar sacral spine, joint movement disorders, joint stiffness, kyphosis of the spine with barrel-shaped chest, ventilation dysfunction, and can promote the occurrence of pulmonary diseases. The heel pad (heel pad) can thicken, the muscles become weak and无力, and even muscle pain may occur. The soft tissue of the wrist can proliferate and compress the median nerve, causing carpal tunnel syndrome. The hypertrophy of the lumbar spine can compress the nerve roots and cause severe pain.

　　I. Spiritual and psychological care. Since the patient has a special abnormal body shape and abnormal facial appearance, there is a certain degree of psychological burden, so ideological work should be done with the patient to increase their confidence in overcoming the disease.

　　II. Mental and psychological care. Due to the special abnormal body shape and abnormal facial appearance of the patient, there is a certain degree of psychological burden, so ideological work should be done with the patient to increase their confidence in overcoming the disease.

　　III. Living care. The patient has an abnormally tall body, and it is difficult to sleep on a general hospital bed, so a longer bed or mattress should be prepared, and the patient should be instructed to bow their head when entering and leaving the ward to avoid bumping their head. For patients with visual field defects, strengthen their living care to prevent accidents.

　　IV. Diet care. High-protein and high-calorie diets should be chosen to ensure sufficient energy supply to the body. For patients with diabetes, sweet foods should be avoided, and follow the diet and care for diabetes. Since the patient's body type is tall, the amount of staple food can be increased appropriately compared to general diabetic patients.

　　V. Observation of the condition. For patients in the late stage with hypopituitarism, close attention should be paid to the changes in their condition, and attention should be paid to vital signs such as blood pressure, heart rate, and respiration. Avoid triggers such as stress, infection, and fatigue. If there are serious gastrointestinal symptoms, mental impairment, and high fever, be alert to the occurrence of a crisis of hypopituitarism and report to the doctor in time for rescue treatment. For patients with pituitary tumors, attention should be paid to the occurrence of sudden headaches, nausea and vomiting, and changes in consciousness, which may indicate a pituitary apoplexy.

　　V. Treatment and nursing. Instruct the patient to take medication on time. If insulin injection is needed for diabetes, help the patient collect four segments of urine four times and check for urine sugar and ketone bodies. Since the amount of insulin required by the patient is higher than that of general diabetic patients, attention should be paid to whether there is a hypoglycemia reaction. For patients with hypopituitarism, they should be examined and reminded not to discontinue medication arbitrarily to avoid triggering a crisis.

　　1. Imaging examination of bone and soft tissue: The typical manifestations of acromegaly in the skull include thickening of the inner and outer plates, with marked thickening of the diploic bone; the ascending ramus of the mandible is elongated, the mandibular angle becomes blunt, and the body protrudes forward. When biting, the lower teeth are in front of the upper teeth. The maxillary sinuses and mastoid processes are all over-gasified; the bones of the hands and feet are characterized by the hyperplasia of the distal phalanx bone plexus in a cluster-like pattern. It may be accompanied by thickening of the bones of the hands and feet, thickening of the bone cortex, widening of the joint spaces, and small exostoses on the heads of the metacarpals and proximal phalanges. Other findings may include enlargement of the vertebral bodies, beak-like deformation of the posterior margin of the vertebral bodies, wedge-shaped deformation of the thoracic vertebral bodies, and畸形 of the spine. The soft tissue of the pad of the acromegaly patient thickens, and X-ray measurement greater than 23mm has clinical significance. However, X-ray measurement may have errors due to different projection magnification in each person, and B-ultrasound, CT, and MRI measurements are more accurate. B-ultrasound is preferred due to its low cost and convenience for follow-up comparison. The normal thickness of the heel pad measured by B-ultrasound does not exceed 21mm.

　　2. Imaging examination for localization of pituitary tumors: Indirect signs in the sellar region can be suggestive during skull X-ray. In patients with large pituitary GH adenomas, the sella is significantly expanded, the sellar floor is double contour, and the tumor can destroy the sellar dorsum and sellar floor when it is very large. MRI and CT of the sellar area are the main localization diagnostic methods for pituitary lesions, and MRI is superior to CT in the detection of pituitary microadenomas, tumor size, and the relationship with adjacent tissues such as the optic chiasm and cavernous sinus. For microadenomas, high-resolution thin-section scanning, enhanced scanning, and dynamic enhanced scanning imaging are clearer, and the contrast between tumor tissue and normal tissue is more significant, which is conducive to improving the detection rate. For large adenomas, these technologies can better help judge the range of the tumor, determine whether the adenoma has invasive growth, whether it compresses or invades the optic chiasm (parasellar or infrasellar, etc.), and provides a basis for formulating a reasonable treatment plan.

　　3. Imaging examination for localization of pituitary tumors: Pituitary external GHRH tumors are very rare. When no pituitary adenoma is found in MRI examination or pituitary pathological examination after surgery is pituitary GH cell hyperplasia, it should be checked whether it may come from a non-pituitary tumor caused by growth hormone-releasing hormone (GHRH) secretion from the chest, abdomen, or pelvic area. At this time, serum GHRH detection is helpful for qualitative diagnosis. Since most GHRH secretion tumors can also express somatostatin receptor, using 111In or 123I labeled octreotide imaging is also helpful for diagnosing GHRH secretion tumors.

　　Dietary health care for patients with acromegaly:

　　1. Provide low-fat, high-protein, and easily digestible foods.

　　2. Supplement adequate fiber.

　　3. Eat more fresh fruits and vegetables.

　　4. Eat light, digestible, low-fat, and high-protein foods.

　　5. Eat less and more meals, and do not overeat at each meal. The dinner should be eaten as little as possible.

　　6. Drink more water.

　　7. Eat more foods rich in vitamin B and vitamin C.

　　8. Eat more fibrous foods.

　　Patients with acromegaly should choose high-protein, high-calorie foods in daily diet to ensure sufficient calories for the body. If there is diabetes, it is generally forbidden to eat sweets, and follow a diabetic diet and care.

　　1. Radiotherapy

　　Internal and external irradiation (such as deep X-ray, cobalt 60, and heavy particles, etc.). 60-90% are more sensitive, and stereotactic radiosurgery with X-knife, γ-knife can also be used.

　　2. Surgical Treatment

　　Most pituitary GH adenomas are首选首选 adenoma resection. The basic surgical approach is divided into transcranial pituitary tumor resection and transsphenoidal pituitary tumor resection.

　　Complete resection of GH tumors can effectively restore normal GH levels, with a sharp decrease in serum GH levels, and IGF-1 levels also decrease accordingly. The normal pituitary function that has been compressed and damaged can also partially or completely recover, and other symptoms compressed by the tumor can also recover. The effectiveness of surgery largely depends on the surgeon's skills, the extent and size of the tumor, and the preoperative GH level. For large GH adenomas, the tumor may have invaded surrounding tissues. Even if the tumor cannot be completely resected, surgery can reduce the tumor burden, and the best results can be achieved by using adjuvant treatment measures such as radiotherapy, drug treatment, or a combination of both.

　　3. Drug Treatment

　　The main drugs for the treatment of acromegaly include somatostatin analogs, dopaminergic agonists, and GH receptor antagonists. Drug treatment is mainly used for: ① Patients who cannot or do not want to undergo surgery; ② To reduce the tumor volume before surgery; ③ Poor or recurrent surgical or radiotherapy effects; ④ Auxiliary treatment after tumor resection; ⑤ Improvement of complications.

　　4. Follow-up

　　After the treatment of acromegaly, regular follow-up should be conducted every 3-6 months to re-evaluate pituitary function; imaging examinations of the sellar region may be necessary if necessary. If the condition is well controlled, lifelong follow-up should still be carried out, and the treatment plan and related complications should be adjusted in a timely manner. Long-term monitoring and follow-up of postoperative patients should include the following:

　　(1) Monitor blood GH and IGF-I changes within one week after surgery

　　(2) At the time of discharge, emphasize health education, remind the importance of long-term follow-up for survival, and provide a follow-up card, inform the follow-up process, sign an informed consent form, and patients will receive follow-up questionnaire surveys annually. If there are changes in address or phone number, inform the follow-up physician in a timely manner.

　　(3) OGTT GH test (75g glucose) should be re-examined after 3 months of surgery

　　(4) Pituitary MRI and OGTT GH test (75g glucose) should be re-examined after half a year of surgery; for patients with complications, follow-up of relevant examination items should be carried out.

　　(5) For patients with good control, blood IGF-I and OGTT GH test (75g glucose) should be re-examined annually after surgery; pituitary MRI should be re-examined at 1st, 2nd, 5th, and 10th year after surgery; for patients with complications, an annual evaluation of complications should be conducted.