gigantism and acromegaly
Introduction
Introduction to giant disease and acromegaly Giant disease and acromegaly are caused by excessive secretion of growth hormone (GH) in the pituitary gland. Adolescents develop giant disease due to patent forcing; after adolescence, osteophytes have merged to form acromegaly; a few puberty from onset to adulthood continue to develop acromegaly. Early stage (formation period) of the disease, general hypertrophy of body and viscera, hyperfunction of anterior pituitary; late stage (recession), physical decline, secondary anterior pituitary dysfunction. basic knowledge The proportion of illness: 0.06% Susceptible people: no specific population Mode of infection: non-infectious Complications: Adrenal insufficiency
Cause
Giant disease and the cause of acromegaly
Pituitary (55%):
The majority. Including GH cell proliferation or adenoma, GH/PRL cell mixed adenoma, prolactin growth hormone cell adenoma, eosinophilic adenoma and the like.
In vitro (25%):
Heterologous GH/ and or GHRH secrete tumors (lung, pancreatic cancer, hypothalamic hamartoma, carcinoid, islet cell tumor). Such tumors often fail to be life-threatening in clinical manifestations of excessive GH secretion.
Prevention
Giant disease and acromegaly prevention
First, mental and psychological care. Because patients have special abnormal body types and abnormal facial appearances, and have different levels of psychological burden, they should do ideological work to patients and increase their confidence in fighting diseases.
Second, life care. The patient's size is abnormally high, and it is difficult for the general bed to fall asleep. Therefore, it is necessary to prepare for the extension of the bed or mattress, and the patient should be lowered into the hospital to avoid bumping the head. If there is a patient with visual field of vision defect, their life care should be strengthened to prevent accidents.
Third, diet care. High-protein, high-calorie diet should be chosen to ensure sufficient heat supply to the body. For patients with diabetes, sweets should be banned. According to the diet and care of diabetes, the amount of staple food can be increased as appropriate for diabetics due to the high size of the patient.
Fourth, the condition observation. For patients with advanced complication of anterior pituitary dysfunction, the changes of the condition should be closely observed, pay attention to life characteristics such as blood pressure, heart rate and respiration. Avoid stress, infection, fatigue and other incentives, such as severe gastrointestinal symptoms, mental disorders, high fever, etc. need to be alert to the occurrence of anterior pituitary hypofunction crisis, timely report to the doctor for rescue treatment. For patients with pituitary tumors, attention should be paid to the occurrence of pituitary apoplexy with severe headache, nausea and vomiting, and altered consciousness.
Fifth, treatment and care. The paralyzed patients take the medicine on time. If there is diabetes, if you need to inject insulin, you need to help the patient to stay four times and four times and check the urine sugar and ketone body. Because the amount of insulin required by the patient is more than that of the general diabetic, it should be observed whether there is low or not. A blood sugar reaction occurs. Patients with hypofunction of the anterior pituitary should be examined and reminded not to stop the drug at random to avoid triggering the crisis.
Sixth, cooperate with the doctor to do a functional test, accurately retain a variety of specimens and send the test, in order to avoid the pain of multiple needle acupuncture, heparinization can be retained in the needle tube, the needle should be blocked during the test, timing push Heparin solution was checked.
Complication
Giant disease and acromegaly complications Complications Adrenal insufficiency
Easy complicated by adrenal insufficiency, gonadal atrophy and sexual dysfunction, hypophyseal anterior pituitary hypofunction.
Symptom
Giant symptoms and acromegaly symptoms Common symptoms Whole body thick skin, excessive hypertrophy, osteoporosis, polydipsia, polythyroid, visual field defect, urinary phosphorus amenorrhea, head enlargement
The onset is slow, early can be asymptomatic, and then gradually grows wide and wide, eyebrows and double humps, giant nose and big ears, thick tongue and tongue, mandibular protrusion, sparse teeth, enlarged nose and throat, language turbid, appearance It is ugly. The toes are short and thick, the palms are thick, and the whole body is thick, sweaty and fatty. A small number of goiter, basal metabolic rate increased, thyroid function is mostly normal, a small number of hyperthyroidism. The internal organs are generally hypertrophied and the thorax is enlarged. Men's sexual desire is excessive, women's majority menstrual disorders, amenorrhea, infertility. Half of the impaired glucose tolerance, polydipsia, and hyperprolactinemia can be emulsified.
Symptoms of tumor compression in the late stage may include headache, visual field defects and hypertension, secondary hypothyroidism, secondary adrenal insufficiency, gonadal atrophy and hypofunction, osteoporosis, spinal activity Limit. Pituitary giant disease manifests itself as overgrowth in childhood, tall and tall, and limbs grow particularly fast. The appetite is too strong and the arm is overpowered. Late (declining) physical strength is declining.
Examine
Giant disease and acromegaly examination
(1) GH measurement: the basic value is >15ug/L, and the active period is as high as 100ug/L or more (normal < 5ug/L).
(2) The growth factor was significantly increased (normal value 75-200 ug/L).
(3) Increased blood sugar, impaired glucose tolerance, glucose inhibition test: oral glucose 100g, 1/2, 1, 2, 3, and 4h after taking sugar and taking blood to measure GH. After normal consumption of sugar, GH 1h fell below 1ug/L, decreased to 5ug/L below 2h, and rose to above 5ug/L after 4h. The GH of this disease is not inhibited by autologous secretion.
(D) Determination of calcium and phosphorus: a small amount of serum calcium, phosphorus increased, urinary calcium increased, urinary phosphorus decreased. Such as persistent or significant hypercalcemia may be associated with other multiple endocrine neoplasias such as hyperparathyroidism.
(5) X-ray examination: the skull is enlarged, the skull plate is thickened; most of the sella are enlarged, the anterior and posterior sinus is destroyed; the sinus is enlarged, the occipital trochanter is prominent; the distal end of the long bone is hyperplasia, and the top of the phalanx is plexiform. . CT scans help to identify patients with microadenomas.
Diagnosis
Diagnosis and identification of giant disease and acromegaly
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
Should be differentiated from skin periosteal hypertrophy, vacuolar saddle and so on.
The X-ray findings of skin periosteal hypertrophy are mainly: periosteal hyperplasia of the extremities and thickening of the diaphysis, which is symmetrical, mainly composed of the tibia and the ulna. The early periosteum is serrated, and is connected to each other in a layered shape as the disease progresses. The periosteum is most obvious at the distal end of the diaphysis, and gradually spreads proximally, generally not involving the epiphysis and metaphysis.
The vacuolar saddle is more common in women (about 90%), especially in middle-aged and more fat women. Headaches are the most common symptoms, sometimes intense, but lacking in characteristics, and may have mild to moderate hypertension. A small number of patients have vision loss and visual field defects, which may be concentric or hemianopia. A small number of patients have benign intracranial hypertension (pseudo-brain tumor), which may be associated with optic nerve head edema and increased cerebrospinal fluid pressure. Some patients have cerebrospinal fluid rhinorrhea, which may be caused by a transient increase in cerebrospinal fluid pressure, causing the passage of the passage between the sella and the oral cavity during the embryonic period. A small number of patients with hypopituitarism, mild gonads and hypothyroidism, and hyperprolactinemia. The function of the posterior pituitary is generally normal, but diabetes insipidus can occur in some children. Children may be associated with skeletal dysplasia syndrome.
