gigantism
Introduction
Introduction to Giant Disease Giant disease is caused by excessive secretion of growth hormone (GH) in the pituitary gland. Adolescents develop giant disease due to patent foramen osteogenesis. After puberty, osteophytes have merged to form acromegaly. A few puberty begins to develop into adulthood and form acromegaly. Sexual giant disease. The early stage of the disease (formation period), physical, visceral general hypertrophy, hyperfunction of the anterior pituitary; late (declining), physical decline, secondary anterior pituitary dysfunction. basic knowledge The proportion of illness: 0.002% Susceptible people: more common teenagers Mode of infection: non-infectious Complications: Adrenal insufficiency
Cause
Cause of giant disease
A large amount of growth hormone promotes excessive growth of long bones and becomes a "giant", so the direct cause of giant disease is due to excessive secretion of growth hormone.
The causes of excessive growth hormone secretion are mainly caused by pituitary and pituitary causes:
1. Pituitary: Most of them, including GH cell hyperplasia or adenoma, GH/PRL cell mixed adenoma, prolactin growth hormone cell adenoma, eosinophilic adenoma.
2. In vitro: heterologous GH/ and or GHRH secreting tumors (lung, pancreatic cancer, hypothalamic hamartoma, carcinoid, islet cell tumor), such tumors often fail to show the clinical manifestations of excessive GH secretion. life.
Prevention
Giant disease prevention
There is no special precautionary method, and the treatment of the cause should be carried out in time for early onset.
Care:
First, mental and psychological care, because patients have special abnormal body shape and abnormal face, but have different levels of psychological burden, so should do mental work to patients, increase their confidence in fighting disease.
Second, life care, the patient's body is abnormally tall, the general bed is difficult to sleep, so it is necessary to prepare to lengthen the bed or mattress, and the patient should go to the ward to pay attention to bowing to avoid bumping the head. If there is a visual field defect, the patient should be strengthened. Life care to prevent accidents.
Third, diet care, should choose high protein, high-calorie diet to ensure adequate supply of heat to the body, for diabetic patients should be banned sweets, according to diabetes diet and care, due to the patient's tall body, the amount of staple food can be increased as the average diabetic.
Fourth, the condition observation, the late patients with anterior pituitary dysfunction should be closely observed the changes in their condition, pay attention to blood pressure, heart rate, breathing and other life characteristics, to avoid stress, infection, fatigue and other incentives, such as severe gastrointestinal symptoms, Mental disorder, high fever, etc. need to be alert to the occurrence of anterior pituitary hypofunction crisis, timely report to the doctor for rescue treatment, patients with pituitary tumors should pay attention to the presence or absence of severe headache, nausea, vomiting and changes in the performance of pituitary apoplexy .
Fifth, treatment and care, patients with medication on time, if you need to inject insulin, you need to help patients stay four times and four times urine and check urine sugar, ketone body, because the amount of insulin required by patients is more than the average person with diabetes, it should be noted Observe the presence or absence of hypoglycemia, and patients with hypopituitar dysfunction should be checked and reminded not to stop taking drugs at random to avoid 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 complications Complications Adrenal insufficiency
Easy complicated by adrenal insufficiency, gonadal atrophy and sexual dysfunction, hypophyseal anterior pituitary hypofunction.
Symptom
Symptoms of giant disease Common symptoms Drinking polyuria Childhood overgrowth Giant yin disease Excessive hypertrophy of the giant finger (toe) Abnormal hyperthyroid thyroid enlargement of the throat
A large number of growth hormones promote long bone overgrowth, development is advanced, height and weight are much higher than normal levels, adulthood often reaches 2 meters, or even 2.5 meters, and body weight can reach more than 150 kilograms. In childhood, he often shows more power. If excessive growth hormone secretion occurs in adults, it causes acromegaly. Because the adult long bones have stopped growing, only the articular cartilage is stimulated to ossify and cause acromegaly; while the periosteum is active, the subperiosteal bones at the upper and lower jaws continue to grow, causing the facial bone to disproportionately proliferate, forming a unique face. It also makes the patient's hands, feet, heads, etc. progressively increase, the palms are wide and fat, the lower jaws are prominent, the lips are thickened, the bridge of the nose is wide and the hair is increased. There may be symptoms such as enlargement of the internal organs such as liver and kidney, and elevated blood pressure.
Excessive secretion of growth hormone and GH tumor performance
(1) Height is due to excessive secretion of GH, which promotes bone growth and development, and the longitudinal growth of long bones accelerates. If GH tumor occurs before bone fusion, the height is significantly longer than that of children of the same age, more than 2SD above the normal range, generally reaching 1.8m (female) and 2.0m after puberty development.
(B) bone GH increases the formation of membranous bone, resulting in bone widening and thickening, and promote the ossification of cartilage to cause bone lengthening. Hyperglycemia occurs in the former of the epiphyseal fusion, long bones caused by excessive growth, is a giant disease; occurs in the latter of the epiphysis, long bones can not be extended, but widened and thickened, for acromegaly.
(3) Changes in skin and soft tissues, glucose metabolism, cardiovascular system, respiratory system, etc. See Baidu Encyclopedia of Acromegaly
(D) pituitary apoplexy GH tumors are mostly large adenomas, rapid growth, more bleeding, infarction or necrosis. Pituitary stroke can occur automatically or have an incentive. The most common cause is pituitary radiation therapy (20% to 57%), and radiotherapy may damage the neovascularization of the tumor. Other causes include increased intracranial pressure, diabetes, and anticoagulant therapy.
The clinical manifestations of pituitary apoplexy depend on the speed, extent and extent of hemorrhage and edema, and can be divided into two types: explosive type and occult type. Explosive patients have large amount of bleeding and sudden onset, and can have the following three symptoms: 1 pituitary tumors rapidly expand, resulting in compression symptoms, such as severe headache, vomiting, visual field defects caused by optic chiasm, invasion of cavernous sinus and oculomotor paralysis Wait. 2 The tumor contents or blood enter the subarachnoid space, causing meningeal irritation such as fever and neck stiffness, and even coma. 3 Other cells in the pituitary gland are destroyed causing temporary or permanent hypogonadism. The pituitary tumor of the occult patient has multiple small hemorrhages, and the patient may have no clinical symptoms at each episode. However, after the pituitary tissue destruction reaches a certain level, the GH secretion function disappears and the corresponding performance of other pituitary dysfunction appears.
Tumor compression performance
The direction and extent of pituitary GH tumor compression on the structure near the sella are related to the size and shape of the sella and the integrity of the saddle. Pituitary GH tumors compress normal pituitary tissue, and patients may develop hypopituitarism. High PRL is more common, about 20% of patients can occur at the same time, female patients often have amenorrhea, galactorrhea, male patients with less breast milk. High PRL may be due to tumor compression of the pituitary stalk and pituitary portal system, so that PRL statin can not reach the pituitary gland and lead to increased secretion of PRL in the pituitary gland, or may be due to the combination of PRL tumor. In addition, the molecular structure of GH has a certain homology with PRL, so GH has galactorrhea activity. Some patients have a family history of multiple endocrine neoplasia syndrome type 1 (MEN-1). Although the incidence of MEN-1 acromegaly is not high, if the patient has hypoglycemia, blood calcium is significantly elevated. Suspected the existence of this disease. The thyroid gland may be nodular or diffuse, and even hyperthyroidism may occur.
(A) headache Most patients complain of headache, early tumor compression of the saddle septum, dura mater or nearby large blood vessels caused by pain in the back of the eye, frontal or ankle. Advanced tumors extend to the posterior superior and involve the third ventricle and interventricular space, affecting the circulation of cerebrospinal fluid and causing an increase in intracranial pressure. There may be a full headache, accompanied by cerebral hypertension such as nausea, vomiting, and papilledema.
(B) visual dysfunction due to pituitary tumor oppression of the optic nerve or blood vessels, optic atrophy leading to visual impairment. The development of visual impairment is generally slow, and the visual acuity caused by pituitary adenomas can gradually develop from blurred vision to loss of vision and even blindness. Vision loss can occur first on one side and then on the other side. Because the optic chiasm is about 25px apart from the saddle septum, in some advanced patients, although hyperglycemia is severe, the optic chiasm can be preserved without normal damage. Individual patients with pituitary apoplexy, due to hemorrhage and edema near the optic chiasm, vision suddenly decreased, or even blindness; if the stroke is relieved, hemorrhage and edema absorption, vision can also be improved.
The examination of visual acuity in patients with pituitary tumors mainly includes: 1 visual field defect, which is different due to the direction of tumor growth and the position of the optic chiasm. The lesion is often asymmetrical, with one side of the lesion first, or one side of the lesion is more focused. Generally, the change in color field appears earlier than the white field of view. The most common visual field defects are bilateral blindness of the eyelids (pressure of the optic chiasm), semi-blind or blindness of the monocular side of the eye, long-term blindness of the eyelid side (pressure in front of the optic chiasm), and ipsilateral semi-blindness of the eyes (optional cross) Pressed at the back). 2 The fundus changes. When the optic nerve is continuously compressed, the optic nerve head becomes pale and pale (simple optic atrophy). Optic papilled edema without optic atrophy is rare in patients with pituitary tumors. 3 oculomotor nerve palsy, when the pituitary tumor enlarges outward and expands to the cavernous sinus involving the IV, VI and V of the cranial sinus, the first and second cranial nerves, the clinical manifestations of diplopia, strabismus, eyeball Loss of activity, ptosis, dilated light, or slow light response are generally seen only in patients with pituitary apoplexy.
(3) Hypothalamic dysfunction may include appetite hyperactivity, obesity, sleep disorders, abnormal temperature regulation, diabetes insipidus and elevated intracranial pressure.
Examine
Giant disease check
(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 glucose, impaired glucose tolerance, glucose inhibition test: oral glucose 100g, 1/2, 1, 2, 3, and 4h after taking sugar, GH, blood GH 1h after normal consumption 1ug / L below 2h fell to 5ug / L or less, after 4h rose back to 5ug / L or more, the disease GH showed autonomous secretion is not inhibited.
(4) Calcium and phosphorus determination: a small amount of serum calcium, phosphorus increased, urinary calcium increased, urinary phosphorus decreased, such as persistent or obvious high blood calcium may be combined with other hyperendocrine adenomas such as hyperparathyroidism.
(5) X-ray examination: the skull is enlarged, the skull plate is thickened; most of the sella are enlarged, and 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 of giant disease
Diagnose based on
1. The height of the child and the length of the limbs are out of balance, and the wingspan far exceeds the height.
2. The movement speed is slow.
3. Parents are shorter and their children are taller.
4. High growth hormone, abnormal metabolism and blood sugar in the body.
5. There are pituitary adenomas in the brain and high growth hormone in the blood.
6. If you don't have surgery, you will soon lose prematurely and your height will shrink.
Differential diagnosis
Should be associated with skin periosteal hypertrophy. Identification of vacuolar sella and other saddles.
The X-ray findings of skin periosteal hypertrophy are mainly: periosteal hyperplasia of the extremities and thickening of the trunk. Symmetrical. Mainly the humerus and the ulna. The periosteum is jagged in the early stage. The layers are connected to each other as the disease progresses; the periosteum is most prominent at the distal end of the diaphysis. And gradually spread to the near end. Generally do not involve the epiphysis and metaphysis.
The vacuolar saddle is more common in women (about 90%). Especially the middle-aged and more fat multi-partum women. Headache is the most common symptom. Sometimes intense. But it lacks characteristics. Can be light. Moderate hypertension. A small number of patients have vision loss and visual field defects. Can be concentric or hemianopia. A small number of patients have benign intracranial hypertension (pseudo-brain tumors). Can be associated with optic nerve head edema and increased cerebrospinal fluid pressure. Some patients have cerebrospinal fluid rhinorrhea. The cause may be a transient increase in cerebrospinal fluid pressure. Causes the passage of the passage between the sella and the oral cavity during the embryonic period. A small number of patients are associated with hypopituitarism. Can be mild gonads and hypothyroidism. And hyperprolactinemia. The function of the posterior pituitary is generally normal. However, diabetes insipidus can occur in individual children. Children may be associated with skeletal dysplasia syndrome.
