An increase in the superciliary arches. Acromegaly: symptoms, causes, treatment. Signs of gigantism in children

The anatomical basis of the disease is a tumor, eosinophilic adenoma of the anterior, glandular, pituitary gland; sometimes this benign adenoma can undergo degeneration into a malignant tumor. The formation of growth hormone is associated with the eosinophilic cells of the glandular lobe of the pituitary gland, the increased secretion of which, apparently, is the essence of the pathological process in acromegaly. Nervous influences, undoubtedly important for disrupting the activity of the pituitary gland, remain insufficiently studied in the clinic of acromegaly. On the part of other organs in acromegaly, the following is found: thickening of the walls of the skull, enlargement of the phalanges, exostoses, hypertrophy of the skin, mucous membranes, visceral organs; only the internal genital organs present atrophic changes.

Causes of acromegaly

The reason is unknown. In pathogenesis, the central place is occupied by increased production of somatotropic hormone (growth hormone) by the anterior pituitary gland, more often due to the development of eosinophilic adenoma or hyperplasia of eosinophilic cells in it.

A number of symptoms observed in acromegaly are due to secondary disorders from other endocrine glands, in particular the sex, adrenal cortex, thyroid gland, as well as changes in the diencephalic region.

Symptoms and signs of acromegaly

The disease often begins at the age of 20-30 years. Acromegaly in childhood and adolescents is rare: similar processes in the pituitary gland in these cases usually lead to gigantism. Patients experience headaches - from occasional, mild to painful constant; pain is usually localized in the temporal and frontal regions. The skin is thickened, often pasty, in places (neck, forehead, scalp) forms deep folds. The hair is thickened, there is hypertrichosis on the trunk and extremities. The hairline in women can be developed according to the male pattern. The muscular system is well developed, muscle strength is increased at the beginning of the disease, but as the disease progresses, severe adynamia develops. Pain in the extremities, decreased sexual potency in men, amenorrhea or other menstrual irregularities in women are noted. In more than half of the cases, there are visual disturbances and limited visual fields from the temporal side (bitemporal hemianopsia) due to pressure of the pituitary tumor on the area of ​​the optic chiasm. On the radiograph of the skull, in addition to the thickening of the bones of the vault, characteristic of acromegaly, changes in the bones of the lower jaw, superciliary arches and zygomatic bones, enlargement of the paranasal sinuses, in almost all cases there is an increase in the size of the Turkish saddle and thinning of its walls due to the pressure of a tumor growing in its cavity (adenoma) pituitary. In the case of predominant tumor growth upwards, the entrance to the Turkish saddle is expanded, destructive changes in the sphenoid processes are noted. A downward-growing tumor (intrasellar adenoma) is characterized by a deepening of the sella turcica and destructive changes in its bottom, while maintaining the normal structure of the entrance to the saddle, as a result of which changes in the eyes and an increase in intracranial pressure may be absent, despite the progression of the disease.

As a rule, the disease proceeds slowly, but with a malignant tumor of the pituitary gland (adenocarcinoma), a rapid course of the disease can be observed with a severe clinical picture due to tumor metastasis to the brain. With a benign adenoma, the disease can last for decades, and patients retain their ability to work for a long time.

The severity of acromegaly is determined by the development of complications and the dynamics of their course.

The most frequent complications: visual disturbances up to complete blindness, diabetes mellitus, a feature of which may be insulin resistance; dysfunction of the thyroid gland (myxedema, thyrotoxicosis). In some patients, acromegaly is complicated by cerebrovascular accident and cerebral hemorrhage. The cause of death in patients with acromegaly may be intercurrent infections, the resistance to which they have significantly reduced.

In the advanced form of the disease, there is a significant increase in the nose, ears, lower jaw, which protrudes forward (prognathism); from the strong growth of the jaw, the gaps between the teeth become larger (diastema); the tongue turns out to be significantly enlarged, thickened, hardly fitting in the oral cavity; the lips are also thickened; there is an increase in the head with a thickening of the walls of the skull, a sharp development of protuberantia occipitalis externa, an increase in the pneumatic cavities of the face, the superciliary arches and cheekbones protrude.

Attention is drawn to the increase in the hands and feet, which become larger and wider; it depends, as radiographs show, on the thickening of the soft parts and bones, mainly the terminal phalanges; such enlarged wide hands with thick fingers are called spade-shaped; in addition to this increase in width, another type of increase in hand length is described, which is more common in acromegaly at an earlier age.

The increased growth of the peripheral parts also applies to the hair; the hair becomes thicker individually and grows more luxuriantly, especially where there were fewer of them - on the limbs, along the white line of the abdomen; in women, vegetation appears on the upper lip and chin.

There is an increased growth of the clavicles, sternum, ribs, an increase in the upper chest, the phenomena of cervicospinal kyphosis and the corresponding lordosis in the lumbar region. The development of exostoses is observed on the bones in the places of muscle attachment. In addition to these more easily observed external changes, there is an increase in internal organs - splanchnomegaly.

In the advanced form of the disease, there is usually another group of symptoms characteristic of acromegaly, often subjectively severe, from the central nervous system; these are, firstly, general symptoms from the brain (headaches, dizziness, vomiting, changes in mood and intelligence, apathy, drowsiness, etc.), and secondly, symptoms indicating the localization of the disease process, and namely visual disorders, of which bitemporal hemianopsia is very often observed.

Severe headaches. Loss of vision is possible due to visual field limitation or atrophy of the optic nerves. Growth of a pituitary tumor can cause pressure on the optic chiasm (chiasm). There is an expansion of the volume of the chest, thickening of the bones of the skull, an increase and prognathism of the lower jaw, divergence of teeth. The volume of internal organs increases: Tongue and nose increase. The skin on the head thickens and forms folds. Muscles at the beginning of the disease are well developed, later their dystrophy occurs. The hair on the head and body grows intensively.

Often there is a violation of carbohydrate metabolism. Diabetes mellitus can be severe. The thyroid gland increases in volume. Its function is enhanced. In women, amenorrhea occurs due to hypofunction of the ovaries, in men - impotence.

The course of the disease is usually slow, lasting for years and decades; it develops gradually, manifesting itself most often at the age of 20-30 years, but there have been cases of acromegaly in adolescence and early childhood, and even a case of acromegalic phenomena in a newborn has been described.

The disease may begin with less definite disorders of the nervous system, such as fatigue, apathy, headaches, muscle pain, etc. A pathognomonic symptom is an increase in certain parts of the face, head, hands and feet; patients begin to notice that their hat, gloves, shoes are becoming small; changes in appearance, facial features in the direction described above reveal the disease.

In addition to these two groups of painful signs - pathological growth disorders and brain tumor phenomena, there are also other painful phenomena in acromegaly. On the part of the cardiovascular system in the advanced stage of the disease, there are signs of atherosclerosis and a significant increase in the heart, which has to be explained not only by atherosclerosis, but also as a frequent phenomenon of splanchnomegaly and myocardial dystrophy.

Often, symptoms of the reproductive apparatus appear quite early in relation to a decrease in its function: sexual desire fades, menstruation stops in women, sexual impotence develops in men; at the same time, atrophic phenomena develop in the internal genital organs, while the external genital organs do not change.

Due to the significant influence of the pituitary gland on metabolism and on other endocrine glands, with acromegaly, diabetes mellitus (pituitary diabetes), as well as signs of Graves' disease or myxedema, are often observed.

Diagnosis of acromegaly

The main diagnostic criteria are an increase in insulin-like growth factor (IGF-1). The results of single measurements of growth hormone are usually unreliable due to the impulsive nature of its secretion and its short existence in plasma. IGF-1, produced in the liver, reflects the level of GH secretion for the previous day. This is a stable, integral criterion for the activity of growth hormone.

The diagnosis of severe cases of acromegaly is not difficult, the appearance of patients changes so much. But still, several painful forms can be named that have a distant external resemblance to this disease. So, with osteoarthropathy of patients with chronic pulmonary suppuration, the distal parts of the limbs (a hand in the form of a shovel, etc.) increase, but not the head and trunk. With leon-tiasis ossea, on the contrary, there is hyperplasia of the bones of the face and skull, and the limbs remain unchanged. In Paget's disease (osteitis deformans), there is kyphosis, as is often the case with acromegaly, as well as an increase in the bones of the skull and limbs, but due to the cranium and long bones of the limbs, and not the facial skull, hands and feet, as in acromegaly. Often, with gigantism, there are features of acromegaly, which makes these diseases, which apparently depend on an excess of growth hormone, come closer together. In the experiment, typical acromegaly and gigantism were induced in puppies by prolonged administration of an anterior pituitary extract into the abdominal cavity.

The diagnosis of acromegaly is confirmed by an x-ray showing enlargement of the sella turcica and peculiar changes in other bones, as well as visual impairment caused by compression of the optic junction by the tumor.

In view of the characteristic changes in the external appearance of patients, the diagnosis is usually not difficult. Of great importance are radiographs of the skeleton and especially the Turkish saddle, as well as tomography of the Turkish saddle. Examination of the fundus and measurement of visual fields allow us to establish atrophy of the optic nerves. The dynamics of changes in the fundus of the eye largely determines the treatment tactics of the doctor.

Recognition is based on the appearance of the patient, changes in the Turkish saddle. A study of vision and neurological condition is necessary. Blood phosphorus and somatotropic hormone in the blood are elevated.

Treatment of acromegaly

Operational or radiation.

Treatment of acromegaly consists in the surgical removal of the tumor of the brain appendage; this technically difficult operation in some cases was carried out with success in patients suffering from severe headaches or a threatening loss of vision. In some cases, X-ray therapy gave favorable results.

With increased growth of the tumor and its pressure on the optic chiasm, the cord is threatened with loss of vision, and hypophysectomy is necessary. X-ray therapy on the pituitary gland gives a good effect in doses of 200-300 r per session (on the temporal, frontal and occipital fields), for a course of 3000-4000 r. Treatment of a diabetes mellitus (see). With amenorrhea, estrogen therapy (0.1% solution of sinestrol, 0.1% solution of estradiol dipropionate, 0.5% solution of diethylstilbestrol and progesterone or pregnin 0.05 g tablets) (see Gigantism). For male impotence, methyltestosterone and testosterone propionate injections.

Surgical treatment is radical - removal of the pituitary tumor. Absolute indications for surgery are changes in the fundus and visual fields, which are growing despite radiotherapy of the tumor. In other cases, X-ray therapy is effective, under the influence of which headaches stop or significantly decrease, the development of changes in the eyes stops, vision improves and the progression of the main symptoms of acromegaly stops. X-ray therapy courses, if indicated, are repeated at intervals of 6-12 months. X-ray therapy is also indicated in cases where the patient who needs surgery categorically refuses it. If acromegaly is complicated by diabetes mellitus, appropriate dietary measures are indicated, and if carbohydrate tolerance is insufficient, insulin or antidiabetic sulfanilamide drugs are indicated.

In women with hypofunction of the gonads with menstrual disorders, depending on age, estrogenic drugs and progesterone are used (as in amenorrhea), and in old age, estrogenic and androgenic drugs (as in menopausal disorders; see Women's menopause). Men are prescribed methyl testosterone. Treatment is carried out in courses of 1 1/2-2 months.

Acromegaly is a very serious disease with equally important causes and symptoms. If you look at the photos of people with such a disease, the scale of the problem immediately catches your eye.

Therefore, in this article we will consider this ailment in more detail. In addition to the causes and symptoms, you will learn how the diagnosis is made. We will also consider in more detail the main methods of treating this disease.

What is acromegaly

is a syndrome that is characterized by chronic hyperproduction of growth hormone (GH) in individuals with complete physiological growth. That is, men and women.

Gigantism- this is the same pathology, only it manifests itself in childhood. It also differs in that bones, tissues and organs grow very quickly in children.

Acromegaly - causes and symptoms with photos

Below we will look at what acromegaly has causes and symptoms with a photo. Please pay attention to the complications of this disease. There are a lot of them.

Causes of acromegaly

Considering the causes of acromegaly, it is worth saying right away that in 99% of cases it is a pituitary adenoma ( brain tumor). There may also be ectopic secretion of somatoliberin/growth hormone.

Cause of acromegaly pituitary adenoma

Well, do not exclude hereditary forms:

• McCune-Albright syndrome
• Familial acromegaly
• Karni Complex
• MEN-1 syndrome

Concomitant diseases that will primarily cause death of a patient with such a disease:

• Ischemic heart disease (CHD)
• Obstructive sleep apnea syndrome (OSAS)
• Hyperparathyroidism
• Thyroid nodules
• Intestinal polyps
• neoplasia

Here are the main symptoms of acromegaly:

• (growth hormone directly acts on the sweat glands, increasing their number and activity)
• Coarsening of the voice (due to an increase in the volume of soft tissues, including in the region of the vocal cords)
• Snoring and sleep apnea (due to an increase in the volume of the soft tissues of the upper respiratory tract)
• Narrowing of the visual fields (this is due to the growth of the adenoma and the pressure of the chiasm of the optic nerve)
• Headache (due to producing pituitary tumor)
• Macroglossia (when talking, they chew words because the tongue swells)
• Pain and limitation of joint mobility
• Somatomegaly (all parenchymal organs are enlarged)
• High strength (disease debut)
• Muscle weakness (prolonged acromegaly)
• Menstrual disorders in women
• Decreased libido in men
• Diplopia
• Arthralgia, arthropathy
• Diastema
• Prognathism

When the fields of vision are narrowed, drivers usually say that from a certain point they began to cut them. The machines themselves appear out of nowhere because a person does not see with peripheral vision.

If the patient crosses the road, then cars can appear out of nowhere. Often such people find themselves in an emergency situation.

In everyday life, when the field of view is narrowed, a person can simply bump into corners and not notice the approaching objects if he does not look straight ahead.

With macroglassia, there may be traces of hemorrhages on the tongue because a person regularly bites it with his teeth.

Hemorrhages on the tongue

Joint damage in acromegaly has several stages:

1. Edema, joint enlargement, limited mobility
2. Destruction of the joint (in the future, it can atrophy and deform)

Mass effect complications

So, let's start with the mass effect of complications. This is when the tumor grows and compresses other pituitary cells. That is, the production of other hormones decreases.

First we will secondary adrenal insufficiency. Its main symptoms are weakness and weight loss.

When doctors have identified a disease, they can conduct additional diagnostics:

• Analysis of daily urine for free cortisol
• Insulin hypoglycemia test. Insulin is administered to a person and blood glucose decreases. This stimulus should increase growth hormone and cortisol. They cause hypoglycemia. If it doesn't, then something is wrong.

Also possible secondary hypothyroidism. Main symptoms:

• Weakness
• Moderate weight gain

Laboratory diagnostics:

• TSH (primary hormone) will be elevated
• Free T4 will be lowered

Secondary hypogonadism. Its main symptoms are decreased libido and menstrual irregularities.

diabetes insipidus The person may have polyuria, polydipsia, or weight loss.

Diagnostics:

1. Plasma osmolality more than 300 mosmol/kg
2. Urine osmolality less than 300 mosmol/kg
3. Low urine density per day (less than 1.008 g/l)

At hydrocephalus the person complains of weakness, unsteady gait, headache, involuntary urination, or loss of consciousness. In this case, an MRI of the brain is performed.

brain MRI

It may also be observed increased intracranial pressure. Headache is localized in the frontal, temporal and behind the orbital region. It is usually neither sharp nor harsh.

Edema

Now consider the second effect (edema), when the growth factor affects the organs and the growth of connective tissue. The first will be respiratory disorders. The most common complaint is the night apnea.

Apnea in acromegaly

That is, a person's tongue increases, as well as connective tissue in the larynx. In the supine position, all this compresses the airways.

The result is sleep apnea. The person breathes badly, constantly wakes up and snores. Also there is no rash and anger in the morning.

Still observed arterial hypertension. When diagnosing, the doctor measures blood pressure and conducts daily monitoring of blood pressure.

Also possible acromegalic cardiomyopathy. As a rule, a person does not complain, since such a process passes without symptoms. Therefore, the doctor will not be able to immediately understand until an ultrasound of the heart is done.

Possible and peripheral nervous system lesions. If swelling of the nerve and compression of the connective tissue develops, then the person experiences pain and paresthesia. An MRI is done for diagnosis.

Now consider metabolic disorders:

• Liver:
• – Stimulation of gluconeogenesis and glycogenolysis
• – Stimulation of the accumulation of triglycerides
• Adipose tissue:
• – Stimulation of lipolysis
• Muscles:
• – Increase the accumulation of free fatty acids
• - Development of insulin resistance
• Pancreas:
• - Stimulation of insulin synthesis
• Bones:
• - Increased bone turnover

There is also diabetes.

At osteopathy possible development of osteoporosis. Especially if a person has hypogonadism. Compression fractures of the vertebrae are also observed.

At arthropathy under the influence of growth hormone, the cartilaginous tissue of the joint and soft circumferential elements (muscles, connective tissue) increase. Further, various ulcerative processes and joint deformity occur.

Increases under the influence of growth hormone thyroid. Such a case is characterized by nodular colloid to varying degrees proliferating goiter. Diagnosis is by ultrasonography and fine needle aspiration biopsy.

Diffuse and nodular goiter

Intestinal polyps include:

• colorectal cancer
• Adenomatous polyps
• Dolichocolon

Uterine fibroids can also grow under the influence of growth hormone.

It also increases the risk cancer development prostate, mammary glands, intestines, lungs and thyroid gland. As a diagnosis, ultrasound, CT, radiography, biopsy and endoscopic methods are performed.

In advanced cases and with the development of external changes, a person also has psychological disorders. They are especially strong in women. These are social isolation and reduced physical activity.

Photo of acromegaly

Below I present to you a selection of photos of acromegaly. You can see sick women in men. To enlarge, just click on the picture.

Now let's talk about the diagnosis of acromegaly. It is a severe, systemic neuroendocrine disease caused by excessive production of growth hormone.

In the image below, you see a patient with pituitary gigantism, as well as developed acromegaly after closure of the growth plates.

A patient with acromegaly

Note that the man has a cane. Most likely, his joints hurt. He also slouches. It is this appearance that is usually associated with patients of this disease.

Clinical picture of the disease (classic signs):

• Enlargement of the hands and feet
• Coarseness and enlargement of facial features
• Rough, thick, oily skin
• coarsening of the voice
• Macroglossia ( man chewing words)
• Augmentation of the lower jaw and brow ridges

Such signs are present in patients with already advanced disease. Below is a drawing with the classic signs of acromegaly.

To date, acromegaly can be diagnosed at an early stage. That is, when it has not yet caused changes in facial features, as well as enlargements of the hands and feet.

If the patient has one or more of the above signs, then this is a reason to exclude acromegaly. In order to exclude it, it is enough to do a blood test for IGF-1 and growth hormone.

The diagnosis is confirmed if:

• Fasting IGF-1 concentration above reference values
• Fasting growth hormone more than 1 ng/ml

If you received normal IGF-1 and growth hormone more than 1 ng / ml, then it is worth oral glucose tolerance test(PGTT) with growth hormone:

• Take blood for growth hormone on an empty stomach
• In 5 minutes, drink 75 g of anhydrous glucose or 82.5 g of glucose monohydrate dissolved in 250 - 300 ml of water
• It is not allowed to smoke during the text and 3 hours before it
• After 30, 60, 90, 120 minutes, blood is taken again for growth hormone
• To exclude diabetes mellitus at points 0 and 120 min, blood is taken for glucose

A contraindication for the test is the presence of diabetes mellitus.

If, according to the results of the test, the growth hormone in any of the points taken does not decrease below 1 ng / ml, then this confirms acromegaly. If the growth hormone at least at one point is below 1 ng / ml, then this is not our disease.

If acromegaly is confirmed by the results of a hormonal blood test, then it is necessary to visualize the source of growth hormone production.

With MRI, regardless of the clinical picture and the duration of the course, it is possible to detect a pituitary microadenoma less than 1 cm.

Pituitary microadenoma less than 10 mm

A macroadenoma (10-25 mm) can be detected.

Macroadenoma more than 1 cm

You can also find a giant adenoma (more than 25 mm) in the patient.

Giant adenoma over 25 mm

Laboratory diagnosis of acromegaly

Laboratory diagnosis of acromegaly is an occasional growth hormone that must be greater than 1.0 ng/mL. With a highly imunetric method, this is more than 0.4 ng / ml. At the same time, blood is taken five times with an interval of 30 minutes.

They also look at insulin-like growth factor 1 (IGF-1), which reflects the daily somatrophin secretory status, regardless of food intake. Therefore, it is diagnostically the most convenient.

IRF-1 (age standards for both sexes)

Age (years)	Norm value (ng/ml)
20 — 25	116 — 358
25 — 30	117 — 329
30 — 35	115 — 307
35 — 40	109 — 284
40 — 45	101 — 267
45 — 50	94 — 252
50 — 55	87 — 328
55 — 60	81 — 225
60 — 65	75 — 212
65 — 70	69 — 200
70 — 75	64 — 188
75 — 80	59 — 177
80 — 85	55 — 166

It can be seen that insulin-like growth factor decreases with age. Also, positive results are possible with liver failure.

Estrogens negatively affect the binding of growth hormone to peripheral tissues.

The minimum level of growth hormones on the background of the oral glucose tolerance test is also a laboratory criterion.

growth hormone concentration

An increase in growth hormone in each of the points of more than 0.4 ng / ml with a highly sensitive immunometric method will indicate a pathology. After all, GH should decrease when glucose enters the blood. Well, its increase will indicate the autonomous nature of the secretion of growth hormone.

Instrumental diagnostics

If the tests are confirmed in the laboratory, then the doctors refer the patient for instrumental diagnosis of acromegaly. It will be an MRI of the pituitary gland with contrast. In this case, tomographs must be at least 1.5 T.

If there are contraindications to MRI, then computed tomography with contrast is performed.

If we did not find a pituitary adenoma, but according to laboratory data there is an increased secretion of growth hormone, then computed tomography of the chest (lungs, thymus) and abdominal cavity (gastrointestinal tract, pancreas) is additionally performed to search for somatoliberin ectopic adenocarcinoma (STL).

But there are also cases of ectopic secretion of somatotropic hormone, which was located in the islets of the pancreas and lymphofollicular tissue.

To confirm the ectopic secretion of somatoliberin, additional laboratory tests are carried out:

1. STL must be greater than 300 pg/ml
2. In the blood test, we additionally look for the presence of prolactin, because the tumor can be mixed and secrete not only growth hormone. This is necessary to determine the nativeness and aggressiveness of the adenoma.

Treatment of acromegaly is a rather problematic, complex and expensive procedure. It requires an individual approach to each patient.

Most patients (about 80%) require conservative (drug) therapy. Such a high percentage of patients dictates the need to achieve hormonal control of the disease. As well as preventing the progression of various complications, achieving a mortality rate comparable to the general population, which is the main ultimate goal of treatment.

To control acromegaly, complex therapy is always required, including suppression of growth hormone hypersecretion, reduction of IGF-1 levels, and control of tumor growth.

Surgical, pharmacological and radiotherapy methods are used to treat acromegaly. However, whatever methods are used, they should all be aimed at achieving target values ​​of biochemical control:

• STH not more than 2.5 ng / ml (not more than 1 μg / l with a highly sensitive method of determination) - in the treatment of SRLs
• Min. GH / OGTT less than 1 ng / ml (less than 0.4 μg / l) - after adenomectomy
• Normalization of IGF-1

Trial test with octreotide

To begin with, trial treatment with octreotide for acromegaly is carried out. This allows you to identify whether there are receptors in the adenoma. If they are not there, then most likely the adenomas there are quite aggressive.

In general, in general, somatotropinomas tend to grow more. They grow rather than produce growth hormone. At least until middle age. But in people over 50, they are usually softer.

But how can you guess?

An objective method is needed here in order to confirm the peculiarity of the course of the disease in different age groups. Accordingly, the octreotide test was used.

Octreotide test

It allows you to find out how the patient tolerates octreotide. Side effects of this medication:

• nausea
• vomit
• diarrhea

As can be seen from the table, 50 mcg is administered for the first time. Nevertheless, even with the introduction of such a sparing dose, the patient may develop a side effect.

One who cannot tolerate a short octreotide will most likely not tolerate a long one either. Only this adventure will be for 28 days, and not for 4 - 5. Therefore, for such cases, you need to know in advance the tolerance of the drug by the patient.

In general, this method is useful for differential diagnosis. We can solve the following tasks:

1. Should the patient be prepared for somatostatin analogues for surgery. After all, they won't help anyone. Only they can reduce the symptoms.
2. Someone has a big adenoma. I would like to reduce it. However, we don't know if octreotide will help or not. This is where testing helps. If IGF-1 decreased by less than 30%, then the person is considered not to have responded to the test. If more than 60% suppression, then this is a very good result. This suggests that a person can be led even to conservative therapies.

The first and only method that can cure the patient once and for all is the surgical treatment of acromegaly. Removing an adenoma, you can completely cure a person. He will no longer need medication and additional help.

The longer the illness goes on, the worse it gets.

Below is a picture that says that no one climbs into the brain itself. They go up the nose. Remove the anterior wall of the sphenoid sinus. Then they pass the anterior wall of the Turkish saddle and find themselves where the pituitary gland is.

As a rule, endoscopic access is used for such an operation. Everything passes through the nose and the adenoma is removed. The pituitary itself is not removed. It is, if possible, unaffected at all.

Thus, the operation is as minimally invasive as possible and localized in the desired area. That is, no one removes an adenoma through the head. This is done only in very rare cases, when a huge adenoma threatens a person's life.

But even very large adenomas are not always removed because they can grow into the brain. Therefore, after their removal, big problems can arise. Then you have to scrape out a piece of the brain, which is also not very good.

That is, if the patient is alive, more or less healthy and can be supported with medication, then sometimes it is better not to touch the adenoma.

But the principle is the following.

The first place of the operation - only it can radically cure a person. If doctors see that the operation cannot radically cure (for example, an adenoma encircles the siphons of the carotid arteries), then sometimes a piece of the volume should be removed to improve the course and help further medical treatment.

The chance of curing a microadenoma with surgery is 70%. For a macroadenoma, this is 30%. It is noteworthy that it is very difficult to identify acromegaly immediately. A person can live 10 to 15 years with this disease. Therefore, doctors do not detect this at an early stage of the disease.

Therefore, during late treatment, a person needs not only to be treated for acromegaly, but also for a bunch of other diseases.

Acromegaly is a common condition. The official prevalence of such a disease is 40 people per million. But the cost per patient exceeds 2-3 million rubles a year. This is a costly disease.

I also note that testing for IGF-1 is also not cheap. With complaints, doctors do not always carry it out, since it is economically unprofitable.

I note that in Germany they tried to conduct such tests for each patient. The prevalence of this disease was 1000 per million. That is much more than in official statistics.

Medical treatment of acromegaly

There is also a drug treatment for acromegaly. It consists of three methods:

1. Somatostatin analogs
2. dopamine receptor agonists

Indications for primary drug therapy:

• Presence of contraindications to surgical treatment
• The categorical refusal of the operation
• Expected unsatisfactory outcome of the operation (pronounced laterosellar adenoma growth)
• Guaranteed preservation of all functions of the hypophysis

Somatostatin analogs

Somatostatin analogues are Sandostatin Lar, Somatulin Autogel, Pasireotide. They are needed for ongoing treatment. People answer them in 80% of cases. The cost of treatment with such drugs is somewhere from 35,000 rubles per month. And this does not include the treatment of other diseases.

In Russia, 4 drugs from the group of analogues of self-tostatin are registered:

1. Somatulin Autogel (lanreotide, original drug, Ipsen, France)
2. Sandostatin Lar (octreotide, originator, Novartis, Switzerland)
3. Octreotide-Long (octreotide, F-Sintez, Russia)
4. Octreotide-Depot (octreotide, Pharmsintez, Russia)

Long-acting formulations of lanreotide and octreotide act primarily on somatostatin subtype 2 receptors and are approximately equally efficacious.

However, lanreotide and octreotide differ in how they are taken, which may affect patient convenience.

Dosage forms of lanreotide long-acting are available in ready-to-use pre-filled syringe tubes, the contents of which are injected subcutaneously. Dosage forms of octreotide long-acting before intramuscular injection require dilution.

Approximately 75% of patients with acromegaly previously treated with LAR octreotide every 4 weeks had normal IGF-1 levels after switching to Somatulin Autogel 120 mg every 6 or 8 weeks.

With the help of various studies, it was revealed that an increase in the interval between injections of the drug, while maintaining the therapeutic effect, can significantly simplify the injection regimen and increase the patients' preference for the therapy.

There is also domestic experience in the use of the drug. We analyzed 65 patients transferred to Lanreotide Autogel according to the following scheme:

• Patients initially treated with 10 mg octreotide every 28 days were switched to injections of 120 mg Somatulin Autogel every 56 days
• Patients initially treated with octreotide 20 mg every 28 days were switched to Somatulin Autogel 120 mg every 42 days
• Patients initially treated with 30 or 40 mg of octreotide every 28 days were switched to injections of 120 mg of Somatulin Autogel every 28 days

In a number of patients, the transfer from octreotide to Somatulin Autogel increased the effectiveness of long-term therapy, which was manifested in the normalization of biochemical parameters (in 12 out of 50 initially decompensated patients) and a moderate decrease in the size of the pituitary adenoma (in 19% of patients).

In this observation group, there were 10 patients who, due to the transfer to Somatulin Autogel 120 mg, managed to increase the intervals between the administration of the drug to 42 (in 8 patients) or 56 days (in 2 patients) without loss of effectiveness.

Thus, the optimal use of monotherapy or combination therapy allows achieving biochemical remission in the majority of patients suffering from acromegaly. At the same time, it is characterized by stable efficiency and safety with long-term use.

With good patient compliance to therapy, loss of efficacy over time against the background of drug therapy is rare.

I would also like to touch on the drug Pasireotide. Its effectiveness has been compared with Octreotide, since the latter acts mainly on type 2 receptors. Pasireotide, on the other hand, is a multi-analogue and acts on almost all receptor subtypes.

As shown by the interim results in the study of this drug, the group of patients treated with Pasireotide to a greater extent achieve normal levels of IGF-1.

However, it should be said that very often, while taking this drug, patients with acromegaly develop such a side effect as the induction of carbohydrate metabolism disorders or the deterioration of carbohydrate metabolism in existing disorders.

As a result, patients constantly need to control carbemia and prescribe hypoglycemic therapy, which worsens the quality of human life.

dopamine receptor agonists

Dopamine agonists are usually cabergoline. This method is used when somatostatin analogs at the maximum dose do not give results.

Cabergoline is always 0.5 mg per tablet. Do not use more than 3 mg per week, as the effectiveness disappears further. You can use up to 7 mg, but just a large amount does not give good results.

Approximately 80% of patients receiving Pegvisomant monotherapy achieve normal levels of IGF-1. If normal values ​​are not reached, it is recommended to combine Pegvisomant with Cabergoline.

growth hormone receptor blockers

Growth hormone receptor blockers are very expensive drugs that will cost you about 80,000 rubles a week. It is used only for the most severe patients who have not been helped by previous treatment options for acromegaly.

Such drugs block the action of growth hormone in the periphery and thereby reduce IGF-1. That is, the effect is excellent.

But the problem is that using only these drugs is still considered unsafe. When they block the production of IGF-1, the pituitary gland inevitably tries to synthesize even more growth hormones. And this can provoke the growth of adenoma.

Therefore, such blockers are usually used in combination with somatostatin analogues.

Radiation therapy for acromegaly

If the previous methods do not help, then begin to use radiation therapy in the treatment of acromegaly. It is basically Gamma Knife and Cyber ​​Knife.

Radiation therapy in the treatment of acromegaly

The effectiveness of radiation therapy is about 30%. But it is worth saying that with patients who are unpromising in treatment plans, this method will not be superfluous.

Previously, proton therapy was still used, but now it is not carried out. Proton therapy- this is one beam of particles that is sent through the pituitary gland. That is, it affects through and through all organs and tissues. It is also worth adding here that panhypopituitarism develops in almost all cases.

Mostly more modern methods are used. Such as gamma knife And cyber knife. They differ in that the beam does not pose any threat to surrounding tissues.

But there are several rays and they all intersect at one point, where the adenoma is located. Accordingly, they have a therapeutic effect. In fact, they burn it out.

In fact, this treatment is very simple. The most important thing is for the doctor to suspect and identify this disease in time. In addition, he still needs to convey to the patient the need for treatment.

Although people with acromegaly may not notice a change in their appearance, there are special questions for patients that reflect the essence of the process. That is, when a doctor asks if the appearance has changed, people usually do not admit it.

Another problem is that they stop being photographed.

Therefore, doctors must understand that if a person does not take pictures, does not participate in competitions, does not go out, then this is already an alarming signal.

As a result, people desocialize.

And when they are explained that they are not alone with such problems, then only at this stage it can become much easier for a person. Therefore, the doctor needs to properly prepare his patient.

Why does the appearance change? There are two factors that make growth hormone:

1. GH causes swelling of all tissues
2. Over time, it causes deposits of connective tissue. In the future, this can lead to cell apoptosis. And then there is fibrosis. If it happened, then it is irreversible. If a person is in the early stages of edema, then everything is reversible. Therefore, this is another reason to diagnose not by external, but by metabolic problems. For example, headache, fatigue, sweating, and so on.

That's all!

Now you know what acromegaly is and what are its causes and symptoms. We also looked at photos. Plus, we talked in more detail about the diagnosis and treatment of such a disease. In general, be healthy!

Acromegaly is a disease associated with pathology in the work of the pituitary gland, which, due to a tumor, begins to produce an increased amount of somatotropin growth hormone. The disease is characterized by enlargement of facial features, ears, nose, lips, as well as an increase in hands and feet. Therefore, you can understand in appearance what acromegaly means - it is difficult to confuse a photo of a person with this disease with a photograph of a healthy one.

This disease begins to develop after full puberty, during which an increased amount of somatotropin growth hormone is normal. Signs of the disease develop over a sufficiently long time, the appearance gradually changes, facial features become larger and rougher. On average, the development period takes about seven years. Men and women are equally affected, whose age is approximately 40-50 years.

The cause of acromegaly is increased secretion of somatotropin by the pituitary gland. Under normal conditions, the secretion process is controlled by the hypothalamus, which secretes substances that can either accelerate (somatoliberin) or slow down the production of growth hormone (somatostatin). Normally, the amount of somatotropin fluctuates significantly during the day, its concentration increases at night, the highest level is reached in the morning. In patients suffering from this disease, there is not only a significantly increased concentration of somatotropin growth hormone, regardless of the time of day, but also a violation of the secretion process.

Scientific studies have proven a direct link between acromegaly and pituitary adenoma. The proliferation of endocrine gland cells leads to the fact that they begin to produce an increased amount of somatotropin, and the process is no longer regulated by the hypothalamus. A benign pituitary tumor can reach a size of several centimeters and significantly compress the healthy tissue of the endocrine gland.

It is with the violation of the activity of the pituitary gland due to adenoma that the appearance of symptoms of the disease is associated (in 90% of cases). A rather large role is played by heredity and genetic predisposition, since most often the disease occurs in relatives.

Disease pathogenesis

Usually, in humans, the growth process in the absence of disease ends already in adolescence. With increased secretion of somatotropin growth hormone, an adult organism cannot grow upwards, therefore it begins to increase in width due to a significant growth of soft tissues and bones of the skeleton, and this growth occurs disproportionately. As a result, the internal organs and limbs increase, but the symptoms of the disease are most pronounced on the face of the patient, whose features become larger and coarser.

The increased work of the pituitary gland leads to the fact that the cells begin to divide intensively, increasing the mass and size of the internal organs, which, due to the growth of connective tissue, cease to function fully, which can lead to their insufficiency due to sclerotic changes. This condition threatens the development of both benign and malignant tumors of organs and tissues.

Stages of development of the disease

As mentioned above, acromegaly does not develop in a month or two, it takes an average of about seven years. The disease develops in the following stages:

• Preacromegaly is characterized by an almost complete absence of symptoms, the disease can only be diagnosed by the results of a blood test, according to which an increased concentration of somatotropin growth hormone is established, as well as after a computed tomography of the brain.
• At the hypertrophic stage of the disease, all the symptoms appear quite clearly;
• At the tumor stage, the adenoma becomes so large that it begins to compress the adjacent sections of the brain, disturbances appear on the part of the optic and other nerves, and intracranial pressure increases.
• At the stage of cachexia, the body comes to exhaustion due to impaired functioning of the patient's internal organs.

Symptoms of the disease

Do not think that the symptoms of acromegaly appear only externally - the work of internal organs is also disrupted.

The main signs of the disease are:

• Change in the appearance of the patient: enlargement and coarsening of the face as a whole, ears, nose, lips and chin, the superciliary arches become more massive, the size of the gap between the teeth increases, the lower jaw protrudes forward, the feet and hands become larger;
• Deepening of the voice due to thickening of the vocal cords;
• Violation of the stable position of the vertebrae and damage to the joints, resulting in constant pain;

• The skin that forms natural folds on the body becomes hyperpigmented;
• Growth of thyroid tissue, possible formation of nodes;
• The appearance of diabetes mellitus, which is not amenable to therapy with insulin;
• Disrupted work of the cardiovascular system, which can lead to coronary insufficiency and death;
• Respiratory system disorders: restrictive insufficiency, in which the lungs do not fully expand, as well as sleep apnea, which occurs due to an increase in the size of the tongue;
• The appearance of carpal tunnel syndrome, the main symptom of which is a decrease in the sensitivity of the fingers on the hands due to the growth of soft tissues that compress the nerve;
• Rapid fatigue, decreased performance, lack of strength.

With the further development of acromegaly disease, when the pituitary tumor increases significantly in size, the functioning of the gland is disrupted due to compression of healthy cells. As a result, there are:

• Severe headaches that do not subside after taking painkillers may also be accompanied by a fear of light and increased work of the lacrimal glands;
• Various neurological disorders;
• Reduced libido and erectile dysfunction in men;
• Disruptions in the menstrual cycle and further infertility in women.

Lack of adequate treatment leads to death, with half of untreated patients dying before the age of fifty, 60% of deaths are due to disorders of the cardiovascular system, 25% due to respiratory diseases, and 15% deaths are caused by malignant tumors of internal organs.

Diagnosis of the disease

The disease in the later stages can be recognized externally: it is the increase in limbs and facial features that can cause an appeal to an endocrinologist. He is obliged to send for further examinations, first of all - to the laboratory for blood donation.

There are only two criteria by which the diagnosis of the disease is made according to the results of the analysis:

• The level of growth hormone somatotropin;
• The level of insulin-like growth factor (IGF).

In almost all patients, an increase in the concentration of somatotropin in the blood is observed, regardless of the time of day. This hormone affects the human body with the help of IGF, and if its level is also elevated, this indicates the presence of a disease.

A blood test for somatotropin after taking 75 grams of glucose is quite effective for diagnosing. Blood is taken every half an hour for two hours. Normally, the level of somatotropin growth hormone should decrease after taking glucose, but in the presence of a disease, the level not only does not decrease, but can even increase. Such a test is usually used if a slight increase in the level of somatotropin was noticed during a blood test, or if it remained normal.

Computed tomography and X-ray of the skull must be done. An x-ray examines an area called the Turkish saddle and is associated with the pituitary gland. Its increase is a sign of disease.

Treatment of the disease

The main goal of treatment is to reduce the concentration of growth hormone in the blood due to the impact on the process of its production. In modern medicine, the following methods of treating acromegaly are used:

• Medication, in which the patient takes drugs that can mimic the effect on the pituitary gland of somatostatin, which slows down the production of somatotropin growth hormone. These drugs include octreotide, somatulin, and sandostatin. In the presence of this disease, the endocrinologist often prescribes additional sex hormones that stimulate the production of dopamine: cabergoline, abergin.
• Radiation, which is used if treatment with medication has not had the desired effect. With this method, the pituitary region is irradiated with gamma rays once.
• The surgical method involves the removal of a tumor of the endocrine gland through the sphenoid bone. If the adenoma was small, then in 85% of cases there is a remission, and the level of somatotropin growth hormone quickly returns to normal. If the tumor has grown to a significant size, then surgery can help in about 30% of cases.
• When using the combined method, all of the above methods of treating the disease can be used simultaneously at the discretion of the doctor.

Forecasts

In the absence of proper therapy, the patient quickly reaches disability, and the risk of premature death increases significantly. Only ten percent of those affected reach the age of sixty years. Most often, death occurs due to pathologies of the cardiovascular system, especially due to coronary insufficiency.

If treatment was started in the early stages of the disease, then the risk of remission after treatment of the disease is very low. Surgical treatment is especially effective in the early stages, but when it is used, mortality is observed in 0.2-5% of cases. If the size of the tumor of the endocrine gland has reached a large size, surgical intervention may not have the desired effect and exacerbations of the disease will periodically occur.

People who have had signs of the disease in their family should know what acromegaly is, since they are at risk of getting this disease. For prevention, it is necessary to monitor the condition of the sinuses, since frequent sinusitis can lead to such a disease, and also to avoid severe traumatic brain injuries. Regular blood tests can detect the development of the disease in the early stages, when it can still be corrected even without surgery.

Bibliography

1. Bessonov P.P., Bessonova N.G. Syndromic diagnosis of chronic liver diseases.
2. A guide for emergency physicians. help. Edited by V.A. Mikhailovich, A.G. Miroshnichenko. 3rd edition. St. Petersburg, 2005.
3. Palshina A.M. Selected issues of cardiology.

Acromegaly is a disease of the pituitary gland associated with increased production of growth hormone - somatotropin, characterized by increased growth of the skeleton and internal organs, enlargement of facial features and other parts of the body, and metabolic disorders. The disease debuts when the normal, physiological growth of the organism has already been completed. In the early stages, the pathological changes caused by it are subtle or not noticeable at all. For a long time, acromegaly progresses - its symptoms increase, and changes in appearance become apparent. On average, it takes 5-7 years from the appearance of the first symptoms of the disease to the diagnosis.

Persons of mature age suffer from acromegaly: as a rule, in the period of 40–60 years, both men and women.

Effects exerted by somatotropin on human organs and tissues

Somatotropic hormone is produced by the cells of the pituitary gland.

The secretion of growth hormone - somatotropin - is carried out by the pituitary gland. It is regulated by the hypothalamus, which, if necessary, produces the neurosecretions somatostatin (inhibits the production of somatotropin) and somatoliberin (activates it).

In the human body, growth hormone ensures the linear growth of the child's skeleton (i.e., its growth in length) and is responsible for the correct formation of the musculoskeletal system.

In adults, somatotropin is involved in metabolism - it has a pronounced anabolic effect, stimulates protein synthesis, helps to reduce fat deposition under the skin and enhance its combustion, and increases the ratio of muscle to fat mass. In addition, this hormone also regulates carbohydrate metabolism, being one of the contrainsular hormones, i.e..

There is also evidence that the effects of somatotropin are also immunostimulatory and increased absorption of calcium by bone tissue.

Causes and mechanisms of development of acromegaly

In 95% of cases, the cause of acromegaly is an adenoma, or somatotropinoma, which provides increased secretion of growth hormone. In addition, this disease can occur when:

• pathology of the hypothalamus, provoking increased production of somatoliberin by it,
• increased production of insulin-like growth factor;
• increased sensitivity of tissues to somatotropic hormone;
• pathological secretion of growth hormone in the internal organs (ovaries, lungs, bronchi, organs of the gastrointestinal tract) - ectopic secretion.

As a rule, acromegaly develops after injuries of the central nervous system, infectious and non-infectious inflammatory diseases.

It has been proven that those persons whose relatives also have this pathology are more likely to suffer from acromegaly.

Morphological changes in acromegaly are characterized by hypertrophy (increase in volume and mass) of the tissues of internal organs, proliferation of connective tissue in them - these changes increase the risk of developing benign and malignant neoplasms in the patient's body.

Clinical manifestations of acromegaly

Subjective signs of this disease are:

• enlargement of brushes, feet;
• an increase in the size of individual facial features - large superciliary arches, a nose, a tongue (there are teeth marks on it), an enlarged protruding lower jaw attract attention; gaps appear between the teeth, skin folds appear on the forehead, the nasolabial fold becomes deeper, the bite changes;
• coarsening of the voice;
• headache;
• paresthesia (feeling of numbness, tingling, crawling in various parts of the body);
• pain in the back, joints, limitation of joint mobility;
• swelling of the upper limbs and face;
• dizziness, vomiting (they are signs of increased intracranial pressure in a pituitary tumor of significant size);
• numbness of the limbs;
• menstrual disorders;
• decreased sexual desire and potency;
• blurred vision (double vision, fear of bright light);
• decreased hearing and sense of smell;
• outflow of milk from the mammary glands -;
• periodic pain in the region of the heart.

An objective examination of a person suffering from acromegaly, the doctor will detect the following changes:

• again, the doctor will pay attention to the enlargement of facial features and limb sizes;
• deformations of the bone skeleton (curvature of the spine, barrel-shaped - enlarged in the anteroposterior size - chest, enlarged intercostal spaces);
• swelling of the face and hands;
• sweating;
• (increased hair growth in women according to the male pattern);
• an increase in the size of the thyroid gland, heart, liver and other organs;
• proximal myopathy (i.e., changes in the muscles located in relative proximity to the center of the body);
• increased blood pressure;
• measurements on the electrocardiogram (signs of the so-called acromegaloid heart);
• elevated levels of prolactin in the blood;
• metabolic disorders (in a quarter of patients there are signs of diabetes mellitus, resistant (resistant, insensitive) to hypoglycemic therapy, including insulin administration).

In 9 out of 10 patients with acromegaly in its advanced stage, symptoms of sleep apnea syndrome are noted. The essence of this condition lies in the fact that due to hypertrophy of the soft tissues of the upper respiratory tract and disruption of the respiratory center in humans, short-term respiratory arrests occur during sleep. The patient himself, as a rule, does not even suspect about them, but relatives and friends of the patient pay attention to this symptom. They note night snoring, which is interrupted by pauses, during which the respiratory movements of the patient's chest are often completely absent. These pauses last a few seconds, after which the patient suddenly wakes up. There are so many awakenings during the night that the patient does not get enough sleep, feels overwhelmed, his mood worsens, he becomes irritable. In addition, there is a risk of death of the patient if one of the respiratory arrests is delayed.

In the early stages of development, acromegaly does not cause discomfort to the patient - not very attentive patients do not even immediately notice an increase in one or another part of the body in size. As the disease progresses, the symptoms become more pronounced, eventually appearing symptoms of heart, liver and lung failure. In such patients, the risk of getting sick with hypertension is an order of magnitude higher than in individuals who do not suffer from acromegaly.

If a pituitary adenoma develops in a child, when the growth zones of his skeleton are still open, they begin to grow intensively - the disease is manifested by gigantism.

Diagnosis and differential diagnosis of acromegaly

In the blood with this pathology, an increased level of somatotropic hormone will be found.

The doctor will suspect this pathology already on the basis of the patient's complaints, the history of the disease (the nature of the symptoms progressing over several years) and the results of an objective examination of the patient (during a visual examination, he will pay attention to enlarged parts of the body, palpation will reveal pathological sizes of internal organs). In order to confirm the diagnosis, the patient will be assigned additional diagnostic methods:

• the level of somatotropic hormone in the blood on an empty stomach and after taking glucose (in a person suffering from acromegaly, the level of somatotropin will always be elevated - including on an empty stomach; after taking glucose, the hormone level is determined three more times - after 30 minutes, 1.5 and 2 hours; in a healthy body, after taking glucose, the content of somatotropic hormone in the blood decreases, and in the case of acromegaly, on the contrary, it increases);
• less often, tests are performed with thyro- or somatoliberin, which stimulate the production of growth hormone, or a test with bromocriptine, which suppresses its secretion in people suffering from acromegaly;
• determination of insulin-like growth factor - IGF-1 (the concentration of this substance in the blood plasma reflects the amount of growth hormone released per day; if IGF-1 is elevated, this is a reliable sign of acromegaly).

When determining the level of somatotropin, it is important to remember that the peak of its secretion in a healthy person occurs at night, and in the case of acromegaly this peak is absent.

The diagnosis of acromegaly is confirmed by the results of the instrumental method of research - x-ray of the skull: the picture will visualize an enlarged sella turcica. To see the pituitary adenoma directly, the patient is given a CT or magnetic resonance imaging.

In case of complaints of the patient about the deterioration of vision, he is prescribed a consultation with an ophthalmologist. During the examination, the latter will find the narrowing of the visual fields characteristic of acromegaly.

If the patient presents with complaints characteristic of the pathology of a particular internal organ, additional research methods are prescribed to him individually.

The differential diagnosis for suspected acromegaly should be with hypothyroidism and Paget's disease.

signs	Acromegaly	Paget's disease	Hypothyroidism
Feelings of the patient	The patient is worried about headache, pain in the back and joints, limitation of their mobility, changes in appearance - an increase in the size of facial features and limbs.	The patient complains of pain in the bones, severe general weakness.	The patient is concerned about the progressive deterioration of memory, hair loss, constipation, chilliness.
Objective changes	The appearance of the patient is changed, the skin is wrinkled, thickened, there are signs of enlargement of internal organs - the liver, spleen.	The proximal (those closer to the center of the body) sections of the tubular bones (shoulder, femur, etc.) are thickened and deformed.	There is dry skin, swelling, the number of heartbeats is reduced - bradycardia. Children are undersized.
Results of the laboratory examination	In the blood, the content of somatotropic hormone and prolactin is increased.	Changes in the level of hormones in the blood are not observed.	The level of thyroid hormones in the blood is reduced, and thyrotropin is elevated.

Treatment of acromegaly

The goal of treatment is to normalize the blood levels of growth hormone (lowering it below 5 ng / ml) and IGF-1.

For treatment, medical, surgical, radiation methods, as well as their combinations, can be used. The choice of method depends on the size and growth rate of the pituitary tumor, the condition of the organ of vision, the level of somatotropic hormone, the age of the patient and the presence of concomitant pathology.

For uncomplicated, non-progressive pituitary adenomas, drug therapy is recommended. Cabergoline (Dostinex) is used at a dose of 0.5 mg twice a week and bromocriptine (Parlodel) 2.5–10 g per day. In recent years, the drug Octreotide, a long-acting synthetic analogue of somatostatin, has proven itself well, suppressing the production of somatotropin by the pituitary gland by reducing the size of the adenoma.

If the adenoma has reached a significant size, or the disease is rapidly progressing, drug therapy alone will not be enough - in this case, the patient is shown surgical treatment. A direct indication for surgery is. The tumor is removed through the sphenoid bone. In 85% of patients, after removal of the tumor, there is a significant decrease in the level of somatotropin up to the normalization of indicators and a stable remission of the disease. With a particularly large size of the tumor, one operation is probably not limited - as a result of it, only a third of patients are cured, while the rest require a second operation or continuation of therapy by other methods.

Acromegaly is a disease of a neuroendocrine nature, the development of which is due to excessively intense production of somatotropin, popularly referred to as growth hormone. In this case, as a rule, only certain parts of the patient's body increase. This pathology is extremely rare and gives patients a lot of torment, both physical and psychological.

Brief description of the pathology

Acromegaly develops, as a rule, with tumor neoplasms localized in the anterior pituitary gland, which is responsible for the production of somatotropin. In patients suffering from this pathology, facial features change (they become larger), hands and foot size increase. In addition, the pathological process is accompanied by painful articular and, there are violations in the reproductive system.

Important! Acromegaly affects only adult patients. Pathology begins to develop at the end of puberty and body growth!

According to statistics, patients in the age group from 40 to 60 years are most susceptible to acromegaly. The pathological process is characterized by a gradual, slow course. In most cases, the disease is diagnosed after 6-7 years from the onset of its development, which significantly complicates subsequent treatment.

Doctors distinguish the following stages of development of the pathological process:

1. At the first stage, the disease proceeds in a latent, latent form, and changes can only be detected when carried out.
2. At this stage, the symptoms characteristic of the pathology manifest themselves especially clearly.
3. At the third stage, there is an increase in the tumor neoplasm located in the anterior lobe of the pituitary gland. At the same time, adjacent brain regions are compressed, which causes the manifestation of specific signs, such as visual impairment, nervous disorders,.
4. The last fourth stage of acromegaly is characterized by the development of cachexia and the complete exhaustion of the patient's body.

An increased concentration of somatotropin contributes to the development of cardiovascular, pulmonary and oncological diseases, which often cause the death of patients suffering from acromegaly.

Causes of acromegaly

To provoke disturbances in the normal functioning of the hypothalamus, and, consequently, the development of acromegaly, according to doctors, the following factors can:

• mental disorders;
• complicated pregnancy and intrauterine developmental pathologies;
• transferred infectious or viral diseases;
• the presence of tumor neoplasms of benign or malignant origin that affect the functioning of the nervous system;
• disturbances in the activity of the endocrine system;
• hypersensitivity to growth hormone;
• pathological processes of secretion of this hormone in the area of ​​internal organs;
• traumatic injuries of the central nervous system;
• hereditary predisposition;
• pituitary adenoma;
• cystic neoplasms localized in the region of the brain.

Note: According to doctors, the gender of the patient also affects the likelihood of developing acromegaly. The fair sex is most susceptible to this pathology.

Symptoms of acromegaly

As a rule, the symptoms of acromegaly appear at the second stage of the course of the pathological process. The signs of this disease are quite specific, and therefore it is difficult not to notice them.

In most cases, patients with acromegaly present with the following clinical symptoms::

Note: Almost all patients diagnosed with acromegaly show signs characteristic of diabetes mellitus, which is associated with endocrine disorders.

In addition, in patients with acromegaly, the structure of the skeleton also changes, the intercostal spaces increase, the spinal column is bent, and the chest becomes noticeably larger and wider.

The fair sex also has an early onset. In men suffering from this disease, erectile dysfunction occurs, develops. The reproductive system in acromegaly is seriously affected, therefore, as a rule, people with this diagnosis have problems with natural conception.

What is dangerous pathology?

In addition to the fact that acromegaly itself spoils the patient's appearance and significantly reduces the quality of his life, in the absence of proper treatment, this pathology can also provoke the development of extremely dangerous complications.

In most cases, the long course of acromegaly leads to the following comorbidities:

Note: Almost half of the patients suffering from acromegaly develop such complications as.

Violations of visual and auditory functions characteristic of this pathology can cause complete deafness and blindness of the patient. Moreover, these changes will be irreversible!

Acromegaly greatly increases the risk of malignant neoplasms, as well as various pathologies of internal organs. Another life-threatening complication of acromegaly is the respiratory arrest syndrome, which mainly occurs in a state of sleep.

That is why a patient who wants to save his life, when the first signs indicating acromegaly appear, should seek professional help from a qualified specialist - an endocrinologist!

How to identify the disease?

In most cases, a specialist can suspect the presence of acromegaly already by the patient's appearance, characteristic symptoms and during the analysis of the collected anamnesis. However, in order to make an accurate diagnosis, determine the stage of the pathological process and the degree of damage to internal organs, patients are prescribed the following diagnostic tests:

• to identify indicators of somatotropin hormone;
• brain;
• skulls.

Important! The main diagnostic method is the analysis of somatotropin using glucose. If the pituitary gland is functioning normally, glucose contributes to a decrease in somatotropin levels, otherwise the hormone level, on the contrary, increases.

To identify concomitant complications provoked by the development of acromegaly, the following additional diagnostic measures are carried out:

• laboratory research;
• internal organs.

After conducting a comprehensive diagnosis, a specialist can not only make an accurate diagnosis, but also identify the presence of concomitant diseases, which allows the patient to prescribe the most complete and adequate therapeutic course for a particular case!

Methods for the treatment of acromegaly

The main task of doctors in diagnosing acromegaly is to achieve stable remission, as well as to normalize the processes of somatotropin production.

For these purposes, the following methods can be used:

• taking medications;
• radiation therapy;
• surgery.

Important! In most cases, effective control of this disease requires complex combination therapy..

conservative methods

To suppress excessively intensive production of growth hormone, patients are prescribed a course of hormone therapy using artificial somatostatin analogues. Often, patients are also prescribed a drug such as Bromocriptine, which is aimed at producing dopamine, which suppresses the processes of synthesizing the somatotropin hormone.

In the presence of characteristic complications and concomitant diseases, appropriate symptomatic treatment is carried out, the scheme of which is developed for each patient individually.

Good results were shown by the use of radiation therapy. This procedure is an impact on the damaged area of ​​the pituitary gland with specific gamma rays. According to statistics and clinical studies, the effectiveness of this technique is about 80%!

One of the most modern methods of conservative treatment of acromegaly is X-ray therapy.. According to experts, exposure to X-ray waves contributes to the active suppression of the growth of tumor neoplasms and the production of somatotropin. A full course of X-ray therapy allows you to achieve stabilization of the patient's condition and eliminate the symptoms characteristic of acromegaly, even the patient's facial features become somewhat thinner!

Surgical treatments for acromegaly

Surgical intervention for acromegaly is indicated with a significant size of the tumor, the rapid progression of the pathological process, and also in the absence of the effectiveness of conservative therapy methods.

Important! Surgery is one of the most effective methods of dealing with acromegaly. According to statistics, 30% of operated patients have completely recovered from diseases, and 70% of patients have a stable, long-term remission!

Surgery for acromegaly is an operation aimed at removing a pituitary tumor neoplasm. In particularly difficult cases, a second operation or an additional course of drug therapy may be required.

How to prevent pathology?

In order to prevent the development of acromegaly, physicians are advised to adhere to the following recommendations.

Acromegaly is a rare and dangerous disease, fraught with a number of complications. However, timely diagnosis and competent, adequate treatment can achieve stable remission and return the patient to a full, normal life!

Sovinskaya Elena, medical columnist