Gigantism is associated with dysfunction of something. Pituitary gigantism (Acromegaly)

Acromegaly and gigantism in children are diseases in which there is a failure of chemical metabolism from the pituitary gland to the brain. As a result, there is an increase in the level of growth hormone - somatotropic hormone or growth hormone, which entails an increase in size musculoskeletal system and disruption of metabolic functions in the body.

HGH has essential functions for the body. Responsible for growth in length and the formation of protein complexes throughout the body, is involved in the production of pancreatic hormone, and increases blood sugar levels.

In acromegaly in children, the growth substances do not close and bone elongations form. The disease is quite rare. In comparison, acromegaly is more common than gigantism in children. On average, cases of the disease in Russia number 45 people per million children. At the age of 1-16 years, with maximum growth activity, gigantism is observed, at the end of the process acromegaly.

If previously, upon diagnosis, children were doomed, now the mood is only for recovery and stabilization of the process.

One of the main causes of acromegaly lies in the excess production of growth hormone. The violation stems from a number of other problems, such as:

  • Pituitary tumors, both malignant and benign;
  • Insulin-like growth factor disorder;
  • A hereditary factor is considered if the disease was observed in the family in one generation and there is a likelihood of manifestation in the next;
  • Inappropriate growth of the pituitary gland due to tumors of other organs.

All processes associated with growth hormone are controlled by the hypothalamus. The hypothalamus produces somatropin and somatostatin. The first stimulates growth. The second one slows him down. At an early age, growth hormone controls the processes of bone formation, ossification and increase in body length. In an adult body - metabolism and water-salt balance.

With acromegaly, the pituitary gland, a gland located at the base of the brain, produces growth hormone, but does not perceive or respond to impulses from the hypothalamus due to a malfunction of the pituitary gland.

Normally, the pituitary gland receives an impulse from the hypothalamus, and the cycle of production and control of pituitary hormones begins. They are in a process dependent on each other, a closed circle of metabolism. At this stage, the liver produces insulin-like growth factor. He, in turn, is responsible for the growth of peripheral parts of the body.

If the processes are not stabilized, the pituitary gland does not stop the production of growth hormone, the level of IGF I also increases and leads to an irreversible process. The body as a whole begins to grow, and internal functions generally lose the ability to transform necessary substances like sugar and fats.

Another cause of gigantism and acromegaly in children is a neoplasm of the pituitary gland - adenoma. The tumor occurs due to traumatic brain injury and inflammation of the sinuses.

The neoplasm in this case serves as a stabilizer for the increased release of growth hormone. As the adenoma progresses, compression of nearby tissues and brain systems occurs, causing functional disorders organ of vision.

As the tumor grows, the production of other pituitary hormones is disrupted. There is a violation of sexual function in women, which, if not treated in a timely manner, will cause infertility. Atypical manifestations of increased levels of growth hormone are observed in tumors of the pancreas, adrenal glands, thyroid gland.

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Symptoms

The disease is characterized by an imperceptible onset and a slight increase in complaints and changes in the personality phenotype. The main symptom of acromegaly in children is a change appearance, in particular facial features and height. Often the diagnosis is made from the moment of illness after 5-7 years.

Signs of acromegaly by appearance:

  • Facial features become rough. The cheek bones and lower jaw become larger, the brow ridges become larger;
  • Enlargement of the nose, lips and ears;
  • Since the lower jaw is enlarged, this leads to a number of consequences: discrepancy between the teeth, resulting in a change in the shape of the jaw, an enlargement of the tongue;
  • The size of shoes, in particular the feet, increases;
  • Arthralgia due to the growth of joint tissue;
  • As a result of increased secretion of sebaceous and sweat glands, in children, increased sweating and deep folds on the scalp;
  • Increase muscle mass, sometimes young men are delighted with this transformation, but after a while myocardial hypertrophy is observed, due to the large production of growth hormone.

Upon inspection:

  • Headache;
  • Memory impairment;
  • Frequent apnea;
  • Frequent detection of clinical diabetes mellitus;
  • Enlargement of the thyroid gland to;
  • Pathological increase in the size of the spleen;
  • Neoplasm of the adrenal glands, uterus, ovary, intestines.

Diagnostics

Since the disease begins to progress 5-7 years from the onset and gives symptoms, it is possible to suspect signs of the disease based on examination. Children have coarse facial features and height does not correspond to body proportions. During an objective examination, patients are found to have an enlarged spleen. If a preliminary diagnosis of acromegaly has been made, laboratory diagnostics must be performed. To do this, a blood test is taken to determine the level of growth hormone. Even if the child does not have a clinical picture, there may already be changes in the analysis.

It is also necessary to conduct a daily analysis of somatotropic hormone; acromegaly is characterized by daily fluctuations in the level of growth hormone.

If the slightest increase in somatotropin levels is detected, tests are carried out with glucose, insulin and thyrotropin-releasing hormone. With acromegaly, the test result may be either an increase in growth hormone or a lack of suppression. Next, IGF I is determined, which has the advantage of increasing. CT and MRI scans determine the size of the pituitary tumor and its progression.

With acromegaly and gigantism in children there is a nonspecific increase in the level alkaline phosphatase and phosphorus level, a biochemical blood test is done to detect it.

Treatment

There are four directions in the treatment of the disease: conservative, surgical, radiation, combined. All methods have one goal: to achieve stabilization of the level of growth hormone.

Operation

At surgical intervention In small tumor sizes, stabilization of the process and improvement in well-being are observed. The predominance of maximum tumor sizes plays a huge role, surgical treatment successful in this case only in a few percent of cases.

Surgical treatment of acromegaly and gigantism in children is carried out with minimal tissue trauma through a transnasal approach.

Radiation treatment is mainly used in a combination of several methods, such as independent method rarely used. The only exception to its use is considered a contraindication for intolerance hormone therapy, to surgery and postoperative relapse. The most commonly used is proton therapy with a dose of up to 100 grams.

Drugs

Medicines are most often used in several groups:

  • somatotropin antagonists Lanreotide, Octreotide;
  • somatotroph receptor blockers Pegvisomantom;
  • dopamine receptor stimulants Dostinex.

It is believed that the funds traditional medicine, also have an auxiliary effect in the treatment of acromegaly and gigantism in children. These are herbs such as: hops, sage, chamomile, linden flowers, licorice root.

Diet

Any treatment other than medication or surgery includes correct image the patient's life. This means you can’t do without a diet. Need to stick proper nutrition, eat foods rich in calcium and estrogens. Estrogens have the properties of inhibiting or completely stopping the production of growth hormone. Calcium will strengthen bones and improve their strength. Products rich in these substances: apricot, cabbage, sour cream, nuts, shrimp. Carbohydrates also slightly reduce the level of growth hormone, but their amount of consumption needs to be controlled.

Forecast

At timely diagnosis disease and treatment, a favorable outcome of the disease is observed. It is worth treating nasopharyngeal disease in the early stages, avoiding hard work, and excluding traumatic brain injuries. If laboratory readings change, drug treatment should be started and the process stabilized.

If the disease progresses and is not treated, the outcome is cachexia. Life expectancy will be 3-4 years. With a slow development of the disease, up to 10 years of life from the onset of the disease.

A child's rapid growth rate is usually less of a concern than slow growth. Especially if one or both of the parents is taller than the average of their gender. However, sudden changes in body size may be symptoms of serious endocrine disorders. Gigantism in preschool and younger children school age diagnosed quite rarely. The manifestation of the disease most often occurs in the prepubertal and pubertal periods.

Appearance of children with pathology

At the very beginning of the disease, the child is practically no different from his peers. Appearance changes as it progresses.

The nature of the changes depends on the type of gigantism:

  • True - all parts of the body grow proportionally. Excess age standards observed from birth. There are no deviations in the psyche or development of internal organs. Treatment in this case is not required.
  • Partial (partial) - certain parts of the body grow faster than others. Most often these are the areas of the arms and legs.
  • Half - one half of the body becomes larger than the other.
  • Eunuchoid - characterized by disproportionate elongation of the arms and legs in relation to the body, complete or partial absence of secondary sexual characteristics in the adolescent.

With a significant increase in the size of individual internal organs, they speak of splanchnomegaly. In this case, protrusion of the damaged organ may be observed.

As separate disease secrete acromegaly, external symptoms which, in addition to gigantism, are disproportions between soft tissues and bony skeleton: facial features become excessively large, feet and toes thicken, and kyphotic curvature of the spine appears. Acromegaly is rarely diagnosed in children because it develops after growth has stopped.

With congenital gigantism or macrodactyly caused by genetic abnormalities, the disease is visible already at birth or in the first months of life. Symptoms of the lesion in this case are mainly the hands and feet of the baby, which acquire an ugly shape.

Main symptoms

Gigantism begins to appear during puberty from 9-10 to 14-15 years. In some cases, the first symptoms may appear as early as 5-6 years of age. The disease occurs more often in boys than in girls.

Gigantism is more common in boys

In addition to increased growth, parents should be wary if their children complain of headaches and dizziness, joint and bone pain. There is weakness and fast fatiguability, visual impairment. The teenager’s memory declines and the ability to adequately respond to school workload deteriorates. Physical indicators can be joined psychical deviations and suppression of sexual functions: amenorrhea in girls, hypogonadism in boys.

If gigantism is not detected in a timely manner and there is no treatment, other hormonal disorders begin to appear - diabetes, an increase or decrease in the functions of the thyroid gland. suffer internal organs- heart, lungs, liver, which do not have time to respond to the rapid growth of the body. Due to low sensitivity to sex hormones, the ossification process is not completed. The patient continues to grow after puberty. Very high chances of becoming infertile.

Reasons for rejection

Macrosomia or gigantism occurs when the pituitary gland excessively secretes the hormone somatotropin (GH). This situation may arise in the following cases:

  1. Enlargement of pituitary cells.
  2. Increased production of growth hormone after traumatic brain injury, brain intoxication, meningitis or encephalitis, pituitary adenoma.
  3. Genetic abnormalities - Sotas, Marfan, Pyle syndromes and other congenital disorders of cell structure.

Diagnosis and treatment

Suspicions of gigantism can be confirmed by taking a blood test and undergoing an examination by an ophthalmologist. Laboratory tests for this pathology will reveal increased content somatotropic hormone. Gigantism will also be indicated congestion in the fundus and restrictions lateral vision. MRI, CT scan and an X-ray of the skull will help identify or rule out pituitary tumors.

A blood test can be done to diagnose the disease.

Treatment of gigantism is carried out depending on the reasons that caused it. Hormonal disorders are eliminated with medications that block the action of growth hormone and enhance the effect of sex hormones on bones. As a result, pathological growth slows down, ossification of the skeleton is completed, and the development of secondary sexual characteristics of the body is restored.

If the cause of macrosomia is a pituitary adenoma, then doctors decide to perform radiation chemotherapy, or surgery to remove the tumor.

Elimination external manifestations partial or half gigantism occurs through surgical interventions.

Timely detection of gigantism in a teenager and A complex approach to its treatment in a child is the key to a favorable outcome in the development of the disease. Any doubts about the child’s excessive growth, accompanied by alarming symptoms deterioration general condition, this is a reason to urgently contact specialists.

Gigantism is a disease characterized by clinical syndrome, which occurs as a result of increased production of somatotropic hormone in childhood or adolescence, and is also manifested by a sufficient increase in the body, especially in length. There is also partial or partial gigantism, which develops due to hypersensitivity in certain locations of tissue receptors for somatotropin. But genetic or constitutional gigantism is not considered an anomaly.

Typically, gigantism results from pituitary adenomas that produce growth hormone; hypogonadism; (with delayed puberty with preservation of growth zones in the bones).

Gigantism is diagnosed based on the external data of patients; X-ray, neurological, ophthalmological studies; determination of pituitary hormones in the blood, as well as family history.

With adequate treatment methods, the prognosis of gigantism is relatively favorable, but at the same time, the patients’ ability to work is reduced. In most cases, patients with gigantism are infertile.

Causes of gigantism

In 99% of cases, as first described in 1900 by K. Benda, the etiology of gigantism is associated with a tumor, somatotropinoma, which develops from eosinophils that form growth hormone (GH). Somatotropinomas are usually classified as benign pathologies, however, can sometimes become infested. Their localization is mainly noted in the adenohypophysis, but they can also be located in the gastrointestinal tract, pancreas, parapharyngeal areas, bronchi and sinuses of the main bones.

Almost one percent of gigantism occurs as a result of hyperproduction of somatocrinin in the hypothalamus and hyperplasia of somatotropes.

Among the risk factors that can provoke eosinophil cells to hyperplasia is even considered. Macroadenoma is the cause of the disease in 90% of cases, and 9% are microadenomas of the adenohypophysis. The rarest cause is “empty sella” syndrome. Also, the occurrence of gigantism can be influenced by the increased sensitivity of tissue receptors of epiphyseal cartilage to growth hormone.

There is a model of genetic pituitary gigantism. This species is based on somatic mutations in somatotrophs that cause a neoplastic reaction.

An excess amount of growth hormone leads to the growth of not only tissues and organs, but also hypertrophies the kidneys and myocardium, develops erythropoiesis and promotes wound healing.

Gigantism symptoms

Gigantism is classified into infantile and acromegalic forms. There are also two stages of the disease, such as stable and progressive.

The clinical picture of gigantism consists of complaints from adolescents in the form of weakness, almost constant headaches, bad memory, pain in the limbs and increased heart rate. In girls, there is an excessive increase in height and foot size, the mammary glands develop poorly, and in boys there is hyposexuality. Girls who are over 190 cm in height, and boys who are over 200 cm in height are very attracted to those around them.

Sometimes, in the symptoms of gigantism, a rough appearance appears in the foreground along with the general infantilism of a person. In addition, macrogenitalism is sometimes observed in young men. Patients with gigantism have a body weight that corresponds to their height, and their limbs are somewhat elongated.

The psyche of many patients is characterized by a certain underdevelopment in the form of average abilities, childishness, a tendency to depression and persecution mania.

People with gigantism always outperform others physically. Somatic organs are large, but this is usually not noticeable with high growth. But the size of the heart does not keep up with the growth of the body, which is why dizziness and fainting occur.

Depending on the hypertrophy and hyperplasia of soft tissues, macroglossia, diastema and enlargement of the larynx are noted. All this leads to changes in voice timbre, i.e. it sounds from the depths, very low. The ears, lips and nose also enlarge, and the lower jaw moves forward (prognathism). As a result of sinusitis, the mouth is often in a half-open position, as a result of which characteristic folds appear on the face. The skin becomes warm, moist, oily, hyperpigmented, and sometimes polyps, sebaceous cysts, acanthosis, etc. appear. The size of the head and foot increases. Sometimes kyphoscoliosis is diagnosed.

With gigantism, glucose perception is reduced, similar to latent and overt diabetes mellitus, with resistance to conventional methods therapy, as well as insulin therapy. But hyperglycemia does not suppress the secretion of growth hormone.

Almost 30% of young people have decreased potency and libido, and girls have irregular periods. In addition, all patients have.

In cases that are difficult to treat, a secondary plan develops.

Gigantism can cause complications, which are manifested by panhypopituitarism, the development of acromegaly, sexual disorders, myocardial dystrophy, increased blood pressure. In more severe forms, patients lose their vision. Somatotropinoma can develop into malignant adenocarcinoma of the pituitary gland.

Partial gigantism

This is a disease that affects individual parts or half of the body as a result of embryogenesis disorders or neurotrophic disorders.

Partial gigantism is extremely rare, so the reasons for its development are not well understood. But there are certain theories that try to presumably explain the emergence of partial gigantism. These include: mechanical, embryonic and neurotrophic theories.

In the first case ( mechanical theory) partial gigantism can form as a result of compression certain parts fetus during pregnancy, as well as incorrect position child in the uterus, can contribute to congestion in any part of the body, and hence provoke their increase.

The embryonic theory explains enhanced growth an organ or part of the body is the result of disturbances during the formation of the organism at the genetic level.

The neurotrophic theory states that partial gigantism develops under the influence of special influences of a neurotrophic nature on a specific part of the body. In this case, the main factor may be damage to the sympathetic fibers.

With partial gigantism, changes that occur in the endocrine system, but this cannot be a basis for searching for its pathogenesis in the endocrine sphere.

Used to treat partial gigantism surgery in relation to enlarged body parts or organs.

Gigantism in children

This is a disease that occurs when chronically excessive reproduction of growth hormone by somatotropes - growth hormone or somatotropin. At early occurrence diseases when bone growth zones remain uncovered, gigantism develops in children, i.e. very tall. If the disease begins to develop after the closure of these zones, then it is characterized by signs of acromegaly. The combination of gigantism and acromegaly is observed mainly among adolescents. At this age, the disease is more common among boys than girls.

Infantile gigantism, which occurs when excess quantity growth hormone produced by the pituitary gland is extremely rare. The cause of its formation is a pituitary tumor of benign etiology. There are suggestions that sometimes gigantism can develop due to the growth of a tumor process in some parts of the hypothalamus, which are responsible for permanent state internal environment the body, its needs, etc. It has been proven that tumors provoke increased production of somatotropin.

As a rule, a rapid increase in a child's height becomes obvious from eight to fourteen years of age, and sometimes such changes occur even at the age of five. The final body length can reach 250 cm and the limbs are relatively long in relation to the body.

After growth stops in children, pathology (enlargement of all organs) is joined by enlargement of the limbs and face. The size of the skull becomes significantly larger, the nose widens, the chin moves down, and the tongue enlarges. As a result of the increase lower jaw, the teeth are very sparsely spaced, and the face looks rather rough. Fingers on the top and lower limbs grow in width, and the legs and arms become thick and resemble a shovel. There is also a curvature of the spine in the form.

Such children get tired very quickly and show signs of delayed puberty. In addition, there appear severe pain in the head, weakness, which later turns into impaired consciousness. And when ophthalmological examinations lack of lateral vision is diagnosed.

When conducting laboratory research an increased level of somatotropin in the blood is detected, almost 400 ng/ml. It has also been recorded to increase during sleep.

A tumor that continues to grow can disrupt the production of gonadotropins, which are involved in sexual function; a hormone that stimulates follicle function; prolactin and LH.

An x-ray can show how the bones inside the skull expand and paranasal sinuses nose, which are located in the face area. Very often, reticular patterns of the bones of the fingers on the limbs and thickening of the bone on the heel are visible. The tumor continues to develop quite slowly, so the disease can last for many years.

But children can also experience macrodactyly, i.e. congenital form of gigantism. This pathological disease caused by processes of disruption of volumetric and linear parameters limbs. They can acquire quite large sizes.

There are three forms of gigantism in children in this form. The first form includes congenital pathology, at which they predominantly increase soft fabrics. In this case, it has peculiar distinctive characteristics, such as increased length and volume of all segments on the affected limb with a sharp increase in the surface of either the hand or foot (the limb becomes ugliness). In addition, the degree of soft tissue in relation to the bones of the skeleton increases in length and width, which does not coincide with the age norm. Clinodactyly is also observed in the area of ​​the interphalangeal joint and metacarpophalangeal affected finger or fingers, as well as hyperextension in them.

The second form of the disease is characterized by congenital gigantism, in which there is an increase in bone content. This type of pathology is manifested by an increase in the length of the modified limb segments ( appearance soft tissues are not changed). And the bones of the skeleton in their increase, especially in length, exceed the increased degree of soft tissue, which also distinguishes them from age norms. Clinodactyly is observed in the metacarpophalangeal and interphalangeal joints of the affected fingers, but hyperextension is completely absent.

In the third form of true congenital gigantism, short muscles are predominantly affected. Gigantism in this case is manifested by an increase in the length of the limb segment, which was damaged due to the metacarpal bones. The volume of soft tissues increases, the feet and palms expand significantly as a result of the formation of spaces between the metacarpal bones. Also, if we compare the increases according to the age grid, they do not correspond to the norms. Clinodactyly and flexion contracture are found in the metacarpal and phalangeal joints, and sometimes there is looseness in the joints of the first finger.

Children with ordinary gigantism are prescribed surgical treatment after confirmation of the tumor process. As a rule, gigantism in children with varying degrees begins to be treated at six or seven months. Basically they can reduce the length of the finger to normal sizes, using shortening resection for the metacarpals and phalanges. Clinodactyly of deformed fingers is eliminated with their simultaneous shortening. is treated after a complete rehabilitation course of the changed fingers to their normal length.

When a finger enlarges by almost 300% or more of normal, the only effective manipulation is amputation. Sometimes they offer a microsurgical operation in the form of finger transplantation, for example, from a healthy foot to an affected hand.

In the first form of the disease, surgery using lateral resection of the phalanges. In the second case, central resection of the phalanges is performed. In the third, the metacarpal bones are brought together in different fixations. This will depend on the severity of the deformity and the age of the child.

During the operation, accompanying deformities are corrected: excess soft tissue is excised, hyperextension of deformed fingers and existing looseness are eliminated.

All these orthopedic and surgical techniques make it possible to bring the affected hands and feet of children closer to the physiological norm, as well as maintain their anatomical proportion.

Treatment of gigantism

For treatment of this disease should use complex therapy taking into account the stage, form and phase of gigantism activity. As a rule, treatment of gigantism is aimed at reducing the level of growth hormone (somatotropin) in the blood by suppressing, destroying or removing the activity of a growth hormone-secreting tumor, which can be achieved with modern and traditional methods treatment. A correctly chosen treatment method prevents the development of various complications. And if they are available, the main method of therapy includes means that correct disorders in the neurological and endocrine systems.

Gigantism begins to be treated with a diet, where proteins are necessarily limited and calories are counted. In addition, treatment may include: drug therapy, radiation exposure and surgery.

Blockade of somatotropin production is achieved with the help of Dopamine antagonists (Lergotril, Lisurgide, Parlodel, Bromocriptine, Metergoline, Dostinex and L-dopa) or GH analogues (Sandostin), which are used long years or for life. At the time of discontinuation of these drugs, the level of somatotropin increases, and gigantism again progresses.

In case of, and also if there is a threat of blindness or is destroyed by a tumor sphenoid bone— carry out radical transsphenoidal hypophysectomy or cryohypophysectomy using liquid nitrogen.

For radiation irradiation, proton beams, heavy L-particles, telegammatherapy are used, and last of all, X-ray therapy of the pituitary gland is used.

Good results can be achieved by implanting radioactive needles made of gold, iridium and yttrium into the pituitary gland.

The form of gigantism without a tumor is treated with Testosterone, which allows the areas of bone growth to be closed.

When pathology occurs in thyroid gland or the appearance of diabetes mellitus, appropriate therapy is prescribed.

For preventive purposes, it is necessary for patients with gigantism to avoid infections, various cranial injuries, and not to use anabolic steroid and questionable in composition nutritional supplements when playing sports and building muscles. It is advisable for the younger generation to limit their use mobile phones. In addition, constantly undergo anthropometric monitoring, especially from 13 to 16 years.

At the time of pre-pubertal development of gigantism, Testosterone is used to stop excessive growth with x-ray monitoring of bone growth zones.

People suffering from gigantism are in a dispensary group and under the supervision of an endocrinologist, neurologist and ophthalmologist. Such patients are never removed from endocrinological registration.

Pregnant women with gigantism are not recommended to have abortions. If during the first pregnancy the child develops gigantism, then subsequent ones are very undesirable. It is important to remember that somatotropinoma refers to a tumor in the brain. It can cause compression inside the skull. Therefore, patients with gigantism should be treated as cancer patients.

This is abnormal tallness for a person, which is caused by the “overproduction” of somatotropic hormone, or as it is also called, growth hormone, by the pituitary gland.
Gigantism is already evident in childhood, and the height of an adult exceeds 1.9 m for women and more than 2.0 m for men. This disease is quite rare and occurs in 1-2 out of 1000 people.

Gigantism: classification

Endocrinology described this disease in great detail and gave it a clear classification. According to the variants of its development, gigantism is:
1. Acromegalic. This type of gigantism is almost completely similar in its characteristics to acromegaly.
2. Eunuchoid. This species is characterized by an almost complete absence of activity of the gonads. You can recognize it by its disproportionately long limbs and the absence of secondary sexual characteristics.
3. Splanchomegalic. Characterized by strong magnification internal organs.
4. True. This type of gigantism is characterized by the absence of any pathologies in the mental and physical development and a proportional increase in the size of the entire body.
5. Cerebral gigantism is caused by damage to the brain and is characterized by a noticeable impairment of intelligence.
6. Half. Characterized by enlargement of one half of the body.
7. Partial gigantism can be recognized by the increased size of individual parts of the body.

Gigantism: reasons

Many gigantism are confused with acromegaly and this is no coincidence, because these diseases have the same causes - increased activity of the pituitary gland, secreted growth hormone (somatotropic) in incredible quantities. And these two diseases differ from each other only in age categories.

Pathology of the pituitary gland, which results in its hyperactivity, can have a different nature:
- intoxication of the body;
- pituitary adenoma;
- traumatic brain injuries;
- neuroinfection (meningitis, encephalitis...).

Another cause of gigantism may be the loss of sensitivity of the epiphyseal cartilages to the effects of sex hormones. These cartilages are “responsible” for the growth of bone tissue and with loss of sensitivity, they still continue their activity (increase bones in length) even in the post-pubertal (adolescent) period.

And lastly: when talking about the causes of gigantism, we cannot ignore the human immune system. It is she who controls the normal functionality of the entire body and its individual organs, and therefore its dysfunctions also manifest themselves in various endocrine diseases. This must be understood when treating gigantism and restoring a person’s normal immune status is one of the main tasks.

Symptoms of gigantism

Gigantism begins to manifest itself with an increase in body length at 9-13 years. On the background rapid growth the following symptoms are observed:
- headaches and dizziness;
- weakness;
- fast fatiguability;
- manifestation of somatics in bones and joints;
- blurred vision;
- muscle weakness;
- decreased memory and intelligence.

Among other things, gigantism can be identified by hormonal disorders: in women - disruption or complete cessation menstrual function, infertility, and in men - hypogonadism (impaired production of sex hormones). Diabetes insipidus It is also a hormonal symptom of gigantism.

Gigantism: complications

This disease can provoke other diseases:
- myocardial dystrophy;
- arterial hypertension;
- heart failure;
- dystrophic change liver;
- diabetes ;
- infertility;
- pulmonary emphysema;
- dysfunction of the thyroid gland.

Gigantism: diagnosis

Diagnostics gigantism always begins with an external examination, then moves on to laboratory and instrumental methods.

Biochemical analysis blood tests determine the increased content of somatotropic hormone in the blood.
Using skull x-ray, computer and magnetic resonance imaging of the brain, a pituitary adenoma is detected.
An ophthalmological examination provides a “picture” of the visual fields and reveals congestion in the fundus.

Gigantism: treatment

Treatment for gigantism is similar to that for acromegaly. Samotostatin analogues and sex hormonal drugs are also used here to “suppress” bone growth zones. It must be taken into account that the use hormonal drugs entails various side effects, which can affect a person differently.

Radiation therapy for pituitary adenoma is also used. But this is also fraught with consequences. This therapy “kills” the immune system, which, in the treatment of gigantism, on the contrary, must be restored first.

Complex therapy and operative method(removal of adenoma) should be carried out using immune drug Transfer factor.
The basis of this drug is made up of immune molecules of the same name, which, when entering the body, perform three functions:
- eliminate disruptions of the endocrine and immune systems;
- being information particles (of the same nature as DNA), transfer factors “record and store” all information about foreign agents that invade the body, and when they invade again, “transmit” this information to the immune system, which neutralizes these antigens;
- eliminate everything side effects caused by the use of other medicines.

There is a whole line of this immunomodulator, from which Transfer Factor Advance and Transfer Factor Glucouch are used in the Endocrine System program for the prevention of endocrine diseases, incl. and gigantism. According to many well-known scientists, for these purposes the best drug No.
It may happen that after complex therapy gigantism with the use of Transfer Factor, there will be no need for surgical intervention. All this is decided by the appropriate doctor, so be sure to consult with him.

Pathological changes in the body that cause the appearance of tallness are called pituitary gigantism. The disease is caused by increased production of growth hormones by the anterior or lower part. The process can begin from an early age.

With the disease, patients experience disproportionality in the development of body parts, height of more than two meters, noticeably elongated limbs and many other signs. Regarding the head, it, compared to all other organs, visually seems very small.

Classification of the disease

In endocrinology, a certain number of options for the development of a disease such as pituitary gigantism in children are identified.

  • Gigantism that has signs of acromegaly is also called acromegalic gigantism. He is the opposite of such a pathology as.
  • When the mass of organs located inside a sick child increases, the pathology is called splanchnomegaly or gigantism of internal organs.
  • Gigantism with complications in the development of the genital organs (the eunuchoid variety occurs in guys in younger age). This type is also typical for women whose gonads completely cease their activity. At the same time, representatives of both sexes experience lengthening of the limbs.
  • A type of gigantism, which is called true, may not pose any serious danger, since there are practically no deviations from the psyche and physiology of the body (in proportionality), excluding size.
  • Gigantism of the partial or partial type is a disease when there is an increase in organs or parts of the body separately.
  • A more dangerous and insidious type of pathology is gigantism of the half type, in which an enlargement of one part of the body occurs, while the other remains normal. Imbalance and poor coordination of movements appear.
  • Gigantism of the cerebral type is mainly caused by lesions of the brain. In this case there is a violation intellectual ability person, as well as some mental indicators.

There is a certain amount various reasons, which influence the development of the disease, even in the womb. This also includes genetic predisposition.

Causes of the disease

According to etiology, this disease is closely related to tumors, somatotropinomas, which develop from eosinophils (the process of formation appears somatotropic hormones). Somatotropic formations, in most situations, are not malignant, but rather benign formations. But sometimes there is a transition from benign process, to malignant. Common cause The appearance of gigantism is pathological changes in parts of the pituitary gland and improper production of hormones.

As for the location of hormones during illness, somatotropinomas accumulate in the area of ​​the adenohypophysis. They are found in other places, including gastrointestinal tract, pancreas, parapharyngeal region, bronchi and bone sinuses.

Risk factors for when a disease such as gigantism may begin to progress in development include the effect of eosinophilic cells on hyperplasia. This process can begin even during pregnancy, with the slightest change in hormone levels. Macroadenoma is considered the main cause of gigantism; it develops in 90% of patients. As for , it develops in 9%. The rarest cause of gigantism is a syndrome of increased sensitivity of tissue and its receptors to the influence of growth hormones.

With the development of pituitary gigantism, the causes are different; it is important to pay attention to somatic (hormonal) mutations in the area of ​​somatotrophs. In this case, a neoplastic reaction occurs. An excessive amount of somatic hormones leads to an increase in not only organs and their tissues - hypertrophy of the kidneys or myocardium appears, erythropoiesis and other diseases often appear.

Gigantism and its pathological symptoms

The appearance of the progress of gigantism is determined without much difficulty. Its main characteristics are a significant increase in the size of the body. More often the arms or legs change.

Children suffering from gigantism disease exhibit the following symptoms:

  • when the pathology just begins to develop (in childhood), the child notices an early increase in strength in the muscles of the arms or legs, but after some time it decreases;
  • a patient suffering from gigantism gets tired quickly;
  • pain in the joints of the arms and legs;
  • emergence sudden dizziness(the heart does not have time to supply blood to all internal organs and their tissues);
  • visual function decreases;
  • memory decreases;
  • Pain often appears in the temporal part of the head.

If the disease is acromegalic in nature, then the symptoms are very diverse. To the above listed manifestations of the disease are added:

  • arches appear above the eyebrows, and the lower jaw increases in size;
  • the appearance of noticeable gaps between the teeth (upper or lower);
  • thickening of the sternum and pelvic bones;
  • lowering the tone of the voice;
  • growth of hands and feet.

If, during the progress of gigantism, another pathology develops in the endocrine system, then patients experience:

  • decreased sexual function in representatives of the stronger sex;
  • the occurrence of infertility, regardless of gender;
  • the appearance of tachycardia;
  • the appearance of thirst.
  • termination menstrual cycle or it does not begin at all, an increase in urine output appears (in women).

Diagnosis of the disease

To establish a diagnosis of “gigantism”, it is important to rely on clinical manifestations diseases. Such patients should be examined with x-rays. If gigantism develops in children, it is not recommended to rush into irradiation; it is important to examine all the signs of the disease and then prescribe treatment. It is imperative to carry out neurological and ophthalmological diagnostic actions.

The laboratory type of diagnostic actions does not carry much information; with such studies, only higher level somatotropin. If there is even the slightest suspicion of gigantism, then you need to undergo studies that use computed tomography of the brain, its results can be viewed in the photo. This is explained by common reasons The disease in question is the occurrence of a tumor-like formation in the area of ​​the lower part of the pituitary gland.

If the patient grows, then an increase in size appears in the area of ​​​​the skull called the “sella turcica”. The tumor, when changing in size, causes vision complications.

When the patient’s skeleton is fully mature, but somatotropin is still produced, formation occurs. In this case, specialist intervention is required.

Treatment of gigantism

In most cases, the treatment of gigantism is carried out by an endocrinologist, who can prescribe additional research and take into account the decisions of other specialists.

Treatment for gigantism is:

Peculiarity drug treatment is that it is based on a decrease in excretion by the body (the pituitary gland is responsible for their production). Today there are many drugs used for gigantism. Below are two of the most popular:

  • Parlodel - this medicine is capable of simulating the work of special receptors located in the human brain, stimulating the production of biologically active substances, including dopamine (this substance is produced in the body to suppress the production of hormones responsible for tissue growth).
  • Octreotide (Somatostatin) - this drug is a substance similar to the natural structure of hormones. When using this medicine, you can notice an improvement in the effect of stopping blood in intragastric or intestinal bleeding. They are responsible for inhibiting the patient's growth. The basis is the inhibition of the production of somatotropic hormones.

The dosage and administration should be prescribed exclusively by a doctor, depending on how the symptoms of the disease are expressed, as well as on the level of somatotropin in the blood.

X-rays are used as radiation therapy. It is based on the principle of waves, they are able to stop the progression of tumors and the body's production of growth hormones.
The dosage is determined exclusively by a specialist, taking into account the activity of the tumor.

Surgical treatment is used when none of the above methods help.

The surgical treatment method is used in practice when the size of the pathological formation exceeds the permissible limits. The method in question is used when tissues around the brain are compressed (with increased pressure inside the skull).

If minor symptoms of the disease appear, and the cause of gigantism is precisely determined, then treatment often remains favorable. It is important to seek help in time.