Few people know about a rare acromegaly disease when parts of the face and body are disproportionately enlarged. At an early stage, the development of pathology can be stopped, and without treatment, it leads to a violation of the functions of internal organs, reduces quality and shortens life expectancy.

What is acromegaly?

Pathology develops with excessive secretion of growth hormone. In children, he is responsible for linear growth, proper formation of bones, muscle tissue. In adults, it participates in metabolism: it stimulates protein synthesis, prevents the deposition of subcutaneous fat, regulates carbohydrate metabolism, and maintains blood glucose levels.

Somatotropin is produced by cells of the upper pituitary, the lower appendage of the brain. The hypothalamus maintains a normal level of STH; it produces peptide hormones somatoliberin and somatostatin. The first stimulates the synthesis and secretion of STH, the second inhibits.

Normally, the concentration of the hormone changes during the day: increases at night, decreases during the day.

If the regulation mechanism fails, the amount of growth hormone increases sharply, the rhythm of secretion is disrupted, the neuroendocrine pathology acromegaly develops. The disease is diagnosed in adults after 20 years, when physical growth ends. It is accompanied by hypertrophic disproportionate proliferation of soft tissues, bones, organs.

The mechanism of development and causes

In 9 out of 10 patients, hormone-active pituitary adenoma provokes acromegaly. The cells of the anterior section intensively multiply, grow, degenerate into a benign neoplasm.It ceases to obey the hypothalamus, intensively produces somatotropin.

Adenoma compresses, destroys neighboring healthy tissues, its size sometimes exceeds the size of the pituitary gland. The appearance of a tumor is often preceded by skull injuries, neuroinfection, chronic inflammation of the ENT organs. The risk of developing adenoma and neuroendocrine disruption increases if the family has already documented cases of the disease.

Less commonly, pathologies are preceded by other premises:

  • ectopic secretion of STH by neoplasms of the lungs, ovaries, bronchi;
  • hypothalamic tumors that enhance somatoliberin production.

The causes and symptoms of acromegaly are similar to the manifestations of gigantism in children and adolescents. However, at an early and young age, the bones continue to grow in length. Tissues, organs increase evenly, the proportions of the body, limbs, faces are not violated. Children stand out against the background of peers with high growth: in adolescent girls it exceeds 190 cm, in boys - 2 m. Acromegaly and gigantism are rarely diagnosed: in 50 adults and 3 children per 1 million population.

Symptoms and signs

The appearance of the disease is signaled by changes in appearance:

  • The protruding parts of the face disproportionately increase: the eyebrows, lower jaw, cheekbones, lips, nose.
  • Interdental spaces expand, bite changes.
  • Due to hypertrophy of the ligaments, the larynx coarsens the timbre of the voice.
  • The feet, wrists, fingers thicken, the skull enlarges. The patient has to wear shoes, clothes 1-2 sizes larger than before.
  • The skin is compacted, covered with folds.

As the tumor grows, squeezes the nerve tissue, neurological symptoms appear, and the state of health worsens:

  • working capacity decreases;
  • intracranial pressure rises, headaches, dizziness occur;
  • vision falls, doubles in the eyes, photophobia develops;
  • smell, hearing worsen;
  • numb hands, feet, fingers;
  • the menstrual cycle in women goes astray;
  • galactorrhea develops: breast milk is secreted from the nipples;
  • decreased potency, sex drive;
  • sweating intensifies, oiliness of the skin increases due to an increase in the number and area of ​​sebaceous, sweat glands;
  • as cartilage and connective tissue grows, joint mobility decreases, arthralgia develops;
  • apnea syndrome appears: short respiratory arrest in a dream;
  • blood pressure rises.

On a medical examination, internal changes are found: the heart, thyroid gland, kidneys, liver, muscle tissue increase. The skeleton is often deformed: the chest is expanding, the gaps between the ribs, the spine is bent. A quarter of patients have metabolic disorders and diabetes mellitus.

Stages of the disease

There are 4 phases of the development of the disease:

  • Preacromegaly. At an early stage, external symptoms are mild. Violation is detected by the amount of hormone in the blood or the results of computed tomography of the brain.
  • Hypertrophic. Symptoms are pronounced.
  • Tumor. Adenoma greatly increases, compresses tissues, ocular, neurological disorders occur.
  • Cachexia is the stage of physical and mental exhaustion.

The disease develops slowly, from the onset of endocrine failure until the onset of the first symptoms, 5-7 years pass.

Diagnostic measures

If you suspect the development of the disease, consult an endocrinologist. The doctor makes a preliminary diagnosis on the basis of external changes, palpation. Laboratory diagnosis of acromegaly includes the following tests:

  • Determining the level of STH. Blood is taken 3-5 times at time intervals of 20 minutes. up to 2.5 hours. Then calculate the average, in healthy people it is not more than 5 ng / ml.
  • Glucose tolerance test. Blood sampling is done first on an empty stomach, then after glucose administration, then the level of growth hormone is compared. In patients, the concentration after taking glucose increases, in healthy it decreases. The test is used during treatment to assess the effect of therapy.
  • Definition of IRF-1. The amount of this substance reflects the daily volume of growth hormone. In patients, the level is higher than normal.

To assess changes in the pituitary gland, X-rays of the skull, MRI or CT scan of the brain are prescribed. If the patient complains of irregularities from other organs, the endocrinologist additionally directs an appointment with other doctors with a narrow profile: cardiologist, ophthalmologist, gastroenterologist.

Disease treatment

The main objective of the treatment of acromegaly is to reduce the amount of growth hormone to normal. To do this, use the following methods:

  • Drug therapy. The patient is prescribed drugs that inhibit the production of STH: synthetic analogues of somatostatin, dopamine antagonists. Medications stop the disease in the initial stage when the adenoma does not progress.
  • Surgical intervention. The operation is prescribed with a rapid increase in the tumor, a sharp deterioration in vision. After removal of the adenoma, the secretion of growth hormone decreases. If the formation reaches gigantic proportions, a second operation will be required.
  • Radiation therapy. This method is used when previous methods of treatment did not lead to the desired results or surgical intervention is contraindicated. The adenoma is affected by gamma rays, its development stops, but the level of the hormone decreases to normal only after years.

Sometimes these methods are combined.

Acromegaly during pregnancy

In the medical literature there are descriptions of slightly more than 100 cases of pregnancy in women with pituitary adenoma. Fetal bearing and childbirth often proceed without complications. Growth hormone does not cross the placenta, so a baby is born without pathologies.

The question of the safety of medicines remains open. Doctors do not give general recommendations, take into account the severity of the condition, the size of the neoplasm. It is often advised to postpone treatment until the end of pregnancy. However, women continue to take medication when the disease progresses after withdrawal. Taking drugs does not affect the development of the fetus.

Preventative measures

The risk of adenoma and endocrine disruption is reduced if the following recommendations are followed:

  • Protect your head from injuries, bruises.
  • Treat infections and inflammation of the nasopharynx.
  • Immediately appear to the endocrinologist with an increase in the size of the limbs, a violation of the proportions of the face.

It is impossible to completely protect yourself from neuroendocrine failure.

Prognosis for the patient

With early diagnosis, proper treatment, stable remission occurs. After removal of microadenomas in 85% of patients, the somatotropin content decreases to normal, stably keeps at the same level.

In advanced cases, the functions of the heart, liver, lungs are disturbed, complications develop:

  • myocardial dystrophy;
  • heart, pituitary insufficiency;
  • hypertension, which is difficult to treat;
  • emphysema;
  • liver dystrophy;
  • diabetes;
  • mastopathy
  • intestinal polyposis;
  • ovarian cysts;
  • uterine fibroids;
  • adrenal hyperplasia.

After a course of therapy or surgery, the patient is observed by an endocrinologist, and every year he is tested. If left untreated, the risk of premature death is increased.

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