What is acromegaly and what treatment options exist?

Hormonal balance determines the health of all organs and systems, and even a person’s appearance. The disease acromegaly, caused by excessive production of somatotropin, a hormone normally responsible for growth in childhood and adolescence, is characterized by characteristic changes in the face, limbs, and figure. However, it also affects the internal state of a person, so the disease cannot be considered exclusively a cosmetic feature. Timely intervention will help alleviate the consequences.

How it proceeds

In the initial stage of the disease, hypersecretion of other tropic hormones of the pituitary gland - gonadotropic, lactotropic, thyrotropic, adrenocorticotropic - may occur. This is explained by an increase in the functionality of the peripheral endocrine glands - the thyroid gland, gonads, and adrenal glands. As the disease develops, increased production of these hormones is replaced by decreased production, which leads to insufficiency of the endocrine glands.

When the pituitary tumor grows to 3-5 cm, symptoms of increased intracranial pressure occur.

If pituitary tumors are malignant, acromegaly develops at an accelerated rate.

Forecast

The outcome of the disease is exhaustion (cachexia). In the absence of appropriate treatment, with an unfavorable course and early onset (at a young age), the life expectancy of patients is 3-4 years. With slow development and a favorable course, a person can live from 10 to 30 years.

With timely treatment, the prognosis for life is favorable and recovery is possible. The patients' ability to work is limited.

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Stages of development of acromegaly

This disease is characterized by a long course, during which acromegaly has four stages:

• the preacromegalytic stage is the onset of the disease. This stage has very mild signs of the disease and therefore it is very difficult to identify pathology at this stage. But still, at this stage, it is possible to diagnose acromegaly using computed tomography of the brain, as well as by the level of somatotropin in the blood;
• the hypertrophic stage is characterized by pronounced symptoms of the disease;
• the tumor stage is dangerous due to the appearance of symptoms of compression of nearby organs and parts of the brain by the tumor, this leads to intracranial hypertension, nervous and ocular disorders;
• the last stage is the stage of cachexia, it is accompanied by exhaustion of the body due to acromegaly.

Prevention

In order to prevent the development of acromegaly, doctors advise adhering to the following recommendations.

• avoid traumatic head injuries;
• promptly treat infectious diseases;
• consult a doctor if there are metabolic disorders;
• carefully treat diseases affecting the respiratory system;
• periodically take tests for somatotropin levels for preventive purposes.

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

Symptoms of acromegaly

As a rule, the first symptoms of acromegaly occur after hypersecretion of growth hormone or the impact of pituitary adenoma on nearby brain structures or optic nerves.

An increased level of somatotropin leads to characteristic changes in patients with acromegaly:

• enlargement of the cheek bones;
• lower jaw;
• brow ridges;
• ear hypertrophy;
• nose;
• lips

This makes facial features rougher.

Enlargement of the lower jaw leads to discrepancies between the teeth and bite deformation. Macroglossia develops (enlargement of the tongue), in which dental typos are visualized. The voice becomes hoarse and low due to the enlargement of the larynx, tongue and vocal cords. External changes occur slowly, so patients cannot immediately notice them. They become noticeable after the patient has to buy hats and shoes several sizes larger than before.

Acromegaly contributes to skeletal deformation:

• curvature of the spine occurs;
• expansion of intercostal spaces;
• enlargement of the chest in the anteroposterior region, so the chest becomes barrel-shaped;
• cartilage and connective tissue hypertrophy, this leads to deformation of the joints and limitation of their mobility, as well as arthralgia.

With this disease, the number of sebaceous glands increases, which causes increased sweating and sebum secretion. The skin thickens, becomes denser, and deep folds of skin form, especially in the area of ​​the scalp.

Medicines

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The term “acromegaly” disease means that it is a disease that occurs in a person whose production of growth hormone is impaired, namely manifestations of the enhanced reproductive function of the somatotropic hormone after the period of maturation. As a result, the proportionality of the growth of the entire skeleton, internal organs and soft tissues of the body is disrupted (this is due to nitrogen retention in the body). Acromegaly is especially pronounced on the extremities of the body, face and entire head.

This disease occurs in both women and men after the end of the growth period. The prevalence of the disease ranges from 45-70 people per one million population. This disease rarely affects children. In rare cases, in growing children, this excess growth hormone leads to a condition called “gigantism.” This change is definitely due to an excessive increase in body weight and bone growth.

Since acromegaly is not very common, and the disease goes away gradually, identifying this disease in the early stages is not easy.

All this is caused not only by a violation of growth hormone, but also by changes in other functions of the glands:

• Endocrine.
• Pancreas.
• Sexual.
• Dysfunction of the adrenal cortex.

Due to acromegaly, metabolism is disrupted, which in most cases leads to diabetes mellitus and poses a huge danger to human life. But do not be upset, there are certain medical procedures that can significantly alleviate the symptoms and reduce the further development of acromegaly.

Symptoms of acromegaly are a slow and subtle manifestation of the clinical development of the disease. This disease occurs due to hormonal imbalance, changes in appearance, as well as deterioration in health. There are patients who made this diagnosis only after 10 years. The main complaints of patients are enlargement of the ears, nose, limbs of the hands and feet.

In developing an effective fight against the disease, it is necessary to avoid two main consequences: malignant neoplasms and pathology of the cardiovascular system. There are four main methods of getting rid of this disease:

• Surgical method. Qualified doctors completely remove tumors. This method allows you to quickly get results. There are some complications after surgery.
• Radiation therapy or radiation. Most often, this method is used in cases where surgery has not helped. Radiation also has certain disadvantages: the optic nerve is damaged and there is a secondary brain tumor.
• Medication method. Acromegaly is treated with the following three types of drugs:

  SST analogues (long-acting (samatulin and sandostatin LAR) and short-acting - sandostatin octroedit).

• STH antagonists.
• Dopamine agonists (ergoline and non-ergoline drugs).

Medicines

But as experience shows, doctors still adhere to drug treatment. This method has less negative effects on the human body.

There is a sufficient list of medications to combat the consequences of acromegaly:

• Genfastat is a homeopathic remedy.
• Octride is a mucolic agent.
• Parlodel is a Mybp bacteriophage.
• Sandotatin – Beta – Adrenergic blocker.
• Samatulin is an antiseptic.

The active ingredient in most of these drugs is octreodite. All dosages and treatment regimens are prescribed only by the attending physician.

What changes occur with acromegaly

With acromegaly, muscles and internal organs (liver, heart, kidneys) increase significantly, while gradual degeneration of muscle fibers occurs. Patients complain of increased fatigue, weakness and decreased performance. First, the myocardium hypertrophies, then myocardial dystrophy occurs and heart failure gradually increases. 90% of patients suffer from sleep apnea syndrome, which is associated with dysfunction of the respiratory center and hypertrophic changes in the soft tissues of the upper respiratory tract; a third of patients are exposed to arterial hypertension.

Patients with this diagnosis have problems with sexual function. In most women, with a deficiency of gonadotropins and an excess of prolactin, menstrual irregularities occur, often infertility, and milk is released from the nipples, which has no connection with pregnancy and childbirth (galactorrhea). A third of men experience reduced potency. In some cases, diabetes insipidus occurs and is facilitated by hyposecretion of antidiuretic hormone.

As the pituitary adenoma enlarges, compression of tissues and nerves occurs, photophobia, pain in the forehead and cheekbones, increased intracranial pressure, double vision, vomiting, dizziness, decreased sense of smell and hearing, and numbness of the extremities appear.

Patients with acromegaly are at risk of developing tumors of the thyroid gland, uterus, and gastrointestinal tract.

What it is?

Acromegaly (from the Greek ἄκρος - limb and the Greek μέγας - large) is a disease associated with dysfunction of the anterior pituitary gland (adenohypophysis); accompanied by enlargement (expansion and thickening) of the hands, feet, skull, especially its facial part, etc.

Acromegaly usually occurs after the body's growth has completed; develops gradually and lasts for many years. Caused by the production of excessive amounts of growth hormone. A similar disruption of the pituitary gland at an early age causes gigantism (if untreated, gigantism may eventually combine with acromegaly).

With acromegaly, headaches, fatigue, weakening of mental abilities, visual disturbances, often impotence in men and cessation of menstruation in women are noted. Treatment is surgery on the pituitary gland, radiotherapy, and the use of hormonal drugs that reduce the production of growth hormone (bromocriptine, lanreotide).

Diagnosis of acromegaly

To determine acromegaly, in most cases, a visual examination of the patient is sufficient, because such patients have a specific appearance. But to document the diagnosis and clarify changes and complications in his body, it is necessary to conduct a series of laboratory and instrumental studies, and conduct additional studies in the form of radiographic examinations and magnetic resonance imaging of the brain.

The main laboratory test is a biochemical study of GH and IGF-1. This analysis allows you to determine the level of growth hormone in the blood and can be performed at any time of the day, regardless of the patient’s food intake.

With acromegaly, the soft tissues of the patients' feet undergo significant changes, clinically this is manifested by the expansion of fatty layers and thickening of the connective tissue septa. X-rays of the foot are often used to diagnose acromegaly.

After receiving the conclusions of laboratory tests and radiographic data, a search for disorders of the hypothalamus or pituitary gland is carried out, for this purpose X-ray examinations of the brain or MRI are performed. Sometimes radiographs and MRI do not show the presence of brain tumors; in such cases, other organs are examined (CT of the chest, pelvic organs, mediastinal organs and retroperitoneal space is performed).

When identifying a pituitary macroadenoma with a suprassellar spread, it is important to clarify the presence of the tumor’s impact on the optochiasmatic region; for this purpose, perimetry and fundus examination are performed.

A complete examination of patients is also carried out to identify complications (diabetes mellitus, intestinal polyposis, multinodular goiter).

Pathogenesis

It has been proven that the development of acromegaly is associated with dysregulation of the somatotropic function of the pituitary gland through the somatotropin-releasing factor produced by the hypothalamus (see Hypothalamic neurohormones), which leads to excessive release of growth hormone from the pituitary gland (see Somatotropic hormone). An increase in the somatotropic function of the pituitary gland, causing the development of acromegaly, can occur with eosinophilic adenoma, adenocarcinoma and other tumors of the hypothalamus and pituitary gland, as well as with functional disorders in the hypothalamic-pituitary system.

Excessive growth hormone secretion in adults who have completed bone growth usually causes acromegaly. In childhood and adolescence, when the epiphyseal growth zones are not closed, increased secretion of somatotropic hormone often causes the development of gigantism.

Complications of acromegaly

Complications of acromegaly affect almost all organs, so several groups of complications can be distinguished. As for the skeletal system, the following changes appear:

• prognathism (lower jaw protruding forward);
• diastema (increased space between teeth);
• osteoarthritis (inflammatory processes in the joints);
• frontal hyperostosis (thickening of the internal frontal plate);
• dorsal kyphosis (curvature of the spine);
• diseases of the temporomandibular joint.

As for the skin, the following processes occur:

• roughening of skin folds;
• warts;
• seborrhea;
• increased sweating;
• hidradenitis

Dysfunction of most organs and systems occurs. Complications of acromegaly also include emphysema, diabetes mellitus, liver dystrophy, and heart failure. Among the severe complications of acromegaly, the appearance of adenomatous polyps and colon cancer are known.

Preparing for your appointment

You'll probably start by seeing your family doctor or general practitioner. However, in some cases, you may be immediately referred to a doctor who specializes in hormonal disorders (endocrinologist).

It's nice to prepare for the meeting. Here's some information to help you prepare for your appointment and know what to expect from your doctor.

What can you do

• Be aware of any prior restrictions. When you make your appointment, ask if there is anything you need to do to prepare for the diagnostic tests.
• Write down the symptoms you experience. Your doctor will want to know about anything that is causing you discomfort or concern, such as headaches, vision changes, or discomfort in your hands, even if these symptoms are not related to the reason you made the appointment.
• Write down key personal information, including any changes in your sex life or, if you're a woman, your menstrual cycle.
• Make a list of all the medications, vitamins, and supplements you take.
• Take old photographs that your doctor can use to compare with your appearance today. Your doctor will likely be interested in photographs from 10 years ago.
• Bring a family member or friend with you if possible. Someone accompanying you may remember things you miss or forget.
• Write down questions to ask your doctor.

Preparing a list of questions will help you make the most of your time with your doctor. For acromegaly, some basic questions to ask your doctor include:

• What could be causing my symptoms or condition?
• Besides the most likely cause, what are the possible causes of my symptoms or conditions?
• What tests do I need?
• What treatments are available for this condition? What approach do you recommend?
• How long do I need treatment before my symptoms improve?
• With treatment, will I return to looking and feeling the way I did before I developed acromegaly symptoms?
• Will I have long-term complications from this condition?
• I have these other health conditions. How can I better manage them together?
• Are there any restrictions that I need to adhere to?
• Should I see a specialist?
• Is there a generic alternative to the medication you are prescribed?
• Are there brochures or other printed materials that I can take with me? What sites do you recommend?

Feel free to ask any other questions you have.

What to expect from your doctor

Your doctor may ask you several questions, including:

• What symptoms are you experiencing and when did they start?
• Have you noticed any changes in the way you feel or look? Has your sex life changed? How do you sleep? Do you have a headache or joint pain or has your vision changed? Have you noticed excessive sweating?
• Does anything seem to improve or worsen your symptoms?
• How much would you say your functions have changed over time? Do you have old photos I can use for comparison?
• Do your old shoes and rings still fit? If not, how much do they change over time?
• Have you had a colon cancer screening?

Treatment of acromegaly

After the diagnosis has been established, the main vector of therapy is aimed at normalizing the level of growth hormone and IGF-1 in the blood.

There are three main treatments for acromegaly: medication, surgery, and radiation therapy. In many cases, it is necessary to turn to a combination therapy method, because one method is not enough. The main goals of treatment are to reduce and stabilize tumor size, reverse the signs and symptoms of acromegaly, and prevent recurrence.

First of all, it is very important to normalize the functioning of the cardiovascular system, the functionality of the lungs and metabolic processes. Also, treatment of acromegaly includes normalizing biochemical parameters to a level that will reduce the risk of mortality. The goal of this treatment is to eliminate the problem without harming other pituitary hormones.

Folk remedies

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Treatment of acromegaly with folk remedies is an excellent preventive measure, which should be agreed with a specialist to achieve maximum effect. In this case, he is an endocrinologist.

What to remember when using folk remedies in the treatment of acromegaly

Decoctions and teas prepared from plants and herbs such as:

• hop;
• sage;
• arnica;
• root part of licorice and ginseng;
• chamomile;
• linden flowers.

Acromegaly, folk remedies for the treatment of which are used only after consultation with a doctor, is quite treatable. It must be remembered that herbal infusions and teas cannot be stored for a long period of time. They should be used within 24 hours after infusion and straining.

This is explained by the fact that if they stand for a long time, they will lose all their healing and restorative characteristics and, even worse, can cause significant harm. When treating acromegaly with traditional recipes, this is unacceptable, because any negative impact will affect the body and the activity of the thyroid gland, which plays one of the main roles in this case.

A mandatory step, which also needs to be agreed upon with a specialist, is diet therapy. It allows you to strengthen the body, speed up metabolism and increase the body's resistance.

Surgery

Surgical treatment consists of removing the pituitary adenoma. Currently in clinical practice there are two approaches to neurosurgical treatment of pituitary tumors: transcranial and transsphenoidal. The choice of access is made by a neurosurgeon.

The goal of surgical treatment of this pathology is complete removal of the tumor in the case of microadenoma and maximum removal of part of the tumor in case of macroadenoma. Surgical treatment provides quick results and is highly effective in relieving headaches and eliminating compression of the optic nerve.

At the moment, the transsphenoidal access method is the most effective method for removing microadenomas. This surgical intervention is performed under endoscopic control, this helps to carry out the operation as successfully as possible. For 50% of patients with a large pituitary tumor, surgical treatment is ineffective, because after surgery the level of somatotropin remains the same.

Causes

Causes of acromegaly In the vast majority of clinical cases, the cause of excessive secretion of growth hormone is a pituitary adenoma, located in the area responsible for the production of growth hormone. Most often, tumor development is provoked by a mutation in the Gs-alpha protein gene. This mutant protein continuously stimulates the enzyme adenylate cyclase, which leads to increased growth of cells that produce somatotropin, and as a result, to an increase in its production.

Sometimes hypersecretion of somatotropin is caused by pituitary hyperplasia or excess secretion of somatoliberin, a releasing hormone secreted by the hypothalamus and stimulating the synthesis of hormones in the anterior lobe of the pituitary gland, including growth hormone.

Acromegaly can be hereditary.

If excessive synthesis of GH is observed in childhood, then the growth of all tissues and organs will be proportional, and gigantism will develop. In adulthood, when the growth plates are already closed, acromegaly develops.

Prognosis for acromegaly

The prognosis for this pathology depends on the timeliness and correctness of treatment. The lack of measures to eliminate acromegaly can lead to disability in patients of working age and active age, and also increases the risk of mortality. Acromegaly affects the life expectancy of patients; in 90% of cases, patients with this pathology do not live to see 60 years of age. The cause of death is usually secondary diseases (cancer pathologies and diseases of the cardiovascular system).

After surgical treatment of a large pituitary tumor, the risk of recurrence increases.

Famous people with acromegaly

In chronological order

• Rondo Hatton (1894–1946) was an American soldier, journalist, and film actor. Most likely (though not certain) the impetus for the disease was given by mustard gas poisoning during the First World War. Becoming an actor, he played ugly villains in horror films.
• Tiye, Maurice (1903−1954) - French wrestler, born in the Urals into a French family; prototype of Shrek.
• Kiel, Richard (1939-2014) - American actor with a height of 2.18 m [4].
• Andre the Giant (1946–1993) was a professional wrestler and actor of Bulgarian-Polish descent.
• Struycken, Karel (born 1948) is a 2.13 m tall Dutch character actor.
• Igor and Grishka Bogdanoff (Igor et Grichka Bogdanoff; born 1949) are French twins of Russian origin, TV presenters and popularizers of space physics[7].
• Big Show (born 1972) is an American wrestler and actor with a height of 2.13 m.
• McGrory, Matthew (1973-2005) - American actor with a height of 2.29 m.
• Valuev, Nikolai Sergeevich (born 1973) - Russian professional boxer and politician.
• The Great Khali (born 1972) is a 2.16 m tall Indian wrestler, actor, and powerlifter.
• Antonio Silva (born 1979) is a Brazilian mixed martial artist.

Prognosis for patients

Acromegaly is a rare but severe pathology. Mortality rates in this group of patients are 3 times higher than in the general population. The most common cause of death is decompensated heart failure. If it is possible to normalize the level of growth hormone in the blood, then the prognosis becomes more optimistic. Good results are achieved when removing a small tumor, while large adenomas are prone to relapse.

Tanya Angus from Las Vegas, suffering from a terrible disease - acromegaly. The photo shows Tatyana before and after the development of the disease.