Acromegaly

If there is a sudden growth of his extremities or protruding body parts, then one is suspected Acromegaly entitled. This is a growth hormone disease, which is also known as Pierre-Marie syndrome. At the first signs, the person concerned should immediately consult a specialist, since the growth of the internal organs associated with the disease can have serious consequences for the patient.

What is acromegaly?

The main cause of the disease is an excess of growth hormones in the blood, which come from the pituitary gland, the so-called pituitary gland, and enable tissue to be built up.

Acromegaly is a special growth hormone disease that usually occurs around the age of 50 in those affected. It is currently assumed that there are around 50 affected people out of one million in Germany.

The main cause of the disease is an excess of growth hormones in the blood, which come from the pituitary gland, the so-called pituitary gland, and enable tissue to be built up.

These growth hormones include the so-called human growth hormone (HGH), growth hormone (GH), somatotropic hormone (STH) and somatotropin. It often takes up to ten years to finally diagnose the disease. The reason for this is the fact that the external signs of the disease are only visible late.

causes

Acromegaly is caused in the majority of cases by an adenoma of the pituitary gland. An adenoma, which is a benign hormone-forming tumor, is the basis of acromegaly in over 95% of cases.

The formation of hormones in the adenomas cannot be interrupted by a normal control loop, so that over the long term there is an overproduction of growth hormones. If the adenoma is smaller than one centimeter, it is called a microadenoma. If they measure more than an inch, they are again called macroadenomas.

In rare cases, an ectopic production of the so-called somatotropin can also be the basis of an acromegaly. This external production can arise from hormone-forming tumors in humans. A malignant pituitary tumor may also be the cause. However, these malignant tumors are very rare as the cause of acromegaly.

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Symptoms, ailments & signs

With acromegaly, symptoms appear over the course of years. At first, those affected feel indefinable headaches as well as increasing tiredness and exhaustion. As the disease progresses, bone pain is added. The hands and feet slowly increase in size, causing complaints such as skin thickening, excess cartilage and joint diseases.

This is accompanied by increased hair growth and often high blood pressure. Typically, the organs also grow - what is known as visceromegaly occurs, which, depending on which organ is affected, can lead to complaints such as digestive disorders, jaundice or cardiac arrhythmias.

In men, acromegaly is also manifested by erectile dysfunction. The woman experiences sexual disorders and missed periods. The overgrowth can be identified at an early stage by means of numbness, tingling sensations and increased sweating. On the face, changes occur in the ears, eye sockets, and chin.

The tongue, jaw, teeth, and lips can also be affected. The multifaceted malformations cause various symptoms and complaints that can only be clearly attributed to acromegaly in the course of the disease. The most obvious symptom is the growing limbs.

Diagnosis & course

The characteristic symptoms of acromegaly only become visible over a long period of time, so that the diagnosis of this disease is considered difficult. In the course of an acromegaly, the hands and feet of those affected grow steadily.

In addition, changes in the face are characteristic, including large ears, bulging eye socket edges and a protruding chin. Affected people also often have a large tongue and a large jaw, which can change the position of the teeth. The increasing growth is often associated with joint pain, headache, visual disturbances or chewing disorders. Most problematic, however, is the enlargement of the internal organs, which can lead to leaky heart valves or cardiac arrhythmias.

If there are any initial indications of a possible growth hormone excess, a specialist should be consulted immediately. In the first step, the amount of GH and the messenger substance insulin-like growth factor 1 (IGF-I) in the blood is measured. If the hormone level is elevated, a glucose tolerance test is carried out as the diagnosis progresses.

The GH level in the patient's blood is then determined. If the GH level shows uncontrolled GH formation, the next step is an MRI. This allows a possible pituitary adenoma to be diagnosed as the cause of the disease at an early stage.

When should you go to the doctor?

With acromegaly that was recognized early, regular visits to the doctor are necessary due to the many side effects and symptoms of the disease. In addition, there is an unusual increase in size, which is soon noticeable. Visits to internists, orthodontists or other medical professionals are the order of the day.

However, if acromegaly occurs before or after puberty, the increased size of the extremities and other outstanding features of the body must first be determined. Due to the rarity of the disease and the insidious course of the disease, this is usually not the case in a timely manner. Most likely, trained endocrinologists determine the cause of the growth in size of certain parts of the body. The patient usually only goes to the doctor when he has noticed the increased growth of certain body parts.

Whether an adenoma or hormonal overproduction is present can be determined by a doctor on the basis of the other symptoms and typical accompanying symptoms. However, since acromegaly is insidious, wrong diagnoses are often made. Individual symptoms are often not related to one another. Without a hormone analysis, the doctors consulted usually come on the trail of acromegaly late.

If an adenoma is present, surgical, drug or radiotherapy treatment options can be chosen. An oncologist or internist should be consulted to verify the presence of tumor-related acromegaly and issue a corresponding recommendation.

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Treatment & Therapy

There are different ways to treat acromegaly. Surgical removal of the pituitary adenoma is a common option. These operations, which usually involve access through the nose, are particularly successful with microadenomas.

In addition to the surgical procedures, however, there is also the option of treating acromegaly with medication. For example, the somatostatin analogues should be mentioned here, which significantly inhibit the release of growth hormones from the pituitary gland. They can also reduce the number of adenomas, but at the same time carry the risk of gallstones.

The drugs are often combined with so-called dopamine agonists, which are also used to treat Parkinson's disease. When using dopamine agonists, however, the liver values ​​should always be checked for a possible increase. If treatment with somatostatin or dopamine agonists is not successful, so-called GH receptor antagonists can also be used. These prevent growth hormones from spreading effectively in the body.

If this measure does not produce any results either, an attempt is made to remove the adenoma using high-precision radiation. If hypophyseal function occurs after this radiation therapy or other operations, hormone replacement therapy is requested. The success of all therapies must be checked by a doctor by constantly checking the levels of growth hormones in the blood.

Outlook & forecast

Acromegaly disrupts the patient's growth. This disorder can also affect the internal organs and lead to various complaints and complications. As a rule, the organs can shift and thereby crush other organs. Acromegaly can therefore lead to life-threatening situations for the patient, which must be treated by a doctor in any case.

In most cases, the patient's resilience drops extremely and the affected person suffers from severe headaches and tiredness. The bones and joints also hurt and limit the patient's everyday life extremely. Furthermore, there is huge growth, which is often accompanied by hair loss and high blood pressure. Children are often teased by the external symptoms. Numbness or paralysis can also occur all over the body.

Acromegaly is usually treated with surgery. There were no further complaints. However, radiation therapy is usually necessary after the procedure to fully combat the disease.

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prevention

A preventive measure against the occurrence of acromegaly is not yet known. Those affected can only make sure to consult a specialist immediately if they experience the first symptoms. This means that no important treatment time goes by.

Aftercare

After the successful treatment of acromegaly, constant follow-up care is recommended. This includes follow-up examinations at regular intervals. The success of the treatment is checked. This is the only way to take timely action if the patient's values ​​change. The follow-up appointments are coordinated with the responsible doctor. During these examinations, blood values ​​and the pituitary gland tumor are checked.

Potential side effects of the medication taken are also monitored. In addition, the general health of the patient is always examined. All relevant additional information helps the attending physician. This includes physical and mental well-being and information on whether other medications are being taken.

Additional follow-up exams may include visual acuity checks, joint and thyroid exams, and cancer screening. These examinations are based on the patient's symptoms. In individual cases this is decided by the attending physician and additional treatment is ordered.

In addition to regular follow-up examinations with a doctor, a healthy lifestyle is also recommended. This can be achieved with a wholesome and healthy diet, sufficient exercise and valuable social contacts. Family and friends in particular can help patients cope with their illness. A psychotherapist can also be called upon if necessary or if there are psychological problems resulting from the illness.

You can do that yourself

In order to control the course of the acromegaly, the patient can contribute a lot. Participation in a self-help group, for example, is considered a helpful measure.

In order for the treatment of acromegaly to go well, it is important that the patient regularly attends the follow-up and check-ups. If necessary, the therapy can be adapted to possible changes at an early stage in this way.

The patient also has the opportunity to become active himself by creating a special diary and entering his symptoms as well as the effects of the medication taken or the results of the check-ups in it. The patient diary thus plays a positive role in monitoring the course of the disease.

The psyche of the person concerned also plays an important role in acromegaly. The illness affects not only the physical, but also the mental and emotional balance. If acromegaly has negative consequences for the psyche, professional advice from a psychologist or doctor can be helpful.

Participation in a self-help group, such as the network “Pituitary and Adrenal Diseases e.V.”, is also considered sensible.

A healthy diet, exercise and support from relatives or friends are also recommended in order to gain strength to deal with the serious illness.