What Is Acromegaly

Acromegaly is a rare medical condition in which the body produces excessive amounts of growth hormone, resulting in abnormal expansion of tissues like organs in the body. When acromegaly is discovered throughout adolescence, it is referred to as gigantism.

Overview

Acromegaly is an uncommon illness characterised by increased production of a hormone known as human growth hormone or somatotropin. This hormone is typically produced by the pituitary gland, which is found near the base of the brain, beneath the hypothalamus. Smaller than a pea in size, this endocrine gland is an important gland that produces a number of vital hormones, including the human growth hormone. 

Because it stimulates growth, the growth hormone is crucial for children's development. When this hormone no longer increases height, it still has other roles, such as assisting in the maintenance of:

• Normal structures and metabolism in organs, bones, and cartilage
• A normal range of blood sugar values

The estimated incidence of acromegaly is 0.2 to 1.1 cases per 100,000 individuals worldwide, and it can affect persons of any age or gender. However, acromegaly is typically diagnosed in adults between the ages of 40 and 47, despite the fact that up to 22% of patients identified range in age from 0 to 19 years thanks to advancements in the field of acromegaly genetics. The acromegaly is called gigantism whenever this augmented production of growth hormone occurs before the fusion of the long bone epiphysis.^4,5

But have you ever wondered about the mechanism behind this abnormal hormone production? 

Causes of acromegaly

How exactly does a growth hormone excess lead to acromegaly? Let's explore the physiological process more thoroughly. 

The overproduction of growth hormone is frequently brought on by hyperplasic glands or tumours, primarily adenomas, in the pituitary gland. Tumours in other parts of the body that also release growth hormones can be another cause of acromegaly, albeit this is extremely unusual. Other potential causes of acromegaly include several hereditary disorders that produce too much growth hormone, like:

• Multiple endocrine neoplasia type I
• Familial acromegaly
• McCune-Albright syndrome
• Carney complex^6,7,8

Signs and symptoms of acromegaly

Typically, this illness advances slowly, and the majority of diagnoses are made in the fifth decade of life. The diagnosis may be delayed as a result of the disease's gradual course and the unclear nature of its symptoms.⁵ Acromegaly patients can have mild to severe presentations of illness alongside disproportionate growth. Some of the symptoms can be a direct consequence of the tumour like:

• Headaches
• Visual deficiency
• Cranial nerve palsy⁹

However, the features that acromegaly patients present and may complain are

• Enlargement of hands and feet due to bony expansion and tissue swelling
• Alterations of facial features leading to the prominent supraorbital bridge, broad nose, large lips and tongue
• Joint and wrists pain
• Acne resulting from oily skin
• Deep voice
• Sleep apnea
• Sweaty palms and soles
• Low sex drive
• Erectile dysfunctions and abnormal menstruation cycles^7,8,9

Moreover, patients can also present type 2 diabetes, cardiovascular problems like hypertension or arrhythmias and hyperpigmentation.⁸

Management and treatment for acromegaly

This disease is treatable, however, not all symptoms can be eradicated. Therefore, the treatment options that exist aim to reduce symptoms as much as possible and improve comorbidities. When acromegaly results from a tumour, another of the treatment goals is to reduce the tumour.⁸

One of the main priorities in the treatment is to reduce the hormone growth and another hormone called insulin-like growth factor – IGF-1 (that is produced in the liver when stimulated by the growth hormone) to normal levels. To do that exist several approaches that doctors then decide which is the best according to the situation.^7,9

Surgery to remove the tumour is thus one method of treating acromegaly. When the cancer is accessible, doctors typically prefer this approach. Endoscopic microsurgery is the type of surgery used, and post-operative recovery may include nasal congestion, sinusitis, or nosebleeds. Although they are uncommon, meningitis, cerebrospinal fluid leakage, and haemorrhaging are some of the more serious post-surgery complications. Blood and urine tests may be necessary right after following surgery to check for normal sodium and blood sugar levels. Patients are usually evaluated again after 6 to 12 weeks to ensure that the surgical procedure was successful.^8,9

When surgery is not an option, either because the patient does not want it or because it is too risky, doctors can give medicine such as

• Somatostatin substitutes, which function to reduce the quantity of growth hormone and insulin-like growth factor hormone. Although there have been reports of side effects like nausea, vomiting, diarrhoea, and increased blood sugar, the body generally tolerates this type of medication well. After 3 months, patients should still be evaluated with blood tests
•  Dopamine agonists that can be used with somatostatin. They are, however, typically considered for acromegaly patients with mild symptoms
•  Antagonists of the growth hormone, which is still relatively new. This form of treatment prevents the effects of growth hormone without necessarily lowering its levels. As a result, the level of the hormone IGF-1 declines, and this parameter is employed to regulate the treatment's efficacy^7,8,9

Whenever a tumour reappears and the prescribed medication does not produce the expected outcomes, the use of radiation is explored. Typically, stereotactic radiotherapy is used, and as a follow-up, patients must have hormonal analyses every year. Some of the hazards are linked to the pituitary gland's diminished hormonal activity.⁹

Diagnosis of acromegaly

Acromegaly is diagnosed using a variety of methods, such as medical evaluations, blood testing, and imaging procedures.

IGF and an oral glucose tolerance test are the two key characteristics of the first analysis, which is often a blood test. The IGF provides data on growth hormone concentration and is the best analysis because it is constant throughout the day. This measure can rule out the suspicion of acromegaly even though it can generate false positives during pregnancy. For the oral glucose intolerance test, 75g of sugar must be ingested, and blood sugar levels must be checked both before and two hours after consumption.^2,8

The imaging processes differ between Magnetic Resonance Imaging and a computed tomography scan of the brain, but the basic purpose remains the same: to learn more about the tumour's measurements and position.⁸ Other analyses can be performed, but their goals are to determine the impact of risk factors that patients may have on their acromegaly.

Risk factors

Since the majority of acromegaly diagnoses are linked to the presence of pituitary gland tumours, the main risk factor is genetic predisposition.^7,8

Complications

Acromegaly is typically treated, despite the fact that the modifications to the bones are irreversible. However, because the condition progresses gradually, problems may appear before a diagnosis. When left untreated, it may potentially result in a shorter life expectancy in addition to a lower quality of life.

A worse prognosis may result from certain comorbidities, such as sleep apnoea, hypertension, type II diabetes, excruciating arthropathies, or carpal tunnel syndrome (which causes pain in the joints and wrists, respectively).^2,7,8

FAQs

How can I prevent acromegaly

Unfortunately, there is no way to stop acromegaly. This is a fairly uncommon disease with symptoms that could also be caused by other conditions. As a result, it cannot be prevented and may be challenging to diagnose.

How common is acromegaly

Acromegaly is extremely rare, and every year are diagnosed around 0.2 to 1.1 cases per 100,000 individuals worldwide. This condition can affect persons of any age or gender, even though is mostly diagnosed in the fifth decade of life.⁴

What’s the difference between acromegaly and gigantism

Whenever this increased synthesis of growth hormone takes place prior to the long bone epiphysis fusing, the acromegaly is referred to as gigantism.

When should I see a doctor

If you experience acromegaly symptoms, you should see a doctor. Doctors follow and do routine examinations on patients who have acromegaly.

Summary

Acromegaly is an extremely rare condition that causes an excess of growth hormone. The vast majority of the time, this is caused by tumours in the pituitary gland, which produce growth hormone. This increase ingrowth hormone leads to an abnormal growth spurt, causing the individual to grow to an unusually large size. The condition is often characterized by an enlarged jaw, hands, and feet, as well as other physical abnormalities. A thorough medical examination is followed by imaging techniques and blood testing to make the diagnosis.

With the exception of bone defects brought on by growth hormones, symptoms are often treated with surgery and medically prescribed medication, and almost all of them are resolved. There are many different forms of treatment, including surgery, prescribed drugs, and even radiotherapy.