ACROMEGALY

ACROMEGALY

INTRODUCTION:

·         Acromegaly comes from the Greek words for extremities and enlargement reflecting one of its most common symptoms- the abnormal growth of the hands and feet.

·         Acromegaly is a hormonal disorder that results from too much growth hormone (GH) in the body. The pituitary, a small gland in the brain makes growth hormone. In Acromegaly, the pituitary produces excessive amounts of Growth hormone. Usually the excess of GH comes from benign or non cancerous tumours on the pituitary. These benign tumours are called adenomas.  

·         Acromegaly is most often diagnosed in middle- aged adults; symptoms can appear at an age. If they are not treated, Acromegaly can result in serious illness and premature death.

·         Acromegaly is treatable in most patients, but because of its slow and often sneaky onset, it often is not diagnosed early or correctly.

·         The most serious health consequences of acromegaly are type2 diabetes, high blood pressure, increased risk of cardiovascular disease and arthritis. Patients with acromegaly are also at increased risk for colon polyps, which may develop into colon cancer if not removed.

                      

Symptoms of Acromegaly:

Swelling of the hands and feet is often an early feature with patients noticing, a change in ring or shoe size. Gradually the bone changes alter the patient’s facial features. The brow and lower jaw protrudes, the nasal bone enlarges and the teeth space out. Outgrowth of bone and cartilage often leads to arthritis. Other symptoms of acromegaly include:

·         Joint acnes

·         Thick, coarse, oily skin

·         Enlarged lips, nose and tongue

·         Deepening of the voice due to enlarged sinuses and vocal cords.

·         Excessive sweating and skin odour.

·         Fatigue and weakness

·         Headache

·         Impaired vision

·         Abnormalities of the menstrual cycle and sometimes breast discharge in women’s

·         Erectile dysfunction in men

·         Decreased libido

Causes of Acromegaly:

·         Acromegaly is caused by prolonged overproduction of Growth hormone by the pituitary gland. The pituitary produces several important hormones that control body function such as growth and development, reproduction and metabolism. But hormones never seem to act simply and directly. They usually flow in a series, affecting each other’s production or release into the bloodstream.

·         Acromegaly is caused by non- cancerous tumours in the pituitary gland which are called pituitary adenomas. The tumour produces too much GH and raises the level of GH in the blood. Too much GH raises the level of Insulin like growth factor (IGF-1), a hormone produced in the liver that also promotes growth. Rarely, acromegaly is caused by hormone producing tumours in other parts of the body.

·         GH is a part of cascade of hormones that as the name implies, regulates the physical growth of the body. This cascade begins in a part of the brain called hypothalamus. The hypothalamus makes hormones that regulates pituitary, one of the hormones in the GH series is GHRH, which stimulates the pituitary gland to produce GH.

·         Secretion of GH by the pituitary into the bloodstream stimulates the liver to produce another hormone called insulin like growth factor (IGF1). IGF-1 is actually cause’s tissues growth in the body. High levels of IGF-1 in turn, signal the pituitary to reduce GH production. The hypothalamus makes another hormone called somatostatin, which inhibits GH production and release. Normally, GHRH, Somatostatin, GH and IGF-1 level in the body are tightly regulated by each other and by sleep, exercise.

·         GH is a hormone that regulates the growth and development of the body. People with acromegaly have too much GH; it accelerates bone growth and organ enlargement. Because of this growth stimulation, the bones and organs of people with acromegaly are much larger than others people’s bones and organs. 

Diagnosis:

A series of tests can confirm the presence of acromegaly:

         

1)      IGF-1 Test:

If acromegaly is suspected, doctor will also do a blood test to check our level of IGF-1. High IGF-1 levels care means that our level of GH is also high. Insulin like IGF-1 is a hormone produced in the liver. It promotes the growth. People with acromegaly usually have high levels of GH which stimulates the production of IGF1.

2)      OGTT test:

Another way to diagnose acromegaly is with an oral glucose tolerance tests. In this test, GH levels in the blood are measured after we drink sugar water. Normally, sugar water will make the pituitary gland stop producing GH and blood levels drop. However, a GH producing primary tumour will not stop making GH, so the levels of GH in the blood will not change. Oral glucose tolerance test (OGTT) can give more accurate reading of GH levels. The patients will need to fast overnight before drinking some concentrated glucose solution and then giving a blood sample. Ingesting the glucose would normally lead to fall in hormone levels. In a person with acromegaly, the hormone levels would remain high because the body is producing excess GH.

3)      Blood test:

This test can determine too much GH, but these are not always accurate because GH levels fluctuate throughout the day.

4)      Imaging Studies:

X- Rays and MRI scan may be ordered to check excess bone growth if our doctor suspects we have acromegaly. Doctors also perform a physical exam and they may order a sonogram to check the size of interior organs. After diagnosed with acromegaly, doctors can use MRI and CT scan to help them find the pituitary tumour and determine its size. If they don’t find a tumour on the pituitary gland, doctor will look forward a tumour in the chest, abdomen or pelvis that may be causing excess GH production.

The NIH estimates that 3—4 out of every 1 million people develop acromegaly every year and that 60 out of every 1 million people have that condition at given time. Since the condition often goes undiagnosed the total number of affected individuals is probably underestimated.

Treatment:

Treatments based on age and overall health. The goals of treatment are:

·         It brings GH production levels back to normal.

·         It relieves pressure around any growing pituitary tumour.

·         Maintain normal around pituitary function.

·         It treats any hormone deficiencies and improves the symptoms of acromegaly.

               

 

1)      Surgery:

Surgery is the best option to remove the tumour causing excess GH, so doctors usually recommend surgery to people with acromegaly. This treatment is quick and effective in reducing GH levels, which can improve symptoms.

2)      Medication:

This treatment is used if surgery is not successful in reducing GH levels and it can also be used to shrink large tumours before surgery. This type of medications is used to regulate or block GH production. Such medications are:

Ø  Somatostatin analogs

Ø  GH receptor antagonists

Ø  Dopamine agonsits

3)      Radiation:

 It may be used to destroy larger tumour or sections of tumour left after surgery or when medications alone are not effective Radiations can slowly help to lower GH levels when used alone with medications. A dramatic increase in GH levels using this type of treatment may take several years with radiation administered in multiple 4-6 weeks session. Radiation can improve your fertility. In rare cases, it can lead to vision loss, brain injury or secondary tumour.

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