Gigantism

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Gigantism

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A 38-year-old woman comes to her primary care physician for evaluation of “changing facial features” that began seven-months ago. The patient describes that her forehead and jaw appear bigger than before. In addition, she endorses deepening of her voice and “being sweaty.” Past medical history is noncontributory. Temperature is 37.5°C (99.5°F), pulse is 72/min, respirations are 18/min, and blood pressure is 128/72 mmHg. As part of the work-up process, serum insulin-like growth factor 1 (IGF-1) levels are measured. In addition, growth hormone (GH) levels are measured before and after the administration of glucose. Which of the following sets of findings will most likely be seen in this patient?

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Gigantism p. 338, 343

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With gigantism, “gigant” refers to giant, so gigantism is a rare hormonal disorder in children and adolescents where there is an excess of growth hormone, and it causes rapid and excessive growth of long bones, like the tibia and humerus.

As an example, the French wrestler André the Giant who played Fezzik in the movie The Princess Bride had gigantism.

In adults, excess growth hormone causes a different disorder, called acromegaly, because their long bones have stopped growing.

Let’s start with how growth hormone, or somatotropin, is made.

Normally, the hypothalamus which is at the base of the brain, secretes growth hormone-releasing hormone in bursts throughout the day - every couple hours, and this can increase based on things like low blood glucose levels, lack of food, increased exercise, increased sleep, and increased stress like trauma.

The growth hormone-releasing hormone goes into the hypophyseal portal system - which is a network of capillaries linking the hypothalamus to the anterior pituitary which is smaller in size than a pea.

The growth hormone-releasing hormone binds to a surface protein on somatotroph and mammosomatotroph cells of the anterior pituitary gland, and in response, they release growth hormone.

Now, growth hormone affects lots of tissues directly and indirectly throughout the body.

Direct effects occur in certain tissues where growth hormone stimulates cellular metabolism and leads to organ growth.

The liver releases more glucose into the blood, the body retains nitrogen leading to more muscle growth, and osteoblasts get stimulated which causes the bones to thicken.

Another direct effect of growth hormone is to increase insulin resistance - making it harder for cells to take in glucose - which leads to an increase in blood insulin levels.

Summary

Gigantism is a hormonal disorder that results in an individual growing to an excessive size. The condition is caused by the overproduction of growth hormone (GH) and insulin-like growth factor-1, usually due to a tumor on the pituitary gland.

Symptoms of gigantism include excessive growth, particularly in the facial area and hands; a deep, hoarse voice; and enlarged organs. Gigantism is also associated with other health problems, including heart disease, joint pain, and diabetes.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Harrison's Endocrinology, 4E" McGraw-Hill Education / Medical (2016)
  6. "Congenital gigantism due to growth hormone-releasing hormone excess and pituitary hyperplasia with adenomatous transformation." The Journal of Clinical Endocrinology & Metabolism (1993)
  7. "Gigantism" The Journal of Clinical Endocrinology & Metabolism (1999)
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