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Gigantism

By: Samuel B Morales

Class : Bio 211-V06

Professor: Cheryl Cook

9/10/2022 Fall semester

Thank You!

What is Gigantism?

Gigantism, also called Pituitary Gigantism and Pediatric Acromegaly, is a very rare condition that happens when a child or adolescent has high levels of growth hormone (GH) in their body, which causes them to grow very tall.

The pituitary gland normally produces GH, but a tumor on their pituitary can produce excess GH in gigantism.

Over Veiw

Of

Gigantism

Allow me to riterate

How It develops pt 1

In addition Children with gigantism can also develop certain symptoms as their pituitary tumor applies pressure to their nearby brain and nerve tissues. The main sign of gigantism is excessive growth. Children with gigantism grow rapidly in height.

For starters Growth hormone (GH) regulates growth during childhood by promoting measured bone, muscle and tissue growth and helping to control metabolism. Growth is normally a relatively stable process until a child reaches puberty.

During puberty, the release of sex hormones (estrogen and testosterone) increases significantly and causes the growth plates (epiphyses) at the ends of your child’s long bones to slowly fuse. Height growth stops at the end of puberty when the growth plates have completely joined.

But during the process, (And the most common cause), a benign tumor grows on the childs pituary gland (Pituitary adenoma) and due to children with giagantism have an enlarged pituitary gland, which over realeases an excess amount of the growth hormone which invades the groth plates and makes the child grow excessively till the end of the pubescent stage of growth.

Origin of Gigantism

Many children with gigantism have a genetic mutation that causes the pituitary tumor to form. The most common genetic mutations associated with gigantism are AIP gene mutations or deletions, making up approximately 29% of the population of people with gigantism.

Gigantism can occur as a part of several rare genetic disorders that lead to an increased risk of developing GH-secreting pituitary tumors, including:

  • Carney complex: This is a genetic condition that affects skin color (pigmentation) and causes benign tumors of your skin, heart and endocrine system. GH-secreting pituitary adenomas occur in about 10% to 13% of Carney complex cases and usually have a slow progression.

  • McCune-Albright syndrome: This is a genetic condition that affects your bones, skin and endocrine system, causing café-au-lait skin pigmentation, scar tissue forming on bones and early (precocious) puberty. Excess GH is present in 20% to 30% of McCune-Albright syndrome cases. It’s often caused by pituitary gland enlargement (hyperplasia).
  • Multiple endocrine neoplasias (MEN) type 1 or type 4: These are genetic conditions in which one or more of your endocrine glands are overactive and/or form a tumor, which can include a GH-secreting pituitary tumor.

  • Neurofibromatosis: This is a condition that’s a part of a group of genetic conditions known as neurocutaneous disorders that affect your skin and nervous system. These types of disorders are caused by abnormal increases in cell growth. This results in the formation of tumors all over your body, which can include a GH-secreting tumor.
  • Familial isolated pituitary adenomas (FIPA): This is an inherited condition characterized by the development of pituitary adenomas, which can include a GH-secreting adenoma.

How it Develops Pt 2

General Symptoms

Aside from being very tall/large for their age, physical characteristics of gigantism include:

  • Very prominent forehead and a prominent jaw.
  • Gaps between their teeth.
  • Thickening of their facial features.
  • Large hands and feet with thick fingers and toes.
  • Enlargement of internal organs, especially your child’s heart.
  • Excessive sweating (hyperhidrosis).
  • Double vision or difficulty with side (peripheral) vision.
  • Headaches.
  • Joint pain.
  • Delayed puberty.
  • Irregular menstruation (periods).
  • Sleeping problems, such as sleep apnea.
  • Muscle weakness.

Densely Granulated GH Cell Adenomas : These constitute about 30% of GH secreting pituitary adenomas and are characterized by cells that are similar to somatotrophs and contain numerous large secretory granules. These are slow-growing tumors which present in middle age.

Sparsely Granulated GH Cell Adenomas: These constitute about 30% of GH secreting pituitary adenomas and are characterized by pleomorphic cells with few secretory granules. These are rapidly growing tumors that present with severe disease at a young age.

Mixed GH Cell and Prolactin Cell Adenoma :These are about 20% of GH secreting pituitary adenomas which are characterized by densely granulated somatotrophs and sparsely granulated lactotrophs. They secrete GH and prolactin.

Acidophilic Stem Cell Adenomas: These are rare tumors and are characterized by giant mitochondria. Clinically these are aggressive tumors that invade the surrounding structures. They also secrete GH and prolactin.

Mammosomatotroph Cell Adenoma: These comprise about 10% of GH secreting pituitary adenomas and are characterized by cells that secrete both GH and prolactin. They commonly occur in children and present with gigantism and hyperprolactinemia.

Plurihormonal Cell Adenomas : As the name suggests, these tumors may secrete multiple hormones GH, prolactin, follicle-stimulating hormone (FSH), Thyrotropin stimulating hormone (TSH), or Adrenocorticotrophic hormone (ACTH); the secondary hormonal products may be silent

Effects at a Cellular & Tissue level

Safely controlling growth hormone (GH) and insulin-like growth factor 1 (IGF1) levels.

Controlling pituitary tumor growth.

Reducing the effects of the pituitary tumor on nearby structures, such as brain tissue and the optic nerve.

Treating or reducing the effects of GH on other body systems.

Medications are available and effective in treating acromegaly (excess GH in adults) but the effects of these drugs on children haven’t been adequately studied.

Healthcare providers typically use a combination of treatments, specifically surgery and radiation therapy, for gigantism.

Treatmeant

Surgery is the most common treatment option for gigantism. The goal is to remove or reduce the size of the pituitary tumor. Since the pituitary tumors that cause gigantism are often large, children with gigantism may need multiple surgeries to remove the tumor and effectively control GH levels.

Your child’s surgeon may use a type of surgery called transsphenoidal surgery to remove the pituitary tumor, which involves going through your child’s nose and sphenoid sinus, a hollow space in your child’s skull behind the nasal passages and below your child’s brain, to perform surgery.

In Addition

References

Beckers A, Petrossians P, Hanson J, Daly AF. The Causes and Consequences of Pituitary Gigantism. (https://pubmed.ncbi.nlm.nih.gov/30361628/) Nat Rev Endocrinol. 2018; 14(12): 705-720. Accessed 9/10/2022.

Bello MO, Garla VV. Gigantism And Acromegaly. [Updated 2021 Apr 7]. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2021. Accessed 9/10/2022.

MedlinePlus. Gigantism. (https://medlineplus.gov/ency/article/001174.htm) Accessed 9/10/2022.

Rostomyan L, Daly AF, Beckers A. Pituitary Gigantism: Causes and Clinical Characteristics. (https://pubmed.ncbi.nlm.nih.gov/26585365/) Ann Endocrinol (Paris). 2015; 76(6): 643-9. Accessed 9/`0/2022.

Society for Endocrinology. Gigantism. (https://www.yourhormones.info/endocrine-conditions/gigantism/) Accessed 9/10/2022.

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