What is it called when a baby is born without a Pituitary Gland?

Question

So my cousin (12 y/o) was born without a Pituitary Gland what is that called? She's in a wheel-chair kind of device, can hardly talk, she need's help with everything...what is this disorder called? How common is it? And what is the life expectancy for people suffering from this disorder? Any other information would be great!

Answer 1

Hypopituitarism
Definition

Hypopituitarism is loss of function in an endocrine gland due to failure of the pituitary gland to secrete hormones which stimulate that gland's function. The pituitary gland is located at the base of the brain. Patients diagnosed with hypopituitarism may be deficient in one single hormone, several hormones, or have complete pituitary failure.


Panhypopituitarism
This condition represents the loss of all hormones released by the anterior pituitary gland. Panhypopituitarism is also known as complete pituitary failure.

Treatment of multiple deficiencies and panhypopituitarism
The treatment of hypopituitarism is usually very straightforward, but must normally continue for the remainder of the patient's life. Some patients may receive treatment with GnRH, the hypothalamic hormone. In most cases, treatment will be based on the specific deficiency demonstrated. Patients with hypopituitarism should be followed regularly to measure treatment effectiveness and to avoid overtreatment with hormone therapy. If the cause of the disorder is a tumor or lesion, radiation or surgical removal are treatment options. Successful removal may reverse the hypopituitarism. However, even after removal of the mass, hormone replacement therapy may still be necessary.

Prognosis

The prognosis for most patients with hypopituitarism is excellent. As long as therapy is continued, many experience normal life spans. However, hypopituitarism is usually a permanent condition and prognosis depends on the primary cause of the disorder. It can be potentially life threatening, particularly when acute hypopituitarism occurs as a result of a large pituitary tumor. Morbidity from the disease has increased, although the cause is not known. It is possible that increased morbidity and death are due to overtreatment with hormones. Any time that recovery of pituitary function can occur is preferred to lifelong hormone therapy.

Prevention

There is no known prevention of hypopituitarism, except for prevention of damage to the pituitary/hypothalamic area from injury.

Answer 2

Underdeveloped Pituitary Gland

Answer 3

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Answer 4

Empty sella syndrome

Definition:
Empty sella syndrome is the absence of the pituitary gland on radiological imaging of the sella turcica, a bony structure that normally partly surrounds the gland.


Causes, incidence, and risk factors:
The pituitary gland is a small gland located at the base of the brain. It makes several hormones that control the function of other glands in the body, including the thyroid, the adrenal glands, and the ovaries or testes.

The pituitary gland is partly surrounded by a bony structure called the sella turcica ("Turkish saddle"). When the pituitary gland is not visible on CT or MRI scans of the sella turcica, the condition is referred to as empty sella syndrome. Primary empty sella syndrome occurs when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the walls of the sella. When the sella is empty because the pituitary gland has regressed following an injury such as head trauma or an event such as surgery or radiation therapy, the condition is called secondary empty sella syndrome.

Primary empty sella syndrome is most often an incidental finding during radiological imaging of the brain. Pituitary function is usually normal, and patients do not have any symptoms. The hormone prolactin is mildly elevated in 10% to 15 % of patients, and the elevated prolactin may interfere with normal function of the testes or ovaries.

Medications such as bromocriptine that suppress prolactin production are effective in correcting the problem.

Patients with an empty sella due syndrome to destruction of the pituitary gland have signs and symptoms caused by partial or complete loss of pituitary gland functions. The causes and symptoms of pituitary insufficiency are reviewed in the article on hypopituitarism.

Empty sella syndrome is often associated with abnormal pituitary function in children. Isolated deficiency of growth hormone (GH) is most common, but other pituitary hormones may also be deficient. One study observed empty sella in 48% of children with GH deficiency or multiple pituitary hormone deficiencies compared to only 2% of children with normal pituitary function.

Answer 5

I'm pro-liberty. That includes the kid. Odd that this situation has occurred when birth control options are finally available. It's not that abortion is new, it's just that it's safer, more available, and convenient. I would like someone to tell me when a human is eligible for Constitutional rights. By my understanding, basic Constitutional rights to life are not given by humans---that includes the government, the father, or the mother. There are serious moral, ethical and political problems with this entire abortion question, and those who wish us the most harm are using it to divide us from both our reason and our fellow citizens.

Answer 6

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Answer 7

I also have hypopituitarism. I was diagnosed at the age of 13 after going to many doctors. One day my mother could not wake me up. She shook me as hard as she could and I still wouldn't wake up. So the doctor we were seeing, Dr. Chipman (who later went to work for Eli Lilly) an endocrinologist, told her to take me to the ER.

Ever since then I have been taking Cortef which is the brand name of the hydrocortisone medicine and Synthroid medicine to replace the hormone that is not being produced by the thyroid because the Pituitary gland controls the Thyroid.

I took estrogen to start puberty and I have been taking estrogen in the form of birth control pills ever since.

In my 30s I had to start taking human growth hormone because my levels were very very low and I believe I will be taking them for the rest of my life as well.

As to how rare this condition is. it is found in 1 out of 200,000 people according to the world health organization.