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Familial Isolated Pituitary Adenoma (FIPA)
Familial Isolated Pituitary Adenoma (FIPA)
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Term
Definition
Acromegaly
A disease caused when a tumour on the pituitary gland produces too much growth hormone.
ACTH
Adrenocorticotrophic Hormone
Adenohypophysis
An alternative name for the anterior pituitary gland.
Adenoma
A benign tumour, a growth which is not cancerous.
ADH
Anti-diuretic Hormone or Vasopressin
Adrenocorticotropic Hormone
This hormone is produced by the pituitary gland and tells the adrenal glands to produce cortisol.
AIP
Aryl hydrocarbon receptor interacting protein
Aryl hydrocarbon receptor interacting protein
A protein important for pituitary cell function, as lack of it predisposes to the development of pituitary adenomas. Abbreviation: AIP
Amenorrhoea
Absence of menstrual periods
Androgens
The group of male sex hormones, which includes testosterone.
Anti-diuretic Hormone
A hormone that the pituitary gland sends to your kidneys to limit the volume of urine produced. It is also known as vasopressin.Abbreviation: ADH or AVP
Apoplexy
Pituitary apoplexy is a rare condition that usually results from problems with the blood supply to, or within, a pituitary tumour. If this occurs, part or the entire tumour may be damaged through lack of oxygen (pituitary infarction) and there may be associated bleeding into the gland
Benign tumour
A growth which is not cancerous
Bromocriptine
A drug used to reduce the levels of both growth hormone and prolactin.
Carney complex
A rare disease with abnormalities in several organs such as the skin, adrenal cortex, heart, the pituitary gland and others
Carpal Tunnel Syndrome
Tingling and sometimes weakness of the hands due to compression of the nerve at the wrist. This is often worse at night. This symptom disappears once treatment has lowered your level of growth hormone.
Cerebrospinal fluid
The watery fluid that bathes the brain and spinal cord and also circulates within the ventricles of the brain and the central canal of the cord.
Circadian rhythm
Also called diurnal rhythm - a regular pattern following a 24 hour cycle. The body's natural release of some hormones, including testosterone, follows such a pattern
Corticotrophin-releasing hormone
A hypothalamic hormone releasing ACTH from the pituitary gland
Cortisol
One of the main hormones produced by the adrenal glands Cortisol controls a number of functions and is particularly important in times of illness and stress
Craniopharyngioma
A benign tumour of the pituitary gland often affecting the hypothalamus and the posterior pituitary as well The tumour is usually filled with a thick fluid, calcium and cysts. Incidence is 1% of adult tumours and 9% of those occurring in children
CRH
Corticotrophin-releasing hormone
Cushing's disease
Excess cortisol (adrenal gland hormone) secretion due to a pituitary adenoma releasing ACTH (Adrenocorticotrophic Hormone)
Cushing's syndrome
A group of symptoms due to excess glucocorticoids as a result of CRH, ACTH or cortisol-secreting tumour or due to drug treatment with glucocorticoids
Deoxyribonucleic acid
Deoxyribonucleic Acid (DNA) is a chemical molecule which is the coding mechanism that allows human cells to replicate and function
Desmopressin
This is the generic name for the medication used to treat Diabetes Insipidus and replaces the hormone vasopressin/ADH. Brand name of the drug can be Desmospray and DDAVP
Diabetes Insipidus
A condition characterised by great thirst and the constant need to pass urine
DNA
Deoxyribonucleic acid
Endocrine system
The body-wide system of hormone-producing glands, and the hormones they make, which control many aspects of life, including growth and reproduction
Endocrinologist
A doctor who specialises in treatment of diseases of the endocrine system
Familial isolated pituitary adenoma
Familial isolated pituitary adenoma (FIPA) occurs if two or more members of a family develop pituitary adenoma with no features of MEN1 or Carney complex. The term was first used by Prof Albert Beckers in 2000. It incorporates other terms such as isolated familial somatotrophinoma, familial acromegaly and pituitary adenoma predisposition.
Familial disease
A disease which is running in the family due to a change in the genetic material DNA (deoxyribonucleic acid) Several family members have abnormal DNA and some or all of these can be suffering from the particular disease
Folliculostellate cells
These cells provide a framework structure to the pituitary gland, they do not secrete any typical pituitary hormones
Follicle-stimulating hormone
This sex hormone (gonadotrophin) contributes to sexual development of children. In women, together with LH, it controls ovulation and is thus essential for a normal menstrual cycle and for fertility. In men, with LH, it stimulates the testes to produce sperm. Abbreviation: FSH
FSH
Follicle-stimulating hormone
Galactorrhoea
Abnormal milk secretion from the breasts
Gastrin
Gastrin is a hormone made in the stomach, duodenum, and the pancreas. It stimulates secretion of gastric acid (HCl) by the parietal cells of the stomach and aids in gastric motility
Geneticist
A doctor who specialises in the diagnosis and counselling of patients with hereditary or familial diseases
GHRH
Growth hormone-releasing hormone
Gigantism
A condition caused by production of too much growth hormone released by a pituitary adenoma during childhood, before growth is complete. It causes people to grow much taller than they would otherwise have done
Glucagon
Glucagon is a hormone, secreted by the pancreas that raises blood glucose levels
Gonadotrophin-releasing hormone
The hormone that controls production of luteinising hormone and follicle stimulating hormone in the pituitary gland
Gonadotrophins
This is a collective term for the sex hormones FSH and LH, which are hormones produced by the pituitary gland. They stimulate the ovaries or testicles
Gonads
The reproductive organs - ovaries in a woman, testes (testicles) in a man
Growth hormone
A hormone produced by the pituitary gland which controls rate of growth. It is mainly produced while you are asleep. Even after growth has ceased, growth hormone has important effects during adult life
Growth hormone-releasing hormone
A hypothalamic hormone which stimulates growth hormone release from the pituitary gland. Abbreviation: GHRH
Gynaecomastia
Enlargement of breast tissue in the male.
Hereditary disease
A disease which is caused by alteration of the genetic material
Hormone
A chemical messenger, which is released at some parts of the body which reaches another part of the body to have its effects
Hydrocortisone
A drug which replaces the natural hormone cortisol
Hyperprolactinaemia
Overproduction of the hormone prolactin
Hypogonadism
Deficiency of sex hormone secretion. This can either be primary, due to a disorder of the testes or ovaries, or secondary when due to disease of the pituitary or hypothalamus.
Hyponatraemia
This occurs when the blood becomes too dilute. It can lead to headaches and feeling generally unwell. With DI it is likely to mean your desmopressin dose is not correct. You should see your doctor if this occurs
Hypophysis
An alternative name for the pituitary gland.
Hypophysitis
A condition resulting in inflammation of the pituitary gland (hypophysis). Variations include Granulomatous Hypophysitis, Lymphocytic Hypophysitis and Tuberculosis Hypophysitis
Hypothalamus
The part of the brain which controls the pituitary gland
Hypothyroidism
Underactivity of the thyroid gland. This condition is sometimes caused by a pituitary problem
IGF-1
Insulin-like Growth Factor-1
Infundibulum
An alternative name for the pituitary stalk, which connects the pituitary gland and the hypothalamus
Insulin
Hormone of the pancreas which reduces blood sugar levels via increasing the uptake of sugar by the cells
Insulin-like Growth Factor-1
The levels of IGF-I in the blood are controlled by GH, and IGF-1 can be used to measure how active your acromegaly is. Abbreviation: IGF-1
Intramuscular injection
An injection deep into the muscle, as opposed to under the skin
Lanreotide
A somatostatin analogue used to reduce levels of growth hormone
Lanreotide
A somatostatin analogue drug
Leydig cells
Cells in the testicles that produce most of a man's testosterone
LH
Luteinising hormone
Luteinising hormone
This sex hormone contributes to sexual development of children. In women, together with FSH, it controls ovulation and is thus essential for a normal menstrual cycle and for fertility. In men, with FSH, it stimulates the testes to produce sperm
Macroprolactinoma
A prolactin-secreting pituitary tumour which is more than 10mm in diameter
MEN1
Multiple Endocrine Neoplasia Type 1, this is a rare inherited condition in which affected individuals tend to develop tumours of the pituitary gland, parathyroid glands and the pancreas
Multiple Endocrine Neoplasia Type 1
This is a rare inherited condition in which affected individuals tend to develop tumours of the pituitary gland, parathyroid glands and the pancreas. Abbreviation: MEN1
Neurohypophysis
An alternative name for the posterior pituitary gland
Non-functioning tumour
A tumour which does not produce any of the pituitary hormones
Octreotide
A somatostatin analogue used to reduce levels of growth hormone
Oestrogen
Female sex hormone, which is produced by the ovaries.
Oligomenorrhoea
Abnormally infrequent menstrual periods. Often the interval between periods exceeds 40 days
Optic Nerve Hypoplasia
Rare congenital underdevelopment of the midline of the brain affecting sight, and sometimes producing hypopituitarism, diabetes insipidus, and possible failure of the adrenal and thyroid glands
Parlodel
The brand name of bromocriptine
Penetrance
Penetrance of a disease-causing mutation is the proportion of individuals with the mutation who exhibit clinical symptoms. If the penetrance is full (100%) all subjects with the mutation will develop the disease at some point in their life. If the penetrance is incomplete then some mutation-carrier subjects can live a long life with no development of the disease
Pegvisomant
A novel drug for the treatment of acromegaly. It inhibits the action of growth hormone, it is a growth hormone receptor antagonist. It has been successfully used in patients who do not respond well to somatostatin analogue therapy. It is usually injected once a day or once or twice in a week.
Pituitary adenoma predisposition
Persons who carry a mutation in the AIP gene have pituitary adenoma predisposition (PAP). This term was first mentioned in the seminal Science paper of Vierimaa and colleagues in 2006.
Pituitary fossa
The bony pocket in the skull where the pituitary gland is located
Pituitary gland
A gland, as small as a pea, located at the base of the brain. It produces hormones, which in turn control the hormone production of many other glands in the body. It has an anterior and posterior part
Pituitary tumour
A tumour, almost always non-cancerous, of the pituitary gland.
Progesterone
Female sex hormone, which is produced by the ovaries.
Prolactin
A hormone often called the 'milk hormone' because its main function is to stimulate the breasts after childbirth. However, men also have prolactin, although the function of this hormone in males is not clear
Prolactinoma
A pituitary tumour which produces prolactin. This can be a microprolactinoma or macroprolactinoma
Radiotherapy
Treatment with X-rays
Recombinant human GH
Synthetically produced growth hormone, which has the same structure as natural human growth hormone.
Sandostatin
The brand name of octreotide
Septo-Optic Dysplasia
Rare congenital underdevelopment of the midline of the brain affecting sight, and sometimes producing hypopituitarism, diabetes insipidus, and possible failure of the adrenal and thyroid glands
Sex hormones
Luteinising hormone (LH) and follicle-stimulating hormone (FSH) are produced by the pituitary gland and stimulate the ovaries to produce oestrogen (in women) and testosterone (in men)
Short stature
Often due to underproduction of growth hormone.
Sleep apnoea
A condition in which you stop breathing for a few moments while asleep. This causes poor quality sleep such that you wake in the morning with a headache and still feeling tired, and can result in you falling asleep during the day. The partners of sufferers typically complain they are bad snorers. Sleep apnoea is a common disorder in patients with acromegaly
Somatostatin
A naturally occurring hormone that reduces GH release from the pituitary gland. Man-made modified versions of somatostatin are known as somatostatin analogues
Somatotrophin
Growth hormone
Somatotrophinoma
A pituitary tumour secreting growth hormone.
Somatuline LA
The brand name of lanreotide
Subcutaneous injection
An injection under the skin, as opposed to deeper into the muscle
Tall stature
A term to use for subjects who are taller than the average for their age and sex in a given ethnic background. It can be a normal variant but it can be caused by a disease such as acromegaly, Marfan syndrome or hypogonadism as well as many others
Testosterone
The main sex hormone in men, produced by the testes.
Thyroid stimulating hormone
A hormone which your pituitary sends to your thyroid gland to stimulate the production of thyroxine. Abbreviation: TSH
Thyrotrophin
Thyroid stimulating hormone, TSH
Thyroxine
A hormone produced by the thyroid gland
Transsphenoidal surgery
A method of operating on the pituitary gland by making an incision in front of the upper teeth and behind the upper lip, or sometimes through the nose
TSH
Thyroid stimulating hormone
TSHoma
A pituitary tumour secreting TSH
Vasointestinal peptide
A hormone secreted by the gut or pancreas. It increases gut motility and secretion of water into the gut. A tumour secreting VIP is called a VIPoma
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