AcromegalyAcromegaly is caused when a tumor on the pituitary gland produces too much
growth hormone (GH). These tumors are almost always benign (i.e. not cancerous)
and therefore do not spread to other areas of the body.
Acromegaly is a very rare condition and usually develops between the ages of 30
and 50. If the condition develops before a person has stopped growing (which
usually occurs between the ages of 15 to 17 years of age), it causes gigantism
because growth hormone promotes growth of bones in the body.
Typical symptoms
- coarsening of facial features
- enlarged hands and feet
- thickening of the soft tissue in the palms and soles of
the feet
- carpal tunnel syndrome (tingling feeling or pains in the hands)
- excessive sweating and oily skin
- headaches
- vision disturbances
- sleep apnea
- general tiredness
- irregular periods (oligomenorrhoea) or loss of normal menstrual function (amenorrhoea)
- adult females
- impotence - adult males
- reduced fertility
- decrease in sex drive
All these symptoms tend to develop gradually and the changes
may not be noticed for some time.
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Adult Growth Hormone Deficiency (AGHD)
Growth Hormone (GH) is a protein made in the pituitary gland and passed from
there into the blood stream. GH has effects on virtually all the organs of the
body, but its primary use during childhood is making children grow.
GH deficiency is usually caused by damage to the pituitary gland or the part of
the brain which controls this gland (the hypothalamus). The damage may be due to
a tumor or to the effects of treatment for the tumor (surgery or radiotherapy)
or to problems with the blood supply to the pituitary gland.
Typical symptoms
- increase in adipose (fatty) tissue (especially around the waist)
- decrease in lean body mass (muscle)
- decrease in strength and stamina, reduction in exercise capacity
- decrease in bone density, increase in rate of fracture in middle age and beyond
- changes in blood cholesterol concentrations (increase in LDL and decrease in HDL)
- excessive tiredness
- anxiety and depression
- feelings of social isolation
- reduction in 'quality of life'
- increased sensitivity to cold or heat
Dwarfism
There are many causes of short stature or dwarfism other than deficient
growth hormone secretion; for example, chromosomal abnormalities,
malnutrition (including poorly controlled diabetes mellitus), thyroid
deficiency, and disorders of bone formation are all examples of dwarfism
with normal GH secretion. Nonetheless, growth hormone deficiency is a fairly
common cause of short stature. Perhaps most frequent is GH deficiency
resulting from damage to the hypothalamus and pituitary during fetal
development or at birth because of trauma, lack of oxygen, or any of a
number of other causes. When damage to the hypothalamus or pituitary is
mild, growth hormone deficiency may be the only detectable manifestation of
a disease state because the somatotrophs are the most sensitive of the
pituitary cells to injury. When all of the cells of the pituitary are
severely damaged or destroyed the patient is said to have panhypopituitarism
(leading to diminished function of the gonads, the thyroid, and the adrenal
glands).
Midgets usually suffer from one of two forms of hereditary (familial)
isolated growth hormone deficiency. In some families the deficiency is the
result of underproduction of GHRH, in which case growth hormone secretion
may be stimulated by infusion of GHRH. In others, the problem lies in the
somatotrophs themselves when they become incapable of manufacturing growth
hormone. Growth hormone levels also tend to fall in some aged persons who
otherwise appear to be normal.
In other forms of dwarfism, the hypothalamus and pituitary function
adequately, and the abnormality lies rather in the lack of response of body
tissues. A well-studied example is that of the Laron dwarf. These children
suffer from a hereditary disorder characterized by the inability of growth
hormone to bind to specific receptors in the body's tissues; circulating GH
levels are elevated but somatomedin levels remain low because GH, unable to
bind to receptors, cannot stimulate somatomedin secretion. Another example
is the African Pygmy, in whom there is a resistance to the administration of
GH. This is caused by an unresponsiveness to somatomedin, which suggests
that there is a defect in the somatomedin receptors.
Growth hormone alone cannot generate growth without an adequate supply of
food, so that in states of malnutrition dwarfism occurs in the face of a
mild elevation in growth hormone concentrations in the blood.
Finally, an example of the effect of emotional and environmental factors on
growth is found in the condition known as psychosocial dwarfism. Such
children suffer emotional deprivation from uncaring or abusive parents.
Growth hormone levels are low but return to normal along with an increased
rate of growth when the children are removed to a more supportive
environment, only to have the cycle repeated when the child is returned to
the custody of the parents. These victims tend to be withdrawn and
apathetic. They have disrupted sleep and bizarre eating and drinking habits.
All of these symptoms are dramatically reversed when the child is removed to
compassionate care in a hospital or foster home.
An adult GH-deficient dwarf has the body proportions of a young child.
Radiographs (X-ray pictures) of growing ends of bone also show growth
retardation in relation to the patient's chronological age. These changes
are not apparent at birth but appear some time within the first two years of
life. Puberty is often delayed, but untreated individuals may be fertile and
give birth to normal children. When it appears in adults, GH deficiency
produces only subtle changes, with minor decreases in strength and in the
density of bones.
Growth hormone-deficient dwarfs respond dramatically to injections of human
growth hormone. Supplies of GH were greatly limited in the past because the
only source was GH extracted from human pituitary glands obtained at
autopsies. With the availability of human GH manufactured by recombinant DNA
technology using bacteria, the supply is potentially unlimited. Most treated
patients achieve normal height, but in some, particularly those with the
hereditary inability to synthesize growth hormone, antibodies to the
injected growth hormone may block the therapeutic action. There is evidence
that children with “constitutional short stature,” that is, children from
otherwise normal families in whom short stature is the rule in the absence
of disease, may also respond to GH treatment.
Excess levels of growth hormone are most often caused by a benign tumor
(adenoma) of somatotrophs of the pituitary gland. Rarely, a tumor of the
lung or the pancreatic islets produces GHRH, which stimulates normal
pituitary somatotrophs to excess secretion when released into the
circulation. Even more rarely is there excessive, ectopic production of GH
by tumor cells that do not ordinarily synthesize GH. If hypersecretion of
growth hormone occurs during childhood, growth progresses at an inordinately
rapid rate to extremes, 8 feet, 11 inches in the case of the “Alton Giant.”
Giantism is rare because such individuals usually have all of the
infirmities described below for acromegaly.
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Craniopharyngioma
Craniopharyngiomas are very rare benign
(non-cancerous) tumors, with 50% occurring in children under 16 years, and
the remainder at any time in adult life.
The tumors can be solid, cystic (full of fluid), calcified, or full of
debris. They are slow-growing tumors that can take 2-3 years (or longer) to
manifest themselves before a diagnosis is made.
Typical symptoms
- headaches (sometimes accompanied by nausea or vomiting)
- diabetes insipidus
- disturbed sleep patterns
- vision disturbance
- behavioural changes, including introversion and inability to
concentrate
- slow growth
- increased sensitivity to cold or heat
- early or delayed puberty - children
- irregular periods or loss of normal menstrual function
- (amenorrhoea) - adult females
- impotence - adult males
- reduced fertility - adults
- decrease in sex drive - adults
- tiredness and susceptibility to infections
- appetite and weight variations
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Cushing's syndrome describes the condition resulting from too much exposure to
steroid hormones.
The commonest cause of Cushing's syndrome (apart from the use of synthetic
steroids to treat other conditions) is Cushing's disease. This is a problem
arising in the pituitary gland caused by a tumor which overproduces a hormone
called ACTH. This in turn stimulates the adrenal glands to overproduce the
steroid hormone cortisol. Cushing's syndrome can also be caused by a small
growth in one, or both, of the adrenal glands.
Cushing's is rare and is more often found in women than in men. It can affect
all age groups, but the peak incidence is in middle age.
Typical symptoms
- behavioral changes, depression and mood swings, occasionally psychological
problems can be severe
- face tends to be rounder (moon face) and redder
- weight gain around the trunk (central obesity)
- muscle wasting and proximal myopathy (patients have difficulty standing from a
seated position without use of arms)
- tendency to bruise easily
- appearance of red 'stretch marks' on the abdomen, similar to those which occur
during pregnancy
- irregular periods (oligomenorrhoea) or loss of normal menstrual function
- (amenorrhoea)
- females
- impotence - males
- reduced fertility
- decrease in sex drive
- increase in hair growth on the face and body (hirsutism)
- increase in blood pressure
- development of mild diabetes mellitus
Because Cushing's progresses slowly and gradually in most cases, it can go
unrecognized for some time.
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Diabetes Insipidus
Diabetes Insipidus (DI) is a disorder in which the kidneys are unable to retain
water. This results in the production of large amounts of urine which in turn
makes you feel dry and very thirsty.
Neurogenic Diabetes Insipidus
This condition is caused by the lack of a water-retaining hormone or chemical in
the blood (called vasopressin or ADH). Neurogenic DI is sometimes referred to as
Cranial, Central or Pituitary DI.
Nephrogenic Diabetes Insipidus
This condition is caused by an abnormality in the kidneys which prevents the
kidneys from responding to the water-retaining hormone.
Diabetes insipidus is not related to the type of diabetes most people have heard
of, diabetes mellitus.
Typical symptoms
- frequent need to pass urine (polyuria) even during the night
- extreme thirst
leading to excessive drinking (polydipsia)
- imbalance of electrolytes (relatively high levels of sodium and potassium)
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Hypopituitarism
The pituitary gland produces a number of hormones or chemicals which are
released into the blood to control other glands in the body. If the pituitary is
not producing one or more of these hormones, or not producing enough, then this
condition is known as hypopituitarism. The term Multiple Pituitary Hormone
Deficiency (MPHD) is sometimes used to describe the condition when the pituitary
is not producing two or more of these hormones. If all the hormones produced by
the pituitary are affected this condition is known as panhypopituitarism.
Hypopituitarism is most often caused by a benign (i.e. not cancerous) tumor of
the pituitary gland, or of the brain in the region of the hypothalamus.
Pituitary underactivity may be caused by the direct pressure of the tumor mass
on the normal pituitary or by the effects of surgery or radiotherapy used to
treat the tumor. Less frequently, hypopituitarism can be caused by infections
(such as meningitus) in or around the brain or by severe blood loss, by head
injury, or by various rare diseases such as sarcoidosis (an illness which
resembles tuberculosis).
More information about conditions which result in hypopituitarism can be found
in the Rarer Disorders section.
Typical symptoms
- excessive tiredness and decreased energy
- muscle weakness
- reduced body hair
- irregular periods (oligomenorrhoea) or loss of normal menstrual function
- (amenorrhoea)
- females
- impotence - males
- reduced fertility
- decrease in sex drive
- weight gain
- increased sensitivity to cold
- constipation
- dry skin
- pale appearance
- low blood pressure and dizziness on standing (postural hypotension)
- headaches
- vision disturbances
- diabetes insipidus
Each of the symptoms described above occur in response to the loss of one or
more of the hormones produced by the pituitary. Decrease in the production of
only one hormone would not lead to all the symptoms described above.
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Non-Functioning Tumor
By far the most common type of tumor of the pituitary gland (about half of all
cases) is the 'non-functioning' tumor. This is a tumor which does not produce
any hormones itself. It can cause headaches and visual problems or it can press
on the pituitary gland, causing it to stop producing the required amount of one
or more of the pituitary hormones, leading to hypopituitarism.
Typical symptoms
- excessive tiredness and decreased energy
- muscle weakness
- reduced body hair
- irregular periods (oligomenorrhoea) or loss of normal menstrual function (amenorrhoea) - females
- impotence - males
- reduced fertility
- decrease in sex drive
- weight gain
- increased sensitivity to cold
- constipation
- dry skin
- pale appearance
- low blood pressure and dizziness on standing (postural hypotension)
- headaches
- vision disturbances
- diabetes insipidus
Each of the symptoms described above occur in response to the loss of one or
more of the hormones produced by the pituitary. Decrease in the production of
only one hormone would not lead to all the symptoms described above.
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Prolactinoma
A prolactinoma is a prolactin-producing tumor of the pituitary gland. These
tumors come in various sizes, but the vast majority are less than 10mm (˝ inch)
in diameter. These are called microprolactinomas. The rarer large tumors are
called macroprolactinomas. Prolactinomas can occur in both men and women.
Very few patients with prolactinomas require surgery, as most prolactinomas
(particularly microprolactinomas) shrink in size following treatment with
medication.
Typical Symptoms
- discharge from breasts (galactorrhoea) - females
- irregular periods (oligomenorrhoea) or loss of normal menstrual function
- (amenorrhoea) - adult females
- impotence - adult males
- reduced fertility
- decrease in sex drive
- headaches
- vision disturbance
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Rarer Pituitary Disorders
Empty Sella Syndrome
This condition occurs when pituitary tissue is destroyed without undergoing
pituitary surgery or radiotherapy, but there is no evidence of a pituitary
tumour. The initial cause of empty sella syndrome may not be clear and may have
occurred much earlier.
Familial
Multiple Endocrine Neoplasia Type 1 (FMEN1)
Familial multiple endocrine neoplasia type 1 is an inherited disorder affecting
the endocrine glands. The disorder affects both males and females equally. FMEN1
is also sometimes called Wermer Syndrome.
People with FMEN1 carry a gene which makes them prone to the development of
pituitary tumors, parathyroid disease, tumors in the pancreas and occasionally
tumors in the other endocrine glands. The parathyroids are the glands most often
affected by FMEN1 and the pituitary becomes overactive in about 1 in 6 persons.
The cause of pitutary overactivity is usually a benign tumour called a
prolactinoma.
Kallmann's Syndrome
Kallmann's syndrome is a form of hypogonadotrophic hypogonadism characterised by
an absence of GnRH, a hormone naturally released by the hypothalamus. Sufferers
fail to go through puberty unless they are given sex hormone replacement therapy
and in addition usually have no sense of smell (anosmia).
Pituitary Infarction
If the blood supply to the pituitary is restricted, the gland tissue can die
resulting in hypopituitarism. Infarctions can occur as a result of impaired
blood flow to the pituitary gland or head trauma.
Rathke's Cleft Cysts
Rathke's cleft cysts are not tumors, but instead are classified as developmental
abnormalities. Like craniopharyngiomas, these cysts form during early
development of the foetus from a structure known as Rathke's pouch. Small
Rathke's cleft cysts are not uncommon and do not usually cause any symptoms.
Problems can occur if these cysts enlarge and interfere with pituitary
production or exert pressure on the optic chiasm.
Septo-Optic Dysplasia (SOD)
Septo-optic dysplasia is a rare condition affecting both children and adults
which consists of three main abnormalities. These are as follows:
Abnormal eye development
Occasionally one or both eyes may be abnormal or the nerves connecting the eyes
to the brain may be abnormal.
Abnormal development of the front part of the brain (forebrain)
Structures called the septum pellucidum and the corpus callosum which divide the
forebrain into two may be absent.
Abnormal pituitary gland development
The pituitary gland produces a number of chemicals called hormones controlling
growth and development in children. Additionally, the posterior part of the
pituitary produces a hormone called vasopressin which makes sure that fluid is
retained in the body and patients do not become dehydrated. The pituitary gland
is very much a master gland which controls other glands in the body such as the
adrenal and thyroid gland.
Adults and children with SOD may have any of these features. Most have two out
of the three cardinal features. Approximately one third will have all three
problems.
Sheehan's Syndrome
The pituitary gland doubles in size during normal pregnancy. Under these
circumstances a sudden drop in blood pressure can result in pituitary infarction
leading to pan-hypopituitarism.
Wolfram Syndrome
Consists of diabetes insipidus, diabetes mellitus, optic atrophy and deafness.
Also known by the acronym DIDMOAD. It is usually a familial disorder.
The following reflexes are used to test the pituitary: It is
important to note that it takes a skilled practitioner to find the root cause of the
person's problem when dealing with endocrine problems.
These reflexes should be tested and treated with the proper supplementation.

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