endocrinology

1. An exposure to panhypo-
putuitarism, gigantism,
and acromegaly
O G B O N N A Y A A K P A R A
E n d o c r i n o l o g y
M e d g a r E v e r s C o l l e g e
S p r i n g 2 0 1 2
5 / 7 / 2 0 1 2

2. PanhyPoPituitarism
Panhypopituitarism is a condition of inadequate production of the anterior pituitary
hormones which result in destruction or loss of function of all or most of the anterior pituitary
gland. It is sometimes referred as hypopituitarism or pituitary dwarfism. It can be either
congenital, which occurs as birth or develops over time. The anterior pituitary secretes the
following hormones: ACTH, GH, PH, LH, FSH, and TSH. The hormones of the anterior
pituitary gland are regulated by the hypothalamus.
Panhypopituitarism is a mega or multi-endocrine disease because it is affected the
secretion of many hormones secreted by the anterior pituitary gland. Panhypopituitarism can
cause by Neoplastic (Pituitary adenoma & Peripituitary tumours), Vascular(infraction),
Anterior
Pituitary
Gland
Lack of Prolactin: Failure of
lactation
Damage!! I cannot
secrete any
hormones

3. Inflammatory/Infiltrative (sarcoidosis), Infection(Tuberculosis, syphilis, mycoses), or damages
to the anterior pituitary gland such as radiation, postpartum hemorrhage, trauma, stroke, birth
defects, and other genetic factors where rare forms of panhypoputuitarism can inherit as
autosomal recessive. For example, children have a high risk inherited the disease if their parents
have the disease. Children tend to show the following symptoms: delayed sexual development
due to deficiency of Gonadotropins, short stature, and Growth retardation. Both adult males and
females show impaired fertility and loss of libido,
Some of the diagnosis methods are: blood tests to measure pituitary and target gland
hormone levels; stimulation tests to test the maximum capacity of the pituitary gland; and semen
analysis in males suspected of infertility. The different treatment methods are: hormone
replacement therapy, tumor removal, and radiation therapy. There is no prevention for
panhypoputuitarism.
. GiGantism
Gigantism is the disease of pre-puberty. It is characterized by extreme physical size and
stature. It is due to over-production of grow hormone (GH) during infancy, childhood or
adolescence. In a more precise and mechanical term, it occurs while epiphyseal growth plates
remain open as shown in figure 2

4. It is reported that there is about 100 cases worldwide. Individual that suffer from gigantism
tend to between 7 to 9 feet long. Gigantism is very common in basketball players. In some cases,
there will die during their 20s. Some causes of gigantism are: tumor of the pituitary gland,
carney complex, McCune-Albright syndrome, and multiple endocrine neoplasia type 1. The
followings are symptoms of the disease: retarded puberty, difficulties in peripheral vision, double
vision, big hands and feet, and excessive sweating.
Disorder
Frequency
of
Gigantism
Age of
Onset of
gigantism
Pituitary
Morphology
Screening
recommendations
Neurofibromatosis
-1
Extremely
rare
6 months on
Optic pathway
tumor with
normal to small
pituitary
Not routine
McCune-Albright
Syndrome
15-20%
Early
childhood on
Pituitary
adenomas or
diffuse pituitary
hyperplasia or no
visible
abnormality
Annually
Multiple Endocrine
Neoplasia Type 1
10-60%
10% by age
40 but has
occurred as
early as age
5
Pituitary
adenoma
Annually beginning at
age 5
Carney Complex 10% Usually 3rd
& Pituitary Annually beginning
Once close,
there is no
more growth
in length

5. 4th
decade
adenoma or
pituitary
hyperplasia
postpubertally
Isolated Familial
Somatotropinomas
100%
Before
3rd
decade
and as early
as age 5
Pituitary
adenoma
As clinically indicated
in unaffected family
members
Table 1.1 This table shown different disorders that affiliated to gigantism.
There are different methods of treatments for gigantism. Surgery aims at removing the
tumor and reducing release or growth hormones. The objective of medical therapy is to restore
GH secretory patterns to normal, prevent recurrence of gigantism, and retain normal pituitary
secretion of other hormones. Medication is among the treatment of gigantism. Dopamine
agonists bind to pituitary dopamine type 2 receptors and suppress GH secretion. Finally,
radiation therapy can also help regulate growth hormone level. However, it causes side effects
such as obesity, emotional alterations, and causes learning disabilities in children.
AcromegAly
Acromegaly is the disease of post-puberty. It affects about 40-60 individuals out of every
one million people in the United States It is occurred as a result of the pituitary gland produces
too much growth hormone after the epiphyseal growth plates of the long bones have fully
stopped growing. The most distinct feature of this disease is enlarged hands and feet. It is
occurred mainly in adulthood. It causes by both pituitary and nonpituitary tumors. The main
cause of acromegaly is benign (noncancerous) tumor of the pituitary gland called a pituitary
adenoma. As the pituitary adenoma grows, growth hormones are produced in excess which cause
bones to grow larger than normal.

6. Some symptoms of acromegaly are: Body odor,enlargement of the lips nose and tongue,
Carpal tunnel syndrome, Decreased muscle strength, large facial bone, large hands and feet,
widely spaced teeth, colonic polyps, hypopituitarism, sleep apnea, uterine fibroids, and excess
hair growth in females, deep voice due to enlarge sinuses and vocal cords, and EKG may show
an enlarged heart. Acromegaly is very difficult to diagnose in middle aged people. Among the
diagnosis methods are; checking growth hormone and insulin-like growth factor 1 levels, spine
x-ray to detect abnormal bone growth, and pituitary MRI to detect a pituitary tumor.
Individuals with acromegaly are more likely to die due to cardiovascular, respiratory,
and brain damage complications caused by the effects of the tumor in the brain or enlargement of
the heart and liver. It is also reported that untreated acromegaly individuals can develop cancer in
the large intestine, which eventually leads to death.
The main objective for the treated acromegaly is to reduce the production of growth
hormone. These are some possibly methods that can be used by physicians; blood test for GH
level, medications, radiation and medical therapies, Medications such as Somatostatin analogues,
which act as a growth hormone releasing inhibiting hormone, in addition, Pegvisomant also
blocks the growth hormone. Radiation therapies methods can be both conventional and
stereotactic radiosurgery. Conventional radiation therapy is given every weekday for four to six
weeks. Stereotactic radiosurgery uses an intense beam which is aimed at the tumor from multiple
directions in order to stink it. There is not a single method to treat acromegaly. In other words,
medical providers often use a combination of treatment to treat patients.