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Dwarfism
Dwarfism
Dwarfism
Nursing care
Advice parent for Adequate head and neck support
Promote good posture by providing a pillow for the lower back and a footstool when your child
is sitting.
Encourage participation in appropriate recreational activities, such as swimming or bicycling,
but avoid sports that involve collision or impact, such as football, diving or gymnastics.
FREOHLICHS
Definition- Froehlich syndrome, also known as adiposogenital dystrophy, is a constellation of
endocrine abnormalities believed to result from damage to the hypothalamus, a part of the brain
that links the nervous system to the endocrine system via the pituitary gland.
Etiology- Froehlich syndrome (hypogonadism with obesity) results from an injury of a part of the
hypothalamus (arcuatus nucleus and ventromedial nueclei.The most frequent cause of Froehlich
syndrome is a specific tumor of the pituitary-hypothalamus area, an expanding hollow (cystic)
lesion (craniopharyngioma) or its surgical treatment (injury of this area during surgery).
Incidence- Froehlich syndrome is a very rare condition that affects more males than females.
S and s-
characterized by increased or excessive eating that leads to obesity
small testes, and a delay in the onset of puberty.
experience the delay in physical growth and the development of secondary sexual
characteristics.
tend to be short in stature.
As a result of tumor growth, some children with Froehlich syndrome may also develop
intellectual difficulties, poor vision – due optic nerve damage-, somnolence, and diabetes
insipidus – known as Infundibulo-tuberal syndrome
Complications- Obesity
Delayed physical and sexual growth
In some cases, even the mental growth is affected
Diabetes mellitus (rare)
Treatment- Control of physiological imbalances and symptomatic treatment approach is followed, for an
individual with Froelich’s Syndrome. These treatment measures include:
Hormonal replacement therapy: The absent or decreased hormones are replaced using
pituitary hormone derivatives
Tumors affecting the pituitary and hypothalamus, may be surgically removed
Good diet control and weight loss regimen can aid overweight individuals. Gastric banding or
gastric bypass surgery for weight loss may be considered
Nursing care
restricting diet until normal weight is achieved.
SIMMOND
DEFINITION
Simmonds' disease or pituitary cachexia is a syndrome ascribed to
destruction or physiological exhaustion of the hypophysis (chiefly
the anterior portion). The destruction may be caused by embolic
infarction, tumor, syphilis, tuberculosis, metastatic abscesses,
inflammation, etc.
ETIOLOGY
Blunt force trauma
Disease – tuberculosis; syphilis
Tumors
Aging
Complications in pregnancy
INCIDENCE
Can occur in both sexes but more prevalent in females. The onset
usually occurs in the postpubertal period.
S&S
Lack of gonadotropin (LH and FSH)
delayed puberty
small and soft testes
lack of beard, pubic hair , primary amenorrhoea .
Lack of growth hormone – bone growth retardation, short stature
Lack of prolactin : absence or decrease in breast milk production . •
Lack of TSH- fatigue , cold , dry skin ,
• Lack of adrenocorticotropic hormone (ACTH)- weakness , nausea ,
vomiting , low blood pressure , low blood sugar , coma , pale skin .
• Pituitary crisis – is a potential life threatening medical condition
requiring immediate emergency treatment.
COMPLICATION
The disease results in emaciation and death if left untreated.
DIAGNOSTIC
History and clinical features; biochemical (low levels of T3, T4,
thyroid-stimulating hormone
adrenocorticotropic hormone (ACTH), and gonadotropins);
anemia; low basal metabolic rate; radiology (pituitary tumor
demonstrated by CT scan of brain
TREATMENT
hormone replacement therapy –
Hydrocortisone (cortisol) for adrenal insufficiency
Levothyroxine for hypothyroidism -Testosterone for male ]
hypogonadism -Estradiol for female hypogonadism
NURSING CARE-
MONITOR VITALS SIGNS ASSESS MENTAL
STATUS ,EMOTIONAL STATE ,ENERGY LEVEL AND APPETITE
ADMINISTER HORMONE REPLACEMENTS AS PRESCRIBES
EXPLAIN THE PATIENT : -THE NEED TO TAKE THE
MEDICATION FOR THE REST OF THE PATIENTS LIFE -THE
NEED FOR THE FREQUENT LABORATORY TESTS 19
GIGANTISM
DEFINITION
Gigantism is a rare condition that causes abnormal growth in
children. This change is most notable in terms of height, but girth is
affected as well. It occurs when your child’s pituitary gland makes
too much growth hormone, which is also known as somatotropin.
ETIOLOGY
Gigantism is a serious condition that is nearly always caused by an
adenoma, a tumor of the pituitary gland. Gigantism occurs in
patients who had excessive growth hormone in childhood. The
pituitary tumor cells secrete too much growth hormone (GH), leading
to many changes in the body.
INCIDENCE
Gigantism is extremely rare, with approximately 100 reported cases
to date. Although still rare, acromegaly is more common than
gigantism, with a prevalence of 36-69 cases per million and an
incidence of 3-4 cases per million per year. Gigantism may begin at
any age before epiphyseal fusion
S&S
a large head.
a very noticeable forehead.
a jaw that seems to jut out.
unusual features of the face, such as a broad nose.
very large hands and feet, with thick fingers and toes.
more sweating than usual.
a very large appetite.
general weakness.
COMPLICATION
Long-term complications that some people with gigantism might
experience due to excessive height and the overall effects of excess
growth hormone include: Mobility issues due to muscle
weakness. Osteoarthritis. Peripheral neuropathy.
DIAGNOSTIC
Growth hormone and IGF-1 (insulin-like growth factor 1) blood tests:
These tests measure different hormone levels in your child’s blood
from a sample taken from their vein. Higher than normal levels may
indicate gigantism.
Glucose tolerance test: This test can assess whether your child’s
growth hormone levels react to glucose (sugar) the way they should.
For this test, a provider will draw blood samples from your child’s
vein at different intervals after your child drinks a glucose (sugar)
solution.
Imaging tests: If blood tests confirm that your child has gigantism,
their provider will probably recommend an MRI (magnetic resonance
imaging) scan or CT (computed tomography) scan, which can
clearly show the size and location of a pituitary tumor and help
determine the proper treatments.
TREATMENT
The goals of treating gigantism include:
Safely regulating growth hormone (GH) and insulin-like growth
factor 1 (IGF1) levels.
Managing 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.
NURSING CARE
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ACROM
DEFINITION
a disorder that occurs when your body makes too much growth
hormone (GH). Produced mainly in the pituitary gland, GH controls
the physical growth of the body. In adults, too much of this hormone
causes bones, cartilage, body organs, and other tissues to increase
in size.
ETIOLOGY
develops when the pituitary gland releases too much GH into the
body over a long period of time. When GH enters the blood, this
signals the liver to produce another hormone, called insulin-like
growth factor I (IGF-I). IGF-I is the hormone that actually causes
bones and body tissue to grow.
INCIDENCE
is a rare disorder that affects males and females in equal numbers.
This disorder occurs in approximately 50 to 70 people per million.
Researchers estimate that three to eleven people out of every
million develop the disorder each year.
S&S
tiredness and difficulty sleeping, and sometimes sleep apnoea.
gradual changes in your facial features, such as your brow, lower
jaw and nose getting larger, or your teeth becoming more widely
spaced. numbness and weakness in your hands, caused by a
compressed nerve (carpal tunnel syndrome)
COMPLICATION
High blood pressure (hypertension)
High cholesterol.
Heart problems, particularly enlargement of the heart
(cardiomyopathy)
Osteoarthritis.
Type 2 diabetes.
Enlargement of the thyroid gland (goiter)
Precancerous growths (polyps) on the lining of your colon.
DIAGNOSTIC
IGF test. Levels of GH in the blood can change throughout the
day. ...
Oral glucose tolerance test. To confirm the diagnosis, your doctor
will order an oral glucose tolerance test.
TREATMENT
Reduce production of GH to normal levels
Stop and reverse the symptoms caused by over-secretion of GH
Correct other endocrine abnormalities (thyroid, adrenal, sex organs)
Reduce the tumor size
NURSING CARE
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