Acromegaly Vs Dwarfism

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ACROMEGALY: Definition Symptoms usually develop very slowly over

time.
Acromegaly, a disorder caused by an excess of
GH in adults, results in enlargement of Acromegaly can cause serious complications
peripheral body parts and soft tissue, after the and premature death if not treated.
fusion of the epiphyseal plates has occurred
(Hickey & Silverstein, 2019), without an In children, excess GH production causes
increase in height with an incidence of between elongation of the bones and associated soft
0.2 and 1.1 cases per 100,000 people tissue swelling. If not treated, children with this
(Lavrentaki, Paluzzi, Wass, et al., 2017). disorder can grow ta height of 7-8 feet.
Although rare, oversecretion of GH in children
before the fusion of epiphyseal growth plates
result in pituitary gigantism; a person may grow Symptoms and complications in adults may
to be 7 or even 8 feet tall. include the following:

Acromegaly is a rare disorder of excessive bone • Abnormally large growth and deformity
and soft tissue growth due to elevated levels of of the:
growth hormone. In young children, prior the
completed fusion and growth of bones, Hands (rings nlonger fit)
excessive growth hormone can cause a similar Feet (need a bigger size shoe)
condition called gigantism. Face (protrusion of brow and lower jaw)
Jaw (teeth dnot line up correctly when
the mouth is closed)
Causes Lips
Tongue
In most cases, acromegaly is caused by over- • Carpal tunnel syndrome
secretion of growth hormone (GH) produced • Skin changes, such as:
by a benign tumor of the pituitary gland. Thickened, oily, and sometimes
darkened skin
In a small number of cases, malignant tumors of Severe acne
other organs (pancreas, adrenal, lung) may be Excessive sweating and offensive body
the source of excess GH. order due tenlargement of the sweat
glands
• Deepening voice due to enlarged
Risk Factors sinuses, vocal cords, and soft tissues of
the throat
• Hereditary • Fatigue and weakness in legs and arms
• Average Age of Diagnosis: 40 – 45 • Sleep apnea
years old. • Arthritis and other joint problems
especially in the jaw
Symptoms • Hypothyroidism
• Enlargement of the liver, kidneys,
spleen, heart, and/or other internal
organs, which can lead to:
Diabetes
High blood pressure
Cardiovascular disease
In women: ACROMEGALY SYMPTOMS

• Irregular menstrual cycles A – Arthralgia / Arthritis


• Galactorrhea (abnormal production of B – Blood Pressure Raised
breast milk) in 50% of cases C – Carpal Tunnel Syndrome
D – Diabetes
In men: E – Enlarged Organs
• In about 50% of cases, impotence F – Field Defect (Visual)

Diagnosis Nursing Management

o Blood tests to measure the level of PREOPERATIVE. At the time of diagnosis, the
insulin-like growth factor (IGF-I), patient requires education and emotional
GRHR, and other pituitary hormones. support. Focus education on the cause of the
disease, the prescribed medical regimen, and
A glucose tolerance test may also be given to see preparation for surgery. Encourage the patient to
if the GH level drops—it will not drop in cases interact with family and significant others.
of acromegaly. Reassure the patient that treatment reverses
some of the physical deformities. If you note
If these tests confirm acromegaly, the following disabling behavior, refer the patient to
may be done to locate the tumor that is causing psychiatric resources.
the disorder:
Prepare the patient and family for surgery.
o Head CT Scan Explain the preoperative diagnostic tests and
o MRI Scan examinations. For a patient who is undergoing a
transsphenoidal hypophysectomy or a
transfrontal craniotomy, explain the
Treatment postoperative need for nasal packing and a
mustache dressing.
Reduce production of GH to normal levels.
Stop and reverse the symptoms caused by over- POSTOPERATIVE. Elevate the patient’s head
secretion of GH. to facilitate breathing and fluid drainage. Do not
Correct other endocrine abnormalities (thyroid, encourage the patient to cough, as this interferes
adrenal, sex organs). with the healing of the operative site. Provide
Reduce the tumor size. frequent mouth care, and keep the skin dry. To
promote maximum joint mobility, perform or
• Surgery assist with range-of-motion exercises.
• Radiosurgery Encourage the patient to ambulate within 1 to 2
• Radiation Therapy days of the surgery. To assure healing of the
incision site, explain the need to avoid activities
Medications that increase intracranial pressure, such as
toothbrushing, coughing, sneezing, nose
• Cabergoline (Dostinex) – given orally blowing, and bending.
• Pergolide (Permax) – given orally
• Bromocriptine (Parlodel) – may be
given before surgery to shrink tumor
• Octreotide (Sandostatin) – injection
• Pegvisomant – injection for px not
responding to other forms of treatment
Documentation Guidelines DWARFISM: Definition

o Physical findings: Respiratory rate and Conversely, insufficient secretion of GH during


pattern; nasal drainage: color, amount, childhood can result in generalized limited
and presence of glucose. growth and pituitary dwarfism.

o Neurological status: Level of As a result of a medical or genetic condition, it


consciousness, motor strength, is described by the advocacy group Little People
sensation, and vision. of America (LPA) as adult height of 4 feet 10
inches or below.
o Presence of postoperative
complications: Diabetes insipidus, The average height of an adult with dwarfism is
hypopituitarism, meningitis. 4 feet, despite the fact that other groups may
raise the standard for some forms of dwarfism to
o Psychosocial assessment: Self-esteem, 5 feet. The majority of illnesses connected to
coping, interpersonal relationships, and dwarfism are genetic, while other ailments have
sexual dysfunction unidentified origins.

Discharge and Home Healthcare Guidelines • Turner Syndrome is a condition that


only affects girls and women and is
Refer patients with advanced acromegaly who caused when a sex chromosome is
experience arthritic changes and require assist missing or partially absent.
devices for ambulation and activities of daily
living to a physical therapist. • Achondroplasia is a condition when a
person has one mutant copy of the gene
ACTIVITY RESTRICTIONS. Instruct the associated with the disorder and one
patient to avoid activities that increase normal copy of the gene.
intracranial pressure for up to 2 months after
surgery. Toothbrushing can be resumed in 2 Dwarfism can be divided into two primary
weeks. Instruct the patient to report increased categories: proportional and disproportionate.
nasal drainage. Incisional numbness and altered
olfaction may occur for 4 months after surgery. Disproportionate dwarfism can either have a
truncated trunk with longer limbs or a
MEDICATIONS. If octreotide is prescribed, the normal-sized torso with shorter arms and
patient will need to be able to demonstrate how legs. The physical parts are in proportion but
to administer a subcutaneous or intramuscular shorter in proportionate dwarfism.
injection.

FOLLOW-UP. Patients need to be monitored for Causes


development of cardiac disease, diabetes
mellitus, and gallstones and a recurrence of • Metabolic or hormonal disorders in
symptoms. Advise the patient to wear a medical infancy or childhood.
identification bracelet. • Chromosomal abnormalities
• Pituitary Gland Disorders-which
influence growth and metabolism
• Absorptive problems when the body
can't absorb nutrients adequately
• Kidney disease
• Achondroplasia
• Turner syndrome
• Genetic Treatment

Dwarfism treatments don't increase stature but


Signs and Symptoms may alleviate problems caused by complications.

o Late development of certain motor Surgical procedures that may correct problems
skills, such as sitting up or walking with bones in people with disproportionate
o Increased susceptibility to ear infections dwarfism.
o Breathing problems
o Weight problems Some people with dwarfism elect to undergo
o A curvature of the spine limb-lengthening surgery.
o Bowed legs
o Joint stiffness and arthritis Growth hormone deficiency is treated with
o lower back pain or numbness in the legs injections of a synthetic version of the hormone.
o Crowding of teeth

Nursing Management
Tests and Diagnosis
Nurses should be familiar with a child's typical
• Measurement of height, weight, and growth and development as well as the need to
head circumference. regularly check a child's weight and height and
• Many distinct facial and skeletal features keep accurate records.
are associated with each of several
dwarfism disorders. Help with making a diagnosis.
• MRI Scan
• Genetic tests Support the parent and child emotionally.
• Family history
Give health instruction.
• Hormone tests
Give them the chance to voice their thoughts on
Prevention
sexual function and body appearance.
If genetically caused, it is impossible to prevent
Furniture and other necessities, such as beds,
but genetic testing can be performed to screen
chairs, and dresses, should be made to fit
for specific variations that result in the
children's sizes for comfort.
condition.

Appropriate diet or hormonal therapy can treat


dwarfism resulting from malnutrition or a
hormonal abnormality. GH deficiency can be
remedied by injection of HGH in early life.

Complications

A – Arthropathy
C – Cataracts
R – Raised intraocular pressure
D – Disproportionate short stature
R – Retinal Detachment

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