Kreatisme, Gigantisme, Akromegali

Linlin Lindayani, PhD

• Kel. thyroid mensekresi :
• h. tiroksin
• triiodotironin
• calsitonin
• Kel. thyroid terdiri dari folikel-2 yg dipenuhi oleh bahan
sekretorik (koloid) dg unsur utama glikoprotein tiroglobulin, dan
sel epitel kuboid
• Tiroglobulin terdiri dari 140 aa tiroksin
• Untuk membentuk hormon tiroksin dan triiodotironin diperlukan
iodium
Efek hormon tiroksin/triiodotironin

• ↑ kecepatan metabolisme (kec. pada otak, retina, limpa, testis dan paru) →↑nafsu makan
• pada anak : merangsang pertumbuhan, khususnya tum-bang otak saat janin – awal
kehidupan (2th)
• ↑ metab. kh. lemak
• ↓ cholesterol plasma, fosfolipid, dan triglicerida
• ↑ enzime → kebutuhan vitamin ↑
• ↑ penggunaan o2 dan sisa metabolisme
• ↑ sekresi & motilitas sal. cerna
• ↑ aktivitas saraf otak
Hypothyroid
• Cretinism Congenital iodine deficiency
syndrome
• a condition of severely stunted physical and mental
growth owing to untreated congenital deficiency of
thyroid hormone (congenital hypothyroidism) usually
owing to maternal hypothyroidism.
Etiology
• Genetic causes account for about 15 to 20 percent of cases
of congenital hypothyroidism.
• The cause of the most common type of congenital
hypothyroidism, thyroid dysgenesis, is usually unknown.
• The abnormal or missing gland cannot produce normal
amounts of thyroid hormones
Mechanism
Sign and symptoms:
• Decreased activity.
• Large anterior fontanelle.
• Poor feeding and weight gain.
• Small stature or poor growth.
• Jaundice.
• Decreased stooling or constipation.
• Hypotonia.
• Hoarse cry.
Is cretinism reversible?
• Early diagnosis is imperative.
• Most of the effects of hypothyroidism are easily reversible.
• However, critical development of the nervous system takes
place in the first few months after birth.
• Thyroid hormone deficiency may result in irreversible damage
to the nervous system with marked mental retardation.
Diagnose

• Routine screening of the newborn using a blood-spot

• Guthrie test
• to detect a high TSH level as an indicator of primary
hypothyroidism is efficient and cost-effective
• usually done at 5-7 days
• Cretinism is prevented if T4 is started within the first few
months of life.
Prevention
• About one billion people worldwide risk the
consequences of iodine deficiency.
• All of which can be prevented by adequate maternal
and infant iodine nutrition.
• Iodized salt is usually the preferred prophylactic vehicle,
but iodized vegetable oil, iodized water, and iodine
tablets are also occasionally used
Acromegaly
• Acromegaly is a disorder that results
from excess growth hormone (GH)
after the growth plates have closed.
• The initial symptom is typically
enlargement of the hands and feet.
• There may also be enlargement of the
forehead, jaw, and nose
Complication
• Severe headache
• Arthritis and carpal tunnel syndrome
• Enlarged heart
• Liver fibrosis and bile duct hyperplasia.
• Hypertension
• Diabetes mellitus (excess of GH leads to insulin resistance)
• Heart failure
• Kidney failure
• Colorectal cancer
• Compression of the optic chiasm leading to loss of vision in the outer visual fields
(typically bitemporal hemianopia.)
• Increased palmar sweating and sebum production over the face (seborrhea) are clinical
indicators of active GH-producing pituitary tumors.
Causes
• About 98% of cases of acromegaly are due to the
overproduction of growth hormone by a benign tumor of the
pituitary gland called an adenoma

• Acromegaly is caused not by pituitary tumors, but by tumors of

the pancreas, lungs, and adrenal glands.
 Diagnostic
• Medical imaging and medical
laboratory investigations are
generally used together to
confirm or rule out the presence
of this condition.
• Frequent blood sampling with
serum GH measurement shows
that in normal subjects (left
panel) GH can fluctuate between
undetectable levels (most of the
time) and peaks of up to 30 μg/l
(90 mIU/l)
Treatment
• There is no known cure for acromegaly
Gigantism
• 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

• hereditary pituitary disorder possibly due to the AIP gene,

causing early onset and familial acromegaly or gigantism.
• Gigantism are caused by a typically benign tumor or adenoma
in the pituitary gland.
• Growth hormone (GH) and insulin-like growth factor-I (IGF-I)
are two substances that have been identified as influencing
growth plate formation and bone growth and, therefore,
gigantism
What height is considered gigantism?

• Gigantism, also known as giantism is a condition characterized

by excessive growth and height significantly above average.
• In humans, this condition is caused by over-production of
growth hormone in childhood resulting in people between 7
feet (2.13 m) and 9 feet (2.75 m) in height.
What age does gigantism occur?

• The specific age of onset for gigantism varies between

patients and gender, but the common age that
excessive growth symptoms start to appear has been
found to be around 13 years.
• Other health complications, such as hypertension, may
occur in pediatric patients with hyper-secretion of
growth hormone.
The difference between acromegaly and gigantism

• The definition of acromegaly is a disease caused by noncancerous

tumors in the pituitary gland that cause secretions of growth
hormone.
• The difference between acromegaly and gigantism is
that acromegaly occurs in adults, typically between the ages of 30
and 50. ... Overall, acromegaly is a rare condition.
Diagnostic testing

• A random blood sample showing markedly elevated GH is

adequate for diagnosis of GH hyper-secretion.
• Insulin-like growth factor-1 (IGF-1) is an excellent test for
evaluation of GH hyper-secretion
Gigantism Treatment

• Pegvisomant is one pharmaceutical drug which has received

attention for being a possible treatment route for gigantism.
• Radiation Therapy.
• For some patients, surgery or medication is not sufficient
• recommend stereotactic radiosurgery.
• Stereotactic radiosurgery is a technique in which doctors aim a
highly focused dose of radiation to the tumor.
Nursing care plan
Pengkajian Keperawatan
1. Riwayat kesehatan klien dan keluarga. Sejak kapan klien
menderita penyakit tersebut dan apakah ada anggota
keluarga yang menderita penyakit yang sama.
2. Kebiasaan hidup sehari-hari seperti
a. Pola makan
b. Pola tidur (klien menghabiskan banyak waktu untuk tidur).
c. Pola aktivitas.
3. Tempt tinggal klien sekarang dan pada waktu balita.
4. Keluhan utama klien, mencakup gangguan pada berbagai
sistem tubuh;
a. Sistem pulmonari
b. Sistem pencernaan
c. Sistem kardiovaslkuler
d. Sistem muskuloskeletal
e. Sistem neurologik dan Emosi/psikologis
f. Sistem reproduksi
g. Metabolik
5. Pemeriksaart fisik mencakup
a. Penampilan secara umum
b. Nadi lambat dan suhu tubuh menurun:
c. Perbesaran jantung
d. Disritmia dan hipotensi
e. Parastesia dan reflek tendon menurun
6. Pengkajian psikososial klien
7. Pemeriksaan penunjang mencakup;
• Pemeriksaan kadar T3 dan T4 serum;
• Pemeriksaan TSH (pada klien dengan hipotiroidisme primer
akan terjadi peningkatan TSH serum, sedangkan pada yang
sekunder kadar TSH dapat menurun atau normal).
Diagnosa Keperawatan