SARAH

STEP 1

Shoulder : Bahu

Rise : naik (tumbuh tinggi)

STEP 2

1. What is the bone-building cells?

2. What is hormones cause growth factors ?
3. What is the etiology of the disease?
4. What is the epidemiology of the disease?
5. What is the pathophysiology of the disease?
6. What is the clinical manifestations of the disease
7. What is the diagnosis of the disease?
8. What is the examination of the disease ?
9. What is the complications of the disease ?
10. What is the management of the disease ?

STEP 3

1. What are the bone-building cells?

Bone cell constituent cells include:

A. Osteoblasts are branched branching cells that function to decrypt cellular matrices.
Osteoblasts will turn into osteocytes when confined within the secreted matrix. These
cells are important in the growth and development of hard bone tissue.
B. Osteoclasts are motile bone cells, branching with 5 to 50 or more intisel. These
cells are derived from the fusion of many monocytes that play a role in phagocytes.
C. Osteocytes are bone cells that reside in the lacunae system of hard bone havers.
These cells come from differentiation of osteoblast cells. These cells play a role in
keeping the extracellular matrix solid. The death of these cells to eat will cause the
decomposition of the extracellular matrix.

2. What hormones cause growth factors ?

Hormones needed for human growth, such as: Growht Hormone, Melatonin, Thyroid
Stimulating Hormone, Insulin, DHEA, Estrogen, Progesterone, Testosterone,
Prolactin, Adrenogen, and others.

3. What is the etiology of the disease?

Gigantism is caused by excessive secretion of GH. This condition can be caused by
pituitary tumors that secrete GH or due to hypothalamic abnormalities leading to
excessive GH release. Gigantism can occur when a state of excess growth hormone
occurs before the bone epiphyseal plate closes or is still in its infancy. The cause of
 excess production of growth hormone is primarily a tumor in somatron cells that
produce growth hormone

There is excessive GH secretion from pituitary adenomas. GH causes excessive

growth of the soft tissues, including skin, tongue, and viscera and bone. This hormone
has antiinsulin properties. (David, et al. Lecture Notes Clinical Medicine)
The causes of ginggatisme can be classified as follows:
1. Primary ghost or hipofisi, imana cause is pituitary adenoma
2. Secondary or hypothalamic ringgatism, caused by GHRH hypersecretion of the
hypothalamus
3. Primary gauss caused by ectopic tumors (lung, pancreas, etc.) that secrete GH or
GHRH
Seeing the size of the tumor, pituitary adeoma can be divided into 2:
1. Mikroadenoma: tumor with a diameter smaller than 10 mm
2. Makroadenima: tumor with a diameter larger than 10 mm

4. What is the epidemiology of the disease?

Gigantism is extremely rare, with about 100 cases occurring to date. Acromegaly is
more common than giantism, with incidents of 3-4 cases per million people per year
and a prevalence of 40-70 cases per million inhabitants.
Sex
Increased IGF-I same in men and women. In the 12th child exam, GH dream secretion
adenoma with a female-to-male ratio of 1: 2. Given the small size of this series, this
disorder will not display sex bias during childhood.
Age
Gigantism can begin every age before epiphyseal closure that is before puberty.

5. What is the pathophysiology of the disease?

In young people with open epiphyses. Excessive GH production results in gigantism.
Gigantism is a disorder caused by excessive secretion of GH, when excess GH occurs
during childhood and adolescence, the longitudinal growth of the patient is very rapid,
and the patient very quickly becomes a giant. After somatic growth is complete, GH
hypersecretion will not cause gigantism, but causes thickening of the bones and soft
tissues. This excess growth hormone occurs after a period of growth passes or the
epiphyseal plate closes. This will cause bone thickening, especially on the bone akral.

6. What are the clinical manifestations of the disease ?

Skeletal abnormalities and signs of glucose intolerance as seen in patients with

acromegaly
Enlargement of pituitary tumors (which cause the loss of other trophic hormones, eg
hormones that stimulate the thyroid, hormones that stimulate follicles and
corticotropins).
.Gigantism may be accompanied by visual impairment when the tumor is enlarged,
thus suppressing the optic khiasma which is the eye nerve pathway.
7. What is the diagnosis of the disease?
GIGANTISME

8. What is the examination of the disease ?

Investigations performed include:
A. Laboratory examination
- Blood glucose examination:
Gigantism (+): increased blood glucose
- Examination Growth Hormone blood or SM-C (IGF 1):
Gigantism (+): elevated GH of blood or SM-C (IGF 1)
- Somatostatin Examination:
Gigantism (+): increased somatostatin
B. Radiological examination
- CT-Scan
- MRI (Magnetic Resonance Imaging)
Roentgenogram of the skull can show enlargement of sella tursika and paranasalis
sinus; Computed tomography scans or magnetic resonance (MRI) images show
tumors. The bonding of the falangs and the increased thickening of the heel cushions
are common. Bone maturation is normal.

Measurement of GH levels through radioimmunoassy, levels only increased in

active disease and not suppressed by glucose in the standard glucose tolerance test
Perimeter to look for defects of visual field of bitemporal (50%)
X-ray of the skull to see cellular enlargement, erosion of the clinical process,
supraorbita groove, and lower jaw. The physiologic fossa of hypopisis usually appears
to be eroded or becomes a double-looking lateral tomogram
CT scan for MRI to see suprasellar extension
Hand x rays to locate the plates on the distal phalanx and increase the cavity
distance between the joints due to hypertrophy of the cartilage. The heel pads are
usually thickened, this test has more interesting elements than the diagnostic
Serum glucose levels may increase
The serum phosphate level during fasting increases but does not have any diagnostic
benefit
Chest X-ray and ECG may show left ventricular hypertrophy due to hypertension

1. Certainty of diagnosis is done by examination of growth hormone. As the SM-G

(IGF-1) examination filter test is probably best considered.
2. CT-Scan head. MRI to determine the presence of macro and macro-pituitary
tumors.
3. Growth hormone suppression test (GD suppressin test) with glucose load 100gr.
Assessed abnormal if there is failure of pressure to below 2g / 1.
9. What are the complications of the disease ?
1. Heart Hypertrophy
2. Hypertension
3. Diabetes Mellitus

Adrenal insufficiency
Diabetes insipidus (rare)
Hypogonadism
Hypothyroidism

10. What is the management of the disease ?

Kraniatomi
Cryial or transfenoidal hypophysectomy or pituitary radiation therapy is performed to
remove the underlying tumor
Thyroid and gonadal hormone replacement and cortisone are performed after
surgery
Bromocriptine (parlodel) and octreotide (sandostatin) are used to inhibit hormone
Gh.

A) Normalize GH or IGF-1.4 levels again

B) Reduces tumor or stabilizes tumor size4
C) Normalize the function of hyposis4
Known 3 kinds of therapy, namely:
A. Surgical therapy4
Surgery is the main treatment. Known two kinds of surgery depends on the size of the
tumor, namely: macro surgery by performing surgery on the skull (TC or trans
cranial) and micro surgery (TESH or trans ethmoid sphenoid hypophysectomy). This
last way (TESH) is performed by surgery through the angle between the infra orbital
gap and the bridge of the nose between the two eyes, to reach the pituitary tumor. The
results obtained are quite satisfactory with the success achieved HP desired levels
reached in 70 - 90% of cases. The success is also largely determined by the size of the
tumor.
Side effects of surgery can occur in 6 - 20% of cases, but can generally be overcome.
Postoperative complications may include spinal cerebrospinal fluid leak (CSF leak),
nasal oro fistula, epistaxis, sinusitis and infection of the surgical wound.
The success of therapy is characterized by a decrease in GH levels below 5 g / l.
With this criterion the success of therapy is achieved in 50-60% of cases, consisting
of 80% of cases of microadenoma, and 20% of macroadenomas.

B. Radiation therapy
Indication of radiation is as a single-choice therapy, if surgery is not possible, and
accompanies surgery if there are still acute symptoms after surgery is performed.
Radiation provides the benefits of tumor shrinkage, lowering GH levels, but may also
affect pituitary function. Decreased levels of GH generally have a correlation with the
duration of radiation carried out. Eastment et al stated that, there was a 50% reduction
in GH from baseline levels after 2 years irradiation, and 75% after 5 years of
irradiation.
Other researchers mentioned that, HP levels can be lowered below 5 g / l after
treatment runs 5 years, in 50% of cases. If treatment is continued for up to 10 years
then, 70% of cases can reach that level.

C. Medical treatment using ocreotide,

A somatostatin analogue is also available. Ocreotide can decrease suppression of GH
and IGF-1 levels, shrink the size of the tumor, and improve the clinical picture.