acromegaly (거인증, 말단비대증)

소개글

acromegaly (거인증, 말단비대증)에 대한 보고서 자료입니다.

목차

ACROMEGALY

INTRODUCTION

ETIOLOGY OF HYPERSOMATOTROPISM

CLINICAL FEATURES OF ACROMEGALY

DIAGNOSIS OF ACROMEGALY

DIFFERENTAIL DIAGNOSIS OF ACROMEGALY

TREATMENT
-Surgery
-Radiation
-Radiation
-Dopamine agonists

CHOOSING A THERAPY

CONCLUSION

본문내용

INTRODUCTION
Elevated levels of growth hormone and insulin-like growth
factor 1 (IGF-1) are hallmark of this syndrome
Usually caused bv growth hormone-secreting pituitary
tumors or rarely, extrapituitary disorder
clinical manifestations include skeletal and soft tissue
growth and deformations, and cardiac, respiratory,
neuromuscular, endocrine, and metabolic complication
Although surgery is considered the treatment of choice,
complete macroadenoma resection, especially if invasive,
is difficult even for the skilled surgeon, and there is
a low rate of biochemical control
Pharmacologic treatment has assumed more importance in
managing these patients using long-acting somatostatin
analogue, dopamine agonists, and growth hormone receptor
antagonist

ETIOLOGY OF HYPERSOMATOTROPISM
Excess growth hormone secretion is associated with
pituitary and extrapituitary tumors
Pure somatotropinomas contain either densely(slow
growing) or sparsely (rapid growing) staining
cytoplasmic growth hormone granule ( 60% )
Bimorphous adenoma, two mature somatotroph and lactotroph
cell type – GH & prolactin secretion
Monomorphous mammosomatotroph cell adenomas arise from a
mature cell that secretes growth hormone and prolactin
Plurihormonal adenomas secrete a combination of growth
hormone and several other hormones, such as prolactin,
TSH, ACTH
Growth hormone-secreting carcinomas with definite proof
of distant metastasis are extremely rare
CLINICAL FEATURES OF ACROMEGALY
attributed to high serum levels of growth hormone,IGF-1,
both
Central features of an expanding pituitary mass are
common, especially with macroadenoma. Headache is often
severe, and chiasmal compression leads to visual
impairment
lateral extension can cuase diplopia or facial pain owing
to compression of cranial nerve
Inferior invasion may cause cerebrospinal fluid rhinorrhea
Skeletal overgrowth owing to periosteal new bone
formation in response to IGF-1 : mandibular overgrowth
with prognathism, widening of the maxilla, tooth
separation, jaw malocclusion, overbite, frontal bossing,
nasal bone hypertrophy, enlargement of paranasal sinuses
Arthropathy occurs in approximately 70% of patients with
monoarticular or polyarticular involvement. : crepitus,
stiffness, tenderness, hypermobility, and debilitating
joint pain. Chronic osteoarthritis causes narrowed and
deformed joint space, osteophyte formation, subchondral
cysts, and lax periarticular ligament with ossification
Skin change : Oily skin and hyperhidrosis, coarsening of
body hair, thickening of the skin, prominent facial
fold, facial wrinkles, a large fleshy nose, skin tag,
heel pad, increased shoe, ring, hat size and Raynaud
phenomenon
Cardiovascular disease : the most important cause of
mortality, responsible approximately 60% of death :
Asymmetric septal hypertrophy, LVH, Cardiomegaly,
cardiac failure -> effective treatment reducing GH and
IGF-1 serum levels improves cardiac function

키워드

acromegaly,   거인증,   말단비대증,   dopamine,   bromocriptine