Acromegalia

Acromegalia

Acromegalia este o maladie endocrin ca rezultat a

producerii excesiv a adenohipofizei de hormon
somatotrop (STH). Dup pubertate.

Denumirea de acromegalie provine de la cuvintele

Greceti acro -"extremiti" i megalos -mari.

Afecteaz adulii cu vrst medie (frecvena 6

bolnavi la 100,000 aduli).

Care sunt cauzele

Acromegaliei?

Producie n exces de durat a STH.

1. Tumoare hipofizar
2. Tumoare extrahipofizar

Etiologie

98%

Tumorile hipofizare

n 90 % acromegalia este cauzat de producie

excesiv de STH, determinat de o tumoare
benign a hipofizei adenom hipofizar.
Aceste tumori produc n exces STH, i cnd ele se
extind pot comprima esuturile adiacente, dintre care
i nervul optic.
De asemenea compresia esutului normal hipofizar
poate duce la dereglarea producerii altor hormoni
hipofizari.
Unele adenome au o cretere rapid, altele
progreseaz lent.

Macroadenom hipofizar

Tumori extrahipofizare

La un procentaj mic de pacieni, acromegalia este

cauzat de o tumoare a pancreasului, pulmonar,
sau a suprarenalelor.

Aceste tumori pot secreta n exces STH, sau frecvent

produc somatoliberin, care stimuleaz sinteza
adenohipofizar de STH.

La aceti pacieni se poate detecta o cantitate

majorat a somatoliberinei n plasma sanguin.

Generaliti despre STH

Este secretat de celulel eozinofile ale adenohipofizei

Aciunea principal este creterea longitudinal

Alte efecte importante: aciune asupra metabolismului

lipidic, glucidic i lipidic

Secreie pulsatil, stimulat de somn, exerciiu fizic i post

alimentar (foame).

Nivel crescut al STH este prezent la tineri, cu vrsta se

observ scderea concentraiei lui.

Nivel redus de STH este determinat la persoanele obeze.

Patogenie
Metabolism

Fiziopatologie

Anomalii biologice

Glucidic

Antagonism insulinic:
micoreaz insulinosecreia,
crete gluconeogeneza,
scade utilizarea periferic

Hiperglicemie
Insulinorezisten
Scade tolerana la
glucoz

Lipidic

Lipoliza

AGL majorai

Mobilizarea

AG din estul adipos

Proteic

Anabolism

Bilan azotic pozitiv

Hidroelectrolitic

Crete reabsorbia de Na

hipervolemie

Fosfor-Ca

Ca ser normal
Ca urin
P n ser

absorbia intestinal de Ca;

reabsorbia tubular de P;
reabsorbia tubular de Ca;
renoirea Ca osos

Clasificarea (Pronin V.1996)

Preacromegalia semne
Stadiul hipertrofic
Stadiul tumoral
Stadiul caectic

Acuze
amenoree/galactoree
impoten sexual
cefalee
tulburri vizuale
dureri osteoarticulare
diabet zaharat
dismorfism

Tabloul clinic

Sindrom tumoral

Sindrom dismorfic

Sindromul endocrino-metabolic
Sindromul dereglrilor reproductive
Sindromul afectrii nervilor cranieni
Sindrom radiologic
Sindrom biologic

Sindrom tumoral
Cefalee - frontal, retroorbitar,
permanente, pronunate, (cauzate de
creterea tensiunii intracraniene i
compresia diafragmei eii turceti ).
Hemianopsie bitemporal

Sindrom somato-visceral

Tegumente groase, cutis

girata, transpiraie excesiv
(creterea numrului i
hipersecreia glandelor
sudoripare i sebacee),
hipertrichoz, acantozis
nigricans.
Mucii n debut hipertrofie cu
creterea forei musculare apoi
degenereaz (are loc proliferarea
esutului conjunctiv i rmnerea
n urm a creterii vaselor
sanguine n comparaie cu masa
muscular).

Masivul facial

Frunte ngust, arcade

sprncenare i
zigomatice proeminente
Nasul, buzele ngroate
Limba - macroglosie,
aspect geografic al limbii,
amprente dentare
Mandibula prognatism,
Dinii diasteme, cad
uor, carii frecvente, cu
apariia tulburrilor
masticatorii

Extremitile

Minele late degete cilindrice, palme

capitonate
Picioarele- late, calcaneu mrit, numrul la
pantofi crete

Acromegalie

Torace i abdomen

Cifoz dorsal, lordoz

Stern proeminent torace globulos abdomen
proeminent

Visceromegalie

Infiltraia laringelui voce rguit

Glanda tiroid hipertrofic, noduli tiroidieni
Cord global mrit, hipertrofie concentric
cardiomiopatie hipertrofic cardiomiopatie
dilatativ ICC; bradicardie, HTA
TD constipaie, hepatosplenomegalie, gastrit
hipertrofic, megacolon, dolicocolon, cancere i
polipi.
Rinichii mari, litiaz urinar

Manifestri asociate

Amenoree, galactoree femei, impoten, hipogonadism

brbai (hipersecreie de prolactin sau de efectele similare
cu lae prolactinei care inhib secreia de gonadoliberine)
Diabet zaharat insulinorezistena,
HTA
CPI
Artralgii, parestezii sindrom de tunel carpal (hipertrofia
esutului cartilaginos)
Apnee n somn (mrirea n volum a esuturilor moi a
cilor respiratorii superioare i afectarea centrelor
respiratorii)
Osteoporoz

Progresarea Acromegaliei

Acromegaly

Overgrowth of bone and soft

tissues due to hypersecretion of
growth hormone
Increase in enchondral bone
formation at the physes (in the
immature skeleton) and at
cartilage-bone junctions in
adults
Radiologic findings in the skull
include enlargement of the
paranasal sinuses, sella turcica,
thickening of the cranial vault,
enlargement of the mandible
and hypercementosis of the
teeth.

Acromegaly

In the hands and wrists,

patients with acromegaly
often have

thickening of the soft

tissues
thickening and squaring of
the phalanges and
metacarpals
overtubulation of the
phalanges, and widened
articular spaces
osseous proliferations at
tendon and ligament
attachments
prominence of the ungual
tufts

Acromegaly

Articular abnormalities in
the appendicular skeleton
typically affect the knees,
hips and shoulders
Thickening of the articular
cartilage can lead to early
radiographic findings of
relative widening of the joint
space
Later in the disease
process, the alteration in
cartilage morphology leads
to degenerative changes,
similar to those of primary
osteoarthritis

Tratamentul Acromegaliei

Chirurgical

Medicamentos

Radio Terapie

Chirurgical

Removal of pituitary tumor is often preferred

treatment; tends to give best results in shortest
time
Results depend on size of tumor & skill of
surgeon
Sometimes, GH levels are reduced by the
operation, but acromegaly is not cured.
Therefore, additional treatment such as drug
therapy and/or radiotherapy necessary

Acces transfenoidal

Terapie medicamentoas

n prezent se utilizeaz 3 grupe de

remedii medicamentoase:
Agonitii

Dopaminei
Analogi ai Somatostatinei
Antagoniti ai receptorilor STH

Agonitii Dopaminei

Inhib direct secreia de STH

Simpli n administrare pe cale oral

Bromocriptine

(denumire comercial Parlodel)

Cabergoline (denumire comercial Dostinex)

Aproximativ la 50% din pacieni se observ

diminuarea nivelului STH i reducerea
dimensiunilor tumorii

Analogi ai Somatostatinei

Inhib secreia STH

Reduce

indirect nivelul IGF-I precum i

dimensiunile tumorii

Octreotide (denumire comercial

Sandostatin)
injecii

s.c. (3 ori/zi) sau

Intramuscular (o dat n lun)

Lanreotide (denumire comercial

Somatuline LA)
I.m.

fiecare 7-14 zile sau

Deep s.c. injection once a month

Antagonitii receptorilor STH

Blocheaz receptorii pentru STH

Pegvisomant: denumire comercial
Somavert
Does not stop the production of GH by
tumor but does block IGF-I secretion
Does not reduce tumor size

Radiation Therapy
X-rays carefully targeted to the tumor
are given in small doses 5 x/week
over about a month
Gradual reduction in tumor size & GH
release
Slow improvement
Drug treatment to lower GH levels
while patient wait for results of
radiotherapy