Flashcards in Acromegaly Deck (10)
1
Definition
Chronic, progressive, multi-systemic excessive secretion of growth hormone
Most commonly due to pituitary somatotroph adenoma
2
What are 5% of cases associated with
MEN 1
3
Pathogenesis
+growth or bone and soft tissue
GH->+IGF-1
4
Key diagnostic factors
Coarsening of facial features
Soft tissue/skin changes
Carpal tunnel syndrome
Acroparasthesia
Joint pain/dysfunction
Snoring
Altered sexual fuunction
Hx/FHx of inherited syndrome
5
Signs of acromegaly
+growth of hands
coarse facial
+supra orbital ridges
Macroglossia
widely spaced teeth
puffy lips/lids
skin darkening
acanthosis nigricans
laryngeal dyspnea
OSA
Goitre
proximal weakness and arthopathy
carpal tunnel
mass effects->headache, VFDs, cranial nerve palsies, features of hypopituitarism
CVS: HTN, hypertrophic cardiomyopathy, arrythmias
Metabolic->glucose intolerance
Effects of prolactin co-secretion->-ve libido, infertility, amenorrhea/oligomenorrhea, galactorrhea, erectile dysfunction
6
Complications
CCF
Ketoacidosis
Glucose intolerance
+BP
Hypertrophic cardiomyopathy
+colon cancer risk
7
Investigations
+Glucose->OGTT
+Calcium
+Phosphate
Serum IGF-1
Random serum GH >0.4 microG/L
ECG/Echo
Tests to consider:
Pituitary MRI/CT
GHRH->elevated in ectopic
Chest/abdominal CT-tumor localisation
Octreoscan
Plasma cortisol-may be low
Prolactin- often elevated
TSH and free T4- may be abnormal
Estradiol or testosterone- often reduced
Visual field testing
8
Management
enclosed pituitary tumour
1st line: transsphenoidal surgery
2nd line: somatostatin analogue (SSA)->octreotide: long acting depot every 4 weeks for 3 months
adjunct: dopamine agonist->cabergoline
3rd line: growth hormone-receptor antagonist (GHRA)->pegvisomant
4th line: radiotherapy: conventional or stereotactic
unresectable pituitary tumour (with neural or vascular impingement/invasion)
1st line: somatostatin analogue (SSA)
adjunct: dopamine agonist
adjunct: debulking surgery
2nd line: growth hormone-receptor antagonist (GHRA)
3rd line: radiotherapy: conventional or stereotactic
non-pituitary adenoma aetiology
1st line: medical and surgical interventions
Ongoing
pituitary adenoma progression or recurrence
1st line: repeat surgery
9
Side effects of somatostatin analogues
Pain at injection site
GIT: abdominal cramps, flatulence, loose stools, gall stones, impaired glucose tolerance
10