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Acromegaly (Complications (Cardiovascular MI, stroke CCH, cardiomyopathy…
Acromegaly
Complications
Cardiovascular
MI, stroke
CCH, cardiomyopathy
HTN
Arrhythmias
Cancer
Colon cancer
Metabolic
DM
Fertility
Subfertile
Diagnosis
Examination
General
Coarse facial features, spade like hands,
widely spaced teeth, puffy lips, eyelids, skin
Derm exam
Acanthosis nigricans, darkened skin
Musculoskeletal exam
Proximal muscle weakness, joint pain,
signs of OA
Neuro exam
CN: reduces acuity, fields, opthalmoplegia, hemianopia
Limbs: proximal muscle weakness, carpal tunnel syndrome
Investigations
Bloods
FBC, CRP, U+E, LFT, TFT,
glucose (hyperglycaemia), bone profile (high ALP, Ca, phos)
Hormones (high ILGF-1; GH difficult to measure as pulsatile)
Imaging
MRI head (pituitary tumour)
ECHO (cardiomegaly)
Bedside
Obs (HTN)
ECG (arrhythmias, LVH)
Old photos (progressive change)
Special tests
OGTT: 75mg glucose then measure up to 2h after
Normally glucose should suppress GH and IGLF-1,
but no suppression in acromegaly
History
DH
Meds, allergies
FH
Acromegaly, cancers (MEN1)
PMH
Endocrine disorders, DM, MEN1
Previous surgeries
SH
Living arrangements, occupation,
smoking, alcohol, diet, exercise
PC/HPC
Altered appearance, headaches,
sweating, muscle weakness, joint pain
Management
Medical
Somatostatin analogues
Indication: 2L (surgery unsuitable)
E.g. octreotide IM (EXPENSIVE!)
MOA: somatostatin inhibits GH action
SEs: injection pain, GIT, gallstones, DM
GH antagonists
Indication: 3L if somatostatin analogues no use
E.g.pegvisomant
Radiotherapy
Indication: adjuvant/alernative to surgery
Surgical
Transphenoidal/transfrontal craniotomy
Indication: 1L tx
MOA: removal of tumour via nose
Conservative
Information, advice, support
Identify and manage cause
Monitoring (bloods, photos, BMI, visual fields)
Pathophysiology
Normal function
GH stimulated by low glucose, exercise, sleep
Released in pulsatile manner
Stimulates bone and tissue growth (linear in children, bone mass, metabolic functions via ILGF-1)
High glucose feeds back to reduce GH
Somatostatin also inhibit GH
Defect
Excess GH secretion (usually tumour)
If before epiphyseal plate fusion, get giganticism, if after,
get acromegaly
Negative feedback via glucose lost (so hyperglycaemia)
Clinical
presentation
Hormone excess
Headaches
Sweating
Hands, jaw and feet growth (inc hat, shoe, ring size)
Coarse facial features (nose, lips, tongue, supraorbital ridges, wide spaced teeth)
Proximal weakness
Arthropathy
Snoring
Mass effects
Reduced acuity
Diplopia
Hemianopia
Epidemiology
Very rare
M=F
Aetiology
Neoplastic
Pituitary tumour (commonest)
Hyperplasia
MEN1 syndromes
Definition
Endocrine disorder of
excess GH production
in a fully grown adult