Acromegaly Flashcards
Define:
Collection of signs and symptoms caused by an excess in growth hormone
In children this is gigantism
Aetiology/risk factors:
99% are caused by GH secreting adenoma
Ectopic tumour releasing GH or GHRH form e.g. bronchial and pancreatic cancer
5% are caused through MEN-1
Epidemiology:
RARE
5/100,000
Affects 40-50 year olds
Symptoms:
Insidious onset Headaches Rings and shoes becoming tight Sweating on face Arthralgia Carpal tunnel syndrome Visual disturbances Hypopituitarism: hypogonadism, hypothyroidism, hypoadrenalism Hypoprolactinemia - impotence, irregular periods and decrease libido
Signs in the hands:
o Large spade-like hands
o Thick greasy skin
o Carpel tunnel syndrome signs
o Premature osteoarthritis
Signs in the face:
o Prominent eyebrow ridge o Prominent cheeks o Broad nose bridge o Prominent nasolabial folds o Thick lips o Increased gap between teeth o Large tongue (macroglossia) o Prognathism o Husky resonant voice (due to thickening of vocal cords) o Growth of jaw o Puffy lips, eyelids and skin o Scalp folds o Darkening of skin
Signs in the neck:
Multi-nodular goitre
Other signs:
Enlarged feet Obstructive sleep apnoea Proximal weakness Organomegaly Bitemporal hemianopia
Investigations:
Bloods, U+ Es –> glucose, calcium and phosphate
IGF-1 (random GH is not useful as it is pulsatile and cyclical)
Oral glucose tolerance test - suppression test (the GH remains high after 75mg of glucose)
MRI of the brain
Visual fields and acuity
Pituitary function tests: o 9am cortisol o Free T4 and TSH o LH and FSH o Testosterone o Prolactin
Management:
Trans-sphenoidal hyposphsectomy
Radiotherapy can act as an adjuvant therapy
Somatostatin analogue e.g. octerotide
Dopamine agonists for the hyperprolactionemia e.g. bromocriptine
GH Antagonist (pegvisomant)
Monitor GH and IGF1 levels can be used to monitor disease control
What are the side effects of somatostatin analogues:
abdominal pain
steatorrhoea
What are the side effects of dopamine agonists:
nausea, vomiting, constipation, postural hypotension, psychosis (RARE)
Complications:
Cardiomegaly Hypertension Obstructive sleep apnoea Colonic polyps Renal stones Diabetes Mellitus Depression Psychosis Hypercalcemia Hyperphophotemia Hypertriglyceremia
Complications of surgery:
o Nasoseptal perforation o Hypopituitarism o Adenoma recurrence o CSF leak o Infection
prognosis:
Good if treated early
Physical changes are irreversible