ACROMEGALY CASE STUDIES

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ACROMEGALY CASE STUDIES

• Margaret E Roberts
• Acromegaly Specialist Nurse
• Christie Hospital Manchester

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Acromegaly
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Acromegaly

• Acromegaly is Rare (3 per million per year)
• Can be disfiguring
• Life shortening (average 10 Years)
• Equally prevalent in both ? and ?
• Average age 44 years at diagnosis
• Delay in diagnosis (8 to 10 years)

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Cause

• Excess of growth hormone
• Slow growing pituitary tumour (99)
• Post puberty
• Overgrowth soft bone tissue
• Bone thickening
• Pre-puberty- gigantism ? GH
• Hypopituitarism
• ? Testosterone and Oestrogen
• Ectopic GHRH
• McCune-Albright Syndrome

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Signs and Symptoms

• Enlargement of hands and feet
• Insidious changes in the face
• Deepening of the voice
• Coarsening of the skin
• Excess sweating
• Hypopituitarism

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Signs and Symptoms

• Severe headache
• Snoring
• Sleep Apnoea
• Lethargy
• Aches and pains
• Menstrual cycle disturbance/impotence

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Acromegaly spade-like hands
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AcromegalyCo-morbidities
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AcromegalyCo-morbidities
Direct tumour effects visual problems
compression hypopituitarism
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Treatment

• Surgery
• Radiotherapy (not widely used)
• Stereotactic Gamma Knife
• Dopamine Agonists ( Cabergoline)
• Somatastatin Analogue (Octreotide LAR, Lanreotide
  Autogel)
• GH receptor Antagonist (Pegvisomant)

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Objectives of treatment

• Reverse the symptoms and signs of acromegaly
• Restore circulating GH (lt2.5 µg/l) and
  Insulin-like growth factor (IGF-1) levels to
  normal
• Restore normal life expectancy
• Remove the pituitary tumour and preserve residual
  
• pituitary function
• Prevent recurrence

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Case Study

• Summary
• 18 year old Iady
• Height 180.5cm (5ft 11½in)
• diagnosed 2006
• Acromegaly with hyperprolactinemia

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History

• From 6 years age taller than friends
• Slept for long periods up to 20hrs at a time.
• Headaches and tiredness
• Pins and Needles in arms and hands
• Mum thought depressed and alcoholic

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• 16 years went to GP referred to local hospital
• Diagnosed acromegaly commenced cabergoline.
• Commenced Menstrual Periods

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• 12 months later referred to Hope Hospital.
• Endocrine blood screen
• Basal pituitary function
• IGF-I
• GHDC
• OGTT
• MRIscan

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Assessment (on cabergoline)

• GHDC mean 14.92mU/L
• IGF-I 109 nmol/L (ref range 21.2-75.9nmol/L)
• Prolactin 129 mU/L (ref range 50-400)
• Non-suppression of GH during OGTT
• MRI scan macroadenoma
• Comfirmed
• Acromegaly with Hyperprolactinemia

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• .
• 2006 referred for surgery had 2 transsphenoidal
  operations not cured.
• Had octreotide LAR injections not controlled.
• 2007 referred to Christie Hospital for further
  opinion.

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Assessment (off cabergoline)

• Prolactin 1810mU/L (ref range 50-400)
• IGF-I 790 mg/l (ref range 151-528)
• Persistent Acromegaly
• Thyroid and Cortisol Deficient
• Diabetes insipidus
• Menstrual Periods stopped

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Medication

• Thyroxine 75mcgs daily
• Hydrocortisone 10/5/5mgs
• Omeprazole
• Desmopressin spray
• Cabergoline

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• Cabergoline effective in 30 patients
• Prolactin and GH co-secreting
• tumours respond well to dopamine agonist
• - biochemical response
• - tumour shrinkage
• Non responsive to somatostatin analogues

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Prolactin responds, better than Growth hormone to
dopamine agonists. Cabergoline effect on heart
no evidence in Acromegaly patients Dopamine
agonist ? ORAL ? CHEAP In this
patient Cabergoline had a much more dramatic
effect. She now feels very well on 3.5mgs
Cabergoline weekly
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• I would like to thank
• Dr Claire Higham for all her help.

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