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MSc Endocrinology > Acromegaly > Flashcards

Acromegaly Flashcards

1
Q

Causes of acromegaly

A
  1. GH secreting pituitary adenoma
  2. Ectopic GHRH secretion
  3. Ectopic GH secretion
  4. Exogenous GH use
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2
Q

IGF-1 in acromegaly

A

If >1.3xULN - acromegaly confirmed

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3
Q

If IGF-1 elevated < 1.3x or normal

A

Oral glucose tolerance test

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4
Q

OGTT criteria for acromegaly

A

Old criteria: Failure of GH to suppress to < 1ug/L

New criteria:
0.4 ug/L in BMI <25
0.2 ug/L in BMI >25

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5
Q

Investigations to find the underlying cause of acreomegaly

A
  1. MRI pituitary
    If no pituitary mass
  2. GHRH level
  3. Whole body PET/CT with 11-C methionine or DOTATATE
  4. Octreotide scan
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6
Q

Other tests to be done in acromegaly

A
  1. Visual field testing
  2. Anterior pituitary hormone profile
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7
Q

Tests for evaluation of complications

A
  1. Diabetes - FBS/HbA1c
  2. Osteoporosis - DEXA
  3. Cardiomyopathy
  4. Colon cancer - colonoscopy
  5. Lipid profile
  6. Sleep study
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8
Q

Ectopic GHRH secreting tumours

A

Pancreatic NETS, bronchial carcinoid

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9
Q

Ectopic GH secreting tumours

A

Rarer - ovarian and pancreatic neoplasms

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10
Q

Difference between GHRH vs GH secreting tumours

A

GHRH levels high in the former
Prolactin levels may be high in the former
Pituitary hyperplasia (former)

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11
Q

First line treatment for acromegaly

A

Trans sphenoidal pituitary surgery

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12
Q

Indications for medical management

A

A. Patient not willing for surgery
B. Cavernous sinus extension without compression of optic chiasm
C. Persistent disease after surgery
D. Pre-op treatment in patients with sleep apnea and high output HF

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13
Q

If surgery not done, what is first medication used?

A

Dopamine agonist

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14
Q

If persistent disease after surgery, what medication is used?

A

1st gen somatostatin receptor ligand (octreotide, lanreotide)

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15
Q

What if no response to DA?

A

Switch to 1st gen SRL

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16
Q

What if there is partial response to 1st gen SRL?

A

If >50% decrease of IGF-1/GH levels but not normalised
Increase dose of the same SRL

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17
Q

Disease not controlled with 1st gen SRL?

A

Decide if concern is tumour burden or impaired glucose metabolism

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18
Q

Disease not controlled with 1st gen SRL
Tumour concern

A

Pasireotide

19
Q

Disease not controlled with 1st gen SRL
Impaired glucose metabolism

A

Pegvisomant
Does not decrease tumour size; may cause growth of tumour

20
Q

Disease not controlled with 1st gen SRL
Both tumour concern and impaired glucose metabolism

A

Pasireotide + 1st gen SRL

21
Q

Disease still not controlled after all medical options

A

Radiotherapy or surgical (re)intervention

22
Q

Risks of radiotherapy

A
  1. Anterior hypopituitarism (high probability)
  2. Cranial neuropathies (low probability)
  3. Possible risks of secondary tumors or stroke (low probability)
23
Q

Post surgery
Desired IGF-1 level

A

18-35 nmol/L after 6 weeks

24
Q

Post surgery
Desired GH level

A

<1 ug/L after 6 weeks

25
Predictors of recurrence in acromegaly
1. Young age 2. Large tumour 3. Aggressive tumour 4. High IGF-1 levels before and after surgery
26
Frequency of SRL injections
Every 4-6 weeks
27
When do you monitor GH and IGF-1 levels in SRL therapy?
After the 3rd injection
28
Levels of response to therapy
A. Complete response - normalised GH and IGF-1 and >20% decrease in tumour size B. Partial response - >50% reduction in GH/IGF-1 and >20% reduction in tumour size C. Resistance - <50% biochemical and <20% anatomical response
29
What percentage of people achieve complete response with SRLs?
55%
30
Incidence and prevalence of acromegaly
Prevalence: 2.8 to 13.7 per 100000 people Annual Incidence: 0.2 to 1.1 cases per 100000 people
31
Percentage of recurrence after surgery only versus surgery + RT versus surgery + medical therapy?
Surgery only 10-20% micro adenoma 30-50% macro adenoma Surgery + RT 10-15% Surgery + medicine Upto 20%
32
Adult growth hormone deficiency diagnosis
If >3 pituitary hormones decreased: Low IGF-1 is confirmatory If not - do dynamic testing
33
Dynamic testing for AGHD
1. Insulin tolerance test 2. GHRH-arginine test 3. Glucagon stimulation test 4. Macimorelin test 5. Clonidine stimulation test
34
Diagnosis of AGHD with ITT
After achieving hypoglycaemia (<2.2 mmol/L), if GH does not rise to >5ng/mL (20 milli units/L) If <3ng/mL - severe GH deficiency
35
Contraindications to ITT
Seizure disorder Ischemic heart disease Adrenal insufficiency
36
What other deficiency can also be diagnosed with ITT?
Cortisol Normal: > 550 nmol/L If not - hypocortisolism
37
Which should be treated first? Thyroid or GH deficiency?
Thyroid GH can increase conversion of FT4 to FT3
38
Macimorelin test
Macimorelin is ghrelin analogue - causes GH release Accuracy may be reduced in patients taking CYP3A4 inducers (e.g., carbamazepine)
39
Indications for treatment of AGHD
1. Severe GH deficiency (peak GH <3 ng/mL with ITT) 2. Deficiency of other pituitary hormones corrected 3. QoL-AGHDA score at least 11
40
Treatment of AGHD
Recombinant human GH <30 years - 0.3-0.5 mg/day 30-60 years : 0.2-0.3 mg/day >60 years: 0.1-0.2 mg/day Women taking OE: 0.3-0.4 mg/day
41
Dosage of rhGH in pediatric patients transitioning to adult care
GH treatment should be stopped for 2 to 3 months GH treatment at adult doses should be re-started only in those satisfying the biochemical criteria for severe GH deficiency
42
Initial monitoring of AGHD treatment
Every 1-2 months: IGF-1 in middle of reference range and QoL questionnaire
43
Monitoring of AGHD treatment after achieving maintenance
IGF-1 and clinical assessment every 6-12 months FBS, HBA1c, lipids every 12 months DEXA every 2 years if abnormal at diagnosis

MSc Endocrinology (5 decks)

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