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Endocrine > Acromegaly > Flashcards

Acromegaly Flashcards

1
Q

What is the difference of acromegaly?

A

Acromegaly occurs AFTER fusion of growth plates

  • Therefore, only soft-tissue and flat-bone enlargement
  • No change in height!

Gigantism occurs BEFORE fusion of epiphyseal plates 🡪 tall stature, long limbs etc

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2
Q

What are the causes of acromegaly?

A

Most commonly pituitary adenoma (>95%)

Genetic causes

  • Mc-Cune Albright
  • MEN 1: Parathyroid, pancreas, pituitary
  • Carney Complex
  • Familial Acromegaly
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3
Q

What are the clinical presentations of Mc-Cune Albright?

A

Polyostotic Fibrous Dysplasia (of the bone)

  • Polyostotic = affecting multiple bones (VS monostotic)
  • Fibrous Dysplasia = skeletal tissue is replaced by fibrous tissue

Precocious Puberty (GnRH independent)

Pigmentation (Café au lait spots)

  • Flat, Hyperpigmented Macules (from light to dark brown in colour)
  • Seen in other disorders & neurocutaneous disorders including: Neurofibromatosis type 1, Tuberous sclerosis, Fanconi anemia

May also be associated with other endocrine abnormalities:

  • Acromegaly (20%)
  • Cushing’s
  • Thyrotoxicosis
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4
Q

What are the presenting complaints of acromegaly due to mass effect?

A

Headache (worst on morning, straining), N&V

Night time awakening

Bitemporal Hemianopsia, BOV due to optic chiasm compression

Central Hypogonadism

  • F: 1’ or 2’ Amenorrhea, Sexual Dysfunction, Infertility, Menopausal S&S
  • M: Sexual Dysfunction, Infertility, Testicular Atrophy, Andropause S&S

Central Hypocortisolism: Hypogly, HypoNa, HypoTN, Lethargy

Central Hypothyroidism: Gain weight, LOA, Constipation, Cold intolerance, Lethargy

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5
Q

What are the presenting complaints of acromegaly due to Hypersecretion of GH + Excessive IGF-1?

A

Hyperhidrosis: Increased sweating (from increased sweat glands)

Thickened skin: do double pinch test

Enlarging spade-like hands & feet. Ask: any change in shoe size? Tightening of rings?

Carpal Tunnel Syndrome

  • May p/w scars from previous release Sx
  • May p/w paraesthesia w/ +ve tinel’s & Phalen’s
  • Due to oedematous swelling of the synovial tendon sheaths and local proliferation of connective tissue.

Proximal Myopathy: Difficulty climbing stairs? Washing hair?

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6
Q

What are the enlarged and coarsened facial features of acromegaly?

A

Enlarged Forehead (from enlarged frontal bone) 🡪 Ask: Any increase in hat size?

Enlarged Nose

Thick lips

Prognathism & Macrognathia (enlarged jaw)

Diastema (i.e. Increased interdental distance)

Macroglossia +/- Cx by OSA due to macroglossia & pharynx soft tissue swelling. Look for dental depression at the sides of the tongue

Deepening of voice

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7
Q

What are the clinical features of acromegaly from insulin intolerance?

A

Acanthosis Nigricans: Ask for other DM S&S – LOW, Polyuria, Polydipsia

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8
Q

What are the skeletal abnormalities of acromegaly?

A

Synovial tissue and cartilage enlarge, causing hypertrophic arthropathy of the knees, ankles, hips, spine, and other joints

Causing Joint Symptoms, Back pain (with kyphosis)

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9
Q

What are the causes of lethargy in acromegaly patient?

A
  • Hypocortisolism
  • Hypothyroid
  • OSA
  • Hypogonadism (low testosterone)
  • Anaemia from colorectal CA
  • Diabetes
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10
Q

What are the causes of breathlessness in acromegaly patient?

A
  • Heart Failure
  • Anaemia
  • Pulmonary HTN
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11
Q

What are the causes of macroglossia ?

A
  • Acromegaly
  • Trisomy 21 – down’s syndrome
  • AML
  • Hypothyroidism
  • Infiltrative disorder (sarcoidosis, amyloidosis)
  • NOT B12 deficiency: will cause glossitis not large tongue
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12
Q

What are the causes of thickened ulnar neve in acromegaly patient?

A
  • Acromegaly
  • Infiltrative: amyloidosis
  • Charcot Marie tooth
  • Hanson’s disease (leprosy
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13
Q

How do the hands of a patient with acromegaly appear O/E?

A
  • Large, spade-like hands
  • Sweating palms (Hyperhidrosis), warmth (increased metabolism)
  • Skin thickening (check via double pinch test)
  • Osteoarthritis (Heberden’s, Bouchard’s)
  • Tinnel’s sign over median nerve (Carpel Tunnels’ syndrome) + Test for numbness if +ve
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14
Q

How do the arms of a patient with acromegaly appear O/E?

A
  • Ulnar nerve thickening (feel behind medial epicondyle) – due to nerve edema
  • Proximal myopathy
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15
Q

How does the face of a patient with acromegaly appear O/E?

A
  • Transfrontal scar (previous surgery)
  • Supraorbital ridge
  • Visual field defects (bitemporal hemianopia in pituitary tumour)
  • Enlarged broad nose
  • Thick lips
  • Macroglossia: Teeth indentation mark on lateral borders of tongue
  • Prognathism (protrusion of the jaw)
  • Diastema / Interdental separation (gum enlargement)
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16
Q

How does the neck of a patient with acromegaly appear O/E?

A

Goitre
- TFT usually NORMAL
OR Subclinical HypoT due to pituitary lesion
- Goitre is not due to thyroid disease, but rather soft tissue swelling 2’ acromegaly

Acanthosis Nigricans

17
Q

How do the legs of a patient with acromegaly appear O/E?

A

Enlarged feet

Proximal myopathy

Osteoarthritis

Pitting edema (from CCF – due to HTN, cardiomegaly. 3/10 pts has CCF)

18
Q

After observing a patient with acromegaly, what other examination steps to perform (percuss, pulpate, auscultate etc etc)

A

CVS

  • Cardiomegaly
  • CCF

Abdomen

  • Hepatosplenomegaly
  • Enlarged kidneys
  • Testicular atrophy

DRE: Colorectal CA: PR bleed, masses

Blood pressure: Hypertension

Urine dipstick: Glycosuria

Fundoscopy

  • Optic atrophy
  • Papilloedema – from raised ICP
  • Cataracts
  • Diabetic retinopathy
  • Hypertensive changes
19
Q

What are the investigations to be performed for acromegaly?

A

Serum IGF1: preferred over GH as it has longer T1/2 (hence more useful)

  • A single plasma level of IGF-1 reflects mean 24-hour GH lvl 🡪 HENCE useful in Dx
  • A normal IGF-1 + a random growth hormone <1 μg/L 🡪 can rule out acromegaly

If ↑ IGF-1 🡪 Perform OGTT 🡪 look for non-suppressibility of growth hormone

  • Measure GH levels at 0th (baseline GH level) & 2nd hour after administration
  • Acromegalics fail to suppress GH below 0.3 μg/L
  • About 25% of acromegalics have a positive diabetic glucose tolerance test.

If non-suppressible GH levels

  • Perform Pituitary MRI
  • Assess for pituitary function (if concurrent hypopituitarism)
20
Q

What are the complications of acromegaly?

A

Endocrine:

  • Panhypopituitarism due to compression of pituitary from adenoma
  • Diabetes

CV (main cause of death): CCF, HTN, HLD, Cardiomyopathy, Valvular Disease

Respiratory: OSA

Neoplasm: Colorectal CA

MSK: carpal tunnel syndrome, osteoarthritis, ulnar nerve palsy, proximal myopathy

21
Q

What is the pharmacological treatment of acromegaly?

A

(1) Pituitary Somatostatin Receptor: Somatostatin analogue (octreotide or lanreotide 1/month injection)

(2) Pituitary Dopamine Receptor
- Dopamine agonists act on D2-R
- Shrinks tumours prior to definitive Mx or to control S&S
- Most effective in mixed GH and Prolactin-producing tumours
- Bromocriptine or cabergoline

(3) Peripheral GH receptor antagonist
- Pegvisomant
- It is given by a daily injection and its main role at the present time is treatment of patients in whom GH and IGF levels cannot be reduced to safe levels with somatostatin analogues alone, surgery or radiotherapy.

Endocrine (18 decks)

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