ACROMEGALY

ACROMEGALY


It is a disorder that results from excess growth hormone(GH) after the growth plates have closed (excess GH after puberty)


It is a diseases of anterior pituitary


  • Somatotrophs are abundant cells in pituitary
  • somatotroph adenoma is most common cause of developing Acromegaly

Mammosomatotroph adenoma
  • growth hormone and prolactin increases
  • clinical features : Galactorrhea

Leading cause of extra pituitary source of excess growth hormone → pancreatic adenoma (islet cells tumor)

Leading cause of excessive GHRH production → CARCINOID TUMOR


CLINICAL FEATURES :


  • ACRAL ENLARGEMENT :
Increase in size of hand/feet
Increase in finger thickness leading to spade like hands
Lower Jaw prominence called as Prognathism
  • Frontal Bossing
  • Heel pad thickness >21 mm → used for monitoring growth in these patients


  • Coarse facies / deep hollow voice ,Hyperhidrosis(excessive sweating) , sebum increase (increase incidence of acne) , acanthosis nigricans , skin tags



  • Premature osteoarthritis → in bilateral limbs → due to weight gain
  • Weakness ,fatigue
  • Increase peripheral resistance → resulting in hypertension
  • Increase growth hormone results in impaired glucose tolerance
FBS (fasting blood sugar) = 100-125 mg%
2 hrs = 140-199 mg%
  • obstructive sleep apnea due to growth of tongue
  • colonic polyps
  • Galactorrhea
  • visual deficit (bilateral hemianopia)



WORKUP :

  • SCREENING : IGF-1(Insulin like growth factor -1)
  • IOC : Failure to suppress growth hormone levels with oral glucose load
  • serum prolactin levels increase
  • MRI head - size of tumor > 10mm
  • X-ray of lateral aspect of foot is done to see "HEEL PAD THICKNESS"


TREATMENT :

  • Trans-sphenoidal surgery, after surgery
first to improve is → soft tissue swelling
After (1-2hrs) → growth hormone levels decreases
After (3-4hrs) → IGF-1 decreases
  • Prior to the surgery,shrink the size of the tumor using drugs → somatostatin analogues
Octreotide
Lanreotide
Pasireotide

acts on SSTR 2 , SSTR 5 Receptors → Growth hormone decrease
  • PEGVISOMANT → Growth hormone receptor antagonist , hence growth hormone levels increase due to feedback (used in case of recurrence of tumor)
  • Radiation

 



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