SPOTTERS MEDICINE

 

 

GIGANTISM 



 

🔹 Definition:

Gigantism is a rare condition caused by excessive secretion of growth hormone (GH) before epiphyseal closure (i.e., in children and adolescents), leading to abnormally increased linear growth and tall stature.


🔹 Cause:

  • GH-secreting pituitary adenoma (most common)
  • Hypothalamic GHRH-secreting tumors (rare)
  • Genetic conditions (e.g., McCune-Albright syndrome)
  • MEN-1 syndrome (rarely associated)

🔹 Pathophysiology:

  • Excess growth hormone → increased IGF-1 from liver
  • IGF-1 stimulates epiphyseal growth plates → excessive height and size
  • Affects long bones, soft tissues, and visceral organs

🔹 Clinical Features:

🔸 General Features:

  • Tall stature beyond genetic potential
  • Large hands and feet
  • Broad facial features
  • Mandibular prognathism (enlarged jaw)
  • Macroglossia (enlarged tongue)

🔸 Systemic Symptoms:

  • Hyperhidrosis (excessive sweating)
  • Headache, visual field defects (due to pituitary tumor compression)
  • Delayed puberty or hypogonadism
  • Joint pains and fatigue
  • Insulin resistance / Diabetes mellitus
  • Hypertension and cardiomegaly

🔹 Diagnosis:

🔸 Hormonal Tests:

  • Elevated serum IGF-1
  • Oral glucose suppression test: GH fails to suppress
  • Elevated GH levels (but may vary due to pulsatile secretion)

🔸 Imaging:

  • MRI brain – shows pituitary adenoma
  • Bone X-rays – open epiphyseal plates, increased bone length

🔹 Differential Diagnosis:

  • Marfan’s syndrome (tall, thin with hypermobility)
  • Klinefelter’s syndrome (tall males with hypogonadism)
  • Constitutional tall stature (familial)

🔹 Treatment:

🔸 Medical:

  • Somatostatin analogs (e.g., Octreotide) – suppress GH
  • GH receptor antagonists (e.g., Pegvisomant)
  • Dopamine agonists (e.g., Cabergoline – adjunct)

🔸 Surgical:

  • Transsphenoidal removal of pituitary tumorfirst-line in most cases

🔸 Radiotherapy:

  • For residual or inoperable tumors

🔹 Complications:

  • Diabetes mellitus
  • Cardiomyopathy and hypertension
  • Pituitary apoplexy
  • Psychological and social difficulties due to extreme height
  • Arthropathy (joint problems due to weight and size)

🔹 Prognosis:

  • Good with early treatment
  • Untreated gigantism leads to severe morbidity and reduced lifespan
  • Requires long-term follow-up for endocrine and growth monitoring

DR.C.GANESAN M.D.

PROFESSOR OF MEDICINE

 

 

 

 

 

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