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Mc Cune Albright syndrome

Clinical Features

  • Precocious Puberty
  • Fibrous Dysplasia of Long Bones
  • Cafe au lait spots( irregular macules confined to one side of the body)

Explanation

McCune Albright Syndrome occurs due to mutation in the GNAS gene which leads to constant G- protein activation which leads to overproduction of pituitary hormones. Thus in addition to precocious puberty ( GnRH independent increase in LH and FSH), MAS can cause an increase in TSH and GH and ACTH leading to Cushing syndrome.

BVC

ABC

Insulinoma reference example[1]

Reference

  1. Metz DC, Jensen RT (November 2008). "Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors". Gastroenterology. 135 (5): 1469–92. doi:10.1053/j.gastro.2008.05.047. PMC 2612755. PMID 18703061.