A boy with McCune-Albright syndrome associated with GH secreting pituitary microadenoma. Clinical findings and response to treatment

Hormones (Athens). 2006 Jul-Sep;5(3):205-9. doi: 10.14310/horm.2002.11186.

Abstract

The McCune-Albright Syndrome (MAS) is a sporadic rare disease first described in 1936 by McCune and separately by Albright. MAS is characterized by a triad of physical signs: café-au-lait spots, polyostotic fibrous dysplasia and autonomous endocrine hyperfunction. MAS is predominantly observed in girls and is rarely reported in males. We report the case of a 9-year old boy with gonadotropin independent precocious puberty, café-au-lait spots, polyostotic fibrous dysplasia and growth hormone hypersecretion.

Publication types

  • Case Reports

MeSH terms

  • Adenoma / complications*
  • Adenoma / diagnosis
  • Adenoma / drug therapy
  • Child
  • Facial Bones / diagnostic imaging
  • Fibrous Dysplasia, Polyostotic / complications*
  • Fibrous Dysplasia, Polyostotic / diagnosis
  • Fibrous Dysplasia, Polyostotic / drug therapy
  • Glucagon / blood
  • Glucose Tolerance Test
  • Gonadotropin-Releasing Hormone / blood
  • Growth Hormone-Secreting Pituitary Adenoma / complications*
  • Growth Hormone-Secreting Pituitary Adenoma / diagnosis
  • Growth Hormone-Secreting Pituitary Adenoma / diagnostic imaging
  • Growth Hormone-Secreting Pituitary Adenoma / drug therapy
  • Humans
  • Male
  • Puberty, Precocious / diagnosis
  • Puberty, Precocious / drug therapy
  • Radionuclide Imaging
  • Skin Pigmentation
  • Somatostatin / analogs & derivatives
  • Somatostatin / therapeutic use
  • Testolactone / therapeutic use

Substances

  • Gonadotropin-Releasing Hormone
  • Somatostatin
  • Testolactone
  • Glucagon