A boy with McCune-Albright syndrome associated with GH secreting pituitary microadenoma. Clinical findings and response to treatment.
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.
Papadopoulou
Maria
M
3rd Department of Paediatrics, Aristotele University of Thessaloniki, Hippocration General Hospital, Thessaloniki, Greece.
Doula
Sofia
S
Kitsios
Kostas
K
Kaltsas
Themistoklis
T
Kosta
Konstantina
K
D000236:Adenoma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002648:Child; D005147:Facial Bones / Q000000981:diagnostic imaging; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005934:Glucagon / Q000097:blood; D005951:Glucose Tolerance Test; D007987:Gonadotropin-Releasing Hormone / Q000097:blood; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D008297:Male; D011629:Puberty, Precocious / Q000175:diagnosis / Q000188:drug therapy; D011877:Radionuclide Imaging; D012880:Skin Pigmentation; D013004:Somatostatin / Q000031:analogs & derivatives / Q000627:therapeutic use; D013738:Testolactone / Q000627:therapeutic use
D007987:Gonadotropin-Releasing Hormone; D013004:Somatostatin; D013738:Testolactone; D005934:Glucagon