title: Tamoxifen Improves Final Height Prediction in Girls with McCune-Albright Syndrome: A Long Follow-Up.
abstract: BACKGROUND
  McCune-Albright syndrome (MAS) is a genetic disorder characterized by the triad of fibrous dysplasia, skin hyperpigmentation, and autonomous hyperfunction of various endocrine organs. MAS frequently presents in females as precocious puberty (PP). Although many treatments have been proposed, the preservation of final height (FH) in these patients remains a challenge.
  
  
  OBJECTIVE
  To evaluate the efficacy of tamoxifen in improving the FH prediction (FHP) in patients with MAS.
  
  
  METHODS
  We retrospectively analyzed 8 female patients with MAS who presented with café-au-lait spots and gonadotropin-independent PP. The patients were followed for a mean period of 8.3 years (range: 3-16).
  
  
  RESULTS
  All patients were treated with tamoxifen (10-20 mg/day) for 3-8 years (mean ± SD: 5.75 ± 2.05), which resulted in the cessation of vaginal bleeding and the stabilization of bone age maturation. There was a significant difference between the FHP at the beginning of treatment and at the end of treatment (145.1 ± 8.6 cm; Z score -2.84 ± 1.44 cm) and at the last evaluation (157.0 ± 9.2 cm; Z score -0.85 ± 0.54 cm; p < 0.001).
  
  
  CONCLUSIONS
  Our results support a role for tamoxifen in improving the FHP in patients with MAS.
authors.affiliation: Pediatric Endocrinology Unit, Instituto da Crianx00E7;a do Hospital das Clx00ED;nicas da Faculdade de Medicina da Universidade de Sx00E3;o Paulo, Sx00E3;o Paulo, Brazil.
authors.forename: Caroline
authors.initials: C
authors.lastname: de G Buff Passone
authors.forename: Hilton
authors.initials: H
authors.lastname: Kuperman
authors.forename: Hamilton
authors.initials: H
authors.lastname: Cabral de Menezes-Filho
authors.forename: Lygia
authors.initials: L
authors.lastname: Spassapan Oliveira Esteves
authors.forename: Rachel
authors.initials: R
authors.lastname: Lana Obata Giroto
authors.forename: Durval
authors.initials: D
authors.lastname: Damiani
mesh_terms: D000293:Adolescent; D001827:Body Height / Q000187:drug effects*; D001846:Bone Development / Q000187:drug effects; D019080:Cafe-au-Lait Spots / Q000150:complications; D002648:Child; D002675:Child, Preschool; D004958:Estradiol / Q000097:blood; D004965:Estrogen Antagonists / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000188:drug therapy* / Q000473:pathology; D005500:Follow-Up Studies; D006062:Gonadotropins / Q000097:blood; D006801:Humans; D010053:Ovary / Q000473:pathology; D011629:Puberty, Precocious / Q000517:prevention & control; D012189:Retrospective Studies; D013629:Tamoxifen / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D004965:Estrogen Antagonists; D006062:Gonadotropins; D013629:Tamoxifen; D004958:Estradiol