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Horm Res Paediatr ; 84(3): 184-9, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26227563

RESUMO

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. OBJECTIVES: To evaluate the efficacy of tamoxifen in improving the FH prediction (FHP) in patients with MAS. METHOD: 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). CONCLUSION: Our results support a role for tamoxifen in improving the FHP in patients with MAS.


Assuntos
Estatura/efeitos dos fármacos , Antagonistas de Estrogênios/uso terapêutico , Displasia Fibrosa Poliostótica/tratamento farmacológico , Tamoxifeno/uso terapêutico , Adolescente , Desenvolvimento Ósseo/efeitos dos fármacos , Manchas Café com Leite/complicações , Criança , Pré-Escolar , Estradiol/sangue , Antagonistas de Estrogênios/efeitos adversos , Feminino , Displasia Fibrosa Poliostótica/patologia , Seguimentos , Gonadotropinas/sangue , Humanos , Ovário/patologia , Puberdade Precoce/prevenção & controle , Estudos Retrospectivos , Tamoxifeno/efeitos adversos , Resultado do Tratamento
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