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Pediatr Blood Cancer ; 55(6): 1187-90, 2010 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-20672367

RESUMO

Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare clinical syndrome characterized by increased bone density and a severe, myelophthisic anemia. Few cases have been reported worldwide and there are no detailed descriptions of the associated hematologic abnormalities and long-term clinical outcomes after treatment. Here, we report two siblings with GHDD who were successfully treated with chronic, low dose, corticosteroid therapy. Although GHDD is uncommon, these cases illustrate the need to consider GHDD in patients with anemia and bone dysplasia and the use of chronic, low-dose steroid therapy.


Assuntos
Anemia Mielopática/etiologia , Anti-Inflamatórios/uso terapêutico , Síndrome de Camurati-Engelmann/complicações , Prednisona/uso terapêutico , Adulto , Anemia Mielopática/tratamento farmacológico , Síndrome de Camurati-Engelmann/diagnóstico , Síndrome de Camurati-Engelmann/tratamento farmacológico , Feminino , Humanos , Lactente , Masculino , Irmãos , Resultado do Tratamento
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