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Successful management of anemia with sirolimus in blue rubber bleb nevus syndrome: update
Fernández Gil, Marta; López Serrano, Pilar; García García, Elena.
Afiliação
  • Fernández Gil, Marta; Fundación Hospital de Alcorcón. Department of Gastroenterology and Hepatology. Alcorcón. Spain
  • López Serrano, Pilar; Fundación Hospital de Alcorcón. Department of Gastroenterology and Hepatology. Alcorcón. Spain
  • García García, Elena; Fundación Hospital de Alcorcón. Department of Gastroenterology and Hepatology. Alcorcón. Spain
Rev. esp. enferm. dig ; 111(8): 643-647, ago. 2019. ilus, tab, graf
Artigo em Espanhol | IBECS | ID: ibc-190339
Biblioteca responsável: ES1.1
Localização: BNCS
ABSTRACT
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic skin hemangiomas and vascular malformations, mostly in the gastrointestinal (GI) tract. The GI lesions are mainly located in the stomach and small intestine, usually more than a hundred, leading to gastrointestinal bleeding and severe chronic anemia. Parenteral iron infusions and scheduled transfusions are frequently necessary. We describe the case of a 21-year-old male with anemia secondary to BRBNS, who becomes unresponsive to octreotide and shows an excellent response to sirolimus (SRL), dismissing the intravenous iron supplementations and being free of transfusions. During the treatment, the patient presents avascular hip necrosis, which is adequately treated with an injection of stem cells with complete recovery, and without the suspension of SRL. Two years later, adequate response persists with no other relevant side effects
RESUMEN
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Assuntos
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Síndrome do Nevo Displásico / Sirolimo / Anemia Limite: Humanos / Masculino Idioma: Espanhol Revista: Rev. esp. enferm. dig Ano de publicação: 2019 Tipo de documento: Artigo Instituição/País de afiliação: Fundación Hospital de Alcorcón/Spain
Buscar no Google
Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Síndrome do Nevo Displásico / Sirolimo / Anemia Limite: Humanos / Masculino Idioma: Espanhol Revista: Rev. esp. enferm. dig Ano de publicação: 2019 Tipo de documento: Artigo Instituição/País de afiliação: Fundación Hospital de Alcorcón/Spain
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