Pulsed-dosing with oral sodium phenylbutyrate increases hemoglobin F in a patient with sickle cell anemia.
Pediatr Blood Cancer
; 50(2): 357-9, 2008 Feb.
Article
en En
| MEDLINE
| ID: mdl-17253639
ABSTRACT
Increasing hemoglobin F (HbF) appears to be beneficial for patients with sickle cell anemia. We previously demonstrated that daily, oral sodium phenylbutyrate (OSPB) induces HbF synthesis in pediatric and adult patients with hemoglobin SS (HbSS). The high doses and need for daily therapy, however, have limited its use. Here, we report a patient treated with pulsed-dosing of OSPB for over 3 years. This patient developed a modest, but sustained elevation in HbF over the course of therapy without side effects. Although larger studies are needed, this case demonstrates that pulsed-dosing with OSPB enhances HbF synthesis.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Fenilbutiratos
/
Hemoglobina Fetal
/
Anemia de Células Falciformes
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2008
Tipo del documento:
Article
País de afiliación:
Estados Unidos