title: Treatment with deferiprone (L1) in a thalassemic patient with bone lesions due to desferrioxamine.
abstract: A male thalassemia major patient who developed bone and cartilage abnormalities with a standard dose of desferrioxamine (DFX) given subcutaneously from the age of 4 years was treated with the oral iron chelator deferiprone (L1). During L1 treatment an improvement of genu valgum, evidence of healing and filling in of bone at the periphery of knee metaphysis and improvement in growth velocity were observed. However, the sitting height had decreased further, confirming the irreversibility of platyspondylosis, so affecting the near final standing height (156.8 cm) which was below the mid-parental height (168 cm). Prospective studies are warranted to determine the effect of different iron chelators on the bone metabolism of patients with thalassemia.
authors.affiliation: Department of Pediatrics, Arcispedale S. Anna, Siracusa, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Mangiagli
authors.forename: V
authors.initials: V
authors.lastname: De Sanctis
authors.forename: S
authors.initials: S
authors.lastname: Campisi
authors.forename: G
authors.initials: G
authors.lastname: Di Silvestro
authors.forename: L
authors.initials: L
authors.lastname: Urso
mesh_terms: D001827:Body Height; D001847:Bone Diseases / Q000139:chemically induced*; D000077543:Deferiprone; D003676:Deferoxamine / Q000009:adverse effects*; D006130:Growth Disorders / Q000139:chemically induced*; D006801:Humans; D007223:Infant; D007502:Iron Chelating Agents / Q000009:adverse effects* / Q000627:therapeutic use; D007717:Knee; D008297:Male; D011728:Pyridones / Q000627:therapeutic use*; D017086:beta-Thalassemia / Q000188:drug therapy*
chemical_list: D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine