title: Severe osteopenia with recurrent fractures after bone marrow transplant for Wiskott-Aldrich syndrome: a case report.
abstract: Wiskott-Aldrich syndrome (WAS) is a rare inherited disorder characterised by thrombocytopenia, eczema, and immunodeficiency. Bone marrow transplantation (BMT) is a well-established modality of treatment now routinely used and often curative. We report the case of a boy who developed osteopenia and sustained multiple long-bone fractures over a 5-year period after bone marrow transplant for WAS. The femora and tibiae of both lower limbs were involved with a clinical presentation similar to osteogenesis imperfecta. After commencing calcitriol treatment at the age of 8 years, the patient has not sustained any further fractures. He is now 11 years old. Although short-term changes in bone metabolism after BMT have been documented, the occurrence of repeated fractures associated with osteopenia has not been previously reported.
authors.affiliation: Department of Orthopaedics, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
authors.forename: I S
authors.initials: IS
authors.lastname: Talkhani
authors.forename: D
authors.initials: D
authors.lastname: Reidy
authors.forename: E E
authors.initials: EE
authors.lastname: Fogarty
authors.forename: A
authors.initials: A
authors.lastname: O'Meara
mesh_terms: D001851:Bone Diseases, Metabolic / Q000188:drug therapy / Q000209:etiology*; D016026:Bone Marrow Transplantation; D002117:Calcitriol / Q000627:therapeutic use; D002120:Calcium Channel Agonists / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications; D014923:Wiskott-Aldrich Syndrome / Q000601:surgery*
chemical_list: D002120:Calcium Channel Agonists; D002117:Calcitriol