title: Diamond-Blackfan anemia: clinical features and treatment results in 4 cases.
abstract: Diamond-Blackfan anemia is a rare hematological disease characterized by selective marrow erythroid hypoplasia. We present the clinical and hematological profile and results of treatment in four children. The median age at presentation was 14 months with a median age of onset of pallor at 7.5 months. Mean Hb at presentation was 2.9 g/dl. All children were started on prednisolone with response in one child. One child was switched to dexamethasone and responded transiently relapsing in 4 months but responded to further dexamethasone. Two children required cyclosporin for 3 and 8 weeks for response. With median follow-up of 59 months, all four children continue in complete hematological remission with no further relapses.
authors.affiliation: Department of Pediatrics, Kalawati Saran Childrens Hospital, Lady Hardinge Medical College, New Delhi 110001, India. dr_rahul6@hotmail.com
authors.forename: Rahul
authors.initials: R
authors.lastname: Naithani
authors.forename: Jagdish
authors.initials: J
authors.lastname: Chandra
authors.forename: Shashi
authors.initials: S
authors.lastname: Narayan
authors.forename: Varinder
authors.initials: V
authors.lastname: Singh
authors.forename: A K
authors.initials: AK
authors.lastname: Dutta
mesh_terms: D029503:Anemia, Diamond-Blackfan / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction
chemical_list: D006454:Hemoglobins; D007166:Immunosuppressive Agents; D003907:Dexamethasone; D016572:Cyclosporine; D011239:Prednisolone