title: Prospective study of histomorphometry, biochemical bone markers and bone densitometric response to pamidronate in β-thalassaemia presenting with osteopenia-osteoporosis syndrome.
abstract: This study aimed to evaluate bone remodelling disorders in thalassaemia by using pamidronate (PD) infusion with or without hormone replacement therapy (HRT) as a diagnostic-therapeutic tool. In this prospective study, 24 adult thalassaemia major (TM) and 10 thalassaemia intermedia (TI) patients received either PD and HRT or HRT only (controls) for 3 years. Eugonadal patients with TI had PD only. Bone remodelling was assessed by dual energy X ray absorptiometry (DXA scan), type 1-collagen biochemical bone markers (BBM) and histomorphometry of iliac crest biopsy before and after PD. As a group, thalassaemics had a significant improvement in spinal and femoral bone mineral density Z scores following PD (P < 0·01) compared to the controls. Although BBM were comparable pre-therapy, they were significantly lower in the PD cohort (P < 0·001) compared to the control group. All patients had osteopenia, diminished osteoid formation and bone volume on histomorphometry pre-therapy with high turnover bone disease (HTO) in TM and low-turnover disease (LTO) in TI. In TM, bone volume improved significantly, whereas TI patients showed little or no response to PD. In conclusion, histomorphometry data suggest that TM patients have a distinct pathology of high turnover bone disease compared to TI patients, who have low-turnover disease.
authors.affiliation: Institute For Women's Health, University College London, London, UK. r.chatterjee@ucl.ac.uk
authors.forename: Ratna
authors.initials: R
authors.lastname: Chatterjee
authors.forename: Farrukh T
authors.initials: FT
authors.lastname: Shah
authors.forename: Bernard A
authors.initials: BA
authors.lastname: Davis
authors.forename: Margaret
authors.initials: M
authors.lastname: Byers
authors.forename: Dev
authors.initials: D
authors.lastname: Sooranna
authors.forename: Rekha
authors.initials: R
authors.lastname: Bajoria
authors.forename: Jean
authors.initials: J
authors.lastname: Pringle
authors.forename: John B
authors.initials: JB
authors.lastname: Porter
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D015519:Bone Density / Q000187:drug effects; D001851:Bone Diseases, Metabolic / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D016723:Bone Remodeling / Q000187:drug effects; D001862:Bone Resorption / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D001842:Bone and Bones / Q000187:drug effects / Q000473:pathology; D016022:Case-Control Studies; D003720:Densitometry; D004164:Diphosphonates / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D010024:Osteoporosis / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D000077268:Pamidronate; D011446:Prospective Studies; D013577:Syndrome; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000188:drug therapy* / Q000473:pathology
chemical_list: D015415:Biomarkers; D004164:Diphosphonates; D000077268:Pamidronate