Pamidronate is an effective treatment for osteoporosis in patients with beta-thalassaemia.
Osteoporosis in beta-thalassaemia is multifactorial; increased osteoclast function seems to play an important role in its pathogenesis. The aim of this study was to evaluate the effect of pamidronate on the osteoporosis of thalassaemia. To this effect we studied 26 patients who received this drug in doses of 30 or 60 mg i.v. once a month over 12 months. The effects were monitored by measuring bone mineral density (BMD) in association with markers of osteoclast function [soluble receptor activator of nuclear factor-kappa B ligand (sRANKL), osteoprotegerin (OPG)] and of bone remodelling [N-telopeptide of collagen type-I (NTX), tartrate-resistant acid phosphatase isoform-5b (TRACP-5b), bone-alkaline phosphatase (bALP), and osteocalcin (OC)]. Thirty healthy individuals were also studied, as controls. NTX, TRACP-5b, bALP and OC levels were significantly higher in thalassaemic patients compared with controls; in contrast, OPG levels were significantly lower, while the levels of sRANKL varied within normal limits. Administration of pamidronate was followed by a clear decrease of NTX, TRACP-5b, OPG, and OC, and by a significant increase in the BMD of the lumbar spine, which was similar in patients of both treatment groups. These data suggest that pamidronate, at a monthly dose of 30 mg, is an effective treatment for thalassaemic osteoporosis.
Voskaridou
Ersi
E
Thalassaemia Centre, Laikon General Hospital, Athens, Greece. ersi_voskaridou@yahoo.com
Terpos
Evangelos
E
Spina
Giovanna
G
Palermos
John
J
Rahemtulla
Amin
A
Loutradi
Aphroditi
A
Loukopoulos
Dimitris
D
D000135:Acid Phosphatase / Q000097:blood; D000328:Adult; D000469:Alkaline Phosphatase / Q000097:blood; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood / Q000652:urine; D001803:Blood Transfusion; D015519:Bone Density / Q000187:drug effects; D002352:Carrier Proteins / Q000097:blood; D016022:Case-Control Studies; D003094:Collagen / Q000652:urine; D024042:Collagen Type I; D004164:Diphosphonates / Q000008:administration & dosage* / Q000627:therapeutic use; D004334:Drug Administration Schedule; D005260:Female; D006023:Glycoproteins / Q000097:blood; D006801:Humans; D007527:Isoenzymes / Q000097:blood; D008159:Lumbar Vertebrae; D008297:Male; D008562:Membrane Glycoproteins / Q000097:blood; D008875:Middle Aged; D015675:Osteocalcin / Q000097:blood; D010024:Osteoporosis / Q000188:drug therapy* / Q000209:etiology / Q000628:therapy; D053244:Osteoprotegerin; D000077268:Pamidronate; D010455:Peptides / Q000652:urine; D053245:RANK Ligand; D053246:Receptor Activator of Nuclear Factor-kappa B; D018160:Receptors, Cytoplasmic and Nuclear / Q000097:blood; D018124:Receptors, Tumor Necrosis Factor; D018709:Statistics, Nonparametric; D000071681:Tartrate-Resistant Acid Phosphatase; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000628:therapy
D000893:Anti-Inflammatory Agents; D015415:Biomarkers; D002352:Carrier Proteins; D024042:Collagen Type I; D004164:Diphosphonates; D006023:Glycoproteins; D007527:Isoenzymes; D008562:Membrane Glycoproteins; D053244:Osteoprotegerin; D010455:Peptides; D053245:RANK Ligand; D053246:Receptor Activator of Nuclear Factor-kappa B; D018160:Receptors, Cytoplasmic and Nuclear; D018124:Receptors, Tumor Necrosis Factor; C505686:TNFRSF11A protein, human; C506030:TNFRSF11B protein, human; C505688:TNFSF11 protein, human; C052929:collagen type I trimeric cross-linked peptide; D015675:Osteocalcin; D003094:Collagen; D000469:Alkaline Phosphatase; C000605510:ACP5 protein, human; D000135:Acid Phosphatase; D000071681:Tartrate-Resistant Acid Phosphatase; D000077268:Pamidronate