Bone density and metabolism in thalassaemia.
Twenty-seven thalassaemic patients (13 F, 14 M, aged 8.1-14.9 yr), regularly transfused and chelated with desferrioxamine (30-40 mg/kg/day) were studied. Every patient was submitted to auxological evaluations, dual X-ray absorptiometry to measure bone mineral density (BMD), and to the determination of bone metabolic markers of osteoclastic activity (total urinary hydroxylysylpyridinoline crosslinks, carboxyterminal pyridinoline crosslinked telopeptide of type I collagen [ICTP]) and of osteoblastic activity (bone Gla protein [BGP] and carboxyterminal propeptide of type I procollagen [PIPC]). The evaluations were repeated after 1 year, during which 13 patients continued desferrioxamine chelation while 14 underwent deferiprone chelation (75 mg/kg/day in 3 doses). The data demonstrate widespread bone alterations consisting of osteoporosis, growth failure and bone age delay. Lumber spine (L2-L4) BMD areal values (Z score) inversely correlated with age, as did height SDS of both male and female patients, indicating osteoporosis progressing with age in parallel with growth insufficiency. No clear-cut alterations in bone mineral metabolism were found in basal state and after 1 year. Extensive MR imaging studies are needed to define the contribution of residual bone marrow hyperplasia to thalassaemic osteopathy suggested by subtle radiological signs as enlargement of bone marrow cavities with thinning of the cortical bone and abnormalities of the trabecules of spongy bone.
Lala
R
R
Department of Paediatric Endocrinology, Regina Margherita Children's Hospital, Turin, Italy.
Chiabotto
P
P
Di Stefano
M
M
Isaia
G C
GC
Garofalo
F
F
Piga
A
A
D015502:Absorptiometry, Photon; D000293:Adolescent; D000365:Age Determination by Skeleton; D000596:Amino Acids / Q000652:urine; D001803:Blood Transfusion; D001827:Body Height; D015519:Bone Density; D002648:Child; D003094:Collagen / Q000652:urine; D024042:Collagen Type I; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008297:Male; D010006:Osteoblasts / Q000378:metabolism; D015675:Osteocalcin / Q000097:blood; D010010:Osteoclasts / Q000378:metabolism; D010446:Peptide Fragments / Q000097:blood; D010455:Peptides / Q000652:urine; D011347:Procollagen / Q000097:blood; D011728:Pyridones / Q000627:therapeutic use; D017086:beta-Thalassemia / Q000378:metabolism / Q000503:physiopathology* / Q000628:therapy
D000596:Amino Acids; D024042:Collagen Type I; D007502:Iron Chelating Agents; D010446:Peptide Fragments; D010455:Peptides; D011347:Procollagen; D011728:Pyridones; C052929:collagen type I trimeric cross-linked peptide; C042813:procollagen type I carboxy terminal peptide; D015675:Osteocalcin; D000077543:Deferiprone; C015484:pyridinoline; D003094:Collagen; D003676:Deferoxamine