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Thalassemia Bone Disease: A 19‐Year Longitudinal Analysis
Journal of bone and mineral research, 2014-11, Vol.29 (11), p.2468-2473
Wong, Phillip
Fuller, Peter J
Gillespie, Matthew T
Kartsogiannis, Vicky
Kerr, Peter G
Doery, James CG
Paul, Eldho
Bowden, Donald K
Strauss, Boyd J
Milat, Frances
2014
Volltextzugriff (PDF)
Details
Autor(en) / Beteiligte
Wong, Phillip
Fuller, Peter J
Gillespie, Matthew T
Kartsogiannis, Vicky
Kerr, Peter G
Doery, James CG
Paul, Eldho
Bowden, Donald K
Strauss, Boyd J
Milat, Frances
Titel
Thalassemia Bone Disease: A 19‐Year Longitudinal Analysis
Ist Teil von
Journal of bone and mineral research, 2014-11, Vol.29 (11), p.2468-2473
Ort / Verlag
United States: Wiley Subscription Services, Inc
Erscheinungsjahr
2014
Quelle
MEDLINE
Beschreibungen/Notizen
ABSTRACT Thalassemia is an inherited disorder of alpha or beta globin chain synthesis leading to ineffective erythropoiesis requiring chronic transfusion therapy in its most severe form. This leads to iron overload, marrow expansion, and hormonal complications, which are implicated in bone deformity and loss of bone mineral density (BMD). In this 19‐year retrospective longitudinal study, the relationships between BMD (determined by dual‐energy X‐ray absorptiometry) and risk factors for osteoporosis in 277 subjects with transfusion‐dependent thalassemia were examined. The mean age at first review was 23.2 ± 11.9 years and 43.7% were male. Hypogonadism was present in 28.9%. Fractures were confirmed in 11.6% of subjects and were more frequent in males (16.5%) compared with females (7.7%). Lumbar spine (LS), femoral neck (FN), and total body (TB) Z‐scores were derived. Patients with transfusion‐dependent thalassemia had a significant longitudinal decline in BMD at the FN and TB. In the linear mixed‐model analysis of BMD and risk factors for bone loss, FN Z‐score was more significantly associated with risk factors compared with the LS and TB. The rate of decline at the FN was 0.02 Z‐score per year and was 3.85‐fold greater in males. The decline in FN Z‐score over the last 5 years (years 15 to 19) was 2.5‐fold that of the previous 7 years (years 8 to 14) and coincided with a change in iron chelator therapy from desferrioxamine to deferasirox. Hemoglobin (Hb) levels positively correlated with higher TB and LS Z‐scores. In conclusion, the FN is the preferred site for follow‐up of BMD. Male patients with β‐thalassemia experienced a greater loss of BMD and had a higher prevalence of fractures compared with females. Transfusing patients (particularly males) to a higher Hb target may reduce the decline in BMD. Whether deferasirox is implicated in bone loss warrants further study. © 2014 American Society for Bone and Mineral Research.
Sprache
Englisch
Identifikatoren
ISSN: 0884-0431
eISSN: 1523-4681
DOI: 10.1002/jbmr.2266
Titel-ID: cdi_proquest_miscellaneous_1615259066
Format
–
Schlagworte
Adult
,
Blood Transfusion
,
BONE DENSITY
,
BONE DISEASE
,
Child
,
Female
,
Follow-Up Studies
,
Humans
,
Iron Chelating Agents - administration & dosage
,
Iron Overload - etiology
,
Iron Overload - metabolism
,
Iron Overload - pathology
,
Iron Overload - therapy
,
Longitudinal Studies
,
Male
,
OSTEOPOROSIS
,
Osteoporosis - etiology
,
Osteoporosis - metabolism
,
Osteoporosis - pathology
,
Osteoporosis - therapy
,
Sex Factors
,
THALASSEMIA
,
Thalassemia - complications
,
Thalassemia - etiology
,
Thalassemia - metabolism
,
Thalassemia - pathology
,
Thalassemia - therapy
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