Low bone mass in prepubertal children with thalassemia major: insights into the pathogenesis of low bone mass in thalassemia.
OBJECTIVE
Low bone mass occurs frequently in the aging thalassemic population. However, limited information exists on bone mass in children with thalassemia major (TM) during their first decade of life.


METHODS
Spinal bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry (DEXA) in 18 children (age 5.8 +/- 1.5 yr; M:F 8:10) with TM on hypertransfusion and iron chelation therapy. Serial BMD measurements were available for 11 of the 18 children.


RESULTS
Weight and height z scores were 0.81 +/- 4.2 and -0.47 +/- 1.7 respectively. At the first BMD, four (22.2%) patients presented with BMD z scores less than -2.5, seven (38.8%) had BMD z scores between -1 and -2.5, while the remaining seven (38.8%) had normal BMDs (z score above -1). The mean decline of BMD z score was -0.38/year (p = ns). BMD z scores correlated with height z scores (p = 0.039), but not with liver enzymes, serum ferritin levels, or thalassemia genotypes.


CONCLUSIONS
Low bone mass is present in most children with TM despite hypertransfusion and optimal chelation, adequate growth and lack of endocrine complications.
Vogiatzi
Maria G
MG
Department of Pediatrics, The New York-Presbyterian Hospital/Weill Medical College of Cornell University, NY, USA.
Autio
Karen A
KA
Schneider
Robert
R
Giardina
Patricia J
PJ
D001803:Blood Transfusion; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000175:diagnosis* / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010024:Osteoporosis / Q000175:diagnosis* / Q000209:etiology*; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy