+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Prevalence of low bone mass among adolescents with nontransfusion-dependent hemoglobin E/β-thalassemia and its relationship with anemia severity

Prevalence of low bone mass among adolescents with nontransfusion-dependent hemoglobin E/β-thalassemia and its relationship with anemia severity

Pediatric Blood and Cancer 65(1)

Low bone mass is common among adolescents with transfusion-dependent β-thalassemia despite adequate transfusion and iron chelation. However, there are few reports regarding bone mineral density (BMD) among adolescents with nontransfusion-dependent thalassemia (NTDT). Indeed, only BMD data in patients with nontransfusion-dependent (NTD) β-thalassemia intermedia have been reported. No previous study has investigated BMD among adolescents with NTD hemoglobin (Hb) E/β-thalassemia. To determine the prevalence of low bone mass among adolescents with NTD Hb E/β-thalassemia and factors relating to low bone mass. We investigated BMD of lumbar spine (L2-L4; BMDLS) and total body (BMDTB), as measured by dual-energy X-ray absorptiometry, in 22 adolescents (aged 13.2-20 years) with NTD Hb E/β-thalassemia. Low bone mass was found to be 18.2% and 22.7% at the lumbar spine (BMDLS Z-score adjusted for bone age and height age) and 13.6% and 9.1% at the total body (BMDTB Z-score adjusted for bone age and height age). Patients with mean Hb level <8 g/dl were more likely to have low bone mass (BMDLS and BMDTB Z-scores adjusted for bone age) compared to those with Hb level ≥ 8 g/dl. Mean Hb level correlated with BMDLS and BMDTB Z-scores adjusted for bone age. We demonstrated that a low Hb level was associated with low bone mass among adolescents with NTD Hb E/β-thalassemia. A significant proportion of low bone mass among these patients highlights the importance of appropriate management, including red cell transfusion, vitamin D and calcium supplementation for improved long-term bone health.

Please choose payment method:

(PDF emailed within 0-6 h: $19.90)

Accession: 060128815

Download citation: RISBibTeXText

PMID: 28801997

DOI: 10.1002/pbc.26744

Related references

A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease). American Journal of Hematology 93(5): 623-629, 2018

Prevalence and Risk Factors for Complications in Patients with Nontransfusion Dependent Alpha- and Beta-Thalassemia. Anemia 2015: 793025, 2015

Relationship of hemoglobin f and alpha thalassemia to severity of sickle cell anemia in the eastern province of saudi arabia. Annals of Tropical Paediatrics 6(4): 261-266, 1986

Detection of abnormal left ventricular diastolic function in nontransfusion-dependent patients with beta-thalassemia/hemoglobin E disease by Doppler echocardiography. Blood 88(10 SUPPL 1 PART 1-2): 27B, 1996

Mutation in a Highly Conserved COOH-Terminal Residue of Krüppel-Like Factor 1 Associated with Elevated Hb F in a Compound Heterozygous β-Thalassemia Patient with a Nontransfusion-Dependent Thalassemia Phenotype. Hemoglobin 40(5): 361-364, 2017

Prevalence and severity of asthma among adolescents and their relationship with the body mass index. Jornal de Pediatria 81(4): 305-309, 2005

Effects of alpha-thalassemia and hemoglobin F level on the severity of sickle-cell anemia. Beuzard, Y, Lubin, B, Rosa, J, INSERM Colloquium; Sickle cell disease and thalassaemias: New trends in therapy, Colloque INSERM; Sickle cell disease and thalassaemias: New trends in therapy 223, 1995

Neuroimaging Abnormalities in Patients With Nontransfusion-dependent Thalassemia. Journal of Pediatric Hematology/Oncology 2019, 2019

Novel approach to reactive oxygen species in nontransfusion-dependent thalassemia. Biomed Research International 2014: 350432, 2015

Epidemiologic and clinical characteristics of nontransfusion-dependent thalassemia in the United States. Pediatric Blood and Cancer 65(7): E27067, 2018

Hydroxyurea for nontransfusion-dependent β-thalassemia: A systematic review and meta-analysis. Hematology/Oncology and Stem Cell Therapy 10(3): 116-125, 2017

Circulating cell-free DNA and ineffective erythropoiesis in nontransfusion-dependent β-thalassemia. American Journal of Hematology 93(11): E365-E368, 2018

Study of alpha hemoglobin stabilizing protein expression in patients with β thalassemia and sickle cell anemia and its impact on clinical severity. Blood Cells, Molecules and Diseases 55(4): 358-362, 2016

Phenotype and Genotype in a Cohort of 312 Adult Patients with Nontransfusion-Dependent Thalassemia in Northeast Thailand. Acta Haematologica 135(1): 15-20, 2016

Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia. Blood Cells, Molecules and Diseases 55(2): 104-107, 2016