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Open Access Highly Accessed Research article

Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran

Alireza Abdollah Shamshirsaz12*, Mir Reza Bekheirnia12, Mohammad Kamgar12, Nima Pourzahedgilani1, Navid Bouzari1, Mohammadreza Habibzadeh1, Reza Hashemi1, Amirhooshang Abdollah Shamshirsaz3, Shahriar Aghakhani1, Hooman Homayoun2 and Bagher Larijani12

Author Affiliations

1 Endocrinology and Metabolism Research Center-Tehran University of Medical Sciences and Health Services, Iran

2 Charity foundation for special diseases, Iran

3 Shahid Beheshti University of Medical Sciences and Health Services, Iran

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BMC Endocrine Disorders 2003, 3:4  doi:10.1186/1472-6823-3-4

Published: 12 August 2003

Abstract

Background

The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients. The main objective of this study is to determine the prevalence of prominent thalassemia complications.

Methods

Two hundred twenty patients entered the study. Physicians collected demographic and anthropometric data and the history of therapies as well as menstrual histories. Patients have been examined to determine their pubertal status. Serum levels of 25(OH) D, calcium, phosphate, iPTH were measured. Thyroid function was assessed by T3, T4 and TSH. Zinc and copper in serum were determined by flame atomic absorption spectrophotometry. Bone mineral density (BMD) measurements at lumbar and femoral regions have been done using dual x-ray absorptiometry. The dietary calcium, zinc and copper intakes were estimated by food-frequency questionnaires.

Results

Short stature was seen in 39.3% of our patients. Hypogonadism was seen in 22.9% of boys and 12.2% of girls. Hypoparathyroidism and primary hypothyroidism was present in 7.6% and 7.7% of the patients. About 13 % of patients had more than one endocrine complication with mean serum ferritin of 1678 ± 955 micrograms/lit. Prevalence of lumbar osteoporosis and osteopenia were 50.7% and 39.4%. Femoral osteoporosis and osteopenia were present in 10.8% and 36.9% of the patients. Lumbar BMD abnormalities were associated with duration of chelation therapy. Low serum zinc and copper was observed in 79.6% and 68% of the study population respectively. Serum zinc showed significant association with lumbar but not femoral BMD. In 37.2% of patients serum levels of 25(OH) D below 23 nmol/l were detected.

Conclusion

High prevalence of complications among our thalassemics signifies the importance of more detailed studies along with therapeutic interventions.