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

Alireza Abdollah Shamshirsaz,Reza Hashemi,Shahriar Aghakhani,Amirhooshang Abdollah Shamshirsaz,Navid Bouzari,Mir Reza Bekheirnia,Mohammadreza Habibzadeh,Hooman Homayoun,Nima Pourzahedgilani,Bagher Larijani,Mohammad Kamgar

doi:10.1186/1472-6823-3-4

Abstract

BackgroundThe 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.MethodsTwo 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.ResultsShort 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.ConclusionHigh prevalence of complications among our thalassemics signifies the importance of more detailed studies along with therapeutic interventions.

Highlights

The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients
Hypogonadism was seen in 22.9% of boys and 12.2% of girls with the mean serum ferritin level equal to 1787 ± 988 μg/l that was significantly higher than patients without hypogonadism (P = 0.036)
It is unclear whether diabetes in β-thalassemia major is related to genetic factors [14,16]

Summary

Introduction

The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients. The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients who can survive into their fourth and fifth decades of life [2,3] On the other hand, frequent blood transfusion in turn can lead to iron overload which may result in hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism and other endocrine abnormalities [4]. Several authors reported a high incidence of endocrine abnormalities in children, adolescents and young adults suffering from thalassemia major. The incidence of the various endocrinopathies changes among different series of the patients due to a mixture of reasons other than iron overloads [5].

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