Beta-thalassaemia Intermedia: Evaluation of Endocrine and Bone Complications

Overview

Journal Biomed Res Int

Publisher Wiley

Specialties Biomedical Engineering
Biotechnology
General Medicine

Date 2014 Aug 12

PMID 25110660

Citations 13

Authors

M Baldini

A Marcon

R Cassin

F M Ulivieri

D Spinelli

M D Cappellini

G Graziadei

Affiliations

Soon will be listed here.

Abstract

Objective: Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients.

Methods: We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentration (LIC) from T2*, transfusion regimen, iron chelation, hydroxyurea, splenectomy, and bone mineralization by dual X-ray absorptiometry.

Results: Thirty-seven (53%) males and 33 (47%) females were studied, with mean age 41 ± 12 years, mean haemoglobin 9.2 ± 1.5 g/dL, median ferritin 537 (range 14-4893), and mean LIC 7.6 ± 6.4 mg Fe/g dw. Thirty-three patients (47%) had been transfused, occasionally (24/33; 73%) or regularly (9/33; 27%); 37/70 (53%) had never been transfused; 34/70 patients had been splenectomized (49%); 39 (56%) were on chelation therapy; and 11 (16%) were on hydroxyurea. Endocrinopathies were found in 15 patients (21%): 10 hypothyroidism, 3 hypogonadism, 2 impaired glucose tolerance (IGT), and one diabetes. Bone disease was observed in 53/70 (76%) patients, osteoporosis in 26/53 (49%), and osteopenia in 27/53 (51%).

Discussion And Conclusions: Bone disease was found in most patients in our study, while endocrinopathies were highly uncommon, especially hypogonadism. We speculate that low iron burden may protect against endocrinopathy development.

Citing Articles

Pancreatic Volume in Thalassemia: Determinants and Association with Alterations of Glucose Metabolism.

Meloni A, Restaino G, Positano V, Pistoia L, Keilberg P, Santodirocco M Diagnostics (Basel). 2025; 15(5).

PMID: 40075815 PMC: 11899254. DOI: 10.3390/diagnostics15050568.

Impact of genotype on multi-organ iron and complications in patients with non-transfusion-dependent β-thalassemia intermedia.

Meloni A, Pistoia L, Ricchi P, Bagnato S, Longo F, Messina G Ann Hematol. 2024; 103(6):1887-1896.

PMID: 38581547 DOI: 10.1007/s00277-024-05741-9.

A retrospective study of glucose homeostasis, insulin secretion, sensitivity/resistance in non- transfusion-dependent β-thalassemia patients (NTD- β Thal): reduced β-cell secretion rather than insulin resistance seems to be the dominant defect for....

De Sanctis V, Daar S, Soliman A, Tzoulis P, Yassin M, Kattamis C Acta Biomed. 2023; 94(6):e2023262.

PMID: 38054678 PMC: 10734240. DOI: 10.23750/abm.v94i6.15001.

Molecular Characterization and Disease-Related Morbidities of β-Thalassemia Patients from the Northeastern Part of Iraq.

Amin S, Jalal S, Ali K, Rasool L, Osman T, Ali O Int J Gen Med. 2020; 13:1453-1467.

PMID: 33335418 PMC: 7737013. DOI: 10.2147/IJGM.S277947.

Beta-Thalassemia Intermedia: A Single Thalassemia Center Experience from Northeastern Iraq.

Amin S, Jalal S, Ali K, Mohammed A, Rasool L, Osman T Biomed Res Int. 2020; 2020:2807120.

PMID: 32190657 PMC: 7066418. DOI: 10.1155/2020/2807120.

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