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Evaluation of endocrine complications in beta-thalassemia intermedia (β-TI): a cross-sectional multicenter study

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Abstract

Background

Data on the prevalence and type of endocrine disorders in β-thalassemia intermedia (β-TI) patients are scarce. This multicenter study was designed to determine the prevalence of endocrine complications and the associated risk factors in a large group of β-TI patients.

Methods

In this cross-sectional multicenter study, 726 β-TI patients, aged 2.5–80 years, registered at 12 thalassemic centers, from nine countries, were enrolled during 2017. In a subgroup of 522 patients (mean age 30.8 ± 12.1; range: 2.5–80 years) from Qatar, Iran, Oman, Cyprus, and Jordan detailed data were available.

Results

Overall, the most prevalent complications were osteopenia/osteoporosis (22.3%), hypogonadism (10.1%), and primary hypothyroidism (5.3%). In the subgroup multivariate analysis, older age was a risk factor for osteoporosis (Odds ratio: 7.870, 95% CI: 4.729–13.099, P < 0.001), hypogonadism (Odds ratio: 6.310, 95% CI: 2.944–13.521, P < 0.001), and non-insulin-dependent diabetes mellitus (NIDDM; Odds ratio: 17.67, 95% CI: 2.217–140.968, P = 0.007). Splenectomy was a risk factor for osteoporosis (Odds ratio: 1.736, 95% CI: 1.012–2.977, P = 0.045). Hydroxyurea was identified as a “protective factor” for NIDDM (Odds ratio: 0.259, 95% CI: 0.074–0.902, P = 0.034).

Conclusions

To the best of our knowledge, this is the largest cohort of β-TI patients with endocrine disorders evaluated in extremely heterogenic thalassemic populations for age, clinical, hematological, and molecular composition. The study demonstrates that endocrine complications are less common in patients with β-TI compared with β-TM patients. However, regular monitoring with timely diagnosis and proper management is crucial to prevent endocrine complications in β-TI patients.

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Author information

Authors and Affiliations

  1. Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

    Mehran Karimi, Tahereh Zarei & Sezaneh Haghpanah

  2. Pediatric Hematology–Oncology, Adult Thalassemia Clinic, Tehran, Iran

    Azita Azarkeivan

  3. Thalassemia Unit, Division of Pediatric Hematology–Oncology, First Department of Pediatrics, University of Athens “Agia Sofia” Children’s Hospital, Athens, Greece

    Christos Kattamis, Vassilis Ladis & Antonios Kattamis

  4. Department of Pediatric Hematology, Medical Faculty, Çukurova University, Adana, Turkey

    Yurdanur Kilinc

  5. Department of Hematology, College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman

    Shahina Daar & Saif Alyaarubi

  6. Department of Hematology, College of Medicine & Health Sciences, Sultan Qaboos University and Muscat, Sultanate of Oman Pediatric Department, Faculty of Medicine, Alexandria University, Alexandria, Egypt

    Doaa Khater, Yasser Wali & Mohamed Elshinawy

  7. Department of General Medicine, Sohar Hospital, Sohar, Oman

    Ali Almadhani

  8. Department of Pediatrics, Division of Endocrinology, Hamad General Hospital Doha, Qatar and Department of Pediatrics, Division of Endocrinology, Alexandria University Children’s Hospital, Alexandria, Egypt

    Mohamed Yassin & Ashraf T. Soliman

  9. Thalassemia Diagnosis Center of Mediterranean Blood Diseases Foundation, Antalya, Turkey

    Duran Canatan

  10. King Abdullah University Hospital, Princess Rahma Teaching hospital, Irbid, Jordan

    Maha Obiedat & Hala Al-Rimawi

  11. Royal Lancaster Infirmary, Lancaster, UK

    Demetris Mariannis

  12. Archibishop Makarios III Hospital, Thalassaemia Clinic, Nicosia, Cyprus

    Constantinos Christodoulides & Soteroula Christou

  13. Department of Endocrinology, Whittington Hospital, University College London, London, UK

    Ploutarchos Tzoulis

  14. Thalassemia Unit, Umberto 1° Hospital, Siracusa, Italy

    Saveria Campisi

  15. Children’s Hospital Santobono-Pausilipon, Naples, Italy

    Salvatore Di Maio

  16. Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy

    Vincenzo De Sanctis

Authors
  1. Mehran Karimi
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  2. Tahereh Zarei
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  9. Shahina Daar
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  14. Ali Almadhani
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  15. Mohamed Yassin
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  16. Ashraf T. Soliman
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  17. Duran Canatan
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  18. Maha Obiedat
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  19. Hala Al-Rimawi
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  20. Demetris Mariannis
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  21. Constantinos Christodoulides
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  22. Soteroula Christou
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  23. Ploutarchos Tzoulis
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  24. Saveria Campisi
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  25. Salvatore Di Maio
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  26. Vincenzo De Sanctis
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Corresponding author

Correspondence to Vincenzo De Sanctis.

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The authors declare that they have no conflict of interest.

Ethical approval

The protocol of the study was approved by Shiraz University of Medical Sciences (Approval code: 1396-01-32-15525).

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Written informed consent was obtained from the patients or their parents or legal guardian before participation in the study.

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Karimi, M., Zarei, T., Haghpanah, S. et al. Evaluation of endocrine complications in beta-thalassemia intermedia (β-TI): a cross-sectional multicenter study. Endocrine 69, 220–227 (2020). https://doi.org/10.1007/s12020-019-02159-6

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