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Evaluation of Endocrine Complications in Beta-Thalassemia Intermedia (Β-Ti): A Cross-Sectional Multicenter Study Publisher Pubmed



Karimi M1 ; Zarei T1 ; Haghpanah S1 ; Azarkeivan A2 ; Kattamis C3 ; Ladis V3 ; Kattamis A3 ; Kilinc Y4 ; Daar S5 ; Alyaarubi S5 ; Khater D6 ; Wali Y6 ; Elshinawy M6 ; Almadhani A7 Show All Authors
Authors
  1. Karimi M1
  2. Zarei T1
  3. Haghpanah S1
  4. Azarkeivan A2
  5. Kattamis C3
  6. Ladis V3
  7. Kattamis A3
  8. Kilinc Y4
  9. Daar S5
  10. Alyaarubi S5
  11. Khater D6
  12. Wali Y6
  13. Elshinawy M6
  14. Almadhani A7
  15. Yassin M8
  16. Soliman AT8
  17. Canatan D9
  18. Obiedat M10
  19. Alrimawi H10
  20. Mariannis D11
  21. Christodoulides C12
  22. Christou S12
  23. Tzoulis P13
  24. Campisi S14
  25. Di Maio S15
  26. De Sanctis V16
Show Affiliations
Authors Affiliations
  1. 1. Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  2. 2. Pediatric Hematology–Oncology, Adult Thalassemia Clinic, Tehran, Iran
  3. 3. Thalassemia Unit, Division of Pediatric Hematology–Oncology, First Department of Pediatrics, University of Athens “Agia Sofia� Children’s Hospital, Athens, Greece
  4. 4. Department of Pediatric Hematology, Medical Faculty, Cukurova University, Adana, Turkey
  5. 5. Department of Hematology, College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman
  6. 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
  7. 7. Department of General Medicine, Sohar Hospital, Sohar, Oman
  8. 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
  9. 9. Thalassemia Diagnosis Center of Mediterranean Blood Diseases Foundation, Antalya, Turkey
  10. 10. King Abdullah University Hospital, Princess Rahma Teaching hospital, Irbid, Jordan
  11. 11. Royal Lancaster Infirmary, Lancaster, United Kingdom
  12. 12. Archibishop Makarios III Hospital, Thalassaemia Clinic, Nicosia, Cyprus
  13. 13. Department of Endocrinology, Whittington Hospital, University College London, London, United Kingdom
  14. 14. Thalassemia Unit, Umberto 1° Hospital, Siracusa, Italy
  15. 15. Children’s Hospital Santobono-Pausilipon, Naples, Italy
  16. 16. Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy

Source: Endocrine Published:2020


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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature.
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