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Endocrine Complications in Pediatric Beta-Thalassemia Major Patients: A Single-Center Experience

Year 2022, Volume: 19 Issue: 3, 556 - 562, 27.12.2022
https://doi.org/10.35440/hutfd.1144199

Abstract

Background: Our aim was to identify risk factors and clinical correlates of endocrine complications in β-thalassemia major (BTM) patients.
Materials and methods: This was a retrospective study carried out in the pediatric hematology and pediatric endocrinology departments of a tertiary healthcare institution with the medical data of 249 children with BTM (108 females, 43.4%; 141 males, 56.6%) with a median age of 7.17 (2 -17.8) years. Baseline descriptive, clinical features including endocrine complications and laboratory data were noted. Correlation between the presence of endocrine complications and demographic, clinical, and laboratory variables were sought. The effects of age, gender, race, height, weight, and splenectomy on endocrine complications were evaluated separately in each complication group.
Results: Vitamin D deficiency/insufficiency is the most common endocrine complication (41.7%). According to Turkish children, Syrian children had also significantly lower vitamin D concentration (p=0.001). At least one endocrinopathy was reported in the majority of BTM patients (67.9%). Accordingly, pubertal status (p=0.014) and Syrian nationality (p=0.007) had significant impacts on TSH levels. Syrian children and those with delayed puber-ty had greater likelihood for subclinical or evident hypothyroidism. The likelihood of suffering from at least one endocrine complication was higher in older children (p=0.042) and those with Syrian nationality (p=0.025)
Conclusion: Disorders of endocrine and metabolic nature are common in children with BTM. Early detection and protocol-based multidisciplinary management of these disorders constitute the most suitable strategies to in-crease patients' quality of life. Surveillance, early detection and treatment, and collaborative follow-up with a multidisciplinary team are the key points in the reduction of the severity and frequency of endocrine complica-tions as well as optimization of therapeutic outcomes. 

References

  • References 1. He LN, Chen W, Yang Y, Xie YJ, Xiong ZY, Chen DY, et al. Elevated Prevalence of Abnormal Glucose Metabolism and Other Endocrine Disorders in Patients with β-Thalassemia Major: A Meta-Analysis. Biomed Res Int. 2019;18;2019:6573497.
  • 2. Altincik A, Akin M. Prevalence of Endocrinopathies in Turkish Children With β-Thalassemia Major: A Single-Center Study. J Pediatr Hematol Oncol. 2016; ;38(5):389-93.
  • 3. Parijat De, Radhika Mistry, Christine Wright, Shivan Pancham, Wyn Burbridge, Kalyan Gangopadhayay, et al. Open J Endocr Metab Dis.2014 ;4(2):25–34.
  • 4. Saffari F, Mahyar A, Jalilolgadr S. Endocrine and metabolic disorders in β-thalassemiamajor patients. Caspian J Intern Med. 2012;3(3):466-72. 5. Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M,et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocr Disord. 2003;12;3(1):4.
  • 6. Isik P, Yarali N, Tavil B, Demirel F, Karacam GB, Sac RU, et al. Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study. Pediatr Hematol Oncol. 2014;31(7):607-15.
  • 7. Najafipour F, Aliasgarzadeh A, Aghamohamadzadeh N, Bahrami A, Mobasri M, Niafar M, et al. A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major. Ann Saudi Med. 2008;28(5):361-6.
  • 8. Adil A, Sobani ZA, Jabbar A, Adil SN, Awan S. Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan. J Pak Med Assoc. 2012;62(3):307-10.
  • 9. Dhouib NG, Ben Khaled M, Ouederni M, Besbes H, Kouki R, Mellouli F, et al. Growth and Endocrine Function in Tunisian Thalassemia Major Patients. Mediterr J Hematol Infect Dis. 2018;10(1):e2018031.
  • 10. De Sanctis V, Soliman AT, Canatan D, Tzoulis P, Daar S, Di Maio S,et al. An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations. Acta Biomed. 2019;89(4):481-489.
  • 11. Multicentre study on prevalence of endocrine complications in thalassaemia major. Italian Working Group on Endocrine Complications in Non-endocrine Diseases. Clin Endocrinol (Oxf). 1995 Jun;42(6):581-6.
  • 12. Al-Elq AH, Al-Saeed HH. Endocrinopathies in patients with thalassemias. Saudi Med J. 2004;25(10):1347-51.
  • 13. Chirico V, Rigoli L, Lacquaniti A, Salpietro V, Piraino B, Amorini M, et al. Endocrinopathies, metabolic disorders, and iron overload in major and intermedia thalassemia: serum ferritin as diagnostic and predictive marker associated with liver and cardiac T2* MRI assessment. Eur J Haematol. 2015;94(5):404-12.
  • 14. Mohammadian S, Bazrafshan HR, Sadeghi-Nejad A. Endocrine gland abnormalities in thalassemia major: a brief review. J Pediatr Endocrinol Metab. 2003;16(7):957-64.
  • 15. De Sanctis V, Soliman AT, Elsedfy H, Pepe A, Kattamis C, El Kholy M, et al. Diabetes and Glucose Metabolism in Thalassemia Major: An Update. Expert Rev Hematol. 2016;9(4):401-8.
  • 16. Oerter KE, Kamp GA, Munson PJ, Nienhuis AW, Cassorla FG, Manasco PK. Multiple hormone deficiencies in children with hemochromatosis. J Clin Endocrinol Metab. 1993;76(2):357-61.
  • 17. Aydinok Y, Darcan S, Polat A, Kavakli K, Nigli G, Coker M, et al. Endocrine complications in patients with beta-thalassemia major. J Trop Pediatr. 2002;48(1):50-4.
  • 18. Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev. 2007;5(2):642-8.
  • 19. Esposito BP, Breuer W, Sirankapracha P, Pootrakul P, Hershko C, Cabantchik ZI. Labile plasma iron in iron overload: redox activity and susceptibility to chelation. Blood. 2003;102(7):2670-7.
  • 20. Karamifar H, Shahriari M, Sadjadian N. Prevalence of endocrine complications in beta-thalassaemia major in the Islamic Republic of Iran. East Mediterr Health J. 2003;9(1-2):55-60.

Pediatrik Beta Talasemi Major Hastalarında Endokrin Komplikasyonlar: Tek Merkez Deneyimi

Year 2022, Volume: 19 Issue: 3, 556 - 562, 27.12.2022
https://doi.org/10.35440/hutfd.1144199

Abstract

Background: We aimed to investigate the risk factors and clinical correlates of endocrine complications in β-thalassemia major (BTM) patients.
Materials and methods: This retrospective study was performed in the pediatric hematology and pediatric endocrinology departments of a tertiary care center using data derived from medical records of 249 children with BTM (108 females, 43.4%; 141 males, 56.6%) with a median age of 7.17 (range: 2 to 17.83) years. Baseline descriptive, clinical features including endocrine complications and laboratory data were noted. Correlation between the presence of endocrine complications and demographic, clinical, and laboratory variables were sought.
Results: The majority of BTM patients (n=169, 67.9%) had at least one endocrine complication. Syrian patients had lower levels of vitamin D and were more likely to have subclinical hypothyroidism. Patients with advanced age, delayed puberty, Syrian nationality, and short stature were more likely to suffer from at least one endocrine complication.
Conclusion: Endocrine and metabolic disorders are very common in children with BTM. Early detection of these disorders, as well as multidisciplinary management with standardized protocols constitutes the most suitable strategy to provide a better quality of life for these patients. Regular surveillance, early diagnosis, treatment, and follow-up with the collaboration of a multidisciplinary team are the key points in the reduction of the severity and frequency of endocrine complications as well as optimization of therapeutic outcomes.

References

  • References 1. He LN, Chen W, Yang Y, Xie YJ, Xiong ZY, Chen DY, et al. Elevated Prevalence of Abnormal Glucose Metabolism and Other Endocrine Disorders in Patients with β-Thalassemia Major: A Meta-Analysis. Biomed Res Int. 2019;18;2019:6573497.
  • 2. Altincik A, Akin M. Prevalence of Endocrinopathies in Turkish Children With β-Thalassemia Major: A Single-Center Study. J Pediatr Hematol Oncol. 2016; ;38(5):389-93.
  • 3. Parijat De, Radhika Mistry, Christine Wright, Shivan Pancham, Wyn Burbridge, Kalyan Gangopadhayay, et al. Open J Endocr Metab Dis.2014 ;4(2):25–34.
  • 4. Saffari F, Mahyar A, Jalilolgadr S. Endocrine and metabolic disorders in β-thalassemiamajor patients. Caspian J Intern Med. 2012;3(3):466-72. 5. Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M,et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocr Disord. 2003;12;3(1):4.
  • 6. Isik P, Yarali N, Tavil B, Demirel F, Karacam GB, Sac RU, et al. Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study. Pediatr Hematol Oncol. 2014;31(7):607-15.
  • 7. Najafipour F, Aliasgarzadeh A, Aghamohamadzadeh N, Bahrami A, Mobasri M, Niafar M, et al. A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major. Ann Saudi Med. 2008;28(5):361-6.
  • 8. Adil A, Sobani ZA, Jabbar A, Adil SN, Awan S. Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan. J Pak Med Assoc. 2012;62(3):307-10.
  • 9. Dhouib NG, Ben Khaled M, Ouederni M, Besbes H, Kouki R, Mellouli F, et al. Growth and Endocrine Function in Tunisian Thalassemia Major Patients. Mediterr J Hematol Infect Dis. 2018;10(1):e2018031.
  • 10. De Sanctis V, Soliman AT, Canatan D, Tzoulis P, Daar S, Di Maio S,et al. An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations. Acta Biomed. 2019;89(4):481-489.
  • 11. Multicentre study on prevalence of endocrine complications in thalassaemia major. Italian Working Group on Endocrine Complications in Non-endocrine Diseases. Clin Endocrinol (Oxf). 1995 Jun;42(6):581-6.
  • 12. Al-Elq AH, Al-Saeed HH. Endocrinopathies in patients with thalassemias. Saudi Med J. 2004;25(10):1347-51.
  • 13. Chirico V, Rigoli L, Lacquaniti A, Salpietro V, Piraino B, Amorini M, et al. Endocrinopathies, metabolic disorders, and iron overload in major and intermedia thalassemia: serum ferritin as diagnostic and predictive marker associated with liver and cardiac T2* MRI assessment. Eur J Haematol. 2015;94(5):404-12.
  • 14. Mohammadian S, Bazrafshan HR, Sadeghi-Nejad A. Endocrine gland abnormalities in thalassemia major: a brief review. J Pediatr Endocrinol Metab. 2003;16(7):957-64.
  • 15. De Sanctis V, Soliman AT, Elsedfy H, Pepe A, Kattamis C, El Kholy M, et al. Diabetes and Glucose Metabolism in Thalassemia Major: An Update. Expert Rev Hematol. 2016;9(4):401-8.
  • 16. Oerter KE, Kamp GA, Munson PJ, Nienhuis AW, Cassorla FG, Manasco PK. Multiple hormone deficiencies in children with hemochromatosis. J Clin Endocrinol Metab. 1993;76(2):357-61.
  • 17. Aydinok Y, Darcan S, Polat A, Kavakli K, Nigli G, Coker M, et al. Endocrine complications in patients with beta-thalassemia major. J Trop Pediatr. 2002;48(1):50-4.
  • 18. Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev. 2007;5(2):642-8.
  • 19. Esposito BP, Breuer W, Sirankapracha P, Pootrakul P, Hershko C, Cabantchik ZI. Labile plasma iron in iron overload: redox activity and susceptibility to chelation. Blood. 2003;102(7):2670-7.
  • 20. Karamifar H, Shahriari M, Sadjadian N. Prevalence of endocrine complications in beta-thalassaemia major in the Islamic Republic of Iran. East Mediterr Health J. 2003;9(1-2):55-60.
There are 19 citations in total.

Details

Primary Language English
Subjects Clinical Sciences
Journal Section Research Article
Authors

Fatma Demir Yenigurbuz 0000-0001-8692-2767

Burcu Akıncı 0000-0001-6026-4786

Ala Üstyol 0000-0001-7903-7356

Deniz Ökdemir 0000-0003-3618-8167

Ahmet Sezer 0000-0001-9380-2771

Publication Date December 27, 2022
Submission Date July 15, 2022
Acceptance Date November 3, 2022
Published in Issue Year 2022 Volume: 19 Issue: 3

Cite

Vancouver Demir Yenigurbuz F, Akıncı B, Üstyol A, Ökdemir D, Sezer A. Endocrine Complications in Pediatric Beta-Thalassemia Major Patients: A Single-Center Experience. Harran Üniversitesi Tıp Fakültesi Dergisi. 2022;19(3):556-62.

Harran Üniversitesi Tıp Fakültesi Dergisi  / Journal of Harran University Medical Faculty