Hormones and metabolic parameters among a sample of beta Thalassemia major patients in Aqrah city- Iraq

Main Article Content

Dleen Hussein Fattah
Dr.Ahmed Mammon Abdulhadi
Ali Shakir Dauod

Keywords

Beta-thalassemia, stunting, anemia, TSH, and PTH

Abstract

Background: Inconveniently, many physicians still emphasize the anemia of thalassemia patients, overlooking the problems of iron overload and its consequences on the development of the child.
Objectives: The main aim of this study is to assess the serum levels of thyroid and parathyroid hormones, calcium, phosphorus, and vitamin D3 among Beta-thalassemia patients.
Methods: Sixty children with thalassemia major with a ratio of males to females 1.222, were included from the 1st of May 2022 to the 15th of July 2022. A detailed medical history is obtained from every patient, and a clinical examination is performed. Blood samples were taken for Hb, thyroid and parathyroid hormones, serum calcium, phosphorous, and vitamin D3.
Results: The mean age (SD) was 11.4 (4.1) years and the largest proportion 25 (41.7%) were aged 10-14 years. Nine children had high TSH (15%), high T4 6 (10%), low PTH 5 (8.3%), low D3 57 (95%), high phosphorus 43 (71.7%), low calcium 28 (46.7%), high ALP 57 (95%), and high ferritin 60 (100%). Half of the children had short stature.
There were significant positive correlations detected between the duration of the disease and variables like ferritin, TSH, and Phosphorus, while the correlation with PTH and height for age were all negative. The mean of TSH among children aged > 10 years (5.05 mIu/ml) was significantly higher than the mean of children aged ≤ 10 years (3.42 Miu/ml). In comparison; the mean of PTH of children aged ≤ 10 years (29.39 pg/ml) was significantly higher than the mean PTH of children aged ≤ 10 years (19.97 pg/ml). No significant differences regarding D3, calcium, ALP, and phosphorus were detected.
Conclusions: Endocrine complications are major health issues in thalassemia despite the medical therapies.

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References

1. Needs T, Gonzalez-Mosquera LF, Lynch DT. Beta Thalassemia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; May 8, 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK531481/
2. Higgs DR, Engel JD, Stamatoyannopoulos G. Thalassaemia. Lancet 2012;379(9813):373-383. https://doi.org/10.1016/S0140-6736(11)60283-3
3. Fahim FM, Saad K, Askar EA, et al. Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt. Int J Hematol Oncol Stem Cell Res 2013;7(4):10-14.
4. https://pubmed.ncbi.nlm.nih.gov/24505537 https://portal.issn.org/resource/ISSN/2008-3009
5. Steensma DP, Hoyer JD, Fairbanks VF. Hereditary red blood cell disorders in middle eastern patients. Mayo Clin Proc 2001;76(3):285-293. https://doi.org/10.4065/76.3.285
6. Alnakshabandi, Abd al-Qadir A., Muhammad, Huda A. Prevalence of β-thalassemia carriers among a cohort of university students in hawler province of Iraqi Kurdistan. Iraqi J Pharm Sci 2009. Vol. 18, no. 2, pp.15-19. https://doi.org/10.31351/vol18iss2pp15-19 7. Al-Allawi NA, Al-Dousky AA. Frequency of haemoglobinopathies at premarital health screening in Dohuk, Iraq: implications for a regional prevention programme. East Mediterr Health J 2010;16(4):381-385.
8. https://apps.who.int/iris/handle/10665/117880#:~:text=ISSN-,1020%2D3397,-Other%20Identifiers
9. De Sanctis V, Eleftheriou A, Malaventura C; Thalassaemia International Federation Study Group on Growth and Endocrine Complications in Thalassaemia. Prevalence of endocrine complications and short stature in patients with thalassaemia major: a multicenter study by the Thalassaemia International Federation (TIF). Pediatr Endocrinol Rev 2004;2 Suppl 2:249-255.
10. https://europepmc.org/article/med/16462705#:~:text=2%3A249%2D255-,PMID%3A%2016462705%C2%A0,-Share%20this%20article https://pubmed.ncbi.nlm.nih.gov/20795420/#:~:text=expand-,PMID%3A%2020795420,-Free%20article
11. Telfer PT, Warburton F, Christou S, et al. Improved survival in thalassemia major patients on switching from desferrioxamine to combined chelation therapy with desferrioxamine and deferiprone. Haematologica 2009;94(12):1777-1778.
12. https://doi.org/10.3324/haematol.2009.009118
13. Delvecchio M, Cavallo L. Growth and endocrine function in thalassemia major in childhood and adolescence. J Endocrinol Invest 2010;33(1):61-68. https://doi.org/10.1007/BF03346551
14. PMID: 20203539.
15. Upadya SH, Rukmini MS, Sundararajan S, et al. Thyroid Function in Chronically Transfused Children with Beta Thalassemia Major: A Cross-Sectional Hospital Based Study. Int J Pediatr. 2018 Sep 16;2018:9071213. https://doi.org/10.1155%2F2018%2F9071213 PMID: 30305822; PMCID: PMC6165584.
16. Sleem GA, Al-Zakwani IS, Almuslahi M. Hypoparathyroidism in adult patients with Beta-thalassemia major. Sultan Qaboos Univ Med J 2007;7(3):215-218.
17. PMID: 21748106; PMCID: PMC3074875. https://pubmed.ncbi.nlm.nih.gov/21748106
18. Hamidieh AA, Moradbeag B, Pasha F, Jalili M, et al. High Prevalence of Hypoparathyroidism in Patients with beta-Thalassemia Major. Int J Hematol Oncol Stem Cell Res. 1;3(3):17-20. https://portal.issn.org/resource/ISSN/2008-3009 https://portal.issn.org/resource/ISSN/2008-2207
19. Liu Y, Zhang L, Hu N, et al. An optogenetic approach for regulating human parathyroid hormone secretion. Nat Commun 2022;13(1):771. https://doi.org/10.1038/s41467-022-28472-9
20. Schafer AL, Shoback DM. Hypocalcemia: Diagnosis and Treatment. [Updated 2016 Jan 3]. In: Feingold KR, Anawalt B, Blackman MR, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK279022/
21. Vogiatzi MG, Macklin EA, Fung EB, et al. Bone disease in thalassemia: a frequent and still unresolved problem. J Bone Miner Res 2009;24(3):543-557. https://doi.org/10.1359/jbmr.080505
22. Najafipour F, Aliasgarzadeh A, Aghamohamadzadeh N, et al. A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major. Ann Saudi Med 2008;28(5):361-366. https://doi.org/10.5144/0256-4947.2008.361
23. 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 Jan-Mar;9(1-2):55-60. PMID: 15562733. https://pubmed.ncbi.nlm.nih.gov/15562733/#:~:text=expand-,PMID%3A%2015562733,-Free%20article
24. De Sanctis V, De Sanctis E, Ricchieri P, et al. Mild subclinical hypothyroidism in thalassaemia major: prevalence, multigated radionuclide test, clinical and laboratory long-term follow-up study. Pediatr Endocrinol Rev 2008;6 Suppl 1:174-180. https://europepmc.org/article/med/19337174#:~:text=1%3A174%2D180-,PMID%3A%2019337174%C2%A0,-Share%20this%20article
25. Sharma R, Seth A, Chandra J, et al. Endocrinopathies in adolescents with thalassaemia major receiving oral iron chelation therapy. Paediatr Int Child Health 2016;36(1):22-27. https://doi.org/10.1179/2046905514Y.0000000160
26. Abdel-Razek AR, Abdel-Salam A, El-Sonbaty MM, Youness ER. Study of thyroid function in Egyptian children with β-thalassemia major and β-thalassemia intermedia. J Egypt Public Health Assoc 2013;88(3):148-152. https://journals.lww.com/ephaj/fulltext/2013/12000/Study_of_thyroid_function_in_Egyptian_chil dren.5.aspx#:~:text=DOI%3A%2010.1097/01.EPX.0000436490.10201.28
27. Seow CE, Goh AS, Lim SL. High prevalence of central hypothyroidism among patients with transfusion dependent thalassemia in Hospital Pulau Pinang: A cross sectional study. Med J Malaysia 2021 Nov 1;76(6):799-803. https://europepmc.org/article/med/34806663#:~:text=6)%3A799%2D803-,PMID%3A%2034806663%C2%A0,-Share%20this%20article http://www.ncbi.nlm.nih.gov/pubmed/34806663
28. Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev 2007;5(2):642-648.
29. https://europepmc.org/article/med/18084158#:~:text=2)%3A642%2D648-,PMID%3A%2018084158,-Review
30. Upadya SH, Rukmini MS, Sundararajan S, et al. Thyroid Function in Chronically Transfused Children with Beta Thalassemia Major: A Cross-Sectional Hospital Based Study. Int J Pediatr 2018;2018:9071213. Published 2018 Sep 16. https://doi.org/10.1155/2018/9071213
31. Mahmoud, R.A., Khodeary, A. & Farhan, M.S. Detection of endocrine disorders in young children with multi-transfused thalassemia major. Ital J Pediatr 2021;47: 165. https://link.springer.com/article/10.1186/s13052-021-01116-2#citeas:~:text=DOI-,https%3A//doi.org/10.1186/s13052%2D021%2D01116%2D2,-Share%20this%20article
32. Shamshirsaz AA, Bekheirnia MR, Kamgar M, et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocr Disord 2003;3(1):4. https://bmcendocrdisord.biomedcentral.com/articles/10.1186/1472-6823-3-4#citeas:~:text=DOI-,https%3A//doi.org/10.1186/1472%2D6823%2D3%2D4,-Share%20this%20article
33. Angelopoulos NG, Goula A, Rombopoulos G, et al. Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia. J Bone Miner Metab 2006;24(2):138-145. https://link.springer.com/article/10.1007/s00774-005-0660-1#citeas:~:text=DOI-,https%3A//doi.org/10.1007/s00774%2D005%2D0660%2D1,-Key%20words
34. Moiz B, Habib A, Sawani S, et al. Anthropometric measurements in children having transfusion-dependent beta thalassemia. Hematology 2018;23(4):248-252. https://doi.org/10.1080/10245332.2017.1396044
35. De Sanctis V, Soliman AT, Canatan D, et al. An ICET- A survey on Hypoparathyroidism in Patients with Thalassaemia Major and Intermedia: A preliminary report. Acta Biomed 2018;88(4):435-444. https://doi.org/10.23750%2Fabm.v88i4.6837
36. El-Din LB, Ebeid FS, Toaima NN, Ibrahim WW. Hypoparathyroidism in children with β-thalassemia major and its relation to iron chelation therapy. Egypt J Haematol 2018;43:63-8 https://www.ehj.eg.net/text.asp?2018/43/2/63/238765
37. De Sanctis V, Eleftheriou A, Malaventura C. Thalassaemia International Federation Study Group on Growth and Endocrine Complications in Thalassaemia. Prevalence of endocrine complications and short stature in patients with thalassaemia major: a multicenter study by the Thalassaemia International Federation (TIF). Pediatr Endocrinol Rev 2004;2 Suppl 2:249-255.
38. PMID: 16462705. https://pubmed.ncbi.nlm.nih.gov/16462705/#:~:text=expand-,PMID%3A%2016462705,-Abstract
39. Pirinççioğlu AG, Söker DG. Parathyroid functions in thalassemia major patients. Ann Clin Endocrinol Metab 2017;1:15-9. https://dx.doi.org/10.29328/journal.hcem.1001003
40. Basha N KP, Shetty B, Shenoy UV. Prevalence of Hypoparathyroidism (HPT) in Beta Thalassemia Major. J Clin Diagn Res 2014;8(2):24-26. https://pubmed.ncbi.nlm.nih.gov/24701472 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3972574/#:~:text=JCDR/2014/6672.3997-,PMCID%3A%20PMC3972574,-PMID%3A%2024701472
41. Hamidieh AA, Moradbeag B, Pasha F, et al. High Prevalence of Hypoparathyroidism in Patients with beta-Thalassemia Major. Int J Hematol Oncol Stem Cell Res 1;3(3):17-20. https://portal.issn.org/resource/ISSN/2008-2207
42. Crabtree NJ, Arabi A, Bachrach LK, et al. Dual-energy X-ray absorptiometry interpretation and reporting in children and adolescents: the revised 2013 ISCD Pediatric Official Positions. J Clin Densitom 2014;17(2):225-242. https://doi.org/10.1016/j.jocd.2014.01.003
43. Al-Elq AH, Al-Saeed HH. Endocrinopathies in patients with thalassemias. Saudi Med J 2004;25(10):1347-1351.
44. https://pubmed.ncbi.nlm.nih.gov/15494799/#:~:text=expand-,PMID%3A%2015494799,-Abstract
45. Eren E, Yilmaz N. Biochemical markers of bone turnover and bone mineral density in patients with beta-thalassaemia major. Int J Clin Pract 2005;59(1):46-51. https://doi.org/10.1111/j.1742-1241.2005.00358.x
46. Napoli N, Carmina E, Bucchieri S, et al. Low serum levels of 25-hydroxy vitamin D in adults affected by thalassemia major or intermedia. Bone 2006;38(6):888-892. https://doi.org/10.1016/j.bone.2005.11.018
47. Soliman A, Adel A, Wagdy M, et al. Calcium homeostasis in 40 adolescents with beta-thalassemia major: a case-control study of the effects of intramuscular injection of a megadose of cholecalciferol. Pediatr Endocrinol Rev 2008;6 Suppl 1:149-154. (PMID: 19337170) Online: ISSN 1468-2044 Print: ISSN 0003-9888 https://pubmed.ncbi.nlm.nih.gov/19337170/#:~:text=expand-,PMID%3A%2019337170,-Abstract
48. Vogiatzi MG, Macklin EA, Trachtenberg FL, et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol 2009;146(5):546-556. https://doi.org/10.1111%2Fj.1365-2141.2009.07793.x
49. Malik S, Syed S, Ahmed N. Complications in transfusion–dependent patients of ß-thalassemia major: A review. Pak J Med Sci 2009;25(4):678-82. ISSN 1681-715X https://www.pjms.com.pk/issues/julsep09/article/article30.html
50. Fahim FM, Saad K, Askar EA, et al. Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt. Int J Hematol Oncol Stem Cell Res 2013;7(4):10-14. https://portal.issn.org/resource/ISSN/2008-3009 https://portal.issn.org/resource/ISSN/2008-2207 https://pubmed.ncbi.nlm.nih.gov/24505537
51. Pirinççioğlu AG, Akpolat V, Köksal O, et al. Bone mineral density in children with beta-thalassemia major in Diyarbakir. Bone 2011;49(4):819-823. https://doi.org/10.1016/j.bone.2011.07.014