Endocrinologic Complications in Patients With Transfusion Dependent Thalassemia

Abstract

Abstract

The survival of the patients with transfusion-induced thalassemia has improvedwith the development of treatment options. However, iron overload in various organs continues to reduce the quality of life. Cardiac toxicity is still the leading cause of death and endocrine complications are the major cause of morbidity. Therefore, the importance of diagnosis and treatment of endocrine complications has increased in order to increase the quality of life of patients. The most common endocrine complications in patients are hypogonadism, diabetes, short stature, hypothyroidism and hypoparathyroidism.

Keywords

• Transfusion dependent thalas-semia,endocrinologic com-plications.

Transfüzyon Bağımlı Talasemi Hastalarında Görülen Endokrin Komplikasyonlar

Öz

Öz

Transfüzyona bağımlı talasemi hastalarında tedavi olanaklarının gelişmesi ile hastaların yaşam süreleri uzamıştır. Ancak çeşitli organlarda aşırı demir yükü yaşam kalitesini düşürmeye devam etmektedir. Kardiyak toksisite halen başlıca ölüm nedeni olup endokrin komplikasyonlar başlıca morbidite nedenidir. Bu nedenle hastaların yaşam kalitelerinin artması için endokrin komplikasyonlarının tanı ve tedavisinin önemi daha da artmıştır. Hastalarda sık görülen endokrin komplikasyonlar hipogonadizm, diyabet, boy kısalığı, osteoporoz gibi kemik hastalıkları, hipotiroidizm ve hipoparatiroidizmdir.

Anahtar Kelimeler

• Transfüzyon bağımlı talasemi,endokrinolojik komplikasyonlar

References

1. 1.Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V. Gui-delines for the management of transfusion dependent thalas-saemia (TDT). 3rd ed. Thalassaemia International Federati-on TIF Publication, 2014; 17-65
2. 2.Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cap-pellini MD,Del Vecchio GC, et al. Survival and complicati-ons in patients with thalassemia major treated with transfu-sion and deferoxamine. Haematologica 2004; 89:1187-93.
3. 3.Vogiatzi MG, Macklin EA, Trachtenberg FL, Fung EB, Che-ung AM, Vichinsky E, et al. Differences in the prevalence ofgrowth, endocrine and vitamin D abnormalities among the va-rious thalassaemia syndromes in North America. Br J Hae-matol 2009; 46:546-56.
4. 4.Wood JC, Noetzl L, Hyderi A, Joukar M, Coates T, MittelmanS. Predicting pituitary iron and endocrine dysfunction. AnnN Y Acad Sci 2010; 1202:123-8.
5. 5.De Sanctis V, Eleftheriou A, Malaventura C. Thalassaemia In-ternational Federation Study Group on Growth and Endocri-ne Complications in Thalassaemia. Prevalence of endocrine com-plications and short tature in patients with thalassaemia ma-jor: a multicenter study by the Thalassaemia International Fe-deration (TIF).Pediatr Endocrinol Rev 2004; 2: 249-55.
6. 6.Karakaş Z, Karadağ Kİ, Gül N, Demir AA, Darendeliler FF,Dursun M. Evaluation Of Pituitary Iron Overload in Patientswith Transfusion Dependent Thalassemia. Meeting Abstract,Thalassemia International Federation Congress, Athen,Greece, Kasım 2017.
7. 7.Kattamis C, Liakopoulou T, Kattamis A. Growth and develop-ment in children with thalassaemia major. Acta Paediatr ScandSuppl 1990; 366:111-7.
8. 8.Aydınok Y, Darcan S, Polat A, Kavakli K, Nigli G, Coker M,et al. Endocrine complications in patients with beta-thalas-semia major. J Trop Pediatr 2002; 48: 50-4.

9. 9.Noetzli LJ, Panigrahy A, Mittelman SD, Hyderi A, Dongelyan A,Wood JC. Piuitary iron and volume predict hypogonadism in trans-fusional iron overload. Am J Hematol 2012; 87:167-171.
10. 10.Güler E, Patıroğlu T, Çaksen H, Özdemir MA, Kurtoğlu S, Ken-dirci M. Talasemi majörlü vakalarda endokrin komplikasyon-ların değerlendirilmesi. Türk Pediatri Arşivi 1999; 34: 174-181.
11. 11.Borgna-Pignatti C, De Stefano P, Zonta L, Vullo C, De Sanc-tis V, Melevendi C, et al. Growth and sexual maturation in tha-lassemia major. J Pediatr 1985; 106:150-5.
12. 12.Landau H, Gross V, Dagan I, Palti Z, Rachmilewitz EA, SpitzIM. Growth and sexual development before and after sex ste-roid therapy in patients with thalassemia major. Arch InternMed 1984; 144: 2341-6.
13. 13.Arrigo T, Crisafulli G, Meo A, Sturiale M, Lombardo F, Mi-celi M, et al. Glucose tolerance, insulin secretion and perip-heral sensitivity in thalassaemia major. J Pediatr EndocrinolMetab 1998; 3: 863-6.
14. 14.De Sanctis V, Zurlo MG, Senesi E, Boffa C, Cavallo L, Di Gre-gorio F. Insulin dependent diabetes in thalassaemia. Arch DisChild 1988; 63:58-62.
15. 15.Zuppinger K, Molinari B, Hirt A, Imbach P, Gugler E, TonzO, et al. Increased risk of diabetes mellitus in beta- thalasse-mia major due to iron overload. Helv Paediatr Acta 1979; 34:197-207.
16. 16.Cavallo-Perin P, Pacini G, Cerutti F, Bessone A, Condo C,Sacchetti L et al. Insulin resistance and hyperinsulinemia inhomozygous beta- thalassemia. Metabolism 1995; 44:281-6.
17. 17.Merkel PA, Simonson DC, Amiel SA, Plewe G, Sherwin RS,Pearson HA, et al. Insulin resistance and hyperinsulinemia inpatients with thalassemia major treated by hypertransfusion.N Engl J Med 1988; 318: 809- 14.
18. 18.Bradley B, Prowse SJ, Bauling P, Lafferty KJ. Desferrioxa-mine treatment prevents chronic islet allograft damage. Dia-betes 1986; 35: 550-5.
19. 19.Kattamis C. Present and Future of Thalasemia the expert po-ints of view. Uluslararası Talasemi Kongresi ve Yaz Okulu Kon-gre Kitabı. Antalya, Talasemi Federasyonu Yayınları,2013;133-141
20. 20.Bradley B, Prowse SJ, Bauling P, Lafferty KJ. Desferrioxa-mine treatment prevents chronic islet allograft damage. Dia-betes 1986; 35: 550-5.
21. 21.Masala A, Meloni T, Gallisai D, Alagna S, Rovasio PP, Ras-su S, et al. Endocrine functioning in multitransfused prepu-bertal patients with homozygous beta-thalassemia. J Clin En-docrinol Metab 1984; 58: 667-70.
22. 22.Magro S, Puzzonia P, Consarino C, Galati MC, Morgione S,Porcelli D, et al. Hypothyroidısm in patients with thalasse-mia syndromes. Acta Haematol 1990; 84:72-6.
23. 23.Jensen CE, Tuck SM, Agnew JE, Koneru S, Morris RW, Yar-dumian A, et al. High incidence of osteoporosis in thalassae-mia major. J Pediatr Endocrinol Metab 1998; 11: 975-7.
24. 24.De Vernejoul MC, Girot R, Gueris J, Cancela L, Bang S, Bie-lakoff J, et al. Calcium phosphate metabolism and bone disea-se in patients with homozygous thalassemia. J Clin Endocri-nol Metab. 1982; 54: 276-81.
25. 25.Mautalen CA, Kvicala R, Perriard D, Bugnard E, Rossi E, Du-hart J. Case report: hypoparathyroidısm and iron storage di-sease. Treatment with 25-hydroxy- vitamin D3. Am J Med Sci1978; 276: 363-8.
26. 26.Borgna-Pignatti C, De Stefano P, Zonta L, Vullo C, De Sanc-tis V, Melevendi C, et al. Growth and sexual maturation in tha-lassemia major. J Pediatr 1985; 106:150-5.
27. 27.Pratico G, Di Gregorio F, Caltabiano L, Palano GM, Caru-so-Nicoletti M. Calcium phosphate metabolism in thalassemia.Pediatr Med Chir 1998; 20:265-8.
28. 28.Voskaridou E, Terpos E. New insights into the pathophysio-logy and management of osteoporosis in patients with beta tha-lassaemia. Br J Haematol 2004; 127: 127-139.
29. 29.Lassman MN, O' Brien RT, Pearson HA, Wise JK, Donabe-dian RK, Felig P, et al. Endocrine evaluation in thalassemiamajor. Ann N Y Acad Sci 1974; 232: 226-37.