An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations

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Vincenzo De Sanctis
Ashraf T Soliman
Duran Canatan
Ploutarchos Tzoulis
Shahina Daar
Salvatore Di Maio
Heba Elsedfy
Mohamed A Yassin
Aldo Filosa
Nada Soliman
Karimi Mehran
Forough Saki
Praveen Sobti
Shruti Kakkar
Soteroula Christou
Alice Albu
Constantinos Christodoulides
Yurdanur Kilinc
Soad Al Jaouni
Doaa Khater
Saif A Alyaarubi
Su Han Lum
Saveria Campisi
Salvatore Anastasi
Maria Concetta Galati
Giuseppe Raiola
Yasser Wali
Ihab Z Elhakim
Demetris Mariannis
Vassilis Ladis
Christos Kattamis

Keywords

Thalassemia major, central hypothyroidism, latent hypocortisolism, growth hormone deficiency, transition phase, ICET-A

Abstract

In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries. The data reported by 15 countries are presented.The commonest endocrine complications registered in 3.114 TM adults are CH and GHD (4.6 % and 3.0 %, respectively), followed by latent hypocortisolism (1.2%). In 13 patients (0.41%) a cytological papillary or follicular thyroid carcinoma was diagnosed in 11 and 2 patients, respectively, and a lobectomy or thyroidectomy was carried out. Of 202 TM patients below the age of 18 years, the  reported endocrine complications were: GHD in 4.5%, latent hypocortisolism in 4.4% and central hypothyrodisim in 0.5%. Transition phase was an area of interest for many clinicians, especially as patients with complex chronic health conditions are responding to new treatments extending their lifespan beyond imagination.. In conclusion, our survey provides a better understanding of  physicians’ current clinical practices and beliefs in the detection, prevention and treatment of some endocrine complications prevailing in adult TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are recommended.

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