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

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

Authors

  • Vincenzo De Sanctis Quisisana Hospital, Ferrara
  • Ashraf T Soliman Department of Pediatrics, Division of Endocrinology, Alexandria University Children’s Hospital, Alexandria, Egypt
  • Duran Canatan Director of Thalassemia Diagnosis Center of Mediterranean Blood Diseases Foundation, Antalya, Turkey
  • Ploutarchos Tzoulis Department of Endocrinology,Whittington Hospital, University College London, London, UK
  • Shahina Daar Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, Sultanate of Oman and Wallenberg Research Centre, Stellenbosch Institute for Advanced Study, Stellenbosch University, Stellenbosch, South Africa
  • Salvatore Di Maio Emeritus Director in Pediatrics, Children’s Hospital “Santobono-Pausilipon”, Naples, Italy
  • Heba Elsedfy Department of Pediatrics, Ain Shams University, Cairo, Egypt
  • Mohamed A Yassin National Center for Cancer Care and Research, Medical Oncology Hematology Section HMC, Doha, Qatar
  • Aldo Filosa UOSD Malattie Rare del Globulo Rosso dell’Azienda Ospedaliera “A. Cardarelli” , Naples, Italy
  • Nada Soliman Primary Health Care, Ministry of Health, Alexandria, Egypt; 11 Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  • Karimi Mehran Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  • Forough Saki Shiraz Endocrinology and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  • Praveen Sobti Professor, Pediatric Hemato-Oncology, Christian Medical College and Hospital, Ludhiana, Punjab, India
  • Shruti Kakkar Department of Pediatrics, Dayanand Medical College & Hospital Ludhiana, Ludhiana, India
  • Soteroula Christou Thalassemia Unit, Nicosia, Cyprus
  • Alice Albu Endocrinology and Diabetes Department - Elias Hospital, Bucharest "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania
  • Constantinos Christodoulides Registrar (Paediatrics), Archbishop Makarios III Hospital, Nicosia, Cyprus
  • Yurdanur Kilinc Cukurova University, Medical Faculty, Department of Pediatric Hematology, Adana, Turkey
  • Soad Al Jaouni Head, Division of Pediatric Hematology Oncology, Deputy Chair of Hematology and Head, Section of Hematology Research Lab, King Fahd Medical Research Center, Department of Hematology Faculty of Medicine, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
  • Doaa Khater Department of Pediatrics, Endocrinology Unit, Alexandria University Children’s Hospital, Egypt and Child Health Department, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman
  • Saif A Alyaarubi Head of Pediatric Endocrine Unit, Department of Child Health, Sultan Qaboos University Hospital, Al-Khoud, Sultanate of Oman
  • Su Han Lum Department of Paediatrics, University Malaya Medical Center, Malaysia
  • Saveria Campisi Thalassemia Unit, Umberto 1° Hospital, Siracusa, Italy
  • Salvatore Anastasi Thalassemia Unit, Maternal and Child Department, Garibaldi Hospital, Catania, Italy
  • Maria Concetta Galati Department of Haematology, Thalassaemia and Prenatal Diagnosis Regional Center, Pugliese-Ciaccio Hospital, Catanzaro, Italy
  • Giuseppe Raiola Department of Paediatrics, Pugliese-Ciaccio Hospital, Catanzaro, Italy
  • Yasser Wali 27 Pediatric Hematology Unit, Child Health Department, Sultan Qaboos University Hospital, Muscat, Oman and Department of Pediatrics, Alexandria University Children’s Hospital, Egypt
  • Ihab Z Elhakim Department of Pediatrics, Ain Shams University, Cairo, Egypt
  • Demetris Mariannis Medical Student, Barts and the London School of Medicine and Dentistry, Nicosia, Cyprus
  • Vassilis Ladis Thalassemia Unit, First Department of Pediatrics National Kapodistrian University of Athens, Athens, Greece
  • Christos Kattamis First Department of Paediatrics, National Kapodistrian University of Athens, Athens, Greece

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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Published

15-01-2019

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ORIGINAL ARTICLES

How to Cite

1.
An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations. Acta Biomed [Internet]. 2019 Jan. 15 [cited 2024 Jun. 13];89(4):481-9. Available from: https://www.mattioli1885journals.com/index.php/actabiomedica/article/view/7774

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