Author/Authors :
De Sanctis, Vincenzo Pediatric and Adolescent Outpatient Clinic - Quisisana Hospital, Ferrara, Italy , Soliman, Ashraf T. Department of Pediatrics - Division of Endocrinology - Alexandria University Children’s Hospital, Alexandria, Egypt , Canatan, Duran Thalassemia Diagnosis Center of Mediterranean Blood Diseases Foundation, Antalya, Turkey , Elsedfy, Heba Department of Pediatrics - Ain Shams University, Cairo, Egypt , Karimi, Mehran Hematology Research Center - Shiraz University of Medical Sciences, Shiraz, Iran , Daar, Shahina Department of Haematology - College of Medicine and Health Sciences - Sultan Qaboos University, Sultanate of Oman , Rimawi, Hala King Abdullah University Hospital, Amman, Jordan , Christou, Soteroula Thalassemia Unit, Nicosia, Cyprus , Skordis, Nicos Division of Pediatric and Adolescent Endocrinology - Pedi Center for Specialized Pediatrics - St. George’s University Medical School at the University of Nicosia, Cyprus , Tzoulis, Ploutarchos Department of Endocrinology - Whittington Hospital - University College London, London, UK , Sobti, Praveen Pediatric Hemato Oncology - Christian Medical College and Hospital, Ludhiana, Punjab, India , Kakkar, Shruti Department of Pediatrics - Dayanand Medical College & Hospital Ludhiana, Ludhiana, India , Kilinc, Yurdanur Department of Pediatric Hematology - Medical Faculty - Çukurova University, Adana, Turkey , Khater, Doaa Department of Pediatrics Endocrinology Unit - Alexandria University Children’s Hospital - Egypt and Child Health Department - Sultan Qaboos University Hospital, Muscat, Sultanate of Oman , Alyaarubi, Saif A Department of Child Health - Sultan Qaboos University Hospital, Al-Khoud, Sultanate of Oman , Kaleva, Valeriya Varna Expert Center for Coagulopathies and Rare Anemias,Varna, Bulgaria , Han Lum, Su Department of Paediatrics - University Malaya Medical Center, Malaysia , Yassin, Mohamed A National Center for Cancer Care and Research - Medical Oncology Hematology Section HMC, Doha, Qatar , Saki, Forough Endocrinology and Metabolism Research Center - Shiraz University of Medical Sciences, Shiraz, Iran , Obiedat, Maha rincess Rahma Teaching Hospital, Amman, Jordan , Anastasi, Salvatore Maternal and Child Department - Garibaldi Hospital, Catania, Italy , Concetta Galati, Maria Department of Haematology - Thalassaemia and Prenatal Diagnosis Regional Center - Pugliese-Ciaccio Hospital, Catanzaro, Italy , Raiola, Giuseppe Department of Paediatrics - Pugliese-Ciaccio Hospital, Catanzaro, Italy , Campisi, Saveria Thalassemia Unit, Umberto 1 Hospital, Siracusa, Italy , Soliman, Nada Primary Health Care - Ministry of Health, Alexandria, Egypt , Elshinawy, Mohamed Department of Pediatrics - Faculty of Medicine - University of Alexandria, Egypt , Al Jaouni, Soad Department of Hematology Faculty of Medicine - King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia , Di Maio, Salvatore Children’s Hospital “Santobono-Pausilipon”, Naples, Italy , Wali, Yasser Department of Pediatrics - Alexandria University Children’s Hospital, Egypt , Elhakim, hab Zaki Department of Pediatrics - Ain Shams University, Cairo, Egypt , Kattamis, Christos First Department of Paediatrics - University of Athens, Athens, Greece
Abstract :
Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chela-tion therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice.Aquestionnaire was sent to all Thalassemia Centres par-ticipating to the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine (ICET-A) Network.Seventeen centers, treating a total of 3023 TM and 739 TI patients, partici-pated to the study. HPT was reported in 206 (6.8%) TM patients and 33 (4.4%) TI patients. In general, ages ranged from 10.5 to 57 years for the TM group and from 20 to 54 years for the TI group. Of the 206 TM patients and 33 TI patients with HPT, 117 (48.9%) had a serum ferritin level >2.500 ng/ml (54.3% TM and 15.1% TI patients) at the last observation. Hypocalcemia varied in its clinical presentation from an asympto-matic biochemical abnormality to a life-threatening condition, requiring hospitalization. Calcium and vita-min D metabolites are currently the cornerstone of therapy in HPT. In TM patients, HPT was preceded or followed by other endocrine and non-endocrine complications. Growth retardation and hypogonadism were the most common complications (53.3% and 67.4%, respectively). Although endocrine complications were more common in patients with TM, non-transfused or infrequently transfused patients with TI suffered a similar spectrum of complications but at a lower rate than their regularly transfused counterparts.In conclu-sion, although a large international registry would help to better define the prevalence, comorbidities and best treatment of HPT, through the result of this survey we hope to give a clearer understanding of the burden of this disease and its unmet needs. HPT requires lifelong therapy with vitamin D or metabolites and is often associated with complications and comorbidities.Therefore, it is important for endocrinologists and other physicians, who care for these patients, to be aware of recent advances of this disorder.(www.actabiomedica.it)
