Han-Ping Wu1,2, Cheng-Li Lin3,4, Yin-Chen Chang5, Kang-Hsi Wu5,6, Rouh-Lih Lei7, Ching-Tien Peng5,8, Tefu Weng5, Yu-Man Tai5, Yu-Hua Chao9,10. 1. Division of Pediatric General Medicine, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan. 2. College of Medicine, Chang Gung University, Taoyuan, Taiwan. 3. Management Office for Health Data, China Medical University Hospital, Taichung, Taiwan. 4. College of Medicine, China Medical University, Taichung, Taiwan. 5. Division of Pediatric Hematology-Oncology, Children's Hospital, China Medical University, Taichung, Taiwan. 6. School of Post-Baccalaureate Chinese Medicine, College of Chinese Medicine, China Medical University, Taichung, Taiwan. 7. Department of Nursing, College of Medicine and Nursing, Hungkuang University, Taichung, Taiwan. 8. Department of Biotechnology and Bioinformatics, Asia University, Taichung, Taiwan. 9. Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan. 10. School of Medicine, Chung Shan Medical University, Taichung, Taiwan.
Abstract
BACKGROUND: While transfusion and iron chelation therapy for thalassemia major (TM) has improved dramatically in recent years, the consequences of this improvement (current rates of survival and TM-related complications) remain unknown. METHODS: This nationwide population-based cohort study analyzed 2007-2011 data obtained from the Taiwanese National Health Insurance Research Database. RESULTS: After excluding those patients receiving hematopoietic stem cell transplantation, we enrolled 454 patients with TM who received transfusion and chelation therapy (median age, 17.2 years). Among these patients, the mortality rate was 2.9% in 2007, 2.3% in 2008, 2.9% in 2009, 2.6% in 2010, and 0.7% in 2011. Heart was the most common target organ of TM-related complications. There were 80 patients (17.6%) with arrhythmia and 86 patients (18.9%) with congestive heart failure. Dysfunction of endocrine organs was common, and the most common endocrinopathy was hypogonadism (23.1%), followed by diabetes (21.2%). There were 75 patients (16.5%) with liver cirrhosis and 79 patients (17.4%) with osteoporosis. CONCLUSIONS: Adequate red blood cell transfusion and iron chelation is available to all patients with TM in Taiwan under the universal health insurance system, and has resulted in reduction of TM-related mortality to very low levels. As these patients get older, early detection of complications and adequate intervention are important to quality-of-life improvement.
BACKGROUND: While transfusion and iron chelation therapy for thalassemia major (TM) has improved dramatically in recent years, the consequences of this improvement (current rates of survival and TM-related complications) remain unknown. METHODS: This nationwide population-based cohort study analyzed 2007-2011 data obtained from the Taiwanese National Health Insurance Research Database. RESULTS: After excluding those patients receiving hematopoietic stem cell transplantation, we enrolled 454 patients with TM who received transfusion and chelation therapy (median age, 17.2 years). Among these patients, the mortality rate was 2.9% in 2007, 2.3% in 2008, 2.9% in 2009, 2.6% in 2010, and 0.7% in 2011. Heart was the most common target organ of TM-related complications. There were 80 patients (17.6%) with arrhythmia and 86 patients (18.9%) with congestive heart failure. Dysfunction of endocrine organs was common, and the most common endocrinopathy was hypogonadism (23.1%), followed by diabetes (21.2%). There were 75 patients (16.5%) with liver cirrhosis and 79 patients (17.4%) with osteoporosis. CONCLUSIONS: Adequate red blood cell transfusion and iron chelation is available to all patients with TM in Taiwan under the universal health insurance system, and has resulted in reduction of TM-related mortality to very low levels. As these patients get older, early detection of complications and adequate intervention are important to quality-of-life improvement.
Authors: M Bordbar; H Bozorgi; F Saki; S Haghpanah; M Karimi; A Bazrafshan; O R Zekavat Journal: J Endocrinol Invest Date: 2019-06-21 Impact factor: 4.256
Authors: Aliya Hisam; Najm Us Sadiq Khan; Naseer Alam Tariq; Hira Irfan; Bushra Arif; Mawra Noor Journal: Pak J Med Sci Date: 2018 Jul-Aug Impact factor: 1.088
Authors: Anuja P Premawardhana; Rasnayaka Mudiyanse; Shamila T De Silva; Nilam Jiffry; Udaya Nelumdeniya; Udaya de Silva; Sanath P Lamabadusuriya; K Pushpakumara; Randima Dissanayaka; M Jansz; I Rifaya; Upul Navarathne; V Thirukumaran; Mahinda Arambepola; Wijesundara Dayanada Bandara; U Vaidyanatha; Devan Mendis; K Weerasekara; Nalika De Silva; D K Shantha Kumara; Sujeewa D Amarasena; K K Hemantha; M A C M Refai; Ishari Silva; Nizri Hameed; F Rajiyah; Sachith Mettananda; Angela Allen; David J Weatherall; Nancy F Oliveri Journal: PLoS One Date: 2019-08-16 Impact factor: 3.240