BACKGROUND: β-Thalassemia is extremely prevalent in Guangxi province, Southern China. However, little is known about the treatment and complications of patients with thalassemia major (TM) in Guangxi. The first thalassemia center in China was opened in Guangxi in 2003. Since that time, more than 400 patients have been enrolled. PROCEDURE: From December 2009 to February 2010, data was collected from TM patients visiting the thalassemia center including the circumstances of diagnosis, biological and clinical data, markers of iron overload and treatment. RESULTS: Data on 231 patients (median age, 5 years; range, 5 months to 21 years) were recorded. Only 44.6% of patients maintained their hemoglobin levels >9.0 g/dl. In 186 patients with ferritin levels >1,000 ng/ml, an iron chelator was used regularly in 44.6%, irregularly in 26.9%, and was not used in 28.5%. The mean serum ferritin level was 3,143 ng/ml and levels increased with age. Height and weight retardation were found in 48.3% and 11.1% patients, respectively. Compared to patients treated outside of the center, patients completing treatment in the thalassemia center had a higher hemoglobin level before transfusion, higher height and weight SD score, and less splenomegaly, but a similar ratio of regular or irregular iron chelation. Six (18.2%) of 33 patients >10 years of age (14.3 ± 2.8 years; range, 11-19 years) were diagnosed as hypothyroid. CONCLUSIONS: Although survival status of patients with TM in Guangxi has improved since the opening of the thalassemia center, TM complications remain high and with an early onset.
BACKGROUND: β-Thalassemia is extremely prevalent in Guangxi province, Southern China. However, little is known about the treatment and complications of patients with thalassemia major (TM) in Guangxi. The first thalassemia center in China was opened in Guangxi in 2003. Since that time, more than 400 patients have been enrolled. PROCEDURE: From December 2009 to February 2010, data was collected from TM patients visiting the thalassemia center including the circumstances of diagnosis, biological and clinical data, markers of iron overload and treatment. RESULTS: Data on 231 patients (median age, 5 years; range, 5 months to 21 years) were recorded. Only 44.6% of patients maintained their hemoglobin levels >9.0 g/dl. In 186 patients with ferritin levels >1,000 ng/ml, an iron chelator was used regularly in 44.6%, irregularly in 26.9%, and was not used in 28.5%. The mean serum ferritin level was 3,143 ng/ml and levels increased with age. Height and weight retardation were found in 48.3% and 11.1% patients, respectively. Compared to patients treated outside of the center, patients completing treatment in the thalassemia center had a higher hemoglobin level before transfusion, higher height and weight SD score, and less splenomegaly, but a similar ratio of regular or irregular iron chelation. Six (18.2%) of 33 patients >10 years of age (14.3 ± 2.8 years; range, 11-19 years) were diagnosed as hypothyroid. CONCLUSIONS: Although survival status of patients with TM in Guangxi has improved since the opening of the thalassemia center, TM complications remain high and with an early onset.
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