Increased prevalence of fractures in inadequately transfused and chelated Indian children and young adults with beta thalassemia major.

Affiliation

Ekbote V(1), Padidela R(2), Khadilkar V(3), Ramanan V(4), Maheshwari A(5), Mughal Z(6), Kariki EP(7), Crabtree N(8), Khadilkar A(9).
Author information:
(1)Growth and Pediatric Endocrine Department, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, 32, Sassoon Road, Pune, Maharashtra 411 001, India. Electronic address: [Email]
(2)Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. Electronic address: [Email]
(3)Growth and Pediatric Endocrine Department, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, 32, Sassoon Road, Pune, Maharashtra 411 001, India; School of Health Sciences, Savitribai Phule Pune University, Ganeshkhind Rd, Ganeshkhind, Pune, Maharashtra 411007, India. Electronic address: [Email]
(4)Clinical Hematology and Transplant, Yashoda Hematology Clinic, Pune, Maharashtra, India. Electronic address: [Email]
(5)Pediatric and Adolescent Endocrinology, Sir Aurobindo Institute of Medical Science, Indore, Madhya Pradesh 453555, India. Electronic address: [Email]
(6)Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. Electronic address: [Email]
(7)Department of Clinical Radiology, Manchester Royal Infirmary, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: [Email]
(8)Department of Endocrinology and Diabetes, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK. Electronic address: [Email]
(9)Growth and Pediatric Endocrine Department, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, 32, Sassoon Road, Pune, Maharashtra 411 001, India; School of Health Sciences, Savitribai Phule Pune University, Ganeshkhind Rd, Ganeshkhind, Pune, Maharashtra 411007, India. Electronic address: [Email]

Abstract

INTRODUCTION: In patients with beta thalassemia major, inadequate transfusion and chelation may compromise bone health and increase risk of fractures. The objective of this study was to describe the prevalence of fractures in Indian inadequately transfused and chelated children, adolescents and young adults with beta thalassemia major. METHODS: We studied 179 patients with beta thalassemia (3.6-28.3 years; 105 boys). Medical, transfusion, chelation and fracture history were recorded. Vertebral fracture assessment (VFA) was performed using lateral spine images acquired using the GE Lunar iDXA (Wisconsin, MD). Fractures were classified according to an adapted semi-quantitative method. RESULTS: History of non-traumatic long bone fractures was observed in 21% patients (n = 37); there were significantly greater (p < 0.05) number of males (n = 30) than females (n = 15). The 21% fracture prevalence in the present study is higher than the reported fractures of 9% in healthy Indian children and adolescents. The prevalence of vertebral fractures was 4.5% (n = 8) in the study group. Of those with fractures, four patients had both long bone and vertebral fractures, and (any, long bone or vertebral fractures) sixteen patients had more than 1 fracture; eleven patients had 2 fractures, four patients had 3 fractures and one patient had 5 fractures. Thus, in 179 patients, there were a total of 68 single fractures which translates to 307 fractures per 10,000 patient years. CONCLUSION: This study found increased prevalence of non-traumatic long bone and vertebral fractures in children and adolescents with thalassemia major.