title: Osteoporosis in thalassemia major: an update and the I-CET 2013 recommendations for surveillance and treatment.
abstract: In recent years, the issue of osteopenia/osteoporosis in children, adolescents and young adults with thalassaemia major (TM) has attracted much attention because it is a prominent cause of morbidity despite adequate transfusion and iron chelation therapy. The reported frequency of osteoporosis, even in well treated TM patients varies from 13.6% to 50% with an additional 45% affected by osteopenia. The pathogenesis of TM-induced osteoporosis is multifactorial. Genetic and acquired factors play role in demineralization of bones in thalassemia. Osteoporosis is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. The significant predictors of fracture prevalence include male gender, hypothyroidism, age, lack of spontaneous puberty in females, active hepatitis, heart disease and diabetes. The early identification of osteopenia and osteoporosis is of paramount importance. This is because delayed diagnosis and inadequate treatment have led to severe osteoporosis, skeletal abnormalities, fractures, spinal deformities, nerve compression and growth failure. dequate hormonal replacement, has been posponed, Effective iron chelation adequate hormonal replacement, improvement of hemoglobin levels, calcium and vitamin D administration and physical activity are currently the main measures for the management of the disease. The use of bisphosphonates in TM patients with osteoporosis is increasing and their positive effect in improving bone mineral density is encouraging. The recommendations of the International Network on Growth Disorders and Endocrine Complications in Thalassaemia (I-CET) for diagnosis and management of osteoporosis in TM are also briefly included in this review.
authors.affiliation: Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy. vdesanctis@libero.it
authors.forename: Vincenzo
authors.initials: V
authors.lastname: De Sanctis
authors.affiliation: Department of Pediatrics, Division of Endocrinology, Hamad General Hospital Doha, Qatar.
authors.forename: Ashraf T
authors.initials: AT
authors.lastname: Soliman
authors.affiliation: Department of Pediatrics, Ain Shams University, Cairo, Egypt.
authors.forename: Heba
authors.initials: H
authors.lastname: Elsedfy
authors.affiliation: Department of Hematology, Alamal Hospital, Hamad Medical Center, Doha, Qatar.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Yassin
authors.affiliation: Director of Hemoglobinopathy Diagnosis Center and President of Mediterranean Blood Diseases Foundation, Antalya, Turkey.
authors.forename: Duran
authors.initials: D
authors.lastname: Canatan
authors.affiliation: Pediatric Hematology and Oncology, Cukurova University, Adana, Turkey.
authors.forename: Yurdanur
authors.initials: Y
authors.lastname: Kilinc
authors.affiliation: Department of Pediatrics, Dayanand Medical College, Ludhiana, Punjab, India.
authors.forename: Praveen
authors.initials: P
authors.lastname: Sobti
authors.affiliation: Department of Paediatrics, Division of Pediatric Endocrinology, Makarios Hospital, Nicosia, Cyprus.
authors.forename: Nicos
authors.initials: N
authors.lastname: Skordis
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
authors.affiliation: Department of Paediatrics, Pugliese Ciaccio Hospital, Catanzaro, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Raiola
authors.affiliation: Department of Haematology, Thalassaemia and Prenatal Diagnosis Regional Center, Pugliese Ciaccio Hospital, Catanzaro, Italy.
authors.forename: Maria Concetta
authors.initials: MC
authors.lastname: Galati
authors.affiliation: Department of Radiology, Hamad General Hospital, Doha, Qatar.
authors.forename: Elsaid
authors.initials: E
authors.lastname: Bedair
authors.affiliation: Department of Pediatrics, NYU School of Medicine, New York, USA.
authors.forename: Bernadette
authors.initials: B
authors.lastname: Fiscina
authors.affiliation: Department of Pediatrics, Ain Shams University, Cairo, Egypt.
authors.forename: Mohamed
authors.initials: M
authors.lastname: El Kholy
mesh_terms: D000293:Adolescent; D000328:Adult; D015519:Bone Density; D002648:Child; D005260:Female; D050723:Fractures, Bone / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods*; D010024:Osteoporosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D012307:Risk Factors; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000453:epidemiology / Q000628:therapy*