Frequency of musculoskeletal and otorhinolaryngologic manifestations in children with B- thalassemia

Background: Beta-thalassaemias have a wide variety of musculoskeletal system manifestations. Spinal involvement related to disease course and treatment is common in patients with thalassemia syndromes. Also, a high frequency sensorineural hearing loss is observed in a large percentage of patients during intensive desferrioxamine therapy. Aim: To study the clinical and radiographic skeletal changes in transfusion-dependent beta-thalassaemia patients and to present a comprehensive overview of spinal involvement in those patients. Patients and Methods: This cross sectional study was carried out on 240 patients with ?-thalassemia major who came weekly for blood transfusion at the hematology outpatient clinic of Pediatric Department at Zagazig University hospital during a period from 2012-2013. In all patients studied, detailed history regarding musculoskeletal involvement was taken and locomotor examinations were performed. Also, preliminary auditory perceptual assessment of patient’s voice and careful laryngeal examination. All patients underwent all routine laboratory investigations (CBC, Serum iron and ferritin, Liver and renal functions ) , Imaging study with standing anteroposterior and lateral X-rays of the spine, Ultra sonography on affected knee (s) joint , augmentation and documentation of the glottic picture and high-fidelity voice recording and acoustic analysis was done. Results : locomotor system involvement was found in 56 patients (23.3 %). Most frequent complaints were arthralgia and low back pain in 21.3% and 6.3% of patients respectively. Scoliosis was detected radiologically in 8 patients (3.3% ). The most common curve pattern in thalassemia was the left lumbar (62.5 %) followed by the right lumbar (37.5). Ultrasonography of affected knee (s) joint showed synovitis , effusions , signs of metaphysis dysplasia and increased power Doppler signals in (23.8% ,14.3 %,85.7% ,19 % respectively ). There was mild to moderat laryngeal congestion nearly in all cases but the vocal fold mobility (crico-arytenoid joint) is not affected. Conclusions: Patients with beta thalassemia have a variety of musculoskeletal problems and spinal involvement is common. Further studies will be needed to detect the risk factors involved in the development of these musculoskeletal problems.