PREVALENCE OF SCOLIOSIS IN BETA-THALASSEMIA

The objective of this study was to determine the prevalence and possib le pathogenesis of scoliosis in beta-thalassemia in our country, and t o compare its characteristics to those of patients with idiopathic sco liosis from the same geographic area. Twenty-four [13 male and 11 fema le thalassemic patients aged 16 +/- 7 years (range 7-32 years)] of 115 examined patients with beta-thalassemia showed scoliosis of 14 degree s +/- 11 (range 10-65 degrees) radiologically. The prevalence of scoli osis in the thalassemic population was 21% in this series, whereas the overall prevalence of scoliosis in the general Greek population was 6 % (Smyrnis PN, Valavanis J, Alexopoulos A, Siderakis G, Giannestras NJ : School screening for scoliosis in Athens. J Bone Joint Surg 61B:215- 217, 1979). The scoliosis prevalence in the general population was sig nificantly higher in the females (5%) than in the males (1%), whereas no difference in prevalence was found between the two sexes in the tha lassemic population. The most common curve pattern in thalassemia was the left lumbar (38%) followed by the right lumbar (21%), whereas in p atients with idiopathic scoliosis the left thoracolumbar most commonly appeared (25%) followed by the left lumbar (14%). No patient with tha lassemia showed radiographic signs of congenital spinal deformities an d spinal fractures, whereas all patients showed a significant retardat ion of their skeletal maturation. The age of the thalassemic patients with scoliosis was significantly (p = 0.0003) higher than in patients without scoliosis. The hematocrit of the thalassemic patients with sco liosis was significantly (p = 0.0012) lower than in those without scol iosis, whereas the rate of transfusions was not correlated with the ma gnitude of the scoliosis. The level of ferritin was significantly (p = 0.025) higher in the thalassemic patients with scoliosis than in thos e without scoliosis. The duration of Desferal treatment was significan tly (p = 0.0357) longer in thalassemic patients with scoliosis when co mpared with those without scoliosis. Thus, the prevalence, curve patte rn, and etiology of scoliosis in beta-thalassemia differ from those of idiopathic scoliosis, indicating that the spinal deformities in thala ssemia represent a distinct type of scoliosis.