ABSTRACT
<p><b>OBJECTIVE</b>To observe the status of iron deposition in patient with β thalassemia major, and to formulate appropriate treatment strategies.</p><p><b>METHOD</b>The data of status of transfusion and chelation in 135 patients aged from 6 years and 4 months to 17 years and 11 months with β thalassemia major were collected and analyzed. Serum ferritin levels were determined and cardiac and hepatic iron deposition was determined using MRI T2(*) technology.</p><p><b>RESULT</b>Of the 135 cases studied, 66 were male, and 69 were female, their average age was 12.1 years. Serum ferritin (SF) was determined for 111 cases, it varied from 1 086.8 µg/L to 15 011.5 µg/L. Among them, 16 cases had SF level <2 000 µg/L (14.5%) , in 41 cases SF were between 2 000 and 4 000 µg/L (36.0%) ;in 54 cases SF >4 000 µg/L (48.7%) . Liver MRI T2(*) results showed that in only 8 cases (5.9%) iron content in the liver was in normal range, 19 cases (14.9%) showed mild liver iron deposition;34 (25.2%) moderate and 74 (54.8%, the youngest one was only 6 years and 4 months of age) had severe iron deposition respectively. Cardiac MRI T2(*) showed that in 89 cases (65.9%) iron content in the heart was in normal range;19 cases (14.1%) had mild cardiac iron deposition and 27 (20.0%) presented severe iron deposition (the youngest one was only 9 years and 3 months of age) . SF level was obviously related to liver and cardiac iron deposition (MRI T2(*)) r and P value were -0.284, 0.003 and -0.374, 0.000 respectively. In 108 cases regular transfusion and chelation were delayed due to financial problem. The late and insufficient dosage administered and irregular chelation caused the higher SF level and the severe iron deposition.</p><p><b>CONCLUSION</b>The survival status of β thalassemia major in China is worrisome. Majority of them had not received regular transfusion and chelation. Liver and cardiac iron deposition occur early and had a high incidence.</p>
Subject(s)
Adolescent , Child , Female , Humans , Male , Ferritins , Blood , Iron , Metabolism , Iron Chelating Agents , Therapeutic Uses , Iron Overload , Epidemiology , Liver , Metabolism , Magnetic Resonance Imaging , Myocardium , Metabolism , Radiography , Retrospective Studies , Transfusion Reaction , beta-Thalassemia , Diagnostic Imaging , Metabolism , TherapeuticsABSTRACT
<p><b>OBJECTIVES</b>To evaluate and compare thoracic vertebrae morphology between patients with idiopathic and normal adolescents through MRI.</p><p><b>METHODS</b>Two-dimensional sagittal MRI of the spine was performed in 10 normal adolescent, 10 patients with mild idiopathic thoracic scoliosis (Cobb angle 15 degrees - 39 degrees ) and 10 patients with moderate thoracic scoliosis (Cobb angle 40 degrees - 75 degrees ), all of them were female and between 13 - 14 years old. Sagittal imaging was reconstructed on image working station (Easy Vision, Philips Medical Systems, Best, Netherlands). Anterior height, posterior height and width of vertebral body as well as length between spinous process were measured on each thoracic spine.</p><p><b>RESULTS</b>Anterior height, posterior height and width of vertebral body increased from T(1) to T(12) with the values from scoliotic groups larger than normal group. The anterior height/width ratio and anterior/posterior column ratio were also larger in scoliotic group especially at apical area.</p><p><b>CONCLUSION</b>The thoracic vertebrae are higher and slimmer in scoliotic patient than in normal age-matched girls which implied that there is abnormal endochondral ossification on spine during adolescent growth spurt.</p>