SHORT STATURE IN HOMOZYGOUS BETA-THALASSEMIA IS DUE TO DISPROPORTIONATE TRUNCAL SHORTENING

Abstract

BACKGROUND AND OBJECTIVE: Growth failure in homozygous beta-thalassaemia has been recognized for many years, and has persisted despite major treatment advances. In this cross-sectional study, sitting and standing height were measured to determine whether growth failure was disproportionate. DESIGN: Patient data were analysed in three age groups, 2-10 years, 11-18 years and 19 and over. Sitting height and subischial leg length were also determined in a cohort of parents (n = 19) and normal Greek adolescents (n = 32). PATIENTS AND MEASUREMENTS: Of the known 156 patients with homozygous beta-thalassaemia in the State of Victoria, 154 (98.7%) attend our institution. Sitting and standing heights ..