Influence of Sickle Hemoglobinopathies on Growth and Development

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Abstract
To determine the influence of hemoglobinopathy on growth and development, we examined the height, weight, and sexual maturation of 2115 patients 2 to 25 years old who had homozygous sickle-cell disease (SS), SC disease (SC), sickle β+ thalassemia (Sβ+), or sickle β0 thalassemia (Sβ0). Using regression analysis of these cross-sectional data to generate growth and maturation curves for each hemoglobinopathy, we found that the curves for all hemoglobinopathy groups were significantly different from published norms for black subjects (P0 were consistently smaller and less sexually developed than those with SC and Sβ+ (P0, 16.0 years for SC, and 16.5 years for Sβ+; among male subjects the corresponding values were 17.6, 18.8, 16.6, and 16.6 years. Discriminant analysis of menarche status, weight, age, and hemoglobinopathy revealed that the influences of age and weight on menarche were similar regardless of hemoglobinopathy. This relationship suggests a consitutional rather than a primary endocrinologic cause of sexual immaturity in patients with hemoglobinopathies. (N Engl J Med 1984; 311:7–12.)