Influence of Sickle Hemoglobinopathies on Growth and Development

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 beta+ thalassemia (S beta+), or sickle beta O thalassemia (S beta O). 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 (P less than 0.001), and that subjects with SS and S beta O were consistently smaller and less sexually developed than those with SC and S beta+ (P less than 0.001). For both sexes and all hemoglobinopathies, low weight was more pronounced than short height and was most apparent in subjects over the age of seven. The median age of the female subjects who had attained at least Tanner Stage V was 17.3 years for those with SS, 17.2 years for S beta O, 16.0 years for SC, and 16.5 years for S beta+; 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 constitutional rather than a primary endocrinologic cause of sexual immaturity in patients with hemoglobinopathies.

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

“…All 4 genotypes were below norms for African Americans. 4 Growth differences were evident even in the youngest group of children (2-4 years old). Numerous other studies have confirmed these observations.…”

Head shape determination and cranial evaluation using anatomical landmarks in children with sickle cell disease (SCD)

“…27 Growth defi cit tends to be greater in width than in height or length and is more severe in patients with sickle cell anemia. 28 The higher cranial index recorded in the sickle cell group corresponds to the brachycephalic head shape dominant in this group. Even though, the brachycephalization is more pronounced in the female sickle cell disease children where all the age groups are brachycephalic except for age group 8-11 years as against the sickle cell males where only age group 12-15 years is brachycephalic all the remaining age groups had the mesocephalic head shape as dominant.…”

Nutritional status, hospitalization and mortality among patients with sickle cell anemia in Tanzania

“…8,9 Poor growth has been previously documented in SCA. [10][11][12] However, the causes and clinical significance are not well understood. 13 In Africa, where dietary intakes are sub-optimal for many populations, we hypothesized that nutrition is an important modifiable risk factor for SCA morbidity and that poor nutritional status is associated with increased mortality and morbidity.…”