Childhood and adolescent growth of patients with sickle cell disease in Aracaju, Sergipe, north-east Brazil.

Overview

Sickle cell disease (SCD) is the most prevalent inherited monogenic pathology in South America. Although children with SCD have normal birthweight, weight deficit is often seen from early childhood. On the other hand, paradoxically, normal final height associated with delayed puberty has been reported from Brazil and Jamaica. This cross-sectional study describes the growth pattern by age and sex in 76 children and adolescents with SCD in Sergipe, north-east Brazil with a median age of 110 months. Median weights and heights for age were below the NCHS standards. The weight and height deficits were statistically significant for boys of all ages, except for 7-year-olds. Most girls have median weights and heights below the NCHS standards but this only becomes statistically significant at 15 years of age. Family channels were calculated from the parents' heights. The observed height was lower than the expected percentile value for the family in seven (41%) children, equal to expected family height in six (35%) and above expected family height in four (24%) of 17 teenagers. Our findings suggest that Brazilian children with SCD do not attain normal height and weight. It is therefore likely that, although maximum height and weight velocity occur significantly later than normal due to delayed puberty, the magnitude of this spurt is less than normal.