Growth and development in sickle cell anemia. Preliminary report.

Historically, adults with sickle cell anemia were described as being short, thin, and eunuchoid in appearance with a particular body habitus. More current investigations in children have suggested decreased height, weight, and hypogonadism although Jamaican studies suggest supranormal heights in adolescence. All studies to date have evaluated children at one point in time. We evaluated children with sickle cell anemia longitudinally at six monthly intervals over 3 years to assess somatic growth and the development of sexual maturation. Our data support reduced height and weight as compared to National Health Statistic norms, with normal skin fold thickness. Bone ages were significantly retarded. When a patient's chronological age was replaced by his bone age and tanner staging was done, sexual development was appropriate for bone age, suggesting delayed sexual maturation. In addition, menarche was significantly delayed. Pituitary gonadotropins showed an appropriate increase with puberty. Gonadal end organ hormones supported normal responsiveness, although an occasional patient showed depressed levels. Longitudinal data is necessary to assess children with suspected delay in somatic and sexual development. Hormonal replacement does not seem warranted in the majority of patients.