Singhal, Atul; Gabay, Leslie A.; Serjeant, Graham R.
Author Affiliation, Ana.
Medical Research Council Laboratories (Sickle Cell); Obstetrics, Gynaecology and Child Health
Testosterone deficiency and extreme retardation of puberty in homozygous sickle-cell disease.
West Indian Medical Journal
Date of Publication
Analysis of growth from children in the Jamaican Cohort Study noted extreme growth retardation , defined as an absence of the adolescent growth spurt and pre-pubertal sexual development (Tanner stage 1 or 2) at age 16 years, in 8/52 (15 percent) SS boys. These and two boys from the general sickle-cell clinic with a similar growth pattern provided a study group of 10 boys who were investigated for a possible endocrine explanation for their extreme retardation of physical maturation. A sub-optimal testosterone response (10 nmol/l) to human chorionic gonadotrophin and an exaggerated gonadotrophin hormone releasing hormone was consistent with poor testicular function in 5 boys. Retardation of adolescent growth and development is common in boys wit SS disease but, when extreme, requires early investigation to identify potentially correctable mechanisms.....