N. Olatunji Olambiwonnu

Sexual maturation in subjects with sickle cell anemia: studies of serum gonadotropin concentration, height, weight, and skeletal age.

Luteinizing hormone and FSH concentrations were determined in sera obtained from 20 males and 20 females, ages 5 to 16 years, with sickle cell anemia (homozygous hemoglobin S). Height, weight, bone age, and stage of sexual development were also determined. Gonadotropin concentrations were increased for stage of sexual development, suggesting transient impairment of gonadal function during the first decade of life. Increased impairment of growth during the second decade of life (18 of 26 subjects, 69%) as compared to the first decade (4 of 14 subjects, 29%) was consistent with prior deficiency of gonadal secretion of steroids. Mean bone age (11.2 +/- 2.1 year) determined in 15 subjects was significantly less than the mean chronologic age (13.0 +/- 1.8 year). Significant additional data, particularly those which would be derived from a longitudinal study, are needed to validate the preliminary hypothesis that transient impairment in gonadal function may, in part, account for the variation in sexual maturation seen in subjects with sickle cell anemia.

CORRELATION OF SERUM LUTEINIZING HORMONE (LH) AND FOLLICLE STIMULATING HORMONE (FSH) CONCENTRATIONS WITH HEIGHT AND WEIGHT

Serum LH and FSH concentrations were determined in 16 normal girls (6-16 yrs of age) and 16 normal boys (9-18 yrs of age) and plotted against height and weight. There was a positive correlation between serum LH and height in girls (r=0.60; p < .01) and in boys (r=0.89; p < .005). A similar positive correlation was found between serum FSH and height in girls and boys (r=0.63; p < .005 for both sexes). A positive correlation was also observed between serum LH and weight in girls (r=0.60; p < .01) and boys (r=0.91; p < .005) and between serum FSH and weight in girls (r=0.47; p < .05) and boys (r=0.71; p < .005).More than two-thirds of the observed concentrations of LH and FSH fell within one standard deviation of calculated regression lines. Speculation: Cell mass can be predicted from the relationship between body composition and height and weight. From infancy to adolescence, there is a linear relationship between basal heat production and visceral cell mass. Serum LH and FSH concentrations would be expected to increase with height and weight if it is assumed that the attainment of a critical metabolic rate may be associated with reduced sensitivity of the hypothalamus to sex steroids giving rise to a change in the gonadal-hypothalamic feedback set-point.

SERUM LUTEINIZING HORMONE |[lpar]|LH|[rpar]| AND FOLLICLE STIMULATING HORMONE |[lpar]|FSH|[rpar]| CONCENTRATIONS IN SUBJECTS WITH SICKLE CELL ANEMIA |[lpar]|SCA|[rpar]|

LH and FSH concentrations were determined in sera obtained from 40 subjects (20 males and 20 females; ages 5-16 years) with SCA (homozygous hemoglobin S). Mean LH concentrations were significantly increased above those of comparable age normal subjects in 5-8 year old males (5.3 ± 0.6 mIU/ml vs. 3.4 ± 0.6 mIU/ml), 5-8 year old females (6.7 ± 1.4 vs. 2.6 ± 0.3) and 9-10 year old females (6.8 ± 1.9 vs. 4.0 ± 1.3). The mean FSH concentration was also significantly increased in 13-14 year old females (12.8 ± 1.5 mIU/ml vs. 8.0 ± 2.9 mIU/ml). The LH concentration in 10 subjects (4 males and 4 females ages 5-8 and 2 females ages 9-10) and the FSH concentration in 7 subjects (all females ages 11-16) were > 2 SD above the mean of normal subjects. The observed increase in LH concentration during the first decade of life is consistent with impaired gonadal function and correlates well with the period of greatest morbidity in SCA. while the observed increase in FSH concentration may represent racial variation in females (Clin. Res. 21:866, 1973), it is also consistent with impaired gonadal function. Transiently impaired gonadal function may, in part, account for the variations in sexual maturation seen in patients with SCA.