Sexual maturation of girls with homozygous sickle disease. About 72 cases.

We carried out a case-control study to investigate the sexual maturation of girls suffering from homozygous sickle cell disease: 72 cases aged 10 to 18 years were compared to 40 controls of the same age. We obtained the following results: In cases, there was a significant delay in signs of physical maturation; puffiness of the mammary glands at 14,4 years +/-1 (as versus 12.4 years +/-1.5 in controls), pubarche occurred at 14.2 years +/- 1, Menarche occurred on average at 15.2 years +/-1.6 (as versus 13.4 years +/- 1.4 for controls) and the lack of the menarche between the ages of 14 and 18 years was observed in 71% of cases as versus 10% of controls. At age 16, only 1% and 3% of cases were respectively at the stage of S5 and P5 as versus 40% for the controls. Thus, 37% of girls suffering from homozygous sickle cell disease were not sexually mature, whereas all controls had reached sexual maturation by the age of 14. In cases, weight remained low, and waist line and the body shape poorly developed in comparison to controls'. The examination by scanner of the pelvis of 20 girls suffering from homozygous sickle cell girls that in 25% of the cases, the ovaries of non mature girls were relatively small.