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Sickle cell disease and age at menarche in Jamaican girls: observations from a cohort study
G R Serjeant1, A Singhal, I R Hambleton
1Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica. grserjeant@cwjamaica.com
Insights
Age at menarche is delayed in sickle cell disease (SCD). In sickle cell haemoglobin C (SC) disease, menarche is delayed by 0.5 years, and in sickle cell (SS) disease, it is delayed by 2.4 years. Weight is the primary factor influencing menarche timing.
Area of Science:
- Hematology
- Reproductive Endocrinology
- Pediatric Health
Background:
- Sickle cell disease (SCD) encompasses genetic blood disorders affecting red blood cells.
- Understanding reproductive health milestones like menarche is crucial for managing SCD.
- Previous studies have indicated potential delays in puberty and menarche in individuals with SCD.
Purpose of the Study:
- To determine the age at menarche in individuals with homozygous sickle cell (SS) disease and sickle cell haemoglobin C (SC) disease compared to controls.
- To identify factors influencing the age of menarche in these populations.
Main Methods:
- A prospective cohort study followed individuals from birth to adulthood (18-26.5 years).
- Participants included 99 with SS disease, 69 with SC disease, and 100 with normal (AA) hemoglobin genotype.
- Regular assessments included height, weight, pubertal stage, and hematological indices.
Main Results:
- Mean age at menarche was 13.0 years in controls, 13.5 years in SC disease, and 15.4 years in SS disease.
- Greater body weight was significantly associated with earlier menarche across all genotypes.
- Fetal hemoglobin and red blood cell count influenced menarche timing in SS disease.
Conclusions:
- Menarche is significantly delayed in individuals with SCD, by 0.5 years in SC disease and 2.4 years in SS disease.
- Body weight is the principal determinant of age at menarche in this cohort.
- Alpha thalassaemia did not appear to affect the timing of menarche.
Aims:
(1) To investigate the distribution of age at menarche in a representative sample of 99 patients with homozygous sickle cell (SS) disease, 69 with sickle cell haemoglobin C (SC) disease, and 100 controls with a normal haemoglobin (AA) genotype followed in a cohort study from birth. (2) To explore the determinants of the age at menarche.
Methods:
Children ascertained in a newborn screening programme were followed prospectively from birth to age 18-26.5 years with regular assessments of height, weight, pubertal stage, and haematological indices at the Sickle Cell Clinic of the University Hospital of the West Indies.
Results:
All subjects have now reached menarche and the mean age in normal controls (13.0 years) was significantly earlier than in SC disease (13.5 years) or SS disease (15.4 years). Greater weight and earlier age at menarche was the only association significant across all genotypes although additional contributions occurred from fetal haemoglobin and red cell count in SS disease. Alpha thalassaemia, which ameliorates many of the effects of SS disease, had no discernible effect on menarche.
Conclusions:
Mean age at menarche is delayed by 0.5 years in SC disease and by 2.4 years in SS disease. Weight appears to be the principle determinant of age at menarche.
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