RESUMO
Objective/Background: Sickle cell disease is a hereditary hemoglobinopathy characterized by abnormal hemoglobin production, hemolytic anemia, and intermittent occlusion of small blood vessels, leading to tissue ischemia, chronic organ damage, and organ dysfunction including endocrine organs. The aim of this work was to evaluate some gonadal hormones in female children with sickle cell anemia [SCA] in correlation with iron overload
Methods: This study was conducted on 40 female children with SCA with a serum ferritin of > 1000 ng/mL, who were attendants at the Hematology Unit, Pediatric Department, Tanta University, Tanta, Egypt in the period from May 2012 to April 2014. Their ages ranged from 11 years to 15 years and the mean age value was 12.63 +/- 1.36 years [Group I]. Forty female children with SCA of matched age with no iron overload served as a control Group [Group II]. For all patients in Groups I and II the following were performed/assessed: complete blood count, hemoglobin electrophoresis, serum iron status, serum estrogen, luteinizing hormone [LH], and follicle-stimulating hormone [FSH]
Results: There were significantly higher serum ferritin and serum iron levels and significantly lower total iron binding capacity, FSH, LH, and estrogen levels in Group I compared with Group II [mean serum ferritin was 2635.1 +/- 918.9 in Group I vs. 292.55 +/- 107.2 in Group II with a p value of .001; mean serum iron was 196.3 +/- 55.6 in Group I vs. 120 +/- 16.57 in Group II with a p value of .001 and mean serum total iron binding capacity was 247.3 +/- 28.6 in Group I vs 327.8.7 +/- 21.96 in Group II with a p value of .001; mean FSH level was 1.36 +/- 0.22 mIU/mL in Group I vs. 2.64 +/- 0.81 mIU/mL in Group II with a p value of .021; mean LH level was 0.11 +/- 0.006 mIU/mL in Group I vs. 1.78 +/- 1.12 mIU/mL in Group II with a p value of .003; mean estrogen level was 21.45 +/- 10.23 pg/mL in Group I vs. 42.36 +/- 15.44 pg/mL in Group II with a p value of 0.001] with significant negative correlation between serum gonadal hormones and serum ferritin [r = 0.835 and p value of .01 for FSH and serum ferritin; r = 0.597 and a p value of .01 for LH and serum ferritin; and r = 0.624 and p value of .01 for estrogen and serum ferritin
Conclusion: Female patients with SCA with iron overload may have gonadal hormone deficiency with significant negative correlations between gonadal hormones including FSH, LH, estrogen, and serum ferritin. Recommendations include regular iron chelation for prevention of irreversible damage of the ovaries and attaining normal sexual maturation, and regular follow up for females with SCA with assessment of puberty as they are more vulnerable to develop hypogonadism and may require hormonal replacement therapy