Iron overload in female patients with sickle cell disease (SCD) has been reported to result in gonadal dysfunction. To date there has been no report in the literature of ovarian sickling being a reason for gonadodysgenesis (premature ovarian failure [POF]) in women. This case report describes POF in a woman with SCD and suggests ovarian sickling as its cause. We propose that frequent episodes of intravascular sickling, vessel occlusion and infarction as well as tissue hypoxia associated with chronic anaemia could account for the ovarian dysgenesis and hence POF.
The objective of this retrospective cohort study from two tertiary centres in the UK was to describe the pregnancy outcomes of women with sickle cell disease (SCD) who booked at these centres between 2004 and 2008, and to compare this with historical data. The study population comprised 122 singleton pregnancies in women with SCD: homozygous sickle cell disease 64, sickle cell haemoglobin C disease 45, sickle b plus thalassaemia 11, sickle cell haemoglobin E disease 1 and sickle cell delta disease 1 from 2004 to 2008 managed in the joint haematology/obstetric antenatal clinics in two tertiary teaching hospitals. The main outcome measures were the frequency of sickle cell crises and obstetric complications. Age and gestation at booking were 18-43 years (mean 29.7) and 9-36 weeks gestation (mean 17.3), respectively. Complications of SCD occurred in 25% of pregnancies. Fifty-four percent of women had induction of labour and 39% were delivered by emergency caesarean section. Thirty-three percent had a postpartum haemorrhage. Nineteen percent of women delivered before 37 completed weeks. Birth weight below 2500 g occurred in 20% of singleton pregnancies. Three neonates developed transient complications related to maternal opiate exposure postnatally. Three intrauterine deaths occurred at 24, 29 and 34 weeks. Two of these had congenital defects, and the other severe intrauterine growth restriction. No maternal deaths occurred. Successful pregnancy outcomes can be achieved in SCD. There has been an improvement in fetal and maternal morbidity and mortality compared with historical data. Pregnancy in women with SCD remains high risk. Early access to antenatal care and to expertise in SCD is essential. A matched control population from the same time period and prospective data collection is needed to address confounders such as ethnicity and deprivation.
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