Objective To determine the outcome of pregnancies complicated by sickle cell disease in the UK during 1991 -1993 and the effect of prophylactic blood transfusion programmes on maternal and fetal outcome.Design A multicentre study.Subjects Eighty-one pregnancies complicated by sickle cell disease and 100 pregnancies from women of black African descent without haemoglobinopathies to act as a comparative group. Pregnancies complicated by sickle cell disease were divided by the type of haemoglobinopathy and also by transfusion regimen. Main outcome measuresAntenatal and postnatal complications of sickle cell disease, proteinuric hypertension, preterm delivery, emergency delivery by caesarean section, fetal distress, birthweight, perinatal and maternal mortality. ResultsThere were two maternal deaths in the 8 1 pregnancies and the perinatal mortality rate was 60/ 1000. Antenatal sickling complications occurred in 46.2 % of pregnancies and postnatal sickling complications occurred in 7.7 % of pregnancies. Pregnancies complicated by sickle cell disease were significantly more likely to be associated with anaemia, preterm delivery, proteinuric hypertension, birthweight below the 10th centile and caesarean section as an emergency procedure than the comparative group. Severe sickling complications occurred more commonly in the third trimester and there was some evidence that a prophylactic transfusion programme reduced the risk of this. Prophylactic transfusion did not improve obstetric outcome when compared with those pregnancies that were untransfused. ConclusionsSickle cell disease remains a severe complicating factor to pregnancy and perinatal mortality and maternal mortality rates in the UK have increased since last reported. A policy of exchange transfusing all women with homozygous sickle cell disease (HbSS) from 28 weeks gestation is recommended to reduce the risk of maternal complications in the thrd trimester and puerperium. There remains a role for earlier prophylactic blood transfusion programmes in women with poor obstetric and haematological histories.Sickle cell disease encompasses a group of inherited disorders characterised by a defect in haemoglobin synthesis which predominantly present with vaso-occlusive symptoms and complications of haemolysis. The three conditions most commonly included under sickle cell disease are sickle cell anaemia (HbSS), sickle cell haem-~~~~~~~~ ~

Aims/hypothesis Concentrations of visfatin are increased in insulin-resistant conditions, but the relationship between visfatin and insulin and/or insulin resistance indices in pregnancy remains unclear. Insulin resistance in pregnancy is further accentuated in women with gestational diabetes mellitus (GDM). Thus we assessed serum levels of visfatin in pregnant women with varying degrees of glucose tolerance. Materials and methods Fasting visfatin levels were measured at 28 weeks of gestation in 51 women divided according to their response to a 50-g glucose challenge test (GCT) and a 75-g OGTT: control subjects (n=20) had normal responses to both a GCT and an OGTT; the intermediate group (IG; n=15) had a false-positive GCT, but a normal OGTT; the GDM group (n=16) had abnormal GCTs and OGTTs. Results There were no age or BMI differences between analysed groups. Across the subgroups there was a progressive increase in glucose and insulin at 120 min of the OGTT (p<0.01). This was accompanied by an increase in visfatin, from 76.8±14.1 ng/ml in the control subjects, to 84.0±14.7 ng/ml in the IG group and 93.1±12.3 ng/ml in the GDM group (p<0.01 for GDM vs control subjects). There was a positive correlation between visfatin and fasting insulin (r=0.38, p=0.007) and insulin at 120 min of the OGTT (r=0.39, p=0.006). Conclusions/interpretation An increase in fasting visfatin, the levels of which correlate with both fasting and postglucose-load insulin concentrations, accompanies worsening glucose tolerance in the third trimester of pregnancy. However, the significance of these findings, and in particular the role of visfatin in the regulation of insulin sensitivity during pregnancy, remains to be elucidated.

Objectives To examine the pregnancies, mode of delivery and outcomes, review the literature on fertility and discuss preconceptual guidance for women with β thalassaemia major. Design An observational study. Subjects Sixteen women with p thalassaemia major. Setting Two collaborating London teaching hospitals. Main outcome measures Pre‐pregnancy assessment, pregnancy course, mode of delivery, gesta‐tional age at delivery and birthweight. Results There were 16 pregnancies in 11 women. Three of these pregnancies were terminated. Of the 13 deliveries, there were two normal deliveries, one forceps delivery and 10 caesarean sections. The main findings in a further five women seeking fertility treatment were of hypogonadotrophic hypogonadism, diabetes and cardiomyopathy. Conclusions Pregnancy in women with p thalassaemia major does not appear to have a deleterious effect on the course of the disease. No increased obstetric complications were encountered except for the high caesarean section rate, essentially due to cephalopelvic disproportion. A high incidence of cardiomyopathy and diabetes dictates a careful assessment before embarking on ovulation induction to treat the hypogonadotrophic hypogonadism which is common in these women.

The incidence of endocrine dysfunction in relation to the detailed genotype of β‐thalassaemia is investigated in this study. In addition, the association of genotype to specific clinical features of β‐thalassaemia is examined, together with the relationship between serum ferritin levels and endocrine complications. Ninety‐seven patients were included, all with transfusion dependent β‐thalassaemia. Patients were divided into 2 categories; group 1 consisted of patients with a β0/β0 genotype with or without a concomitant α‐globin gene deletion as well as patients with β0/β+ or β+/β+ genotype and normal α‐globin chain synthesis. Group 2 included patients with β+/β+ or β+/β0 genotype and one α‐globin chain deletion and those with a moderate amount of β‐globin chain synthesis (β++) and normal α‐globin chain synthesis. The results showed that group 1 patients were more likely to have severe clinical disease (p = 0.005). Sixty‐four patients (66%) had at least 1 endocrine disorder and 39 (40%) had multiple endocrinopathies; the most common abnormality was hypogonadotrophic hypogonadism (HH). There was a significant association between patients with group 1 genotypes and the presence of HH and impaired glucose tolerance or diabetes. A positive correlation was demonstrated between serum ferritin concentrations and the presence of thyroid or parathyroid dysfunction.

Over the last 15 years, 22 women with thalassemia major have completed 29 pregnancies at the Royal Hospital in London. The major pre-pregnancy issues, medications, and pregnancy care are reviewed. Experience suggests that, with proper care and guidance, pregnancies among women with thalassemia major are practical and can have successful outcomes.