Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region
Sickle cell anaemia is one of the most common hemoglobinopathies worldwide and an important public health problem in Brazil. This study evaluated the prevalence of sickle cell anaemia and its traits in newborns from the Amazon-Savanna Transition Region in the state of Maranhão, Brazil. A cross-sectional study was carried out, based on data from neonatal screening tests performed in 2013–2015 in Maranhão. The Hardy-Weinberg theorem was applied to analyse the frequency of expected homozygotes based on HbSS phenotype. A spatial-temporal distribution analysis was performed to delimit the regions with the greatest number of newborn cases with sickle cell anaemia. Of 283,003 newborns, 162 were found to have sickle cell anaemia, while 10,794 had a sickle cell trait, with a prevalence of 0.05% and 3.8%, respectively. The prevalence of expected homozygotes was higher in the North Region and in the state capital of Maranhão. This study may contribute to existing social and public health actions or the creation of new strategies for sickle cell disease in endemic areas in Brazil to improve the quality of life.
Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study
Background Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose—drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables. Methods The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use. Results The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences. Conclusions The impact of SCD on patients’ lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. Trial registration Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.
Pregnancy loss after first-trimester viability in women with sickle cell trait: time for a reappraisal?
OBJECTIVE
The purpose of this study was to evaluate the obstetric outcomes and pathologic findings in women with sickle cell trait.


STUDY DESIGN
In this retrospective case control study, pregnant women with sickle cell trait were studied over a 4-year period (2001-2005). The women who were delivered at > 16 weeks of gestation were compared with a cohort group of subjects with normal hemoglobin levels, and the placentas were sent for pathologic evaluation.


RESULTS
A total of 180 pregnancies were studied with a like number of control patients. Subjects who had sickle cell trait demonstrated shorter average duration of pregnancy (233 +/- 45 days vs 255 +/- 34 days; P < .001) and lower birth weight (2114 +/- 1093 g vs 2672 +/- 942 g; P < .001). The rate of fetal death was significantly higher among study group patients (3.5% vs 9.7%; P = .015) when compared with the control group. Additionally, in study women, acute ascending amniotic infection and meconium histiocytosis were noted much more frequently. Sickling in the intervillous space and decidual vessels that were not associated with artifactual change was also found among patients sickle cell trait.


CONCLUSION
Patients with sickle cell trait appear to be at increased risk for fetal loss compared with women with normal hemoglobin levels, and placental abnormalities may play a causal role.