Maternal and Perinatal Outcome of Women With Sickle Cell Disease of a Tribal Population in Central India

Key Points• Pregnant women with sickle cell disease have high risks of adverse maternal and perinatal outcomes.• The risks are greatest for those with HbSS disease (vs HbSC disease) and those in low income (vs high income) countries.A systematic review and meta-analysis of observational studies were conducted to quantify the association between sickle cell disease in pregnancy and adverse maternal and perinatal outcomes. 32-5.98). Meta-regression demonstrated that genotype (HbSS vs HbSC), low gross national income, and high study quality were associated with increased RRs. Despite advances in the management of sickle cell disease, obstetrics, and neonatal medicine, pregnancies complicated by the disease remain associated with increased risk of adverse maternal and perinatal outcomes. (Blood. 2015;125(21):3316-3325)

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“…[24][25][26][27][28][29][30][31] All studies were categorized as pregnancy cohort studies. Thirteen studies originated For personal use only.…”

Section: Study Characteristicsmentioning

confidence: 99%

Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis

Oteng‐Ntim

Meeks

Seed

et al. 2015

Blood

197

223

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“…Women with pre-eclampsia also have a reduced plasma volume at the time of diagnosis and associated intrauterine growth restriction; under-activity of the RAAS has been found in such women as well. 17 , 18 Pregnant HbSS women have been reported to have a higher incidence of pre-eclampsia 19 - 22 although not all studies have found this association. 4 , 23 , 24 …”

Section: Introductionmentioning

confidence: 99%

Volume regulatory hormones and plasma volume in pregnant women with sickle cell disorder

Afolabi

Oladipo

Abudu

et al. 2016

J Renin Angiotensin Aldosterone Syst

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Background:Sickle cell disease (haemoglobin SS (HbSS)) mainly affects those of West African origin and is associated with hypervolaemia. Plasma volume rises by up to 50% in normal pregnancy but was previously found to be paradoxically contracted in late sickle cell pregnancy. The renin–angiotensin–aldosterone system is activated very early in human pregnancy to support the plasma volume expansion. We hypothesised that activation of the renin–angiotensin–aldosterone system would be blunted in pregnant women with sickle cell disease.Materials and methods:We measured plasma volume and concentrations of plasma renin, angiotensinogen, aldosterone and other volume-related hormones in a cross-sectional study of pregnant and non-pregnant Nigerian women with HbSS or HbAA.Results:Plasma volume was higher in non-pregnant HbSS than HbAA women, but had not risen by 16 weeks, unlike plasma volume in HbAA women. The concentration of plasma renin also rose significantly less by 16 weeks in HbSS; angiotensinogen and aldosterone concentrations increased.Conclusions:The lower plasma renin concentration at 16 weeks with HbSS could be either primary or secondary to vasoconstriction related to inadequate vasodilator activity. The contracted plasma volume might then stimulate aldosterone synthesis by non-angiotensin II dependent stimulation. Studies of vasodilators such as nitric oxide, vasodilator eicosanoids or the PlGF/VEGF/sFlT-1 axis in pregnant HbSS and HbAA women will test this hypothesis.

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“…There were 25 deliveries to women with sickle cell disease and preeclampsia and disseminated intravascular coagulation were common problems. There was no maternal mortality; however, there were five intrauterine foetal deaths and one early neonatal death 50 .…”

Section: Clinical Presentation and Care Of Sickle Cell Disease Cases mentioning

confidence: 86%

Sickle cell disease in tribal populations in India

Colah

Mukherjee

Martin

et al. 2015

Indian Journal of Medical Research

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The sickle gene is widespread among many tribal population groups in India with prevalence of heterozygotes varying from 1-40 per cent. Co-inheritance of the sickle gene with β-thalassaemia, HbD Punjab and glucose-6-phosphate dehydrogenase (G6PD) deficiency has also been reported. Most of the screening programmes in India now use high performance liquid chromatography (HPLC) analysis although the solubility test is also sensitive and cheap. Sickle cell disease (SCD) among tribal populations is generally milder than among non-tribal groups with fewer episodes of painful crises, infections, acute chest syndrome and need for hospitalization. This has partly been attributed to the very high prevalence of α-thalassaemia among these tribes as well as higher foetal haemoglobin levels. However, the clinical presentation is variable with many cases having a severe presentation. There is not much information available on maternal and perinatal outcome in tribal women with sickle cell disease. Newborn screening programmes for SCD have recently been initiated in Maharashtra, Gujarat, Odisha and Chattisgarh and monitoring these birth cohorts will help to understand the natural history of SCD in India. Prenatal diagnosis is acceptable by tribal families in India. The Indian Council of Medical Research and the National Rural Health Mission in different States are undertaking outreach programmes for better management and control of the disease.

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Maternal and Perinatal Outcome of Women With Sickle Cell Disease of a Tribal Population in Central India

Cited by 22 publications

References 20 publications

Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis

Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis

Volume regulatory hormones and plasma volume in pregnant women with sickle cell disorder

Sickle cell disease in tribal populations in India

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