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Background: Laboratory results are frequently abnormal in pregnant mothers. Abnormalities usually relate to pregnancy or associated complications. Hematological abnormalities and age in pregnancy may increase the likelihood for transfusion and mortality., Study Design and Methods: Hematological profiles and transfusion history of pregnant mothers presenting to a tertiary hospital, were evaluated over 2 years. Age, anemia, leukocytosis and thrombocytopenia were assessed for transfusion likelihood. Iron deficiency and coagulation were assessed in transfused patients. Anemia, leukocytosis, thrombocytopenia, human immunodeficiency virus (HIV) and transfusion were assessed for mortality likelihood., Results: There were 12,889 pregnant mothers included. Mothers <19-years-old had the highest prevalence of anemia (31.5%) and proportion of transfusions (19%). The transfusion likelihood was increased in mothers with anemia (odds ratios [OR] = 6.41; confidence intervals at 95% [95% CI] 5.46-7.71), leukocytosis (OR = 2.35; 95% CI 2.00-2.76) or thrombocytopenia (OR = 2.71; 95% CI 2.21-3.33). Mothers with prolonged prothrombin times received twice as many blood products as their normal counterparts (p = .03) and those with iron deficiency anemia five times more blood products (p < .001). Increased likelihood for mortality was seen in patients with anemia (OR = 4.15, 95% CI 2.03-8.49), leukocytosis (OR = 2.68; 95% CI 1.19-6.04) and those receiving blood transfusion (OR = 3.6, 95% CI 1.75-7.47)., Discussion: Adolescence, anemia, leukocytosis and thrombocytopenia expose mothers to a high risk for transfusion and/or mortality. These risk factors should promptly trigger management and referral of patients. Presenting hematological profiles are strong predictors of maternal outcome and transfusion risk., (© 2024 The Authors. Transfusion published by Wiley Periodicals LLC on behalf of AABB.)

Objective: To document pregnancy outcome in homozygous sickle cell (SS) disease and in age-matched controls with a normal haemoglobin genotype followed from birth for up to 45 years., Methods: A total of 100 000 consecutive non-operative deliveries screened for sickle cell disease at the main Government maternity hospital in Kingston, Jamaica between 1973 and 1981 detected 311 (149 female) babies with SS disease who were matched by age and gender with 250 (129 female) controls with an AA haemoglobin phenotype. These individuals have been followed from birth with prospective assessment of menarche and detailed documentation of all pregnancies., Results: There were 177 pregnancies in 71 SS patients and 226 pregnancies in 74 AA controls. Mothers with SS disease had more spontaneous abortions (adjusted relative risk [aRR] 3.2, 95% CI 1.6-6.1), fewer live births (aRR 0.7, 95% CI 0.6-0.9) and their offspring were more likely to have a gestational age <37 weeks (aRR 2.1, 95% CI 1.1-3.7) and low birthweight <2.5 kg (aRR 3.0, 95% CI 1.6-5.3). They were more prone to acute chest syndrome (aRR 13.7, 95% CI 4.1-45.5), urinary tract infection (aRR 12.8, 95% CI 1.3-125.9), pre-eclampsia/eclampsia (aRR 3.1, 95% CI 1.1-8.8), retained placenta (aRR 10.1, 95% CI 1.1-90.3), sepsis (Fisher's Exact test 0.04) and pregnancy-related deaths (Fisher's Exact test 0.02). Four of five deaths were attributable to acute chest syndrome. There was no genotypic difference in pregnancy-induced hypertension or postpartum haemorrhage., Conclusion: Pregnancy in SS disease carries risks for both mother and child. The variable characteristics of pregnancy-related deaths complicate their prevention., Tweetable Abstract: Pregnancy in SS disease compared with controls showed increased abortions and stillbirths, fewer live births and maternal deaths in 7% patients., (© 2021 John Wiley & Sons Ltd.)

Objective: To estimate the incidence of anemia in pregnancy and compare the maternal and perinatal outcomes of women with and without anemia., Methods: We conducted a population-based retrospective cohort study on all pregnant women in British Columbia who had a live birth or stillbirth at or after 20 weeks of gestation between 2004 and 2016. Women were diagnosed with anemia based on two criteria: third-trimester hemoglobin value or a delivery admission diagnosis of anemia (made before delivery). Anemia was categorized into no anemia (hemoglobin 11 g/dL or greater), mild (9-10.9 g/dL), moderate (7-8.9 g/dL), severe (less than 7 g/dL), or anemia of unspecified severity (with diagnosis made before delivery). Logistic regression was used to estimate adjusted odds ratios (aOR) and 95% CIs expressing the association between anemia and maternal and perinatal outcomes., Results: Of 515,270 women in the study population, 65,906 (12.8%) had anemia: 11.8%, 0.43%, and 0.02% had mild, moderate, and severe anemia, respectively, and 0.58% had anemia of unspecified severity. Anemic women had longer hospitalization duration and more antenatal admissions, and rates of preeclampsia, placenta previa and cesarean delivery were higher among women with anemia. The intrapartum-postpartum blood transfusion rate was 5.1 per 1,000 among women without anemia, and higher among women with anemia (aOR 2.45, 95% CI 1.74-3.45 for mild anemia; 21.3, 95% CI 12.2-37.3 for moderate anemia; not analyzable for severe anemia; and 48.3, 95% CI 6.60-353.9 for anemia of unspecified severity). Anemia was associated with preterm birth (mild anemia, aOR 1.09, 95% CI 1.05-1.12; moderate anemia, aOR 2.26, 95% CI 2.02-2.54; anemia of unspecified severity, aOR 2.27, 95% CI 2.06-2.50), small-for-gestational-age live birth, low 5-minute Apgar score, neonatal death, and perinatal death., Conclusion: Maternal anemia in pregnancy represents a common and potentially reversible risk factor associated with antepartum, intrapartum, and postpartum maternal morbidity and perinatal morbidity and mortality.

Objective: To analyze quality improvement opportunities (QIOs) identified through review of cases of maternal death from obstetric hemorrhage by the California Pregnancy-Associated Mortality Review Committee., Design: Qualitative descriptive using thematic analysis., Sample: A total of 159 QIOs identified from 33 cases of pregnancy-related deaths from obstetric hemorrhage in California from 2002 to 2007., Methods: We coded and thematically organized the 159 QIOs using three of the four domains commonly applied in quality improvement initiatives for maternal health care: Readiness, Recognition, and Response. Data did not include reporting issues, so the Reporting domain was excluded from the analysis., Results: Thematic findings indicated that facility Readiness would be improved through practice standardization, better organization of equipment to treat hemorrhage, and planning for care of women with risk factors for hemorrhage. Recognition of hemorrhage by health care providers could be improved through accurate assessment of blood loss, risk factors, and early clinical signs of deterioration. Provider Response could be improved through reducing delays in administering blood, seeking consultations, transferring women to higher levels of care within or outside of the facility, and moving on to other treatments if a woman does not respond to current treatment., Conclusion: Hemorrhage is the most preventable cause of maternal death in California. Morbidity and mortality from hemorrhage can be prevented if birth facilities and maternity care clinicians align local practices with national safety guidelines., (Copyright © 2019 AWHONN, the Association of Women’s Health, Obstetric and Neonatal Nurses. Published by Elsevier Inc. All rights reserved.)

Cardiovascular disease contributes to approximately one third of all maternal mortality and remains a significant source of peri‑ and postpartum morbidity. As more women at risk for and with cardiovascular disease are desiring pregnancy, it is imperative that general cardiologists and obstetricians participate collaboratively in preconception counseling and are more facile with management of these lesions during peri‑ and postpartum periods. This review aims to address this growing need and highlights the management strategies for some of the major high risk cardiac conditions encountered during pregnancy including anticoagulation, cardiomyopathies as well as severe mitral and aortic stenosis; aortopathy, pulmonary hypertension, and severe congenital heart lesions will not be addressed., (Copyright © 2018 Elsevier Inc. All rights reserved.)

In Africa, the maternal mortality rate in sickle cell disease (SCD) is ~10%. Our team previously demonstrated an 89% decrease in mortality rate in a before-and-after feasibility study among women with SCD living in low-resource setting in Ghana. In the same cohort including additional participants with and without SCD, we used a prospective cohort design to test the hypothesis that implementing a multidisciplinary care team for pregnant women with SCD in low-resource setting will result in similar maternal and perinatal mortality rates compared to women without SCD. We prospectively enrolled pregnant women with and without SCD or trait and followed them up for 6-week postpartum. We tested the newborns of mothers with SCD for SCD. We recruited age and parity matched pregnant women without SCD or trait as the comparison group. Maternal and perinatal mortality rates were the primary outcomes. A total of 149 pregnant women with SCD (HbSS, 54; HbSC, 95) and 117 pregnant women without SCD or trait were included in the analysis. Post-intervention, maternal mortality rates were 1.3% and 0.9% in women with and without SCD, respectively (P = 1.00); the perinatal mortality rates were 7.4% and 3.4% for women with and without SCD, respectively (P = 0.164). Among the mothers with SCD, ~15% of newborns had SCD. Multidisciplinary care of pregnant women with SCD may reduce maternal and perinatal mortality rates to similar levels in pregnant women without SCD in low-resource settings. Newborns of mothers with SCD have a high rate of SCD., (© 2018 Wiley Periodicals, Inc.)

Introduction: Sickle cell disease in pregnancy carries increased risk of maternal and perinatal morbidity and mortality. Past studies on pregnancy complications in sickle cell disease women were limited by relatively small sample sizes, and use of retrospective and hospital discharge data., Study Design: This prospective case-control study compared booked pregnant Haemoglobin (Hb) SS women with AA controls from two tertiary centres in Lagos, in order to precisely identify their complication and mortality rates and identify associated factors. Eligible pregnant HbSS and HbAA women were recruited from antenatal clinics at booking and follow-up visits. Information was collected on a proforma and data was analyzed using IBM SPSS version 20., Results: We found higher complication rate in HbSS group, commonest complications being vaso-occlusive crisis (RR 1.47, 95% CI 1.22 - 1.78), pregnancy induced hypertension (RR 1.31, 95% CI 1.08 - 1.57), urinary tract infection (RR 1.32, 95% CI 1.12 - 1.57), and intrauterine growth restriction (RR 1.2, 95% CI 1.05 - 1.34). HbSS group had higher systolic and mean arterial blood pressure values in early puerperium compared to HbAA group (p = 0.014 and 0.024 respectively). No maternal death recorded in both group. Incidence of low birth weight <2.5Kg was 38% in HbSS and 4% in HbAA subjects, p = 0.001. However, overall maternal and perinatal outcomes were comparable in both groups (p = 1.000)., Conclusion: Although sickle cell disease poses higher obstetric risk in pregnancy, maternal and perinatal outcome can be as good as in the non-sickle cell pregnant women if adequate and prompt individualized care is given to this group of women., Competing Interests: None

Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. Recognition of depression by routine screening evaluations is essential; treatment of depression is effective. Minor cognitive impairment is also common. The recognition that cognitive impairment is related to the preceding TTP can provide substantial emotional support for both the patient and her family. Because TTP commonly occurs in young black women, the frequency of systemic lupus erythematosus, as well as other autoimmune disorders, is increased. Because there is a recognized association of TTP with pregnancy, there is always concern for subsequent pregnancies. In the Oklahoma Registry experience, relapse has occurred in only 2 of 22 pregnancies (2 of 13 women). The frequency of new-onset hypertension is increased. The most striking evidence for the impact of morbidities following recovery from TTP is decreased survival. Among the 77 patients who survived their initial episode of TTP (1995-2017), 16 (21%) have subsequently died, all before their expected age of death (median difference, 22 years; range 4-55 years). The conclusion from these observations is clear. Following recovery from TTP, multiple health problems occur and survival is shortened. Therefore, careful continuing follow-up is essential., Competing Interests: Conflict of interest: The author declares no competing financial interests., (© 2018 by The American Society of Hematology. All rights reserved.)

Background: Anaemia affects as many as half of all pregnant women in low-income and middle-income countries, but the burden of disease and associated maternal mortality are not robustly quantified. We aimed to assess the association between severe anaemia and maternal death with data from the WHO Multicountry Survey on maternal and newborn health., Methods: We used multilevel and propensity score regression analyses to establish the relation between severe anaemia and maternal death in 359 health facilities in 29 countries across Latin America, Africa, the Western Pacific, eastern Mediterranean, and southeast Asia. Severe anaemia was defined as antenatal or postnatal haemoglobin concentrations of less than 70 g/L in a blood sample obtained before death. Maternal death was defined as death any time after admission until the seventh day post partum or discharge. In regression analyses, we adjusted for post-partum haemorrhage, general anaesthesia, admission to intensive care, sepsis, pre-eclampsia or eclampsia, thrombocytopenia, shock, massive transfusion, severe oliguria, failure to form clots, and severe acidosis as confounding variables. These variables were used to develop the propensity score., Findings: 312 281 women admitted in labour or with ectopic pregnancies were included in the adjusted multilevel logistic analysis, and 12 470 were included in the propensity score regression analysis. The adjusted odds ratio for maternal death in women with severe anaemia compared with those without severe anaemia was 2·36 (95% CI 1·60-3·48). In the propensity score analysis, severe anaemia was also associated with maternal death (adjusted odds ratio 1·86 [95% CI 1·39-2·49])., Interpretation: Prevention and treatment of anaemia during pregnancy and post partum should remain a global public health and research priority., Funding: Barts and the London Charity., (Copyright This is an Open Access article published under the CC BY 3.0 IGO license which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In any use of this article, there should be no suggestion that WHO endorses any specific organisation, products or services. The use of the WHO logo is not permitted. This notice should be preserved along with the article's original URL.)

Differential treatment strategies are applied in thrombotic microangiopathy (TMA) according to the sub-classifications. Hence, it is worthwhile to overview clinical manifestations and outcomes of overall TMA patients according to sub-classifications. We analyzed TMA patients whose serum lactate dehydrogenase levels >250 IU/L, with the presence of schistocytes in their peripheral blood smear, or with typical vascular pathologic abnormalities in their renal biopsy. We compared clinical manifestations including overall survival (OS) and renal survival according to TMA causes. A total of 117 TMA patients (57 primary and 60 secondary TMA) were analyzed. Renal symptom was the most common manifestation in whole patients, while renal function at diagnosis was worst in pregnancy-related TMA group. Primary TMA patients had more frequent CNS symptom and hematologic manifestation compared to secondary TMAs. Among secondary TMAs, pregnancy- and HSCT-related TMA patients showed prevalent hemolytic features. During 150.2 months of follow-up, 5-year OS rate was 64.8%. Poor prognostic factors included older age, combined hematologic and solid organ malignancies, lower hemoglobin levels, and lower serum albumin levels. There was no significant difference in OS between primary and secondary TMAs. Seventy-eight percent of patients experienced AKI during TMA. Five-year death-censored renal survival rate was poor with only 69.2%. However, excellent renal outcome was observed in pregnancy-associated TMA. TMA showed various clinical manifestations according to their etiology. Notably, both OS and renal survival were poor regardless of their etiologies except pregnancy-associated TMA. Physicians should differentiate a variety of TMA categories and properly manage this complex disease entity.

Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic. The pre-intervention period was assessed through a retrospective chart review to identify every death and the post-intervention period was assessed prospectively. Interventions consisted of joint obstetrician and hematologist outpatient and acute inpatient reviews, close maternal and fetal surveillance, and simple protocols for management of acute chest syndrome and acute pain episodes. Primary outcomes included maternal and perinatal mortality rates before and after the study period. A total of 158 and 90 pregnant women with SCD were evaluated in the pre- and post- intervention periods, respectively. The maternal mortality rate decreased from 10 791 per 100 000 live births at pre-intervention to 1176 per 100 000 at post-intervention, representing a risk reduction of 89.1% (P = 0.007). Perinatal mortality decreased from 60.8 per 1000 total births at pre-intervention to 23.0 per 1000 at post-intervention, representing a risk reduction of 62.2% (P = 0.20). A multidisciplinary obstetric and hematology team approach can dramatically reduce maternal and perinatal mortality in a low-resource setting., (© 2017 Wiley Periodicals, Inc.)

As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject. Patient A was a 28-year-old woman with sickle cell anemia with multiple complications. At 30 weeks' gestation she developed hemolysis associated with poor placental function necessitating delivery by C-section. The fetus was delivered successfully but she died due to multi organ failure after delivery. Autopsy showed pulmonary and amniotic fluid embolization. Patient B was a 37-year-old woman with uncomplicated sickle cell anemia who presented with pre term labor and crisis, then ACS and fetal distress. The infant was delivered successfully but the patient died after cardiovascular collapse. Autopsy results showed fat and bone marrow embolization as the cause of death. Pregnancy continues to be high risk for patients with sickle cell anemia including those with mild disease. Maternal perinatal mortality could be unpredictable due to serious complications of sickle cell disease. More studies to assess maternal perinatal mortality are needed.

Background: Postpartum haemorrhage (PPH) is a leading cause of maternal death in Sokoto State, Nigeria, where 95% of women give birth outside of a health facility. Although pilot schemes have demonstrated the value of community-based distribution of misoprostol for the prevention of PPH, none have provided practical insight on taking such programs to scale., Methods: A community-based system for the distribution of misoprostol tablets (in 600ug) and chlorhexidine digluconate gel 7.1% to mother-newborn dyads was introduced by state government officials and community leaders throughout Sokoto State in April 2013, with the potential to reach an estimated 190,467 annual births. A simple outcome form that collected distribution and consumption data was used to assess the percentage of mothers that received misoprostol at labor through December 2014. Mothers' conditions were tracked through 6 weeks postpartum. Verbal autopsies were conducted on associated maternal deaths., Results: Misoprostol distribution was successfully introduced and reached mothers in labor in all 244 wards in Sokoto State. Community data collection systems were successfully operational in all 244 wards with reliable capacity to record maternal deaths. 70,982 women or 22% of expected births received misoprostol from April 2013 to December 2014. Between April and December 2013, 33 women (< 1%) reported that heavy bleeding persisted after misoprostol use and were promptly referred. There were a total of 11 deaths in the 2013 cohort which were confirmed as maternal deaths by verbal autopsies. Between January and December of 2014, a total 434 women (1.25%) that ingested misoprostol reported associated side effects., Conclusion: It is feasible and safe to utilize government guidelines on results-based primary health care to successfully introduce community distribution of life saving misoprostol at scale to reduce PPH and improve maternal outcomes. Lessons from Sokoto State's at-scale program implementation, to assure every mother's right to uterotonics, can inform scale-up elsewhere in Nigeria.

Objective: The purpose of the present study is twofold: (a) to investigate the etiology of disseminated intravascular coagulopathy (DIC) caused by obstetrical conditions and (b) to present parameters that can be used in predicting DIC-related mortality in obstetrical patients., Material and Method: Obstetrical patients who had a delivery at or were referred (after delivery) to Obstetrics and Gynecology Clinic of Dicle University between July 2006 and December 2013 were retrospectively analyzed in this study. Those patients diagnosed with DIC were included in the study., Results: Fifty-six obstetrical patients carrying the diagnosis of DIC were included in this study. The overall mortality rate was 25% among these patients. More specifically, the mortality rate was 10.7% among patients with a DIC score ≤5 and 40.7% among those with a DIC score > 5. Multiple logistic regression analysis resulted in the finding that international normalized ratio (INR) and urea were among those factors affecting mortality in obstetrical DIC [OR: 8.44 (CI: 1.9-36.8), OR: 1.05 (CI: 1.0-1.1), respectively]., Conclusion: DIC is a syndrome that might be caused by obstetrical conditions. It is associated with high mortality and morbidity rates. In obstetrical DIC, urea is the most important factor affecting mortality. In addition, we are of the opinion that DIC score might guide mortality predictions as a determinant of prognosis.

Physiologic changes of pregnancy result in a hypercoagulable state, placing the risk for venous thromboembolic events at 1 in 1600 births. Venous thromboembolic events are one of the leading causes of maternal mortality. A correlation among venous thromboembolic events, pregnancy complications, and inherited thrombophilia continues to be investigated. This article primarily focuses on the impact of inherited thrombophilias on pregnancy, labor, and birth and yet also addresses acquired thrombophilia. Prophylactic and therapeutic perinatal anticoagulation are lifesaving and pregnancy-sparing interventions. Interprofessional management of these high-risk pregnancies allows for increased surveillance to reduce perinatal morbidity and mortality.

Pregnancy in women with sickle cell disease is associated with adverse maternal and neonatal outcomes. Studies assessing the effects of prophylactic red blood cell transfusions on these outcomes have drawn inconsistent conclusions. The objective of this systematic review was to assess the effect of prophylactic compared with on-demand red blood cell transfusions on maternal and neonatal outcomes in women with sickle cell disease. A systematic search of several medical literature databases was conducted. Twelve studies involving 1291 participants met inclusion criteria. The studies had moderate to high risk of bias. Meta-analysis demonstrated that prophylactic transfusion was associated with a reduction in maternal mortality (7 studies, 955 participants; odds ratio [OR], 0.23; 95% confidence interval [CI], 0.06-0.91), vaso-occlusive pain episodes (11 studies, 1219 participants; OR, 0.26; 95% CI, 0.09-0.76), pulmonary complications (9 studies, 1019 participants; OR, 0.25; 95% CI, 0.09-0.72), pulmonary embolism (3 studies, 237 participants; OR, 0.07; 95% CI, 0.01-0.41), pyelonephritis (6 studies, 455 participants; OR, 0.19; 95% CI, 0.07-0.51), perinatal mortality (8 studies, 1140 participants; OR, 0.43; 95% CI, 0.19-0.99), neonatal death (5 studies, 374 participants; OR, 0.26; 95% CI, 0.07-0.93), and preterm birth (9 studies, 1123 participants; OR, 0.59; 95% CI, 0.37-0.96). Event rates for most of the results were low. Prophylactic transfusions may positively impact several adverse maternal and neonatal outcomes in women with sickle cell disease; however, the evidence stems from a relatively small number of studies with methodologic limitations. A prospective, multicenter, randomized trial is needed to determine whether the potential benefits balance the risks of prophylactic transfusions., (© 2015 by The American Society of Hematology.)

Patients who have recovered from their acute episode of acquired ADAMTS13-deficient thrombotic thrombocytopenic purpura (TTP) were once thought to have complete recovery except for risk of relapse. Data from previous publications from the Oklahoma TTP-hemolytic uremic syndrome (HUS) Registry are summarized. Patients have decreased cognitive function and increased prevalence of hypertension, systemic lupus erythematosus, major depression, and albuminuria as compared to the expected values from the US population. The proportion of patients that died during the follow-up period was greater than expected based on the US population reference population. Among women who had a pregnancy following recovery from TTP, relapse during pregnancy or postpartum is uncommon, but the occurrence of preeclampsia may be increased. Thirteen of 16 pregnancies in these women resulted in healthy children. Increased morbidity and mortality in TTP patients following recovery suggest that TTP may be more of a chronic disorder than a disorder with acute episodes and complete recovery., (© 2015 International Society on Thrombosis and Haemostasis.)

Background: Sickle cell disease (SCD) is associated with chronic hemolysis and painful episodes. Pregnancy accelerates sickle cell complications, including prepartum and postpartum vasoocclusive crisis, pulmonary complications, and preeclampsia or eclampsia. Fetal complications include preterm birth and its associated risks, intrauterine growth restriction, and a high rate of perinatal mortality. The purpose of this study was to evaluate pregnancy outcomes in patients with SCD who underwent planned preventive red blood cell exchange (RBCX)., Study Design and Methods: We retrospectively evaluated the complications of SCD in 37 pregnant patients. Patients with SCD who had undergone prophylactic RBCX were compared with a control group who had not undergone RBCX during pregnancy., Results: Forty-three exchange procedures were performed in 24 patients. The control group comprised 13 patients with a mean age of 27.4 ± 3.3 years who had not undergone RBCX during pregnancy. Four of the five patients who developed a vasoocclusive crisis died. There was a significant difference in maternal mortality between the study and control groups (p = 0.011). There was also a significant difference in the incidence of vasoocclusive crisis between the study and control groups. One fetal death occurred in the 20th gestational week in a patient in the control group, although there were no postpartum complications in either the babies or the mothers in the control group., Conclusion: This study has demonstrated that prophylactic RBCX during pregnancy is a feasible and safe procedure for prevention of complications. Given the decrease in the risks of transfusion, RBCX warrants further study., (© 2014 AABB.)

Thrombotic thrombocytopenic purpura (TTP) is an uncommon, severe, potentially life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, altered mental status, fever, and renal abnormalities. It can be seen at any age or sex but affects women of childbearing age more commonly. Pregnancy is known as one of the most common precipitating events for the onset of TTP and occurs mostly in the late third trimester or during the puerperium. Because of relatively low prevalence of pregnancy-related TTP, here we report the clinical characteristics and successful outcomes of 7 women with pregnancy-related TTP. Median age of patients was 25 (19-32). While 4 out of 7 women were primiparous, others were multiparous. Total plasma exchange (TPE) procedure was started within 24h after admission to our hospital. All patients got into complete remission without any maternal mortality. Fetal mortality was found to be 28%. Pregnancy-related TTP is still associated with high maternal and fetal mortality rates. However, the prognosis of TTP has improved dramatically with early diagnosis and plasma- based therapies., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: Previous studies showed a higher risk of maternal morbidity amongst black and other minority ethnic (BME) groups, but were unable to investigate whether this excess risk was concentrated within specific BME groups in the UK. Our aim was to analyse the specific risks and to investigate reasons for any disparity., Methods: Unmatched case-control analysis using data from the United Kingdom Obstetric Surveillance System (UKOSS), February 2005-January 2013. Cases were 1,753 women who experienced severe morbidity during the peripartum period. Controls were 3,310 women who delivered immediately before the cases in the same hospital. Multivariable logistic regression modelling was used to adjust for known confounders and to understand their effects., Results: Compared with white European women, the odds of severe maternal morbidity were 83% higher among black African women (adjusted odds ratio (aOR) = 1.83; 95% Confidence Interval (CI) = 1.39-2.40), 80% higher among black Caribbean (aOR = 1.80; 95% CI = 1.14-2.82), 74% higher in Bangladeshi (aOR = 1.74; 95% CI = 1.05-2.88), 56% higher in other non-whites (non-Asian) (aOR = 1.56; 95% CI = 1.05-2.33) and 43% higher among Pakistani women (aOR = 1.43; 95% CI = 1.07-1.92). There was no evidence of substantial confounding. Anaemia in current pregnancy, previous pregnancy problems, inadequate utilisation of antenatal care, pre-existing medical conditions, parity>3, and being younger and older were independent risk factors but, the odds of severe maternal morbidity did not differ by socioeconomic status, between smokers and non-smokers or by BMI., Discussion: This national study demonstrates an increased risk of severe maternal morbidity among women of ethnic minority backgrounds which could not be explained by known risk factors for severe maternal morbidity.

Objective: Venous thromboembolism (VTE) is amongst the main causes of maternal death in the developed world. The objective of this study is to elucidate risk factors of VTE and specifically, predictors of fatal thromboembolic disease during the delivery., Study Design: We conducted a population-based cohort study on 8 million birth records using the Healthcare Cost and Utilisation Project-Nationwide Inpatient Sample from 1999 to 2008 to estimate the incidence and case fatality of VTE's during labour admission. Logistic regression was used to calculate the odds ratio (OR) and corresponding 95 % confidence intervals (CIs) of demographic and obstetrical determinants of VTEs and fatal VTEs., Results: The overall incidence of VTE was 167.7/100,000 births, increasing over the 10-year period, with an average case fatality rate of 0.41 %. VTE was associated with maternal age above 25, elderly primigravida, multigravida, black race, smoking, thrombophilia, cardiovascular disease, hypertension, obesity, postpartum haemorrhage and blood transfusion. Predictors of VTE fatalities included black race, hypertension, caesarean section and transfusion., Conclusion: VTE is a rare but serious condition that is increasing in incidence and is associated with a significant degree of maternal morbidity and mortality. Further research targeting prevention among high-risk groups is warranted.

Background: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies. The present review replaces and updates a Cochrane review that was withdrawn in 2006., Objectives: To assess the benefits and harms of a policy of prophylactic versus selective blood transfusion in pregnant women with sickle cell disease., Search Methods: We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 October 2013) and reference lists of retrieved studies. We did not apply any language restrictions., Selection Criteria: Randomised and quasi-randomised trials evaluating the effects of prophylactic versus selective (emergency) blood transfusion in pregnant women with sickle cell disease. Trials were considered for inclusion whether the unit of randomisation was at individual or cluster level, however, no cluster-randomised trials were identified., Data Collection and Analysis: Two review authors independently assessed trials for inclusion and assessed trial quality. Two review authors independently extracted data. Data were checked for accuracy., Main Results: Out of six relevant reports identified by the search strategy, two trials involving 98 women with sickle cell anaemia (HbSS) met our inclusion criteria. The two trials were at moderate risk of bias. Overall, there were few events for most of the reported outcomes and the results were generally imprecise. One trial (involving 72 women) reported no maternal mortality occurring in women who received either prophylactic or selective blood transfusion. The same trial (involving 72 women) indicated no clear differences in maternal mortality, perinatal mortality (risk ratio (RR) 2.85, 95% confidence interval (CI) 0.61 to 13.22) or markers of severe maternal morbidity [pulmonary embolism (no events); congestive cardiac failure (RR 1.00, 95% CI 0.07 to 15.38); acute chest syndrome (RR 0.67, 95% CI 0.12 to 3.75)] between the treatment groups (prophylactic blood transfusion versus selective blood transfusion). Prophylactic blood transfusion reduced the risk of pain crisis compared with selective blood transfusion (RR 0.42, 95% CI 0.17 to 0.99, two trials, 98 women); however, the margin of uncertainty around the effect estimate ranged from very small to substantial reduction. One trial (involving 72 women) indicated no differences in the occurrence of acute splenic sequestration (RR 0.33, 95% CI 0.01 to 7.92) and haemolytic crises (RR 0.33, 95% CI 0.04 to 3.06) and delayed blood transfusion reaction (RR 2.00, 95% CI 0.54 to 7.39) between the comparison groups., Authors' Conclusions: Evidence from two small trials of low quality suggests that prophylactic blood transfusion to pregnant women with sickle cell anaemia (HbSS) confers no clear clinical benefits when compared with selective transfusion. Currently, there is no evidence from randomised or quasi-randomised trials to provide reliable advice on the optimal blood transfusion policy for women with other variants of sickle cell disease (i.e. HbSC and HbSβThal). The available data and quality of evidence on this subject are insufficient to advocate for a change in existing clinical practice and policy.

The antiphospholipid syndrome (APS) is defined by venous or arterial thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPLs). Catastrophic APS is the most severe form of APS, which is associated with rapid development of microvascular thrombosis resulting in multiorgan failure in patients with aPLs. Patients with APS and catastrophic APS are recognized to have a high risk of recurrent thrombosis that can occur despite anticoagulant therapy. Although antithrombotic therapy remains the mainstay of treatment, bleeding manifestations can complicate management and contribute to increased morbidity. Patients with persistently elevated aPL levels, particularly those who exhibit positive testing for lupus anticoagulant, anticardiolipin antibodies, and anti-β2GPI antibodies (triple positivity), appear to be at increased risk for thrombosis and pregnancy complications, whereas isolated positivity for aPLs appears to be associated with low risk. Recognizing that patients with APS have different thrombotic risk profiles may assist clinicians in assessing the risks and benefits of anticoagulation. The optimal type, intensity, and duration of anticoagulation in the treatment of APS remain controversial, particularly for arterial thrombosis and recurrent thrombosis. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.

Background: Compared to women in their twenties, adolescents are more likely to die during childbirth. Moreover, more than half of women in sub-Sahara Africa deliver their first child before the age of 20 years., Objective: To determine the institutional maternal mortality ratio among adolescents, its risk factors/causes and suggest ways of preventing or reducing the deaths., Methods: A descriptive study based on hospital case records of all adolescent maternal deaths at Usmanu Danfodiyo University Teaching Hospital (UDUTH) Sokoto between January 2000 and December 2009., Results: There were 165 maternal deaths and 3,047 live births among the adolescent mothers within the period giving an adolescent maternal mortality ratio of 5415/100,000 live births. The main causes of death were eclampsia (53.9%), prolonged obstructed labour (15.3%), anaemia (14.5%) and haemorrhage (9.1%). Interestingly, abortion (2.4%) was an uncommon cause of adolescent maternal mortality in this study. Identified risk factors included lack of prenatal care/labour supervision and illiteracy., Conclusion: The adolescent maternal mortality ratio in the institution is remarkably high and is tragically due to preventable causes in most cases. The role of promotion of female education and utilization of family planning,prenatal and intrapartum services in reducing adolescent maternal death cannot be overemphasized.

Introduction: Thrombocytopenia affects about 10% of all pregnancies. Preeclampsia/HELLP syndrome induced thrombocytopenia may associate perinatal morbidity, preterm delivery, or low-birth-weight newborns., Objective: To assess perinatal outcome and complications of pregnancy in women presenting with thrombocytopenia., Methods: We retrospectively analyzed 936 consecutive pregnant women admitted during a 6-month period., Results: Incidence of thrombocytopenia in pregnancy was 11.11% (104/936). Thrombocytopenia represented a risk factor for premature delivery - highest risk for severe thrombocytopenia (RR=8.69, p<0.01). Thrombocytopenic preeclampsia or HELLP syndrome associated the highest rates of prematurity (RR=7.97, p=0.00, respectively 12.32). Thrombocytopenia also represented a risk factor for low-birth-weight newborns, especially severe thrombocytopenia - 2047.50 ± 938.98 g (p=0.02) versus 3224.86 ± 496.00 g in controls. Again, thrombocytopenic preeclampsia was significantly associated with low-birth-weight newborns (RR=11.94, p=0.00), with medium weight of 2462.05 ± 794.54 g versus 2932.37 ± 708.91 g in thrombocytopenic pregnancies, respectively 3224.86 ± 496.00 g (p=0.00) in normal pregnancies., Conclusions: Thrombocytopenia in pregnancy was associated with perinatal morbidity, with the strongest association for preeclampsia and HELLP syndrome - for both prematurity and low-birth-weight: the lower the platelet count, the higher the risks for the fetus/newborn. Therefore, we strongly recommend close surveillance of thrombocytopenic mothers and their babies, in order to establish the etiology and the best moment for intervention.

Objective: To investigate maternal thrombophilia in cases of Stillbirth (SB), also an uncertain topic because most case series were not characterised for cause/associated conditions of death., Study Design: In a consecutive, prospective, multicentre design, maternal DNA was obtained in 171 cases of antenatal SB and 326 controls (uneventful pregnancy at term, 1:2 ratio). Diagnostic work-up of SB included obstetric history, neonatologist inspection, placenta histology, autopsy, microbiology/chromosome evaluations. Results audited in each centre were classified by two of us by using CoDAC. Cases were subdivided into explained SB where a cause of death was identified and although no defined cause was detected in the remnants, 64 cases found conditions associated with placenta-vascular disorders (including preeclampsia, growth restriction and placenta abruption - PVD). In the remnant 79 cases, no cause of death or associated condition was found. Antithrombin activity, Factor V Leiden, G20210A Prothrombin mutation (FII mutation) and acquired thrombophilia were analysed., Results: Overall, the presence of a thrombophilic defect was significantly more prevalent in mothers with SBs compared to controls. In particular, SB mothers showed an increased risk of carrying Factor II mutation (OR=3.2, 95% CI: 1.3-8.3, p=0.01), namely in unexplained cases. Such mutation was significantly associated also with previous SB (OR=8.9, 95%CI 1.2-70.5). At multiple logistic regression, Factor II mutation was the only significantly associated variable with SB (adj OR=3.8, 95% CI: 1.3-13.5)., Conclusion: These data suggest that Factor II mutation is the only condition specifically associated with unexplained SB and could represents a risk of recurrence. PVD-associated condition is unrelated to thrombophilia., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

The maternal death rate in the United States has shown no improvement in several decades and may be increasing. On the other hand, hospital systems that have instituted comprehensive programs directed at the prevention of maternal mortality have demonstrated rates that are half of the national average. These programs have emphasized the reduction of variability in the provision of care through the use of standard protocols, reliance on checklists instead of memory for critical processes, and an approach to peer review that emphasizes systems change. In addition, elimination of a small number of repetitive errors in the management of hypertension, postpartum hemorrhage, pulmonary embolism, and cardiac disease will contribute significantly to a reduction in maternal mortality. Attention to these general principles and specific error reduction strategies will be of benefit to every practitioner and more importantly to the patients we serve., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Disseminated Intravascular Coagulation (DIC) is a complex systemic thrombohaemorrhagic disorder characterised by widespread endothelial damage. Aim of this study was to assess the prevalence of DIC in different obstetrical conditions., Methods: This descriptive study was carried out in the Department of Obstetrics and Gynaecology Unit 'A', Ayub Medical College, Abbottabad from January 2010 to December 2011. All 40 diagnosed cases of DIC were included, and their risk factors and maternal/foetal outcome were evaluated., Results: Out of 4,334 obstetrical admissions, DIC was diagnosed in 40 (0.92%) patients. Risk factors noted were eclampsia 28 (70%), abruptio placentae 7 (17.5%), septicaemia 3 (7.5%), pancytopenia 1 (2.5%), and 1 (2.5%) patient had DIC secondary to haemorrhagic shock due to placenta previa. Mean age range of patients was 31 +/- 6.69 (19-48) year, and parity was 3.17 +/- 2.56 (0-10). Mode of delivery of 34 (85%) patients was by caesarean section, and vaginal delivery occurred in 3 (7.5%) patients. Eleven (27.5%) patients had caesarean hysterectomy. Maternal mortality was 25% and perinatal mortality was (47.5%). Majority of our patients were critical and were managed in ICU., Conclusion: DIC is serious life threatening condition secondary to any underlying pathology. There is spontaneous resolution of DIC after correction of pathology.

Background: Decreases in direct maternal deaths in Jamaica have been negated by growing indirect deaths. With sickle cell disease (SCD) a consistent underlying cause, we describe the epidemiology of maternal deaths in this population., Methods: Demographic, service delivery and cause specific mortality rates were compared among women with (n = 42) and without SCD (n = 376), and between SCD women who died in 1998-2002 and 2003-7., Results: Women with SCD had fewer viable pregnancies (p: 0.02) despite greater access to high risk antenatal care (p: 0.001), and more often died in an intensive care unit (p: 0.002). In the most recent period (2003-7) SCD women achieved more pregnancies (median 2 vs. 3; p: 0.009), made more antenatal visits (mean 3.3 vs. 7.3; p: 0.01) and were more often admitted antenatally (p:<0.0001). The maternal mortality ratio for SCD decedents was 7-11 times higher than the general population, with 41% of deaths attributable to their disorder. Cause specific mortality was higher for cardiovascular complications, gestational hypertension and haemorrhage. Respiratory failure was the leading immediate cause of death., Conclusions: Women with SCD experience a significant excess risk of dying in pregnancy and childbirth [MMR: (SCD) 719/100,000, (non SCD) 78/100,000]. MDG5 cannot be realised without improving care for women with SCD. Tertiary services (e.g. ventilator support) are needed at regional centres to improve outcomes in this and other high risk populations. Universal SCD screening in pregnancy in populations of African and Mediterranean descent is needed as are guidelines for managing SCD pregnancies and educating families with SCD.

Background: According to a World Health Organization (WHO) review of nationally representative surveys from 1993 to 2005, 42% of pregnant women have anemia worldwide. Almost 90% of anemic women reside in Africa or Asia. Most countries have policies and programs for prenatal iron-folic acid supplementation, but coverage remains low and little emphasis is placed on this intervention within efforts to strengthen antenatal care services. The evidence of the public health impact of iron-folic acid supplementation and documentation of the potential for scaling up have not been reviewed recently., Objective: The purpose of this review is to examine the evidence regarding the impact on maternal mortality of iron-folic acid supplementation and the evidence for the effectiveness of this intervention in supplementation trials and large-scale programs., Methods: The impact on mortality is reviewed from observational studies that were analyzed for the Global Burden of Disease Analysis in 2004. Reviews of iron-folic acid supplementation trials were analyzed by other researchers and are summarized. Data on anemia reduction from two large-scale national programs are presented, and factors responsible for high coverage with iron-folic acid supplementation are discussed., Results: Iron-deficiency anemia underlies 115,000 maternal deaths per year. In Asia, anemia is the second highest cause of maternal mortality. Even mild and moderate anemia increase the risk of death in pregnant women. Iron-folic acid supplementation of pregnant women increases hemoglobin by 1.17 g/dL in developed countries and 1.13 g/dL in developing countries. The prevalence of maternal anemia can be reduced by one-third to one-half over a decade if action is taken to launch focused, large-scale programs that are based on lessons learned from countries with successful programs, such as Thailand and Nicaragua., Conclusions: Iron-folic acid supplementation is an under-resourced, affordable intervention with substantial potential for contributing to Millennium Development Goal 5 (maternal mortality reduction) in countries where iron intakes among pregnant women are low and anemia prevalence is high. This can be achieved in the near term, as policies are already in place in most countries and iron-folic acid supplements are already in lists of essential drugs. What is needed is to systematically adopt lessons about how to strengthen demand and supply systems from successful programs.

Anaemia is a major health problem among woman of reproductive age group, particularly in developing countries. We undertook this study to determine the maternal and perinatal outcome in patients with severe anaemia in pregnancy, with a haemoglobin concentration of < 7 g/dl. The in-hospital data were analysed for 12 months between January 2007 and December 2007 and 2.15% (n = 96) of women were found to have severe anaemia. Out of these, 18.75% had pre-term premature rupture of membranes and 5.12% of all deliveries were pre-term. Hypertensive diseases of pregnancy were seen in 17.7%; abruption in 3.12% and 9.37% had congestive cardiac failure. Postpartum haemorrhage was seen in 25.5% of the patients and 8.33% had puerperal pyrexia. Fetal distress was seen in 26% of and 33.33% had small for gestational age neonates; there were 16.66% stillbirths and 4.16% neonatal deaths. Of the 96 severely anaemic women, six died after admission. Our study shows that efforts must be taken towards safe motherhood and spreading awareness about the various consequences of anaemia, which is usually preventable with early correction.

Objective: To determine the frequency of near-miss cases, nature of near-miss events and mortality among obstetric patients., Study Design: Cross-sectional, observational study., Place and Duration of Study: The study was conducted in the Obstetric Unit of Fatima Hospital, Baqai Medical University, from January 2006 to December 2006., Methodology: Near-miss case definition was based on validated specific criteria comprising of five diagnostic features: haemorrhage, hypertensive disorders in pregnancy, dystocia, infection and anemia. The main outcome measures were frequency and characteristics of near-miss cases, total hospital stay, high dependency unit/ICU stay and development of multiple organ dysfunction. Near-miss events and maternal deaths were described with respect to disease profiles. Mortality indices were determined for various disease processes to appreciate the standard of care provided for near-miss cases. The maternal death to near-miss ratio was calculated., Results: There were 868 deliveries in the year 2006, 44 near-miss cases, 47 near-miss events and 6 maternal deaths. The maternal death to near-miss ratio was 1:7. The most common type of near-miss events were obstetrical haemorrhage, anemia and dystocia responsible for 51%, 21.2% and 14.8% respectively. Severe hypertensive disorders in pregnancy and infections accounted for 8.5% and 4.2% of near-miss events. Postpartum haemorrhage was responsible for 83.3% and infection for 16.6% of the maternal deaths. The mortality index was higher for infections (33.3%) than for haemorrhage (17.2%). Organ system dysfunction/failure was diagnosed in 18.1% of near-miss cases., Conclusion: This study showed that for every 7 women who survived life threatening complications, one died. However, the underlying disease processes for near-miss and mortalities were almost same. Evaluation of the circumstances surrounding near miss cases could act as proxy for maternal deaths in the studied population.

Objective: Through the Australian and New Zealand Haemostasis Registry, we report on the Australian and New Zealand experience with recombinant activated factor VII (rFVIIa) in obstetric patients., Methods: The role of rFVIIa for off-label indications, including trauma, cardiac surgery, and severe postpartum hemorrhage, remains controversial. The Haemostasis Registry established by Monash University in Melbourne, Australia monitors off-label use of rFVIIa across Australia and New Zealand. The purpose of this study was to summarize Registry data for all obstetric hemorrhage patients treated with rFVIIa at participating hospitals between January 2002 and July 2008. The primary outcome measures were reduction or cessation of bleeding (positive therapeutic response), mortality, and hysterectomy rate., Results: During the study period, the Registry received data for 2128 patients. This included 110 cases of administration of rFVIIa in obstetric patients from 38 hospitals, comprising 5% of the total Registry population, 105 of whom were treated for acute hemorrhage. Women received median (interquartile range) individual doses of 92 microg/kg (73-100) of rFVIIa (median total dose 92 microg/kg [58-108]), and 78% of patients received a single dose. The positive response rate to rFVIIa was 76% with 64% responding to the first dose. Ninety-one percent of women were alive at 28 days. Forty-three women (41%) underwent hysterectomy before receiving rFVIIa and, of those remaining, 13 (21%) required hysterectomy after rFVIIa therapy. Two thromboembolic events (1 pulmonary embolism and 1 deep venous thrombosis) and 1 case of hypoxic-ischemic encephalopathy resulting from severe anoxia were reported., Conclusions: The reported effect of rFVIIa in many, but not all, obstetric cases was positive. There was no mortality as a result of thromboembolic complications. Randomized, controlled trials are required to confirm its safety and efficacy and to assess the possibility that use at an earlier stage in treatment of severe postpartum hemorrhage may avoid the need to resort to postpartum hysterectomy for control of bleeding, thus preserving fertility.

The management of the unconscious pregnant patient encompasses many aspects of obstetrics and critical care. It is not uncommon to have to manage such a patient, therefore one needs to be well prepared. There is a spectrum of altered consciousness, brain death being the most extreme. The causes of unconsciousness can be general or pregnancy specific. It is important to consider the physiological changes in pregnancy when managing these patients. The immediate resuscitative measures are mostly the same as for the nonpregnant with a few modifications. It is important to remember that there are two patients involved and this can complicate management issues. A multidisciplinary approach would be prudent. The intermediate and long-term management should also involve the family. The issues of perimortem and somatic support for foetal maturity are also discussed.

Upshaw-Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic purpura (TTP) due to mutations in the gene that encodes for ADAMTS13 (ADAMTS13), but its clinical signs may be mild or absent during childhood. We have identified 37 patients with USS (24 females, 13 males) belonging to 32 families. The nine women from six families who were diagnosed during their first pregnancy are the focus of this report. Six of the nine women had episodes of thrombocytopenia during childhood misdiagnosed as idiopathic thrombocytopenic purpura. Thrombocytopenia occurred during the second-third trimesters in each of their 15 pregnancies, with 16 babies (one twin pregnancy), often followed by TTP. Of 15 pregnancies, eight babies were stillborn or died soon after birth, and the remaining seven were all premature except one, who was born naturally following plasma infusions to the mother that had started at 8 weeks' gestation. All nine USS women had severely deficient ADAMTS13 activity. ADAMTS13 analyses demonstrated that eight women were compound heterozygotes of Y304C/G525D (2 siblings), R125VfsX6/Q1302X (2 siblings), R193W/R349C (2 siblings), I178T/Q929X, and R193W/A606P; one woman was homozygous for R193W. Only the R193W mutation has been previously reported. These observations emphasize the importance of measuring ADAMTS13 activity in the evaluation of thrombocytopenia during childhood and pregnancy.

Women with sickle cell disorder are historically known to have significant maternal and perinatal complications but recent studies from developed countries show a change in this trend. This study was a retrospective, case-controlled study of 75 women with haemoglobin SS (HbSS) and 150 with haemoglobin AA (HbAA). Data were analysed using chi(2)-test and independent t-test as appropriate. There were more perinatal (18.7 vs 8.8, p<0.05) and maternal (5.3% vs 0, p<0.05) deaths in HbSS women compared with HbAA. Birth weight, gestational age at delivery and 1 and 5 min Apgar scores were also significantly lower in the HbSS women. There was no significant difference in the incidence of pre-eclampsia and urinary tract infection between the two groups. Pregnancy in HbSS women is still fraught with maternal and fetal complications. Prospective studies may help clarify the relationship between SCD and specific maternal complications.

The report describes successful management of 10 women in 2nd and 3rd pregnancy trimesters with EchiTab IgG antivenom after carpet viper (Echis ocellatus) envenoming. All women survived but foetal loss in a victim with delayed presentation and a case of mild hypersensitivity reaction were recorded. Excellent outcomes can be achieved in rural and semi-nomadic populations without specialized care and immediate access and provision of effective antivenoms is paramount in curtailing snakebite maternal morbidity, mortality and foetal loss.

The aim of this report was to establish the national maternal mortality rate in Bahrain over the period 1987-2004, to identify preventable factors in maternal deaths and to make recommendations for safe motherhood. There were 60 maternal deaths out of 243 232 deliveries giving an average maternal mortality rate of 24.7 per 100 000 total births. The main causes of death were sickle-cell disease (25.0%), hypertension (18.3%), embolism (13.3%), haemorrhage (13.3%), heart disease (11.7%), infection (8.3%) and other (10.0%). In an audit of care, 17 (28.3%) out of 60 deaths were judged to be avoidable, nearly half of which were due to a shortage of intensive care beds. We recommend that a confidential enquiry of maternal deaths be conducted at the national level every 3 to 5 years.

Background: In 1998 a national program for first-trimester screening for red cell (RBC) antibodies in all pregnant women was implemented. The aim of our study was to assess the impact on perinatal mortality caused by Kell alloimmunization, Study Design and Methods: Prospectively collected data on all pregnant women referred to our center from 1988 until 2005 for intrauterine transfusion (IUT) for fetal anemia due to Kell alloantibodies were analyzed. The cohort was divided into two groups, those treated before 1998 and those treated after 1998. The primary outcome was fetal and neonatal survival. Secondary outcome variables were gestational age, fetal hemoglobin (Hb) levels at first IUT, severity of hydrops, and total number of IUTs per pregnancy. Causes for mortality were analyzed in detail., Results: A total of 43 pregnancies were included, 18 before introduction of screening and 25 thereafter. Perinatal survival increased from 61 percent in the first period to 100 percent after introduction of screening. After 1998, fetal hydrops was generally less severe at first IUT, while gestational age and fetal Hb levels at first IUT were similar., Conclusion: Implementation of routine screening for Kell antibodies in pregnancy was associated with an increased referral rate for suspected fetal anemia, more timely referrals, and a higher perinatal survival rate after intrauterine treatment.

Objective: Recent studies have reported increased fetal loss and preeclampsia in women with sickle cell trait (hemoglobin [Hb] AS). There is a paucity of studies of outcomes in carriers of hemoglobin C. We examined the prevalence of hemoglobin C and S carrier status (Hb AC and Hb AS, respectively) and their effect on pregnancy outcomes., Methods: This was a retrospective cohort study using data prospectively collected from 1991 to 2006. Perinatal and maternal outcomes for African-American women with Hb AS and Hb AC were compared with those with normal hemoglobin (Hb AA). Multivariable regression was performed by applying generalized estimating equations to account for correlation between births from the same woman., Results: Among 22,096 eligible African-American women (36,897 pregnancies) with routine antenatal hemoglobin electrophoresis, 88.5% had a normal (Hb AA) pattern. Hemoglobin AS was identified in 8.2% and Hb AC in 2.4% of women. Hemoglobin SS and Hb SC each accounted for less than 0.2% and Hb CC for 0.01%. Prevalence and relative risks for adverse outcomes in 3,019 AS pregnancies (3,062 births) and 875 AC (886 births), compared with 32,724 AA pregnancies (33,213 births), were not increased. Adjusted relative risks (95% confidence intervals) for perinatal mortality and preeclampsia were 0.7 (0.5-1.0) and 1.0 (0.8-1.2), respectively, for AS and 0.7 (0.3-1.4) and 1.0 (0.6-1.3), respectively, for AC. Risks of stillbirths and pregnancy-associated hypertension were also not increased., Conclusion: Contrary to other recent reports, perinatal mortality and preeclampsia are not increased in carriers of sickle cell trait or hemoglobin C., Level of Evidence: II.

Unlabelled: Because women with paroxysmal nocturnal hemoglobinuria (PNH) are especially vulnerable to thromboembolic phenomena, pregnancy is a time of increased risk for both mother and fetus. However, pregnancies in affected women are rare; only case reports and small studies have been reported so far. We present the case of a 20-year-old woman with PNH who, while undergoing medical tests in preparation for a bone marrow transplant, was discovered to be pregnant. We also review the obstetric literature on pregnancy complicated by PNH, which indicates that both maternal and fetal mortality is exceptionally high (11.6% and 7.2%) with the major cause of maternal mortality being thromboembolism. Major maternal complications are more frequent postpartum (30.2%) than antepartum or intrapartum (16.3%)., Target Audience: Obstetricians & Gynecologists, Family Physicians., Learning Objectives: After completion of this article, the reader should be able to recall that paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy increases adverse events for both the mother and the fetus, state that maternal and fetal mortality are both high, and explain that the major complications occur in the postpartum period.

Malaria and anaemia contribute tremendously to maternal and prenatal morbidity and mortality. This study was carried out to document the magnitude of the problem in pregnancy with a view to identifying areas of intervention. The subjects were 108 consecutive pregnant women aged 18 to 44 years recruited from the antenatal clinics. 23 (21.3%) had malaria, 35 (32.4%) had anaemia while 20(18.5%) had both malaria and anaemia. The highest incidence of malaria occurred in the second trimester, while anaemia was most prevalent in the third trimester (62.86%) and among primigravidae (37.14%). All the cases of malaria were due to plasmodium falciparum. Six out of the 20 women with both anemia and malaria were admitted and treated. Two low birth weight babies were delivered among the malaria and anaemia group. The incidence rates of malaria and anaemia were 215 and 327 per 1000 pregnant women respectively while the incidence rate of anaemia due to malaria was 571 per 1000 infected pregnant women. There is a need for a more effective intervention to reduce the incidence of both malaria and anaemia in pregnancy.

Venous thromboembolism is the leading cause of pregnancy-associated morbidity and mortality. Women with thrombophilia have an increased risk of VTE in pregnancy and puerperium. In individuals with hereditary thrombosis risk factors a relative risk of pregnancy associated VTE ranging from 3.4 to 15.2 has been found. Women with previous VTE have an approximately 3.5-fold increased risk of recurrent VTE during pregnancy compared to non-pregnant periods. Data on the association of thrombophilia and pregnancy loss and pre-eclampsia are conflicting. Besides an established association with antiphospholipid antibodies, available data suggest associations for antithrombin deficiency, hyper-homo-cysteinemia, factor V Leiden, prothrombin G20210A variation and protein S-deficiency. A contribution of thrombophilia to the risk of pre-eclampsia is less well established. A limited number of prospective studies did not reveal an increased risk of pregnancy complications in unselected women with thrombosis risk factors. Data of only one controlled trial on the prevention of pregnancy loss with low molecular weight heparin (LMWH) are available, which revealed a strikingly positive effect. Thrombophilia screening might be justified in women with pregnancy loss and treatment with LMWH might be considered in those with pregnancy loss and thrombophilia. Further prospective studies and controlled interventional trials are urgently needed.