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pregnancy and sickle cell disease

Norita Hussein, Stephen F Weng, Joe Kai, Jos Kleijnen, Nadeem Qureshi
BACKGROUND: Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting...
March 14, 2018: Cochrane Database of Systematic Reviews
Javon Prophet, Kalifa Kelly, Julian Domingo, Helen Ayeni, Xaviera Pascale Djoko Mekouguem, Breana Dockery, Farida Allam, Manvir Kaur, Javon Artis, Kiara K Spooner, Jason L Salemi, Omonike A Olaleye, Hamisu M Salihu
OBJECTIVE: The relationship between sickle cell disease (SCD) and severe pre-eclampsia is poorly established. It is also unknown whether the occurrence of HIV infection among women with SCD modifies their risk level for severe pre-eclampsia. We hypothesized that pregnant women with SCD are at an elevated risk for severe pre-eclampsia as a result of heightened endothelial damage; and the combination of SCD-HIV augments the inflammatory processes of endothelial damage leading to amplified risk for severe pre-eclampsia...
January 2018: Pregnancy Hypertension
Affette M McCaw-Binns, Leroy V Campbell, Simone S Spence
OBJECTIVE: Describe trends in indirect cause-specific pregnancy-related mortality from 1998-2015. DESIGN: Secondary analysis of annual, national cross-sectional database of maternal and late maternal deaths, identified through active surveillance of deaths among women 10-50 years. SETTING: Jamaica, a middle-income Caribbean country. POPULATION: Maternal and late maternal deaths. METHODS: Descriptive trend analyses of demographic and cause-specific maternal and pregnancy-related mortality ratios undertaken comparing the periods 1998-2003, 2004-9 and 2010-15...
February 8, 2018: BJOG: An International Journal of Obstetrics and Gynaecology
Dongmei Sun, Anne McLeod, Shital Gandhi, Ann Kinga Malinowski, Nadine Shehata
Importance: Anemia is common in pregnancy, ranging from 5.4% in developed countries to more than 80% in developing countries. Anemia in pregnancy has been associated with prematurity, low birth weight, and adverse pregnancy outcomes. Objective: This review uses clinical vignettes to illustrate the clinical presentations, approach to diagnosis, maternal and fetal implications, and treatment for the common etiologies of anemia in pregnancy. Evidence Acquisition: Literature review...
December 2017: Obstetrical & Gynecological Survey
Mariachiara Lodi, Elena Bigi, Giovanni Palazzi, Lara Vecchi, Riccardo Morandi, Monica Setti, Silvana Borsari, Giuliano Bergonzini, Lorenzo Iughetti, Donatella Venturelli
The implementation of screening programs for early detection of patients with sickle cell disease has become necessary in Italy as a result of the high rate of migration from areas with a high prevalence of the disease (Sub-Saharan Africa, Middle East and the Balkans). Following a pilot study performed in the province of Modena, Italy in 2011-2013, an official screening program was established on May 31 2014 for all pregnant women, free-of-charge for the family according to the National Guidelines for Physiological Pregnancy...
July 2017: Hemoglobin
Cathi Phillips, Margaret Peggy Boyd
Sickle cell disease is the genetic disorder most commonly detected with state-mandated newborn screening. Women with sickle cell disease struggle with psychosocial, emotional, and physical challenges throughout their lives. Pregnancy for women with sickle cell disease brings greater risk for maternal and fetal morbidity and mortality and increased likelihood of hospitalization for complications, including sickle cell pain crisis. Chronic maternal opioid use for pain can place newborns at risk for neonatal abstinence syndrome...
December 2017: Nursing for Women's Health
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
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...
July 2017: Hemoglobin
Evgeni Chubar, Naiel Bisharat
Delayed haemolytic transfusion reaction is a rare, life-threatening complication of blood transfusion that has been typically described among patients with sickle cell disease (SCD) due to alloimmunisation induced by their exposure to red blood cell antigens through recurrent transfusions. We report the case of a patient who suffered from fatal delayed haemolytic transfusion reaction (DHTR) occurring 1 week after blood transfusion. Indirect antiglobulin testing confirmed the presence of anti-Kell antibodies that were absent in the pretransfusion sample...
November 3, 2017: BMJ Case Reports
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar, Vladimir Gotlieb
Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia...
October 16, 2017: Hematology/oncology and Stem Cell Therapy
Eric S Donkor, Jonathan A Osei, Isaac Anim-Baidoo, Samuel Darkwah
Asymptomatic bacteriuria (ASB) is benign except in certain medical conditions such as pregnancy and immunosuppression. In Ghana, there are hardly any studies on urinary infections among sickle cell disease (SCD) patients, and the few studies carried out in Africa focused on pediatric SCD populations. The current study aimed to investigate the risk of ASB among SCD patients at a tertiary hospital in Ghana. This was a cross-sectional study involving 110 SCD patients and 110 age and sex matched healthy controls...
February 15, 2017: Diseases (Basel)
Nabilah F Lari, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is one of the most common hemoglobinopathy, affecting a considerable proportion of black populations of African origin, Middle East and in the Indian sub-continent. Women with SCD are more likely to experience adverse pregnancy and delivery outcomes. Hypertensive diseases in pregnancy such as preeclampsia and eclampsia are more common in women with sickle cell disease. Areas covered: This review examined the influence of hypertension and SCD in pregnancy, and provides the preliminary evidence that the traditional systolic and diastolic blood pressure thresholds for hypertensive disorders such as pre-eclampsia and eclampsia may require reassessment in pregnant women with SCD...
November 2017: Expert Review of Hematology
Djamila L Ghafuri, Sarah-Jo Stimpson, Melissa E Day, Andra James, Michael R DeBaun, Deva Sharma
Sickle cell disease (SCD) represents one of the most common monogenic blood disorders worldwide, with an incidence of over 300,000 newborns affected per year. Reproductive challenges for men and women with SCD have been previously reviewed; however, evidence-based strategies to prevent and manage infertility and increase fecundity are lacking in women with SCD, which is one of the most important factors for quality of life. Areas covered: This review article summarizes the known risk factors for infertility, low fecundity, and premature menopause related to SCD...
October 2017: Expert Review of Hematology
Opeyemi Abayomi Obilade, Alani Suleimon Akanmu, Fiona Broughton Pipkin, Bosede Bukola Afolabi
BACKGROUND: Pregnancy increases the risk of morbidity and mortality in sickle cell disease. We previously showed pregnant women with sickle cell disease to have a relatively low plasma renin concentration in late pregnancy, associated with a lack of the expected plasma volume expansion. We hypothesized this to be due to increased systemic vascular resistance through an imbalance between the vasodilator prostacyclin and vasoconstrictor thromboxane, associated with decreased glomerular filtration rate (GFR)...
2017: PloS One
Blaise Felix Faye, Kouassi Berenger Kouame, Moussa Seck, Abdou Aziz Diouf, Macoura Gadji, Nata Dieng, Sokhna Aissatou Touré, Abibatou Sall, Awa Oumar Toure, Saliou Diop
OBJECTIVES: The aim of this study was to evaluate the maternal and fetal complications in pregnant patients with sickle cell disease (SCD) and find risk factors of stillbirth. METHOD: We conducted a prospective study in pregnant women with SCD. Demographic characteristics, maternal and fetal morbi-mortality, and outcome of pregnancies were described. Risk factors of fetal loss were evaluated by comparing the parameters of the pregnancies that led to a live birth with those interrupted...
January 2018: Hematology (Amsterdam, Netherlands)
Michelle Matthews, Rebecca Pollack
PURPOSE: Hematopoietic stem cell transplantation (HSCT) is a cure for sickle cell disease (SCD) but frequently results in permanent sterility. The complications associated with oocyte cryopreservation and risks of future pregnancy are increased in SCD patients. This case report discusses risk reduction strategies and includes a literature review of pregnancy after HSCT. CASE: A 23-year-old woman underwent ovarian stimulation for fertility preservation resulting in cancelation due to acute pain crisis...
November 2017: Journal of Assisted Reproduction and Genetics
J Sharif, L Byrd, K Stevenson, J Raddats, E Morsman, K Ryan
INTRODUCTION: Sickle cell disease in pregnancy carries a high risk of maternal and fetal adverse outcomes. The use of prophylactic transfusions to reduce the risk of sickle complications is controversial. Current UK standards do not recommend the routine use of transfusion for sickle pregnancy. We examined transfusion episodes during sickle pregnancies in a single centre over an 11-year period. METHODS: We conducted a retrospective observational study of all pregnancies in patients with sickle cell disease who attended the joint obstetric/haematology clinic over an 11-year period...
July 27, 2017: Transfusion Medicine
Soyoung Choi, Adam M Bush, Matthew T Borzage, Anand A Joshi, William J Mack, Thomas D Coates, Richard M Leahy, John C Wood
Sickle cell disease (SCD) is a life-threatening genetic condition. Patients suffer from chronic systemic and cerebral vascular disease that leads to early and cumulative neurological damage. Few studies have quantified the effects of this disease on brain morphometry and even fewer efforts have been devoted to older patients despite the progressive nature of the disease. This study quantifies global and regional brain volumes in adolescent and young adult patients with SCD and racially matched controls with the aim of distinguishing between age related changes associated with normal brain maturation and damage from sickle cell disease...
2017: NeuroImage: Clinical
Eugenia Vicky Asare, Edeghonghon Olayemi, Theodore Boafor, Yvonne Dei-Adomakoh, Enoch Mensah, Harriet Ghansah, Yvonne Osei-Bonsu, Selina Crabbe, Latif Musah, Charles Hayfron-Benjamin, Brittany Covert, Adetola A Kassim, Andra James, Mark Rodeghier, Michael R DeBaun, Samuel A Oppong
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...
September 2017: American Journal of Hematology
Ryan M Antiel, Scott D Halpern, Evelyn M Stevens, Jesse D Vrecenak, Chavis A Patterson, Trudy Tchume-Johnson, Kim Smith-Whitley, William H Peranteau, Alan W Flake, Lamia P Barakat
BACKGROUND: In utero hematopoietic cell transplantation (IUHCT) has curative potential for sickle cell disease (SCD) but carries a risk of fetal demise. METHODS: We assessed the conditions under which parents of children with SCD and young adults with SCD would consider IUHCT in a future pregnancy, given a 5% fixed risk of fetal demise. Participants were randomized to consider a hypothetical cure rate (20%, 40%, or 70%). Subsequently, cure rate was either increased or decreased depending on the previous answer to reveal the lowest acceptable rate...
May 1, 2017: Medical Decision Making: An International Journal of the Society for Medical Decision Making
Patricia A R Brunker, Keerthana Ravindran, R S Shirey
Alloimmunization to red blood cell antigens is unpredictable and poorly understood. Patients who are negative for high-incidence antigens (HIAs) are at risk for developing the corresponding antibodies. Molecular methods can easily predict the lack of an antigen and thus, the risk of an individual to become immunized. We examined the prevalence and risk factors for HIA alloimmunization in patients at risk based on genotyping results. Genotyping using a molecular method (HEA BeadChip™, Immucor, Warren, NJ) was performed on all patient specimens referred for molecular testing over 45 months; serologic and clinical data were analyzed...
January 2017: Immunohematology
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