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pregnancy and sickle cell disease

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https://www.readbyqxmd.com/read/29102975/fatal-delayed-haemolytic-transfusion-reaction-in-a-patient-without-previous-transfusions-but-with-an-obstetric-history-of-13-pregnancies
#1
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
https://www.readbyqxmd.com/read/29079125/hereditary-persistence-of-hemoglobin-f-is-protective-against-red-cell-sickling-a-case-report-and-brief-review
#2
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
https://www.readbyqxmd.com/read/28933357/risk-of-asymptomatic-bacteriuria-among-people-with-sickle-cell-disease-in-accra-ghana
#3
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)
https://www.readbyqxmd.com/read/28905692/the-emerging-challenge-of-optimal-blood-pressure-management-and-hypertensive-syndromes-in-pregnant-women-with-sickle-cell-disease-a-review
#4
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
https://www.readbyqxmd.com/read/28891355/fertility-challenges-for-women-with-sickle-cell-disease
#5
REVIEW
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
https://www.readbyqxmd.com/read/28880908/prostacyclin-thromboxane-and-glomerular-filtration-rate-are-abnormal-in-sickle-cell-pregnancy
#6
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
https://www.readbyqxmd.com/read/28846064/challenges-in-the-management-of-sickle-cell-disease-during-pregnancy-in-senegal-west-africa
#7
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...
August 28, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28801781/acute-pain-crisis-in-a-patient-with-sickle-cell-disease-undergoing-ovarian-simulation-for-fertility-preservation-prior-to-curative-stem-cell-transplantation-case-report-and-literature-review
#8
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...
August 11, 2017: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/28749037/transfusion-for-sickle-cell-disease-in-pregnancy-a-single-centre-survey
#9
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
https://www.readbyqxmd.com/read/28540180/hemoglobin-and-mean-platelet-volume-predicts-diffuse-t1-mri-white-matter-volume-decrease-in-sickle-cell-disease-patients
#10
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
https://www.readbyqxmd.com/read/28512745/implementation-of-multidisciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#11
MULTICENTER STUDY
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
https://www.readbyqxmd.com/read/28490230/acceptability-of-in-utero-hematopoietic-cell-transplantation-for-sickle-cell-disease
#12
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
https://www.readbyqxmd.com/read/28425750/modeling-alloantibody-formation-to-high-incidence-red-blood-cell-antigens-in-immune-responders-using-genotypic-data
#13
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
https://www.readbyqxmd.com/read/28422403/blood-chimerism-in-dizygotic-monochorionic-twins-during-5-years-observation
#14
M H Dziegiel, M H Hansen, S Haedersdal, A N Barrett, K Rieneck, K M Main, A T Hansen, F B Clausen
Dizygotic monochorionic twin pregnancies can result in blood chimerism due to in utero twin-to-twin exchange of stem cells. In this case, we examined the proportion of allogeneic red blood cells by flow cytometry and the proportion of allogeneic nucleated cells by digital polymerase chain reaction at 7 months and again at 5 years. We found an increase in the proportion of allogeneic cells from 63% to 89% in one twin, and a similar increase in autologous cells in the other twin from 57% to 84%. A paradigm for stem cell therapy could be modeled on this case: induction of tolerance and chimerism by antenatal transfusion of donor stem cells...
April 19, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28323673/clinical-pearls-of-maternal-critical-care-part-2-sickle-cell-disease-in-pregnancy
#15
Vinod Patil, Gamunu Ratnayake, Galina Fastovets
PURPOSE OF REVIEW: The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy. RECENT FINDINGS: Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises and chest crisis are also outlined...
June 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28258065/pregnancy-in-women-with-sickle-cell-disease-is-associated-with-risk-of-maternal-and-perinatal-mortality-and-severe-morbidity
#16
Eugene Oteng-Ntim
No abstract text is available yet for this article.
March 3, 2017: Evidence-based Nursing
https://www.readbyqxmd.com/read/28251585/voluntary-premarital-screening-to-prevent-sickle-cell-disease-in-jamaica-does-it-work
#17
G R Serjeant, B E Serjeant, K P Mason, F Gibson, R Gardner, L Warren, M Jonker
To determine whether identifying haemoglobin genotype, and providing education and counselling to senior school students will influence their choice of partner and reduce the frequency of births with sickle cell disease. The Manchester Project provided free voluntary blood tests to determine haemoglobin genotype to the fifth and sixth forms (grades 11-13), median age of 16.7 years, of all 15 secondary schools in the parish of Manchester in south central Jamaica. A total of 16,636 students complied, and counselling was offered to carriers of abnormal genes over 6 years (2008-2013)...
April 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28159390/sickle-cell-disease
#18
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28109314/sickle-cell-disease-and-pregnancy-outcomes-a-study-of-the-community-based-hospital-in-a-tribal-block-of-gujarat-india
#19
Gayatri Desai, Ankit Anand, Pankaj Shah, Shobha Shah, Kapilkumar Dave, Hardik Bhatt, Shrey Desai, Dhiren Modi
BACKGROUND: Sickle cell disease (SCD) is a hereditary blood disorder prevalent in tribal regions of India. SCD can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. This study reports the analysis of tribal maternal admissions in the community-based hospital of SEWA Rural (Kasturba Maternity Hospital) in Jhagadia block, Gujarat. The objective of the study is to compare the pregnancy outcomes among SCD, sickle cell trait and non-SCD admissions...
January 21, 2017: Journal of Health, Population, and Nutrition
https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#20
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
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...
December 22, 2016: Cochrane Database of Systematic Reviews
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