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Sickle cell crisis pregnancy

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...
March 18, 2017: Current Opinion in Anaesthesiology
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
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
Manickam Niraimathi, Rakhee Kar, Sajini Elizabeth Jacob, Debdatta Basu
Vaso-occlusive crisis in sickle cell anaemia is one of the commonest presentations and a leading cause of death. Death can be sudden and unexpected. Herein we present three cases of sickle cell anaemia with sudden death within 3 days of hospitalisation. All the three cases presented with fever and jaundice. Two cases presented consecutively in the same year within a span of 5 months while the other case had presented 2 years prior to these two cases. Infection was the precipitating event in two cases and pregnancy with infection in one...
June 2016: Indian Journal of Hematology & Blood Transfusion
Narcisse Elenga, Aurélie Adeline, John Balcaen, Tania Vaz, Mélanie Calvez, Anne Terraz, Laetitia Accrombessi, Gabriel Carles
Sickle cell disease is a serious genetic disorder affecting 1/235 births in French Guiana. This study aimed to describe the follow-up of pregnancies among sickle cell disease patients in Cayenne Hospital, in order to highlight the most reported complications. 62 records of pregnancies were analyzed among 44 females with sickle cell disease, between 2007 and 2013. Our results were compared to those of studies conducted in Brazil and Guadeloupe. There were 61 monofetal pregnancies and 2 twin pregnancies, 27 pregnancies among women with SS phenotype, 30 SC pregnancies, and five S-beta pregnancies...
2016: Obstetrics and Gynecology International
Paolo Perseghin
Hematological diseases in pregnancy should be carefully managed with a multidisciplinary approach, which should include obstetrics, hematology and, in selected patients, apheresis professionals. Hematological malignancies in pregnant women are rare, but the attending physicians should be aware that the use of cytotoxic drugs, tyrosine-kinase inhibitors or differentiating agents such as all-trans retinoic acid (ATRA) during the first trimester of pregnancy might be teratogenic and, in turn, induce fetal abnormalities or abortion...
December 2015: Transfusion and Apheresis Science
Nicholas J Schott, Mark H Yazer, Robert Krohner, Jonathan H Waters
Cell salvage is a process whereby the bloodshed from the operative field is collected and returned to the patient. It can be especially useful when allogeneic red blood cell (RBC) units are not readily available such as when the recipient has multiple alloantibodies. We report on the anesthesia and transfusion strategies for managing a pregnant patient with sickle cell disease (SCD) with HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome. A pregnant patient with twins at 30 weeks of gestation was admitted in an SCD crisis...
December 2014: Journal of Extra-corporeal Technology
Craig D Seaman, Jonathan Yabes, Jie Li, Charity G Moore, Margaret V Ragni
INTRODUCTION: The risk of venous thromboembolism (VTE) is higher during pregnancy, with an incidence between 0.05 and 0.2%, and among persons with sickle cell disease (SCD), yet the rates and risk factors, such as pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS), associated with pregnancy-related VTE are not firmly established in SCD. METHODS: Inpatient hospital discharge data from 2007-2011 were obtained from the Pennsylvania Health Care Cost Containment Council to estimate the rate of VTE among African American delivery hospitalizations with SCD and to compare pregnancy complications and medical comorbidities among pregnant women with SCD...
December 2014: Thrombosis Research
Suheyl Asma, Ilknur Kozanoglu, Ebru Tarım, Cagla Sarıturk, Cigdem Gereklioglu, Aydan Akdeniz, Mutlu Kasar, Nurhilal H Turgut, Mahmut Yeral, Fatih Kandemir, Can Boga, Hakan Ozdogu
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)...
January 2015: Transfusion
Khushnooma Italia, Dipti Upadhye, Pooja Dabke, Harshada Kangane, Stacy Colaco, Pratibha Sawant, Anita Nadkarni, Ajit Gorakshakar, Dipty Jain, Yazdi Italia, Kanjaksha Ghosh, Roshan Colah
BACKGROUND: Co-inheritance of structural hemoglobin variants like HbS, HbD(Punjab) and HbE can lead to a variable clinical presentation and only few cases have been described so far in the Indian population. METHODS: We present the varied clinical and hematological presentation of 22 cases (HbSD(Punjab) disease-15, HbSE disease-4, HbD(Punjab)E disease-3) referred to us for diagnosis. RESULTS: Two of the 15 HbSD(Punjab) disease patients had moderate crisis, one presented with mild hemolytic anemia; however, the other 12 patients had a severe clinical presentation with frequent blood transfusion requirements, vaso occlusive crisis, avascular necrosis of the femur and febrile illness...
April 20, 2014: Clinica Chimica Acta; International Journal of Clinical Chemistry
Nada Alayed, Abbas Kezouh, Lisa Oddy, Haim A Abenhaim
OBJECTIVE: To estimate the prevalence of sickle cell disease (SCD) in pregnancy, and to measure risk factors, morbidity, and mortality among women with SCD with and without crisis at the time of birth. METHODS: We conducted a population-based, retrospective cohort study on all births in the Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) from 1999 to 2008. Births to SCD with and without crisis were identified using ICD-9 codes. Adjusted effects of risk factors and outcomes were estimated using logistic regression analyses...
July 2014: Journal of Perinatal Medicine
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 3, 2013: Cochrane Database of Systematic Reviews
Mayoor M Daigavane, Rabindra K Jena, Tushar J Kar
Sickle cell anemia, the homozygous genotype of sickle cell disease is one of the most common heritable diseases in the world. The Arab-Asian haplotype present in India is one of the least severe of all haplotypes. Many sickle cell anemia patients are now leading a symptom-free productive life due to hydroxyurea (HU) and better supportive care. Although pregnancy in sickle cell anemia patients is considered a high-risk category, it perinatal outcome is least studied, particularly among carriers of the Arab-Asian haplotype...
2013: Hemoglobin
Marc R Parrish, John C Morrison
Pregnant women with sickle cell disease appear to be more likely to experience antepartum, intrapartum, and postpartum complications when compared with unaffected women. Access to high-risk obstetric care, patient education, and close follow-up is important to minimize maternal morbidity and mortality. A high index of suspicion and good diagnostic acumen is necessary to obtain optimal results in the pregnant patient affected by sickle cell crisis.
August 2013: Seminars in Perinatology
Shumaila Zia, Muhammad Rafique
OBJECTIVE: To compare the pregnancy and neonatal outcome in sickle cell disease and trait women. METHOD: The retrospective comparative study was conducted at Abha General Hospital, Abha, Saudi Arabia, from January 2009 to December 2011. The records of women having sickle cell disease and trait were reviewed for antenatal and postnatal complications. Pregnancy and foetal outcome was also compared in both the groups. Chi-square, Fisher's Exact and Student's t tests were used for statistical analysis...
June 2013: JPMA. the Journal of the Pakistan Medical Association
K Khozaim, M Schellinger, O Cummings, B Robinson
Acute liver failure during pregnancy is a rare and potentially fatal occurrence. Sickle cell (SC) crises, when precipitated by pregnancy, usually affect patients with SC disease. We report a case of peripartum acute liver failure due to SC vaso-occlusive crisis of the liver in a patient with SC trait.
August 2013: Journal of Perinatology: Official Journal of the California Perinatal Association
Minerva M Thame, Clive Osmond, Graham R Serjeant
OBJECTIVES: To assess fetal growth and whether lower birthweight to mothers with homozygous sickle cell (SS) disease is related to maternal body composition or to clinical events in pregnancy. STUDY DESIGN: A prospective study of 41 pregnant women with SS disease and 41 women with a normal (AA) phenotype attending the antenatal clinic, University Hospital of the West Indies, Kingston, Jamaica. Maternal anthropometry, body composition and fetal sonographic measurements were assessed at 15, 25, and 35 weeks' gestation from December 2005 to April 2008...
September 2013: European Journal of Obstetrics, Gynecology, and Reproductive Biology
Maíra da Silva Caracas, Sumatra P Jales, Levi H Jales Neto, Joice Carla da Silva Castro, Liliana Mitie Suganuma, Guilherme Henrique Hencklain Fonseca, Sandra Fatima Menosi Gualandro, José Tadeu Tesseroli de Siqueira
We report a rare case of aseptic arthritis in the temporomandibular joint of a patient with sickle cell anemia. A 22-year-old woman with sickle cell disease, in the 18th week of gestation, was referred by her hematologist to investigate a sudden mouth opening limitation and severe pain on her left cheek. The patient received a standard pain assessment protocol, clinical examination, and complementary exams (complete blood count, hemoglobin electrophoresis, blood solubility test, panoramic radiograph, and magnetic resonance imaging [MRI])...
February 2013: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Maryam A Al Kahtani, Mohammad AlQahtani, Mashael M Alshebaily, Mohamed Abd Elzaher, Ashraf Moawad, Naji Aljohani
OBJECTIVE: To identify morbidity and pregnancy outcomes associated with sickle cell disease (SCD) among pregnant Saudi women. METHODS: A 10-year retrospective study was conducted at King Khalid University Hospital in Riyadh, Saudi Arabia, that included 392 cases of SCD in pregnancy and 784 controls with normal hemoglobin phenotype, who were selected and matched for age, parity, and delivery outcome. The main outcome measures were morbidity, maternal outcomes, and fetal outcomes...
December 2012: International Journal of Gynaecology and Obstetrics
Sören Verstraete, Rik Verstraete
We describe a 19-year-old Cameroonian primigravid young woman with sickle cell disease who was admitted to a local hospital in Cameroon where the first author performed his internship gynecology and obstetrics. She presented at 28 weeks of gestation with severe pain in her left leg caused by a vaso-occlusive crisis. As recommended, high doses of intravenous morphine were administered, but without significant pain relief. She received a single bolus injection of 5 mg morphine, followed by a continuous infusion of 0...
October 2012: Journal of Anesthesia
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