Complications in pregnant women with sickle cell disease.

Complications in pregnant women with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 06;2019(1):359-366 Authors: Smith-Whitley K Abstract Pregnancy in women with sickle cell disease (SCD) is associated with increased maternal and fetal morbidity and mortality. Outcomes vary widely owing to methodological limitations of clinical studies, but overall, hypertensive disorders of pregnancy, venothromboembolism, poor fetal growth, and maternal and perinatal mortality are increased globally. Few therapeutic interventions have been explored other than prophylactic and selective transfusion therapy. Unfortunately, existing data are limited, and it remains unclear whether prophylactic use of chronic transfusions will improve pregnancy outcomes. Management of pregnant women with SCD is best accomplished with a multidisciplinary team that includes a sickle cell expert and an obstetrician familiar with high-risk pregnancies. Women with SCD should have individualized care plans that outline management of acute pain and guidelines for transfusion therapy. Neonates require close monitoring for neonatal abstinence syndrome and hemolytic disease of the newborn. Ideally all young women with SCD will have a "reproductive life plan" developed as a component of preconception counseling and health promotion. Research leading to improved pregnancy management focused on diminishing adverse maternal and neonatal outcomes is overdue. International co...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

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In this study, we used HUT as the means to provide an all-encompassing assessment of cardiac and/or peripheral autonomic function in normal controls, SCD subjects and non-SCD subjects with chronic anemia. We hypothesized that by identifying different categories of HUT response among these subjects, we would be able to isolate the autonomic phenotypes that might place SCD subjects at increased risk for microvascular occlusion and VOC. We then employed the causal modeling approach, which utilizes signal analysis and system identification techniques, to probe and disentangle the functional mechanisms involved in the cardiovas...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Sickle cell disease (SCD) is characterized by chronic hemolytic anemia and vaso-occlusion. Hypocholesterolemia has been long recognized in SCD patients, and while it has been postulated that it is secondary to increased erythropoiesis, these mechanisms are not fully understood. Despite their name, angiopoietin-like proteins (ANGPTLs) are family of regulators of angiogenesis unable to bind to receptors classically targeted by angiopoetins. ANGPTL3 and ANGPTL4 are also inhibitors of lipoprotein lipase (LPL), and have become pharmacological targets in the treatment of dyslipidemia. We hypothesized that ANGPTL levels would be ...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster I Source Type: research
Introduction. Despite the increasing of number of patients with Sickle Cell Disease (SCD) in Italy, due to multi-ethnic migratory phenomena, a large percentage of Caucasian sickle population is already present in Italy mainly with b-thal/HbS genotype. Red cell transfusion is one effective treatment for both acute and chronic complications of SCD, while hydroxycarbamide (HC) is used to reduce the frequency of painful vaso-occlusive crises (VOCs) and decrease the need for blood transfusion.Through the National Comprehensive Reference Centers for SCD, the Italian Society of Thalassemia and Hemoglobinopathies (SITE), in collab...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research
Publication date: November 2018Source: The Lancet Haematology, Volume 5, Issue 11Author(s): Barnabas Gellen, Laurent A Messonnier, Frédéric Galactéros, Etienne Audureau, Angèle N Merlet, Thomas Rupp, Sandrine Peyrot, Cyril Martin, Léonard Féasson, Pablo Bartolucci, Anoosha Habibi, Emma Guillet, Justine Gellen-Dautremer, Jean-Antoine Ribeil, Jean-Benoit Arlet, Sarah Mattioni, Jugurtha Berkenou, Noemie Delrieux, François Lionnet, Jean-François GrenotSummaryBackgroundExercise could be a triggering factor for vaso-occlusive crises in patients with sickle-cell disease. We ...
Source: The Lancet Haematology - Category: Hematology Source Type: research
This report would examine developments at the state and federal-level, court cases, and current views from stakeholders. Policy Questions Which states have PAS laws and what do those laws provide? What protections against abuse of PAS?What have the Supreme Court and lower courts held regarding individuals’ rights under PAS laws? The laws themselves?Is there evidence that persons with disabilities are being denied treatment by insurance companies but offered PAS instead, as NCD predicted?How is PAS viewed by disability organizations? Has this evolved in the past 13 years? If so why? If not, why?Are persons with disabi...
Source: blog.bioethics.net - Category: Medical Ethics Authors: Tags: Health Care syndicated Source Type: blogs
Discussion In the US during 2011, ~60,000 youth were incarcerated at some time in a correctional facility. Boys are detained more than girls (86% vs 14% respectively in the US, 95% male in the United Kingdom in 2014) but girls have more health issues. There are racial and ethnic differences with 38-40% of detainees being black, 23% being Hispanic/Latino and 32% being white, and 5% other. For detained youth, 5% are for violent crimes, 22% for non-violent property crimes and the majority of the rest are for non-violent offices such as substance use. The average length of detainment is 3-4 months and unfortunately the rates o...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
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. T1 weighted images were acquired on 33 clinically asymptomatic SCD patients (age=21.3±7.8; F=18, M=15) and 32 racially matched control subjects (age=24.4±7.5; F=22, M=10). Exclusion criteria included pregnancy, previous overt stroke, acute chest, or pain crisis hospitalization within one month. All brain volume comparisons were corrected for ag...
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
Authors: Howard J Abstract Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome,...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
By JESS JACOBS This has been a very sad weekend for me personally, the wider health care community and for anyone who knew Jess Jacobs, who died on Saturday. She was only 29 years old, and was smart, funny, enthusiastic, and brave well beyond her years. She suffered from two very rare diseases, but was also working to push health policy forward at ONC, FDA and Aetna, and she really knew her stuff. Jess was a marvel and a rarity in more ways than one. She was #UnicornJess. (That link will take you to the twitter memorial on Sunday night, but also check out remembrances from Ted Eytan &Carly...
Source: The Health Care Blog - Category: Consumer Health News Authors: Tags: THCB #UnicornJess Hospital Care Jess Jacobs Source Type: blogs
Discussion Appendicitis results from a closed loop obstruction of a blind-ending tubular structure arising from the cecum. It is a common cause of abdominal pain. It is the most frequent condition leading to emergent abdominal surgery in pediatrics. The combination of obstruction, edema, bacterial overgrowth, increased inflammatory process and increased intraluminal pressure leads to abdominal pain and possibly perforation. Appendicitis occurs in all age groups but is rare in neonates. The peak age is 6-10 years old. Pelvic inflammatory disease (PID) is an inflammatory disease of the uterus, fallopian tubes and adjacent p...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
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