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fetal anemia

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https://www.readbyqxmd.com/read/28801981/pregnancy-outcomes-in-mothers-of-offspring-with-inherited-bone-marrow-failure-syndromes
#1
Neelam Giri, Helen D Reed, Pamela Stratton, Sharon A Savage, Blanche P Alter
BACKGROUND: Children with inherited bone marrow failure syndromes (IBMFSs) may be symptomatic in utero, resulting in maternal and fetal problems during the pregnancy. Subsequent pregnancies by their mothers should be considered "high risk". METHODS: We retrospectively analyzed outcomes of 575 pregnancies in 165 unaffected mothers of offspring with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS) for events noted during pregnancy, labor, and delivery...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28800727/a-phased-snp-based-classification-of-sickle-cell-anemia-hbb-haplotypes
#2
Elmutaz M Shaikho, John J Farrell, Abdulrahman Alsultan, Hatem Qutub, Amein K Al-Ali, Maria Stella Figueiredo, David H K Chui, Lindsay A Farrer, George J Murphy, Gustavo Mostoslavsky, Paola Sebastiani, Martin H Steinberg
BACKGROUND: Sickle cell anemia causes severe complications and premature death. Five common β-globin gene cluster haplotypes are each associated with characteristic fetal hemoglobin (HbF) levels. As HbF is the major modulator of disease severity, classifying patients according to haplotype is useful. The first method of haplotype classification used restriction fragment length polymorphisms (RFLPs) to detect single nucleotide polymorphisms (SNPs) in the β-globin gene cluster. This is labor intensive, and error prone...
August 11, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28796996/call-for-caution-neonatal-portal-vein-thrombosis-following-enbucrilate-embolization-of-placental-chorioangioma
#3
Hian Yan Voon, Rafaie Amin, Juan Loong Kok, Kia Sing Tan
We illustrate a case of giant placental chorioangioma presenting at 20 weeks of gestation. Subsequent monitoring revealed enlargement of the lesion, associated with fetal anemia and cardiac failure, prompting in utero intervention. Amnioreduction followed by percutaneous embolization of the tumour with enbucrilate:Lipiodol Ultra-Fluid™ at a dilution of 1:5 was successfully performed. No repeat intervention or additional supportive measures were required throughout pregnancy and the baby was delivered at 36 weeks of gestation, following spontaneous labour...
August 11, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28783617/an-age-dependent-response-to-hydroxyurea-in-pediatric-sickle-cell-anemia-patients-with-alpha-thalassemia-trait
#4
Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W Cohen, Catherine Driscoll, Deepa Manwani
Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait...
July 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28782230/fetal-anemia
#5
EDITORIAL
N Abbasi, J-A Johnson, G Ryan
No abstract text is available yet for this article.
August 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28759962/effects-of-hydroxyurea-on-blood-rheology-in-sickle-cell-anemia-a-two-years-follow-up-study
#6
Nathalie Lemonne, Berenike Möckesch, Keyne Charlot, Yohann Garnier, Xavier Waltz, Yann Lamarre, Sophie Antoine-Jonville, Maryse Etienne-Julan, Marie-Dominique Hardy-Dessources, Marc Romana, Philippe Connes
The aim of the present study was to test the effects of hydroxyurea (HU) therapy on clinical, hematological and hemorheological parameters in adult patients with sickle cell anemia (SCA). Hematological and hemorheological parameters were measured in 28 SCA patients before HU therapy (i.e., baseline) and at 6, 12 and 24 months of treatment. RBC deformability was determined by ektacytometry at 30 Pa. RBC aggregation properties were investigated by light-backscatter method. Blood viscosity was measured at 225 s-1 by a cone-plate viscometer...
July 25, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28747161/factors-associated-with-perinatal-mortality-among-public-health-deliveries-in-addis-ababa-ethiopia-an-unmatched-case-control-study
#7
Yemisrach Getiye, Mesganaw Fantahun
BACKGROUND: perinatal mortality is the sum of still birth (fetal death) and early neonatal death (ENND) i.e. death of live newborn before the age of 7 completed days. Perinatal mortality accounts three fourth of the deaths of the neonatal period and is one of the major challenges for under-five mortality. Therefore this study was conducted to better understand the common and avoidable factors that affect perinatal mortality in Addis Ababa, Ethiopia. METHODS: An unmatched case control study design using secondary data as a source of information was conducted...
July 26, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/28743859/let-7-microrna-dependent-control-of-leukotriene-signaling-regulates-the-transition-of-hematopoietic-niche-in-mice
#8
Xuan Jiang, John S Hawkins, Jerry Lee, Carlos O Lizama, Frank L Bos, Joan P Zape, Prajakta Ghatpande, Yongbo Peng, Justin Louie, Giorgio Lagna, Ann C Zovein, Akiko Hata
Hematopoietic stem and progenitor cells arise from the vascular endothelium of the dorsal aorta and subsequently switch niche to the fetal liver through unknown mechanisms. Here we report that vascular endothelium-specific deletion of mouse Drosha (Drosha (cKO)), an enzyme essential for microRNA biogenesis, leads to anemia and death. A similar number of hematopoietic stem and progenitor cells emerge from Drosha-deficient and control vascular endothelium, but Drosha (cKO)-derived hematopoietic stem and progenitor cells accumulate in the dorsal aorta and fail to colonize the fetal liver...
July 25, 2017: Nature Communications
https://www.readbyqxmd.com/read/28739199/results-of-the-first-american-prospective-study-of-intravenous-iron-in-oral-iron-intolerant-iron-deficient-gravidas
#9
Michael Auerbach, Stephanie E James, Melissa Nicoletti, Steven Lenowitz, Nicola London, Huzefa F Bahrain, Richard Derman, Samuel Smith
BACKGROUND: Anemia affects up to 42% of gravidas. Neonatal iron deficiency is associated with low birth weight, delayed growth and development, and increased cognitive and behavioral abnormalities. While oral iron is convenient, up to 70% report significant gastrointestinal toxicity. Intravenous iron formulations allowing replacement in one visit with favorable side-effect profiles decrease rates of anemia with improved hemoglobin responses and maternal fetal outcomes. METHODS: 74 oral iron intolerant, 2(nd) and 3(rd) trimester iron deficient gravidas were questioned for oral iron intolerance and treated with intravenous iron...
July 21, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28736939/fetal-hemoglobin-in-sickle-cell-anemia-the-arab-indian-haplotype-and-new-therapeutic-agents
#10
REVIEW
Alawi H Habara, Elmutaz M Shaikho, Martin H Steinberg
Fetal hemoglobin (HbF) has well-known tempering effects on the symptoms of sickle cell disease and its levels vary among patients with different haplotypes of the sickle hemoglobin gene. Compared with sickle cell anemia haplotypes found in patients of African descent, HbF levels in Saudi and Indian patients with the Arab-Indian (AI) haplotype exceed that in any other haplotype by nearly twofold. Genetic association studies have identified some loci associated with high HbF in the AI haplotype but these observations require functional confirmation...
July 24, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28728448/pregnancy-and-child-health-outcomes-in-pediatric-and-young-adult-leukemia-and-lymphoma-survivors-a-systematic-review
#11
Ksenya Shliakhtsitsava, Sally A D Romero, Samantha Rose Dewald, H Irene Su
As long-term survival is high for children and young adults diagnosed with leukemia and lymphoma, delineating maternal, fetal and offspring health risks is important to their family planning. This systematic review examined data comparing these health risks between leukemia and lymphoma survivors and women without a history of cancer. Following a search of Embase, PubMed, CINAHL, Cochrane, and Web of Science, 142 articles were screened and 18 were included in this review. No higher risks of spontaneous abortion, maternal diabetes and anemia, stillbirth, birth defects, or childhood cancer in offspring were observed in survivors compared to controls...
July 21, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28700372/immunohistochemical-demonstration-of-parvovirus-b19-viral-protein-2-in-periflexural-exanthema-in-an-adult-supporting-antibody-dependent-enhancement-as-means-of-endothelial-uptake-of-the-virus
#12
Carlos Santonja, Úrsula Pielasinski, Jorge Polo, Heinz Kutzner, Luis Requena
Human parvovirus B19 (B19V) causes a number of skin exanthemas and has been related to both cutaneous and systemic diseases. Tropism of the virus for the rapidly proliferating erythroid progenitor cells in the bone marrow and fetal liver explains the pathogenesis of anemia and fetal hydrops. The cutaneous lesions of erythema infectiosum and other B19V-related exanthemas have been attributed to the deposition of immune complexes in the skin. We report on the immunohistochemical detection of B19V protein in the cytoplasm of dermal endothelial cells in a case of periflexural exanthema in a 28-year-old woman...
July 6, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28694332/the-ets2-repressor-factor-erf-is-required-for-effective-primitive-and-definitive-hematopoiesis
#13
Ioanna Peraki, James Palis, George Mavrothalassitis
Erf is a ubiquitously expressed ets-DNA-binding containing transcriptional repressor. Erf haploinsufficiency causes craniosynostosis in human and mice, while its absence in mice leads to failed chorioallantoic fusion and death at E10.5. In this study, we show that Erf is required in all three waves of embryonic hematopoiesis. Mice lacking Erf in embryo proper exhibited severe anemia and died around embryonic day (E) 14.5. Erf epiblast specific knockout embryos had reduced numbers of circulating blood cells from E9...
July 10, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28675906/early-evaluation-of-the-fetal-heart
#14
Edgar Hernandez-Andrade, Manasi Patwardhan, Mónica Cruz-Lemini, Suchaya Luewan
Evaluation of the fetal heart at 11-13 + 6 weeks of gestation is indicated for women with a family history of congenital heart defects (CHD), a previous child with CDH, or an ultrasound finding associated with cardiac anomalies. The accuracy for early detection of CHD is highly related to the experience of the operator. The 4-chamber view and outflow tracts are the most important planes for identification of an abnormal heart, and can be obtained in the majority of fetuses from 11 weeks of gestation onward...
July 5, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28667380/variants-in-the-non-coding-region-of-the-tlr2-gene-associated-with-infectious-subphenotypes-in-pediatric-sickle-cell-anemia
#15
Susana David, Pedro Aguiar, Liliana Antunes, Alexandra Dias, Anabela Morais, Anavaj Sakuntabhai, João Lavinha
Sickle cell anemia (SCA) is characterized by chronic hemolysis, severe vasoocclusive crises (VOCs), and recurrent often severe infections. A cohort of 95 SCA pediatric patients was the background for genotype-to-phenotype association of the patient's infectious disease phenotype and three non-coding polymorphic regions of the TLR2 gene, the -196 to -174 indel, SNP rs4696480, and a (GT)n short tandem repeat. The infectious subphenotypes included (A) recurrent respiratory infections and (B) severe bacterial infection at least once during the patient's follow-up...
June 30, 2017: Immunogenetics
https://www.readbyqxmd.com/read/28667041/placental-fetal-thrombotic-vasculopathy-occurring-in-association-with-megacystis-microcolon-intestinal-hypoperistalsis-syndrome-a-case-report
#16
Anneliese Vélez-Pérez, Pamela Younes, Nina Tatevian
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare disease of functional obstruction affecting the bladder and intestines, characterized by a markedly distended bladder, microcolon, and decreased or absent intestinal peristalsis. Afflicted neonates have very poor prognosis, usually with fatal outcomes in first days to months of life. Placental fetal thrombotic vasculopathy (FTV) is a thrombo-occlusive disorder of the chorionic plate and fetal circulation. Herein, we describe an undocumented association of MMIHS and placental FTV...
May 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28663682/hepatitis-e-virus-induced-acute-liver-failure-with-scrub-typhus-coinfection-in-a-pregnant-woman
#17
Nipun Verma, Megha Sharma, Manisha Biswal, Sunil Taneja, Nitya Batra, Abhay Kumar, Radha K Dhiman
Coinfections contribute significantly to diagnostic challenges of acute febrile illnesses, especially in endemic areas. The confusion caused by overlapping clinical features impedes timely management. Herein, we report an unusual, previously unreported case of a pregnant woman suffering from a coinfection of scrub typhus and hepatitis E virus. A 25-year-old, 31-week pregnant woman presented with jaundice for 5 days and altered sensorium for 2 days. She had features of both viral acute liver failure (ALF) and tropical infections mimicking ALF, including hyperbilirubinemia, coagulopathy, anemia, thrombocytopenia, intravascular hemolysis, and hepatosplenomegaly...
June 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28651846/molecular-basis-of-%C3%AE-thalassemia-and-potential-therapeutic-targets
#18
REVIEW
Swee Lay Thein
The remarkable phenotypic diversity of β thalassemia that range from severe anemia and transfusion-dependency, to a clinically asymptomatic state exemplifies how a spectrum of disease severity can be generated in single gene disorders. While the genetic basis for β thalassemia, and how severity of the anemia could be modified at different levels of its pathophysiology have been well documented, therapy remains largely supportive with bone marrow transplant being the only cure. Identification of the genetic variants modifying fetal hemoglobin (HbF) production in combination with α globin genotype provide some prediction of disease severity for β thalassemia but generation of a personalized genetic risk score to inform prognosis and guide management requires a larger panel of genetic modifiers yet to be discovered...
June 20, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28648573/personalized-treatment-with-immunoadsorption-and-intravenous-immunoglobulin-in-a-case-of-severe-rh-alloimmunization-during-pregnancy-unresponsive-to-plasma-exchange
#19
Anna Colpo, Tiziana Tison, Maria Teresa Gervasi, Cinzia Vio, Maria Vicarioto, Giustina De Silvestro, Piero Marson
INTRODUCTION: Despite prophylaxis, a small proportion of RhD-negative women may develop anti-D antibodies after a sensitizing event occurring during pregnancy or delivery of a D-positive baby. Intrauterine transfusion (IUT) is the treatment of choice in case of fetal anemia, but it cannot be performed early during pregnancy. Combined treatment with therapeutic plasma-exchange (TPE) and intravenous immunoglobulin (IVIG) can avoid or delay IUT. Immunoadsorption (IA) could represent a more effective treatment in selected cases...
June 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28633140/early-intrauterine-transfusion-in-fetuses-with-severe-anemia-caused-by-parvovirus-b19-infection
#20
Astrid Hellmund, Annegret Geipel, Christoph Berg, Rainer Bald, Ulrich Gembruch
OBJECTIVE: To describe procedure-related complications and perinatal survival after intrauterine transfusion (IUT) before 20 weeks of gestation in fetuses with severe anemia due to human parvovirus B19 infection. MATERIALS AND METHODS: A retrospective study was conducted of all fetuses requiring IUT before 20 weeks of gestation in two tertiary referral centers between January 2002 and July 2015. Gestational age (GA) at first IUT, fetal blood sampling results, and presence of hydrops were related to procedure-related complications, fetal death (FD), and perinatal outcome...
June 21, 2017: Fetal Diagnosis and Therapy
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