Base de dados : MEDLINE
Pesquisa : C15.378.071.141.150.150.440 [Categoria DeCS]
Referências encontradas : 586 [refinar]
Mostrando: 1 .. 10   no formato [Detalhado]

página 1 de 59 ir para página                         

  1 / 586 MEDLINE  
              next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28466542
[Au] Autor:Möckesch B; Connes P; Charlot K; Skinner S; Hardy-Dessources MD; Romana M; Jumet S; Petras M; Divialle-Doumdo L; Martin C; Tressières B; Tarer V; Hue O; Etienne-Julan M; Antoine S; Pialoux V
[Ad] Endereço:Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France.
[Ti] Título:Association between oxidative stress and vascular reactivity in children with sickle cell anaemia and sickle haemoglobin C disease.
[So] Source:Br J Haematol;178(3):468-475, 2017 08.
[Is] ISSN:1365-2141
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Oxidative stress and haemolysis-associated nitric oxide (NO) depletion plays a crucial role in the development of vasculopathy in sickle cell anaemia (SS). However it remains unknown whether oxidative stress and haemolysis levels influence vascular function in patients with sickle haemoglobin C disease (SC). Microvascular response to heat (using Laser Doppler flowmetry on finger), oxidative stress biomarkers, NO metabolites, endothelin-1 and haematological parameters were compared between patients with SS and SC. Vascular function, oxidative and nitrosative markers were also measured in healthy (AA) children. SS and SC had increased plasma advanced oxidation protein products (AOPP), malondialdehyde, plasma antioxidant activities and NO end products, compared to AA. SC had lower catalase activity compared to AA and SS. Haemolytic rate, glutathione peroxidase and nitrotyrosine concentrations were significantly increased in children with SS compared to SC and AA. SS and SC had impaired microvascular reactivity compared to AA. In SS, the plateau phase of the response to local thermal heating was negatively associated with nitrotyrosine and AOPP. No association between vascular function parameters and oxidative stress markers was observed in SC. Mild haemolysis in SC, compared to SS, may limit oxidative and nitrosative stress and could explain the better preserved microvascular function in this group.
[Mh] Termos MeSH primário: Anemia Falciforme/fisiopatologia
Estresse Oxidativo/fisiologia
[Mh] Termos MeSH secundário: Adolescente
Produtos da Oxidação Avançada de Proteínas/sangue
Antioxidantes/metabolismo
Viscosidade Sanguínea/fisiologia
Estudos de Casos e Controles
Criança
Endotelina-1/sangue
Feminino
Dedos/irrigação sanguínea
Doença da Hemoglobina SC/fisiopatologia
Hemólise/fisiologia
Seres Humanos
Fluxometria por Laser-Doppler/métodos
Masculino
Malondialdeído/sangue
Microcirculação/fisiologia
Óxido Nítrico/sangue
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Advanced Oxidation Protein Products); 0 (Antioxidants); 0 (Endothelin-1); 31C4KY9ESH (Nitric Oxide); 4Y8F71G49Q (Malondialdehyde)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170504
[St] Status:MEDLINE
[do] DOI:10.1111/bjh.14693


  2 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28489923
[Au] Autor:Garnier Y; Ferdinand S; Etienne-Julan M; Elana G; Petras M; Doumdo L; Tressières B; Lalanne-Mistrih ML; Hardy-Dessources MD; Connes P; Romana M
[Ad] Endereço:Unité Biologie Intégrée du Globule Rouge, Université des Antilles, Inserm 1134, laboratoire d'Excellence GR-Ex, Paris, France.
[Ti] Título:Differences of microparticle patterns between sickle cell anemia and hemoglobin SC patients.
[So] Source:PLoS One;12(5):e0177397, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry. Most of circulating MPs were derived from platelets (PLTs) and red blood cells (RBCs) in the two SCD syndromes. Moreover, we showed that HbSC patients exhibited lower blood concentration of total MPs compared to SCA patients, resulting mainly from a decrease of MP levels originated from RBCs and to a lesser extent from PLTs. We did not detect any association between blood MP concentrations and the occurrence of painful vaso-occlusive crises, acute chest syndrome and pulmonary hypertension in both patient groups. We also demonstrated for the first time, that whatever the considered genotype, RBC-derived MPs exhibited higher externalized phosphatidylserine level and were larger than PLT-derived MPs.
[Mh] Termos MeSH primário: Anemia Falciforme/patologia
Plaquetas/patologia
Micropartículas Derivadas de Células/patologia
Eritrócitos/patologia
Doença da Hemoglobina SC/patologia
[Mh] Termos MeSH secundário: Adolescente
Anemia Falciforme/sangue
Criança
Feminino
Doença da Hemoglobina SC/sangue
Seres Humanos
Masculino
Fosfatidilserinas/análise
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Phosphatidylserines)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170908
[Lr] Data última revisão:
170908
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170511
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0177397


  3 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
Texto completo
[PMID]:28426137
[Au] Autor:Nevitt SJ; Jones AP; Howard J
[Ad] Endereço:Department of Biostatistics, University of Liverpool, Block F, Waterhouse Building, 1-5 Brownlow Hill, Liverpool, UK, L69 3GL.
[Ti] Título:Hydroxyurea (hydroxycarbamide) for sickle cell disease.
[So] Source:Cochrane Database Syst Rev;4:CD002202, 2017 04 20.
[Is] ISSN:1469-493X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Register, comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched online trial registries.Date of the most recent search: 16 January 2017. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials, of one month or longer, comparing hydroxyurea with placebo, standard therapy or other interventions for people with SCD. DATA COLLECTION AND ANALYSIS: Authors independently assessed studies for inclusion, carried out data extraction and assessed the risk of bias. MAIN RESULTS: Seventeen studies were identified in the searches; eight randomised controlled trials were included, recruiting 899 adults and children with SCD (haemoglobin SS (HbSS), haemoglobin SC (HbSC) or haemoglobin Sߺthalassaemia (HbSߺthal) genotypes). Studies lasted from six to 30 months.Four studies (577 adults and children with HbSS or HbSߺthal) compared hydroxyurea to placebo; three recruited individuals with only severe disease and one recruited individuals with all disease severities. There were statistically significant improvements in terms of pain alteration (using measures such as pain crisis frequency, duration, intensity, hospital admissions and opoid use), measures of fetal haemoglobin and neutrophil counts and fewer occurrences of acute chest syndrome and blood transfusions in the hydroxyurea groups. There were no consistent statistically significant differences in terms of quality of life and adverse events (including serious or life-threatening events). Seven deaths occurred during the studies, but the rates by treatment group were not statistically significantly different.Two studies (254 children with HbSS or HbSߺthal also with risk of primary or secondary stroke) compared hydroxyurea and phlebotomy to transfusion and chelation; there were statistically significant improvements in terms of measures of fetal haemoglobin and neutrophil counts, but more occurrences of acute chest syndrome and infections in the hydroxyurea and phlebotomy group. There were no consistent statistically significant differences in terms of pain alteration and adverse events (including serious or life-threatening events). Two deaths occurred during the studies (one in a the hydroxyurea treatment arm and one in the control arm), but the rates by treatment group were not statistically significantly different. In the primary prevention study, no strokes occurred in either treatment group but in the secondary prevention study, seven strokes occurred in the hydroxyurea and phlebotomy group (none in the transfusion and chelation group) and the study was terminated early.The quality of the evidence for the above two comparisons was judged as moderate to low as the studies contributing to these comparisons were mostly large and well designed (and at low risk of bias); however evidence was limited and imprecise for some outcomes such as quality of life, deaths during the studies and adverse events and results are applicable only to individuals with HbSS and HbSߺthal genotypes.Of the remaining two studies, one (22 children with HbSS or HbSߺthal also at risk of stoke) compared hydroxyurea to observation; there were statistically significant improvements in terms of measures of fetal haemoglobin and neutrophil counts but no statistically significant differences in terms of adverse events (including serious or life-threatening events).The final study (44 adults and children with HbSC) compared treatment regimens with and without hydroxyurea - there was statistically significant improvement in terms of measures of fetal haemoglobin, but no statistically significant differences in terms of adverse events (including serious or life-threatening events). No participants died in either of these studies and other outcomes relevant to the review were not reported.The quality of the evidence for the above two comparisons was judged to be very low due to the limited number of participants, the lack of statistical power (as both studies were terminated early with approximately only 20% of their target sample size recruited) and the lack of applicability to all age groups and genotypes. AUTHORS' CONCLUSIONS: There is evidence to suggest that hydroxyurea is effective in decreasing the frequency of pain episodes and other acute complications in adults and children with sickle cell anaemia of HbSS or HbSߺthal genotypes and in preventing life-threatening neurological events in those with sickle cell anaemia at risk of primary stroke by maintaining transcranial doppler velocities. However, there is still insufficient evidence on the long-term benefits of hydroxyurea, particularly in preventing chronic complications of SCD, recommending a standard dose or dose escalation to maximum tolerated dose. There is also insufficient evidence about the long-term risks of hydroxyurea, including its effects on fertility and reproduction. Evidence is also limited on the effects of hydroxyurea on individuals with HbSC genotype. Future studies should be designed to address such uncertainties.
[Mh] Termos MeSH primário: Anemia Falciforme/tratamento farmacológico
Antidrepanocíticos/uso terapêutico
Hidroxiureia/uso terapêutico
[Mh] Termos MeSH secundário: Adulto
Anemia Falciforme/sangue
Anemia Falciforme/mortalidade
Antidrepanocíticos/efeitos adversos
Terapia por Quelação
Criança
Transfusão de Eritrócitos
Genótipo
Doença da Hemoglobina SC/sangue
Doença da Hemoglobina SC/tratamento farmacológico
Seres Humanos
Hidroxiureia/efeitos adversos
Flebotomia/efeitos adversos
Ensaios Clínicos Controlados Aleatórios como Assunto
Conduta Expectante
[Pt] Tipo de publicação:JOURNAL ARTICLE; META-ANALYSIS; REVIEW
[Nm] Nome de substância:
0 (Antisickling Agents); X6Q56QN5QC (Hydroxyurea)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170801
[Lr] Data última revisão:
170801
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170421
[St] Status:MEDLINE
[do] DOI:10.1002/14651858.CD002202.pub2


  4 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28110132
[Au] Autor:Gupta S; Carmona R; Malvar J; Young G
[Ad] Endereço:Division of Transfusion Medicine and Coagulation, Baylor College of Medicine, Houston, TX, United States. Electronic address: shveta85g@gmail.com.
[Ti] Título:Thromboelastographic characterization of the activated clotting system in children with sickle cell trait or sickle cell disease.
[So] Source:Thromb Res;151:44-50, 2017 Mar.
[Is] ISSN:1879-2472
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Recent epidemiological evidence suggests sickle cell disease (SCD) and sickle cell trait (SCT) is a risk factor for venous thromboembolism. The increased in-vivo markers of thrombin generation support the notion that such patients are in a chronic hypercoagulable state. In an attempt to better understand the underlying mechanism, global hemostatic assays including thrombin generation assay (TGA) and thromboelastography (TEG) have been utilized by several groups, but thus far, have shown inconsistent results either due to small sample size or technical differences. OBJECTIVES: Global hemostatic characterization of children with SCD or SCT by using TGA and modified TEG methods. MATERIALS AND METHODS: In this pilot study, we obtained TGA, TEG and other hemostatic data on specimens from 13 patients with SCD, 14 with SCT and 12 race-matched healthy controls (NC). RESULTS: R time and K time with modified TEG methods were significantly shorter in SCD when compared to SCT and NC. Alpha and MA did not show any significant differences between the groups. There was no difference seen between SCT and NC. TGA profiles did not show any difference between the three groups. As expected the in-vivo markers of thrombin generation and activation of fibrinolysis including D dimer and thrombin-antithrombin complexes were significantly higher in SCD subjects as compared to SCT and NC. CONCLUSION: The modified TEG methods are able to detect the activated coagulation system for the SCD population but a larger and more homogenous SCT cohort needs to be studied for more conclusive results.
[Mh] Termos MeSH primário: Coagulação Sanguínea
Doença da Hemoglobina SC/sangue
Traço Falciforme/sangue
Trombofilia/sangue
[Mh] Termos MeSH secundário: Adolescente
Adulto
Antitrombina III
Criança
Feminino
Produtos de Degradação da Fibrina e do Fibrinogênio/análise
Doença da Hemoglobina SC/complicações
Seres Humanos
Masculino
Peptídeo Hidrolases/sangue
Projetos Piloto
Traço Falciforme/complicações
Tromboelastografia
Trombina/análise
Trombofilia/etiologia
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Fibrin Fibrinogen Degradation Products); 0 (antithrombin III-protease complex); 0 (fibrin fragment D); 9000-94-6 (Antithrombin III); EC 3.4.- (Peptide Hydrolases); EC 3.4.21.5 (Thrombin)
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170428
[Lr] Data última revisão:
170428
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170123
[St] Status:MEDLINE


  5 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27984631
[Au] Autor:Drasar E; Fitzpatrick E; Gardner K; Awogbade M; Dhawan A; Bomford A; Suddle A; Thein SL
[Ad] Endereço:Faculty of Life Sciences & Medicine, Molecular Haematology, King's College London, London, UK.
[Ti] Título:Interim assessment of liver damage in patients with sickle cell disease using new non-invasive techniques.
[So] Source:Br J Haematol;176(4):643-650, 2017 Feb.
[Is] ISSN:1365-2141
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:We explored transient elastography (TE) and enhanced liver fibrosis (ELF ) score with standard markers of liver function to assess liver damage in 193 well patients with sickle cell disease (SCD). Patients with HbSS or HbSß thalassaemia (sickle cell anaemia, SCA; N = 134), had significantly higher TE results and ELF scores than those with HbSC (N = 49) disease (TE, 6·8 vs. 5·3, P < 0·0001 and ELF, 9·2 vs. 8·6 P < 0·0001). In SCA patients, TE and ELF correlated significantly with age and all serum liver function tests (LFTs). Additionally, (weak) positive correlation was found with lactate dehydrogenase (TE: r = 0·24, P = 0·004; ELF: r = 0·26 P = 0·002), and (weak) negative correlation with haemoglobin (TE: r = -0·25, P = 0·002; ELF: r = -0·25 P = 0·004). In HbSC patients, correlations were weaker or not significant between TE or ELF, and serum LFTs. All markers of iron loading correlated with TE values when corrected for sickle genotype (serum ferritin, ß = 0·25, P < 0·0001, total blood transfusion units, ß = 0·25, P < 0·0001 and LIC ß = 0·32, P = 0·046). The exploratory study suggests that, while TE could have a role, the utility of ELF score in monitoring liver damage in SCD, needs further longitudinal studies.
[Mh] Termos MeSH primário: Anemia Falciforme/complicações
Hepatopatias/etiologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Anemia Falciforme/patologia
Técnicas de Imagem por Elasticidade
Feminino
Doença da Hemoglobina SC
Hemoglobina Falciforme
Seres Humanos
Sobrecarga de Ferro/diagnóstico
Cirrose Hepática/diagnóstico
Hepatopatias/patologia
Testes de Função Hepática
Masculino
Meia-Idade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Hemoglobin, Sickle)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170730
[Lr] Data última revisão:
170730
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161217
[St] Status:MEDLINE
[do] DOI:10.1111/bjh.14462


  6 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27982424
[Au] Autor:Pecker LH; Schaefer BA; Luchtman-Jones L
[Ad] Endereço:Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC, USA.
[Ti] Título:Knowledge insufficient: the management of haemoglobin SC disease.
[So] Source:Br J Haematol;176(4):515-526, 2017 Feb.
[Is] ISSN:1365-2141
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC.
[Mh] Termos MeSH primário: Doença da Hemoglobina SC/terapia
[Mh] Termos MeSH secundário: Gerenciamento Clínico
Eritrócitos Anormais/patologia
Genótipo
Doença da Hemoglobina SC/diagnóstico
Seres Humanos
Qualidade de Vida
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170608
[Lr] Data última revisão:
170608
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161217
[St] Status:MEDLINE
[do] DOI:10.1111/bjh.14444


  7 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27957790
[Au] Autor:Vieira C; de Oliveira CN; de Figueiredo LA; Santiago RP; Adanho CS; Santana SS; Burak CL; Lyra IM; Goncalves MS
[Ad] Endereço:Ambulatório Pediátrico de Doença Cerebrovascular, Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Bahia, Brazil.
[Ti] Título:Transcranial Doppler in hemoglobin SC disease.
[So] Source:Pediatr Blood Cancer;64(5), 2017 May.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Stroke is a severe clinical disorder in sickle cell disease (SCD), and few studies have evaluated transcranial Doppler (TCD) flow velocities in hemoglobin SC disease (HbSC). The guidelines for stroke risk are based on evaluations in sickle cell anemia (SCA) or HbS/ß thalassemia. PROCEDURE: In this study, we compare cerebral blood flow in patients with SCD stratified by genotypes. A total of 1,664 pediatric patients with SCD underwent TCD velocity screening, and the time-averaged maximum mean velocity (TAMM) was determined in the middle cerebral artery (MCA), anterior cerebral artery (ACA), and distal intracranial internal carotid artery (ICA). RESULTS: Abnormal velocities were not identified in the ACA; therefore, we only use ICA and MCA velocities. TAMM from the left and right in the ICA and MCA was 134.3 ± 32.0 and 134.4 ± 32.6 cm/s in patients with SCA, and 105.2 ± 20.6 and 104.7 ± 20.0 cm/s in the patients with HbSC, respectively. Mean TAMM between right and left ICA/MCA was 134.5 ± 30.5 cm/s in the SCA group, and 104.9 ± 19.3 cm/s in the HbSC group. Notably, our data show that TCD velocities were significantly lower among the patients with HbSC compared to SCA. TAMM was negatively correlated with hemoglobin and hematocrit in both genotypes. CONCLUSION: These results suggest that a different cut-off value for abnormal TCD velocities could be considered for patients with HbSC. Additional studies are warranted to determine the actual risk of stroke in HbSC genotype associated with this possible TCD risk value.
[Mh] Termos MeSH primário: Velocidade do Fluxo Sanguíneo/fisiologia
Circulação Cerebrovascular/fisiologia
Doença da Hemoglobina SC/diagnóstico por imagem
Acidente Vascular Cerebral/prevenção & controle
Ultrassonografia Doppler Transcraniana/métodos
[Mh] Termos MeSH secundário: Adolescente
Artérias Cerebrais/fisiologia
Criança
Pré-Escolar
Feminino
Hemoglobinas/análise
Hemoglobinas/genética
Seres Humanos
Masculino
Acidente Vascular Cerebral/diagnóstico por imagem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Hemoglobins)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170626
[Lr] Data última revisão:
170626
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161214
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26342


  8 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Costa, Fernando F
Texto completo
[PMID]:27546026
[Au] Autor:Baptista LC; Costa ML; Ferreira R; Albuquerque DM; Lanaro C; Fertrin KY; Surita FG; Parpinelli MA; Costa FF; Melo MB
[Ad] Endereço:Center for Molecular Biology and Genetic Engineering (CBMEG), University of Campinas - UNICAMP, Campinas, SP, Brazil.
[Ti] Título:Abnormal expression of inflammatory genes in placentas of women with sickle cell anemia and sickle hemoglobin C disease.
[So] Source:Ann Hematol;95(11):1859-67, 2016 Oct.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Sickle cell disease (SCD) is a complex disease that is characterized by the polymerization of deoxyhemoglobin S, altered red blood cell membrane biology, endothelial activation, hemolysis, a procoagulant state, acute and chronic inflammation, and vaso-occlusion. Among the physiological changes that occur during pregnancy, oxygen is consumed by fetal growth, and pregnant women with SCD are more frequently exposed to low oxygen levels. This might lead to red blood cells sickling, and, consequently, to vaso-occlusion. The mechanisms by which SCD affects placental physiology are largely unknown, and chronic inflammation might be involved in this process. This study aimed to evaluate the gene expression profile of inflammatory response mediators in the placentas of pregnant women with sickle cell cell anemia (HbSS) and hemoglobinopathy SC (HbSC). Our results show differences in a number of these genes. For the HbSS group, when compared to the control group, the following genes showed differential expression: IL1RAP (2.76-fold), BCL6 (4.49-fold), CXCL10 (-2.12-fold), CXCR1 (-3.66-fold), and C3 (-2.0-fold). On the other hand, the HbSC group presented differential expressions of the following genes, when compared to the control group: IL1RAP (4.33-fold), CXCL1 (3.05-fold), BCL6 (4.13-fold), CXCL10 (-3.32-fold), C3 (-2.0-fold), and TLR3 (2.38-fold). Taken together, these data strongly suggest a differential expression of several inflammatory genes in both SCD (HbSS and HbSC), indicating that the placenta might become an environment with hypoxia, and increased inflammation, which could lead to improper placental development.
[Mh] Termos MeSH primário: Anemia Falciforme/genética
Citocinas/biossíntese
Regulação da Expressão Gênica
Doença da Hemoglobina SC/genética
Inflamação/genética
Placenta/metabolismo
Complicações Hematológicas na Gravidez/genética
Receptores de Citocinas/biossíntese
[Mh] Termos MeSH secundário: Adulto
Anemia Falciforme/complicações
Estudos de Casos e Controles
Citocinas/genética
Feminino
Perfilação da Expressão Gênica
Doença da Hemoglobina SC/complicações
Seres Humanos
Inflamação/etiologia
Placenta/patologia
Gravidez
Reação em Cadeia da Polimerase em Tempo Real
Receptores de Citocinas/genética
História Reprodutiva
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Cytokines); 0 (Receptors, Cytokine)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170207
[Lr] Data última revisão:
170207
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160823
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-016-2780-1


  9 / 586 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27485695
[Au] Autor:Eid R; Raj A; Farber D; Puri V; Bertolone S
[Ad] Endereço:School of Medicine, University of Louisville, Louisville, Kentucky; and.
[Ti] Título:Spinal Cord Infarction in Hemoglobin SC Disease as an Amusement Park Accident.
[So] Source:Pediatrics;138(3), 2016 Sep.
[Is] ISSN:1098-4275
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Spinal cord infarction (SCI) is extremely rare in children, and only 2 other reports have described the occurrence of SCI in patients with hemoglobin SC disease (HbSC). Amusement park accidents are serious injuries. Patients with preexisting conditions, such as hypertension, cardiac disease, and recent back or neck injuries, may be at an increased risk. We report the case of a 12-year-old girl with HbSC with a past history of only 2 admissions for pain crises, who presented to the emergency department with symptoms of SCI after riding a roller coaster. Fibrocartilaginous embolism (FCE) is an increasingly recognized cause of SCI after events that put strain on the axial skeleton, such as many amusement park rides. Although radiologic criteria for FCE have been proposed, FCE remains a diagnosis of exclusion. To the best of our knowledge, this is the first documented case of SCI in a patient with HbSC and the first case of FCE after an amusement park accident. This case report highlights that HbSC may confound the differential diagnosis of SCI and aims to document an association with FCE in pediatric patients.
[Mh] Termos MeSH primário: Acidentes
Embolia/etiologia
Doença da Hemoglobina SC/complicações
Infarto/etiologia
Isquemia do Cordão Espinal/etiologia
Medula Espinal/irrigação sanguínea
[Mh] Termos MeSH secundário: Vértebras Cervicais
Criança
Diagnóstico Diferencial
Embolia/diagnóstico
Feminino
Seres Humanos
Infarto/diagnóstico
Recreação
Isquemia do Cordão Espinal/diagnóstico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170615
[Lr] Data última revisão:
170615
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:160804
[St] Status:MEDLINE


  10 / 586 MEDLINE  
              first record previous record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27235631
[Au] Autor:Thame MM; Singh-Minott I; Osmond C; Melbourne-Chambers RH; Serjeant GR
[Ad] Endereço:Department of Child and Adolescent Health, University of the West Indies, Mona, Kingston, Jamaica. Electronic address: Minerva.thame@uwimona.edu.jm.
[Ti] Título:Pregnancy in sickle cell-haemoglobin C (SC) disease. A retrospective study of birth size and maternal weight gain.
[So] Source:Eur J Obstet Gynecol Reprod Biol;203:16-9, 2016 Aug.
[Is] ISSN:1872-7654
[Cp] País de publicação:Ireland
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To assess pregnancy and fetal outcomes in Jamaican subjects with sickle cell-haemoglobin C (SC) disease. STUDY DESIGN: A retrospective chart review over 21 years (1992-2012) of all pregnancies in SC disease and a comparison group matched by gender and date of delivery in mothers with a normal haemoglobin (AA) phenotype at the University Hospital of the West Indies, Jamaica. There were 118 pregnancies in 81 patients with SC disease and 110 pregnancies in 110 in the normal comparison group. Corrections were made for repeat pregnancies from the same mother. Outcome measures included maternal weight at 20, 25, 30, 35 and 38 weeks gestation, maternal pregnancy complications, birth weight, head circumference and crown heel length and were used to analyse possible predictors of birth weight. RESULTS: First antenatal visits occurred later in women with SC disease, who also had lower haemoglobin level and lower systolic blood pressure. The prevalence of pregnancy-induced hypertension, pre-eclampsia, ante-partum or postpartum haemorrhage did not differ between genotypes. Maternal weight gain was significantly lower in SC disease and there was a significantly lower birth weight, head circumference, and gestational age. CONCLUSIONS: Pregnancy in SC disease is generally benign but mothers had lower weight gain and lower birth weight babies, the difference persisting after correction for gestational age.
[Mh] Termos MeSH primário: Peso ao Nascer/fisiologia
Doença da Hemoglobina SC/fisiopatologia
Hipertensão Induzida pela Gravidez/epidemiologia
Complicações Hematológicas na Gravidez/fisiopatologia
Ganho de Peso/fisiologia
[Mh] Termos MeSH secundário: Adulto
Feminino
Seres Humanos
Hipertensão Induzida pela Gravidez/fisiopatologia
Gravidez
Resultado da Gravidez
Cuidado Pré-Natal
Prevalência
Estudos Retrospectivos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160529
[St] Status:MEDLINE



página 1 de 59 ir para página                         
   


Refinar a pesquisa
  Base de dados : MEDLINE Formulário avançado   

    Pesquisar no campo  
1  
2
3
 
           



Search engine: iAH v2.6 powered by WWWISIS

BIREME/OPAS/OMS - Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde