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[PMID]:28121068
[Au] Autor:Yang E; Voelkel EB; Lezon-Geyda K; Schulz VP; Gallagher PG
[Ad] Endereço:Department of Pediatrics, George Washington University School of Medicine, Washington, District of Columbia.
[Ti] Título:Hemoglobin C trait accentuates erythrocyte dehydration in hereditary xerocytosis.
[So] Source:Pediatr Blood Cancer;64(8), 2017 Aug.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:A 17-year-old male presented with acute hemolysis with stomatocytosis, elevated mean corpuscular hemoglobin concentration (MCHC), and osmotic gradient ektacytometry consistent with marked erythrocyte dehydration. Erythrocytes from both parents also demonstrated evidence of dehydration with elevated MCHC and abnormal ektacytometry, but neither to the degree of the patient. Genetic studies revealed the patient had hereditary xerocytosis (HX) due to a novel PIEZO1 mutation inherited from his mother and hemoglobin C (HbC) trait inherited from his father. HbC trait accentuated the erythrocyte dehydration of HX. Coinheritance of interrelated disorders and/or modifier alleles should be considered whenever severe erythrocyte dehydration is observed.
[Mh] Termos MeSH primário: Anemia Hemolítica Congênita/complicações
Anemia Hemolítica Congênita/genética
Eritrócitos/patologia
Doença da Hemoglobina C/complicações
Doença da Hemoglobina C/genética
Hidropisia Fetal/genética
[Mh] Termos MeSH secundário: Adolescente
Anemia Hemolítica Congênita/sangue
Índices de Eritrócitos
Doença da Hemoglobina C/sangue
Seres Humanos
Hidropisia Fetal/sangue
Canais Iônicos/genética
Masculino
Mutação
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Ion Channels); 0 (PIEZO1 protein, human)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170914
[Lr] Data última revisão:
170914
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170126
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26444


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[PMID]:26546258
[Au] Autor:Bissonnette ML; Henriksen KJ; Delaney K; Stankus N; Chang A
[Ad] Endereço:Departments of Pathology and.
[Ti] Título:Medullary Microvascular Thrombosis and Injury in Sickle Hemoglobin C Disease.
[So] Source:J Am Soc Nephrol;27(5):1300-4, 2016 May.
[Is] ISSN:1533-3450
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Sickle cell nephropathy is a common complication in patients with sickle cell hemoglobinopathies. In these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells, which can cause endothelial cell injury in the kidney that may lead to thrombus formation when severe or manifest by multilayering of the basement membranes (glomerular and/or peritubular capillaries) in milder forms of injury. As the injury progresses, the subsequent ischemia, tubular dysfunction, and glomerular scarring can result in CKD or ESRD. Sickle cell nephropathy can occur in patients with homozygous hemoglobin SS or heterozygous hemoglobin S (hemoglobin SC, hemoglobin S/ß(0)-thalassemia, and hemoglobin S/ß(+)-thalassemia). Clinical manifestations resulting from hemoglobin S polymerization are often milder in patients with heterozygous hemoglobin S. These patients may not present with clinically apparent acute sickle cell crises, but these milder forms can provide a unique view of the kidney injury in sickle cell disease. Here, we report a patient with hemoglobin SC disease who showed peritubular capillary and vasa recta thrombi and capillary basement membrane alterations primarily involving the renal medulla. This patient highlights the vascular occlusion and endothelial cell injury in the medulla that contribute to sickle cell nephropathy.
[Mh] Termos MeSH primário: Anemia Falciforme/complicações
Doença da Hemoglobina C/complicações
Nefropatias/etiologia
Medula Renal/irrigação sanguínea
Trombose/etiologia
[Mh] Termos MeSH secundário: Adulto
Biópsia
Feminino
Seres Humanos
Medula Renal/patologia
Microvasos
Traço Falciforme
Trombose/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170524
[Lr] Data última revisão:
170524
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151108
[St] Status:MEDLINE
[do] DOI:10.1681/ASN.2015040399


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[PMID]:26337929
[Au] Autor:Benites BD; Benevides TC; Valente IS; Marques JF; Gilli SC; Saad ST
[Ad] Endereço:Hematology and Hemotherapy Center-University of Campinas/Hemocentro-UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, University of Campinas, Campinas, São Paulo, Brazil.
[Ti] Título:The effects of exchange transfusion for prevention of complications during pregnancy of sickle hemoglobin C disease patients.
[So] Source:Transfusion;56(1):119-24, 2016 Jan.
[Is] ISSN:1537-2995
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease. MATERIALS AND METHODS: Patients were divided into two groups according to the type of transfusion support received: 10 women received prophylactic erythrocytapheresis or manual exchange transfusion at 28 weeks of gestation, and 14 received transfusions only on demand, due to acute complications, or received no transfusions at all. RESULTS: Our results indicated higher frequencies of SCD-related complications in the group that did not receive prophylactic transfusion support (35.7% vs. only 10% in the erythrocytapheresis group). Furthermore, these complications were more severe in this group and included all cases of acute chest syndrome. A significant difference was observed concerning gestational age at birth (38.7 weeks in the transfusion group vs. 34.4 weeks, p = 0.037), with a higher frequency of preterm births in the nontransfused group (69.23% vs. 30% in the transfusion group). CONCLUSION: We demonstrated a clear reduction of unfavorable outcomes in patients receiving prophylactic transfusions, probably reflecting better maternal and fetal conditions, which corroborated to the more satisfactory indices of vitality, observed in newborns. Considering that erythrocytapheresis or manual exchange transfusions both represent feasible and safe procedures, they could represent important tools for the optimal management of these patients.
[Mh] Termos MeSH primário: Remoção de Componentes Sanguíneos
Transfusão de Eritrócitos
Transfusão Total
Doença da Hemoglobina C/terapia
Complicações Hematológicas na Gravidez/terapia
Cuidado Pré-Natal/métodos
[Mh] Termos MeSH secundário: Adulto
Feminino
Seres Humanos
Recém-Nascido
Gravidez
Complicações na Gravidez/prevenção & controle
Resultado do Tratamento
[Pt] Tipo de publicação:EVALUATION STUDIES; JOURNAL ARTICLE
[Em] Mês de entrada:1605
[Cu] Atualização por classe:160113
[Lr] Data última revisão:
160113
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150905
[St] Status:MEDLINE
[do] DOI:10.1111/trf.13280


  4 / 446 MEDLINE  
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[PMID]:25335812
[Au] Autor:Lemonne N; Billaud M; Waltz X; Romana M; Hierso R; Etienne-Julan M; Connes P
[Ad] Endereço:Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, 97159 Pointe-à-Pitre, Guadeloupe.
[Ti] Título:Rheology of red blood cells in patients with HbC disease.
[So] Source:Clin Hemorheol Microcirc;61(4):571-7, 2016.
[Is] ISSN:1875-8622
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Patients with hemoglobin C disease (CC) usually do not develop severe complications in comparison with individuals with sickle cell anemia (SS) or with sickle cell hemoglobin C disease (SC). The present study compared the hematological, biochemical, hemorheological and clinical characteristics of CC patients to those of SS, SC and healthy individuals (AA). Blood viscosity was measured at 225 s(-1) with a cone plate viscometer. The hematocrit-to-blood viscosity ratio (HVR), i.e. an index of red blood cell (RBC) oxygen transport effectiveness, was calculated. RBC deformability was determined at 30 Pa by ektacytometry, and RBC aggregation properties by syllectometry. CC and SC had higher blood viscosity and lower HVR than AA. Nevertheless, HVR was higher in CC compared to SS and tended to be higher than in SC. The CC group exhibited very rigid hyperchromic RBC compared to the three other groups. RBC aggregation abnormalities were observed in CC: low RBC aggregation index and high RBC aggregates strength. Despite these hemorheological abnormalities, CC never had hospitalized painful vaso-occlusive crisis or acute chest syndrome. In contrast, all of them had splenomegaly. Of note, 2 out of 7 CC developed retinopathy or otologic disorders. Whether the blood hyperviscosity and decreased RBC deformability are responsible for these complications is unknown. The higher oxygen transport effectiveness (i.e., HVR) of CC compared to SS is probably at the origin of the very low risk of medical complication in this population.
[Mh] Termos MeSH primário: Eritrócitos/metabolismo
Doença da Hemoglobina C/sangue
Hemorreologia
[Mh] Termos MeSH secundário: Adulto
Viscosidade Sanguínea
Contagem de Eritrócitos
Deformação Eritrocítica
Feminino
Doença da Hemoglobina C/patologia
Seres Humanos
Masculino
Meia-Idade
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1701
[Cu] Atualização por classe:170104
[Lr] Data última revisão:
170104
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141023
[St] Status:MEDLINE
[do] DOI:10.3233/CH-141906


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[PMID]:25488433
[Au] Autor:Bain BJ
[Ad] Endereço:St Mary's Hospital Campus of Imperial College Faculty of Medicine, St Mary's Hospital, Praed Street, London, N1 1NY, UK.
[Ti] Título:Hemoglobin C disease.
[So] Source:Am J Hematol;90(2):174, 2015 Feb.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Eritroblastos/patologia
Doença da Hemoglobina C/patologia
Hemoglobina C/química
[Mh] Termos MeSH secundário: Colecistectomia
Cálculos Biliares/complicações
Cálculos Biliares/patologia
Cálculos Biliares/cirurgia
Doença da Hemoglobina C/complicações
Doença da Hemoglobina C/diagnóstico
Seres Humanos
Masculino
Meia-Idade
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
9008-00-8 (Hemoglobin C)
[Em] Mês de entrada:1503
[Cu] Atualização por classe:150123
[Lr] Data última revisão:
150123
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141210
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.23915


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[PMID]:25271992
[Au] Autor:Vovor A; Fétéké L; Kueviakoe IM; Kpatarou L; Mawussi K; Magnang H; Ségbéna AY
[Ad] Endereço:Laboratory Hematology the CHU-Sylvanus Olympio , Lomé , Togo .
[Ti] Título:Blood typing profile of a school-aged population of a North Togo township.
[So] Source:Hemoglobin;38(5):316-8, 2014.
[Is] ISSN:1532-432X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:The aim of this study was the determination of hemoglobin (Hb) variants and ABO blood groups in a school population aged 6 to 9 years in the township of Agbandé-Yaka in North Togo. A cross-sectional study was carried out on 570 children of four primary schools at Agbande-Yaka, between March and July 2010. Hemoglobin characterization was done by alkaline buffer electrophoresis and the blood types ABO-Rhesus (Rh) D by immuno-hematological methods. A Hb variant was detected in 37.0% of the schoolchildren. Among them, the AS trait accounted for 11.9% and the AC trait for 20.2%. Homozygous Hb S (HBB: c.20A>T) was not found but Hb C (HBB: c.19G>A) appeared at a frequency of 3.3%, while compound heterozygotes carrying Hb SC were seen at a frequency of 1.6%. The O, B and A blood groups accounted for 49.0, 26.8 and 21.9%, respectively. The Hb anomalies reached a high prevalence in this school population. These results are remarkable by the absence of homozygous Hb S individuals compared to homozygous Hb C individuals, which were as numerous as expected. The frequencies of the ABO blood groups are similar to what has been found in other West African populations.
[Mh] Termos MeSH primário: Sistema do Grupo Sanguíneo ABO/sangue
Doença da Hemoglobina C/epidemiologia
Hemoglobina C/análise
Doença da Hemoglobina SC/epidemiologia
Hemoglobina Falciforme/análise
Polimorfismo de Nucleotídeo Único
Sistema do Grupo Sanguíneo Rh-Hr/sangue
[Mh] Termos MeSH secundário: Alelos
Criança
Estudos Transversais
Feminino
Frequência do Gene
Hemoglobina C/genética
Doença da Hemoglobina C/sangue
Doença da Hemoglobina C/genética
Doença da Hemoglobina SC/sangue
Doença da Hemoglobina SC/genética
Hemoglobina Falciforme/genética
Heterozigoto
Homozigoto
Seres Humanos
Masculino
Programas de Rastreamento
Prevalência
Instituições Acadêmicas
Togo/epidemiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (ABO Blood-Group System); 0 (Hemoglobin, Sickle); 0 (Rh-Hr Blood-Group System); 9008-00-8 (Hemoglobin C)
[Em] Mês de entrada:1506
[Cu] Atualização por classe:141002
[Lr] Data última revisão:
141002
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141002
[St] Status:MEDLINE
[do] DOI:10.3109/03630269.2014.963234


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[PMID]:24633868
[Au] Autor:Lemonne N; Lamarre Y; Romana M; Hardy-Dessources MD; Lionnet F; Waltz X; Tarer V; Mougenel D; Tressières B; Lalanne-Mistrih ML; Etienne-Julan M; Connes P
[Ad] Endereço:pconnes@yahoo.fr.
[Ti] Título:Impaired blood rheology plays a role in the chronic disorders associated with sickle cell-hemoglobin C disease.
[So] Source:Haematologica;99(5):74-5, 2014 May.
[Is] ISSN:1592-8721
[Cp] País de publicação:Italy
[La] Idioma:eng
[Mh] Termos MeSH primário: Doença da Hemoglobina C/sangue
Doença da Hemoglobina C/etiologia
Hemorreologia
[Mh] Termos MeSH secundário: Seres Humanos
[Pt] Tipo de publicação:LETTER
[Em] Mês de entrada:1504
[Cu] Atualização por classe:150514
[Lr] Data última revisão:
150514
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:140318
[St] Status:MEDLINE
[do] DOI:10.3324/haematol.2014.104745


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[PMID]:23302597
[Au] Autor:Lamarre Y; Hardy-Dessources MD; Romana M; Lalanne-Mistrih ML; Waltz X; Petras M; Doumdo L; Blanchet-Deverly A; Martino J; Tressières B; Maillard F; Tarer V; Etienne-Julan M; Connes P
[Ad] Endereço:Inserm U665, Pointe-à-Pitre, F-97159 Guadeloupe, Université des Antilles et de la Guyane, Guadeloupe.
[Ti] Título:Relationships between systemic vascular resistance, blood rheology and nitric oxide in children with sickle cell anemia or sickle cell-hemoglobin C disease.
[So] Source:Clin Hemorheol Microcirc;58(2):307-16, 2014.
[Is] ISSN:1875-8622
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Vascular function has been found to be impaired in patients with sickle cell disease (SCD). The present study investigated the determinants of systemic vascular resistance in two main SCD syndromes in children: sickle cell anemia (SCA) and sickle cell-hemoglobin C disease (SCC). Nitric oxide metabolites (NOx), hematological, hemorheological, and hemodynamical parameters were investigated in 61 children with SCA and 49 children with SCC. While mean arterial pressure was not different between SCA and SCC children, systemic vascular resistance (SVR) was greater in SCC children. Although SVR and blood viscosity (ηb) were not correlated in SCC children, the increase of ηb (+18%) in SCC children compared to SCA children results in a greater mean SVR in this former group. SVR was positively correlated with ηb, hemoglobin (Hb) level and RBC deformability, and negatively with NOx level in SCA children. Multivariate linear regression model showed that both NOx and Hb levels were independently associated with SVR in SCA children. In SCC children, only NOx level was associated with SVR. In conclusion, vascular function of SCC children seems to better cope with higher ηb compared to SCA children. Since the occurrence of vaso-occlusive like complications are less frequent in SCC than in SCA children, this finding suggests a pathophysiological link between the vascular function alteration and these clinical manifestations. In addition, our results suggested that nitric oxide metabolism plays a key role in the regulation of SVR, both in SCA and SCC.
[Mh] Termos MeSH primário: Anemia Falciforme/sangue
Doença da Hemoglobina C/sangue
Óxido Nítrico/metabolismo
[Mh] Termos MeSH secundário: Viscosidade Sanguínea
Criança
Feminino
Seres Humanos
Masculino
Reologia
Resistência Vascular
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
31C4KY9ESH (Nitric Oxide)
[Em] Mês de entrada:1508
[Cu] Atualização por classe:170220
[Lr] Data última revisão:
170220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:130111
[St] Status:MEDLINE
[do] DOI:10.3233/CH-121661


  9 / 446 MEDLINE  
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[PMID]:24263962
[Au] Autor:Steinberg MH; Chui DH
[Ad] Endereço:Department of Medicine, Boston University School of Medicine, Center of Excellence for Sickle Cell Disease, Boston Medical Center, Boston, MA.
[Ti] Título:HbC disorders.
[So] Source:Blood;122(22):3698, 2013 Nov 21.
[Is] ISSN:1528-0020
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Doença da Hemoglobina C/genética
Homozigoto
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
[Pt] Tipo de publicação:COMMENT; JOURNAL ARTICLE
[Em] Mês de entrada:1401
[Cu] Atualização por classe:131122
[Lr] Data última revisão:
131122
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:131123
[St] Status:MEDLINE
[do] DOI:10.1182/blood-2013-09-526764


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[PMID]:24137818
[Au] Autor:Dalia S; Zhang L
[Ad] Endereço:H. Lee Moffitt Cancer and Research Institute, USA.
[Ti] Título:Homozygous hemoglobin C disease.
[So] Source:Blood;122(10):1694, 2013 Sep 05.
[Is] ISSN:1528-0020
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Doença da Hemoglobina C/genética
Homozigoto
[Mh] Termos MeSH secundário: Adulto
Feminino
Doença da Hemoglobina C/complicações
Doença da Hemoglobina C/tratamento farmacológico
Seres Humanos
Dor/etiologia
Varfarina/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
5Q7ZVV76EI (Warfarin)
[Em] Mês de entrada:1310
[Cu] Atualização por classe:140107
[Lr] Data última revisão:
140107
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:131019
[St] Status:MEDLINE



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