Base de dados : MEDLINE
Pesquisa : C15.378.071.085.080 [Categoria DeCS]
Referências encontradas : 6 [refinar]
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  1 / 6 MEDLINE  
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[PMID]:23417980
[Au] Autor:Cambot M; Mazurier C; Canoui-Poitrine F; Hebert N; Picot J; Clay D; Picard V; Ripoche P; Douay L; Dubart-Kupperschmitt A; Cartron JP
[Ad] Endereço:Institut National de la Transfusion Sanguine (INTS), 75015, Paris, France.
[Ti] Título:In vitro generated Rh(null) red cells recapitulate the in vivo deficiency: a model for rare blood group phenotypes and erythroid membrane disorders.
[So] Source:Am J Hematol;88(5):343-9, 2013 May.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen expression, membrane deformability, and gas transport function, providing the proof of principle for their use as model of Rh(null) syndrome and to investigate Rh complex biogenesis in human primary erythroid cells. Using this model, we were able to reveal for the first time that RhAG extinction alone is sufficient to explain ICAM-4 and CD47 loss observed on native Rh(null) RBCs. Together with the effects of RhAG forced expression in Rh(null) progenitors, this strongly strengthens the hypothesis that RhAG is critical to Rh complex formation. The strategy is also promising for diagnosis purpose in order to overcome the supply from rare blood donors and is applicable to other erythroid defects and rare phenotypes, providing models to dissect membrane biogenesis of multicomplex proteins in erythroid cells, with potential clinical applications in transfusion medicine.
[Mh] Termos MeSH primário: Proteínas Sanguíneas/metabolismo
Antígeno CD47/metabolismo
Moléculas de Adesão Celular/metabolismo
Células Eritroides/metabolismo
Doenças Genéticas Inatas/metabolismo
Glicoproteínas de Membrana/metabolismo
Sistema do Grupo Sanguíneo Rh-Hr/metabolismo
[Mh] Termos MeSH secundário: Células-Tronco Adultas/citologia
Células-Tronco Adultas/metabolismo
Anemia Hemolítica Congênita/metabolismo
Anemia Hemolítica Congênita/patologia
Anemia Hipoplástica Congênita/metabolismo
Anemia Hipoplástica Congênita/patologia
Proteínas Sanguíneas/antagonistas & inibidores
Proteínas Sanguíneas/genética
Diferenciação Celular
Linhagem Celular
Células Cultivadas
Células Eritroides/patologia
Células Precursoras Eritroides/citologia
Células Precursoras Eritroides/metabolismo
Feminino
Sangue Fetal
Células-Tronco Fetais/citologia
Células-Tronco Fetais/metabolismo
Doenças Genéticas Inatas/sangue
Doenças Genéticas Inatas/patologia
Seres Humanos
Glicoproteínas de Membrana/antagonistas & inibidores
Glicoproteínas de Membrana/genética
Porfiria Eritropoética/metabolismo
Porfiria Eritropoética/patologia
Gravidez
Interferência de RNA
RNA Interferente Pequeno
Reticulócitos/metabolismo
Reticulócitos/patologia
Sistema do Grupo Sanguíneo Rh-Hr/sangue
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Blood Proteins); 0 (CD47 Antigen); 0 (CD47 protein, human); 0 (Cell Adhesion Molecules); 0 (ICAM4 protein, human); 0 (Membrane Glycoproteins); 0 (RHAG protein, human); 0 (RNA, Small Interfering); 0 (Rh-Hr Blood-Group System); 0 (Rho(D) antigen)
[Em] Mês de entrada:1306
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:130219
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.23414


  2 / 6 MEDLINE  
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[PMID]:21671367
[Au] Autor:Rochowski A; Sun C; Glogauer M; Alter BP
[Ad] Endereço:Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland 20852-7231, USA.
[Ti] Título:Neutrophil functions in patients with inherited bone marrow failure syndromes.
[So] Source:Pediatr Blood Cancer;57(2):306-9, 2011 Aug.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The inherited bone marrow failure syndromes (IBMFS) include Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome (SDS). Previous studies reported decreased neutrophil chemotaxis in patients with SDS; there are no reports of neutrophil function in other IBMFS. In this study we examined neutrophil respiratory burst function in IBMFS patients. PROCEDURE: Samples from 43 IBMFS patients and 61 healthy family members were collected, shipped, and analyzed within 24 hr. We also studied samples from 12 healthy control persons immediately after collection. Neutrophils were stimulated with phorbol 12-myristate acetate (PMA) and N-formyl-methyonyl-leucyl-phenylalanine (fMLP), and respiratory burst analyzed by reduction of dihydro-rhodamine and cytochrome c. RESULTS: There was no significant difference in the degree of fMLP or PMA-driven respiratory burst activity between each of the IBMFS subgroups and their respective family members. There was also no difference in respiratory burst activity between any IBMFS, pooled group of all healthy family members and healthy controls. CONCLUSIONS: Neutrophil respiratory burst activity from IBMFS patients does not differ from that of healthy family members and controls.
[Mh] Termos MeSH primário: Anemia Hipoplástica Congênita/imunologia
Disceratose Congênita/imunologia
Neutrófilos/metabolismo
Explosão Respiratória
[Mh] Termos MeSH secundário: Doenças da Medula Óssea/imunologia
Estudos de Casos e Controles
Insuficiência Pancreática Exócrina/imunologia
Seres Humanos
Lipomatose
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL; RESEARCH SUPPORT, N.I.H., INTRAMURAL; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Mês de entrada:1108
[Cu] Atualização por classe:161122
[Lr] Data última revisão:
161122
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:110615
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.22885


  3 / 6 MEDLINE  
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[PMID]:18084332
[Au] Autor:Gluckman E; Wagner JE
[Ad] Endereço:Hematology Department, Eurocord Hôpital, Saint Louis, Paris, France. eliane.gluckman@sls.aphp.fr
[Ti] Título:Hematopoietic stem cell transplantation in childhood inherited bone marrow failure syndrome.
[So] Source:Bone Marrow Transplant;41(2):127-32, 2008 Jan.
[Is] ISSN:0268-3369
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Aplastic anemia is a rare disease in children that is most commonly idiopathic and less often a hereditary disorder. Hereditary bone marrow failure (BMF) syndromes, however, should be considered both in children and in adults before any attempt at treatment. Precise diagnosis is important because it will modify prognostic treatment options and the results of bone marrow transplantation. In this review, we will report recent results of treatment of Fanconi anemia and other hereditary BMF syndromes.
[Mh] Termos MeSH primário: Anemia Hipoplástica Congênita/terapia
Transplante de Células-Tronco de Sangue do Cordão Umbilical
Transplante de Células-Tronco Hematopoéticas/métodos
[Mh] Termos MeSH secundário: Anemia Hipoplástica Congênita/genética
Pré-Escolar
Seres Humanos
Estimativa de Kaplan-Meier
Agonistas Mieloablativos/uso terapêutico
Relações entre Irmãos
Condicionamento Pré-Transplante/métodos
Transplante Homólogo/métodos
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Myeloablative Agonists)
[Em] Mês de entrada:0803
[Cu] Atualização por classe:101118
[Lr] Data última revisão:
101118
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:071218
[St] Status:MEDLINE


  4 / 6 MEDLINE  
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[PMID]:15782403
[Au] Autor:Steele M; Hitzler J; Doyle JJ; Germeshausen M; Fernandez CV; Yuille K; Dror Y
[Ad] Endereço:Marrow Failure and Myelodysplasia Programme, and Blood and Marrow Transplantation Section, Division of Haematology and Oncology, Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
[Ti] Título:Reduced intensity hematopoietic stem-cell transplantation across human leukocyte antigen barriers in a patient with congenital amegakaryocytic thrombocytopenia and monosomy 7.
[So] Source:Pediatr Blood Cancer;45(2):212-6, 2005 Aug.
[Is] ISSN:1545-5009
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare inherited bone marrow failure syndrome that has the potential to progress to pancytopenia and acute myeloid leukemia. Hematopoietic stem-cell transplantation (HSCT) is presently the only curative treatment approach. We used a reduced intensity transplantation regimen in a CAMT patient with aplastic anemia and monosomy 7 who had no matched related donor. The patient had rapid and durable engraftment with minimal complications and is well 24 months post-transplantation. Thus, reduced intensity conditioning might be a feasible approach to stem-cell transplantation in patients with CAMT who do not have a related donor and who are at increased risk of toxicity from standard conditioning regimens.
[Mh] Termos MeSH primário: Anemia Hipoplástica Congênita/terapia
Transplante de Células-Tronco Hematopoéticas/métodos
Trombocitopenia/congênito
Trombocitopenia/terapia
Condicionamento Pré-Transplante/métodos
[Mh] Termos MeSH secundário: Criança
Cromossomos Humanos Par 7
Feminino
Seres Humanos
Megacariócitos
Monossomia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:0509
[Cu] Atualização por classe:090112
[Lr] Data última revisão:
090112
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:050323
[St] Status:MEDLINE


  5 / 6 MEDLINE  
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[PMID]:15525619
[Au] Autor:Wong AY; Chan RS; Irwin MG
[Ad] Endereço:Department of Anaesthesiology F2, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, China. wongyca@so-net.com.hk
[Ti] Título:Anesthetic management of Cesarean delivery in a patient with hypoplastic anemia and severe pre-eclampsia.
[So] Source:Can J Anaesth;51(9):923-7, 2004 Nov.
[Is] ISSN:0832-610X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To describe the anesthetic management of Cesarean delivery in a patient with hypoplastic anemia and severe pre-eclampsia. CLINICAL FEATURES: A 28-yr-old parturient with a history of thrombocytopenia was admitted with signs of pre-eclampsia (blood pressure of 140/90 mmHg, heavy proteinuria and moderate bilateral ankle edema) at 25 weeks of gestation. Laboratory studies revealed pancy-topenia (hemoglobin 6.4 g.dL(-1), white cell count 3.43 x 10(9).L(-1), platelet count 20 x 10(9).L(-1)) and bone marrow biopsy showed hypoplastic anemia. As pre-eclampsia worsened, a Cesarean delivery was performed at 27 weeks with prophylactic platelet transfusion and meticulous blood pressure control. The procedure was uneventful, conducted under general anesthesia with an estimated blood loss of around 600 mL and a live female baby was delivered. Postoperatively her blood pressure and neurological symptoms improved but thrombocytopenia remained at discharge. CONCLUSIONS: Hypoplastic anemia is rare in pregnancy but it poses an increased risk for both mother and fetus. The mother is at risk of life-threatening episodes of bleeding and infection and a multidisciplinary team approach (obstetrician, anesthesiologist, hematologist and pediatrician) is essential. An accurate assessment of the hematological condition should be made and abnormalities corrected before surgery. Regional anesthesia may not be possible in this circumstance.
[Mh] Termos MeSH primário: Anemia Hipoplástica Congênita/complicações
Anestesia Geral
Anestesia Obstétrica
Cesárea
Pré-Eclâmpsia/complicações
Complicações Hematológicas na Gravidez
[Mh] Termos MeSH secundário: Adulto
Anemia Hipoplástica Congênita/terapia
Pressão Sanguínea/efeitos dos fármacos
Feminino
Seres Humanos
Nifedipino/uso terapêutico
Transfusão de Plaquetas
Pré-Eclâmpsia/tratamento farmacológico
Gravidez
Complicações Hematológicas na Gravidez/terapia
Vasodilatadores/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Vasodilator Agents); I9ZF7L6G2L (Nifedipine)
[Em] Mês de entrada:0503
[Cu] Atualização por classe:131121
[Lr] Data última revisão:
131121
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:041105
[St] Status:MEDLINE


  6 / 6 MEDLINE  
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[PMID]:15275990
[Au] Autor:Bizzarro MJ; Colson E; Ehrenkranz RA
[Ad] Endereço:Department of Pediatrics, Yale University School of Medicine, 333 Cedar Street, P.O. Box 208064, New Haven, CT 06520-8064, USA.
[Ti] Título:Differential diagnosis and management of anemia in the newborn.
[So] Source:Pediatr Clin North Am;51(4):1087-107, xi, 2004 Aug.
[Is] ISSN:0031-3955
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Neonatal anemia is a condition with a diverse etiologic spectrum.Therefore, in order to form a focused differential diagnosis, it is important for the caregiver to have some knowledge of the more common causes of low hemoglobin and hematocrit concentrations in the neonate. Proper history taking, physical examination, and interpretation of diagnostic tests can narrow this focus and aid in establishing an accurate diagnosis and in directing the appropriate therapeutic interventions.
[Mh] Termos MeSH primário: Anemia/diagnóstico
[Mh] Termos MeSH secundário: Algoritmos
Anemia/epidemiologia
Anemia/etiologia
Anemia/terapia
Anemia Hemolítica Congênita/diagnóstico
Anemia Hemolítica Congênita/epidemiologia
Anemia Hemolítica Congênita/terapia
Anemia Hipoplástica Congênita/diagnóstico
Anemia Hipoplástica Congênita/epidemiologia
Anemia Hipoplástica Congênita/terapia
Diagnóstico Diferencial
Transfusão de Eritrócitos
Seres Humanos
Recém-Nascido
Ferro/uso terapêutico
Valores de Referência
Estados Unidos/epidemiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
E1UOL152H7 (Iron)
[Em] Mês de entrada:0409
[Cu] Atualização por classe:131121
[Lr] Data última revisão:
131121
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:040728
[St] Status:MEDLINE



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