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1.
Br J Haematol ; 194(3): 537-541, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33991422

RESUMO

Infections are one of the well-known precipitating factors for relapses in patients with immune thrombocytopenia (ITP). Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection can sometimes lead to or be associated with thrombocytopenia due to an increase in peripheral platelet destruction from inflammatory hyperactivation. Currently, we do not know if SARS-CoV-2 infection modifies the natural evolution of chronic or persistent ITP or if previous immunosuppression of patients with ITP influences the incidence and severity of coronavirus disease 2019 (COVID-19) in this group. The present study was an observational, multicentre, national series of 32 adult patients with pre-existing ITP and subsequent SARS-CoV-2 infection, collected by the Spanish ITP Group [Grupo Español de Trombocitopenia Inmune (GEPTI)].


Assuntos
COVID-19/epidemiologia , Púrpura Trombocitopênica Idiopática/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/diagnóstico , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , SARS-CoV-2/isolamento & purificação , Espanha/epidemiologia
2.
Blood Cells Mol Dis ; 86: 102505, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32979651

RESUMO

BACKGROUND: Ten years after their availability, thrombopoietin receptor agonists (TPO-RA) have heralded a paradigm shift in the treatment of immune thrombocytopenia (ITP). This study was aimed to analyze the implementation of current recommendations in the standard practice of adult ITP patients, and how age may influence those changes. METHODS: We included 121 adult patients (> 65 years, n = 54; younger individuals, n = 67) who initiated treatment with TPO-RA between January 2012 and December 2014. RESULTS: Patients older than 65 years treated with TPO-RA presented at diagnosis with significantly higher platelet counts, less bleeding, and a more prothrombotic profile than younger ones. The high efficacy rates of TPO-RA, preferentially used during the last decade in non-chronic phases, precluded from further therapies in the majority of ITP patients. Their administration was associated with a sharp decline in the last decade in the use of splenectomy and intravenous immunoglobulin, especially in younger ITP individuals. CONCLUSION: These results confirm (1) that there is a preferential use of TPO-RAs in elderly ITP patients with fewer bleeding complications but more unfavorable prothrombotic conditions than in younger individuals, and (2) that early use of these agents has been established as an effective therapeutic alternative to other second line therapies.


Assuntos
Púrpura Trombocitopênica Idiopática/terapia , Receptores de Trombopoetina/agonistas , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Gerenciamento Clínico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
3.
Int J Mol Sci ; 22(13)2021 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-34199099

RESUMO

Eltrombopag is a thrombopoietin receptor (MPL) agonist approved for the treatment of primary immune thrombocytopenia (ITP). Recent evidence shows that some patients may sustain platelet counts following eltrombopag discontinuation. The systemic immunomodulatory response that resolves ITP in some patients could result from an increase in platelet mass, caused either by the direct action of eltrombopag on megakaryocytes through MPL stimulation, or potential MPL-independent actions on other cell types. To uncover the possible mechanisms of action of eltrombopag, in silico analyses were performed, including a systems biology-based approach, a therapeutic performance mapping system, and structural analyses. Through manual curation of the available bibliography, 56 key proteins were identified and integrated into the ITP interactome analysis. Mathematical models (94.92% mean accuracy) were obtained to elucidate potential MPL-dependent pathways in non-megakaryocytic cell subtypes. In addition to the effects on megakaryocytes and platelet numbers, the results were consistent with MPL-mediated effects on other cells, which could involve interferon-gamma, transforming growth factor-beta, peroxisome proliferator-activated receptor-gamma, and forkhead box protein P3 pathways. Structural analyses indicated that effects on three apoptosis-related proteins (BCL2L1, BCL2, BAX) from the Bcl-2 family may be off-target effects of eltrombopag. In conclusion, this study proposes new hypotheses regarding the immunomodulatory functions of eltrombopag in patients with ITP.


Assuntos
Benzoatos/farmacologia , Hidrazinas/farmacologia , Imunomodulação/efeitos dos fármacos , Púrpura Trombocitopênica Idiopática/etiologia , Púrpura Trombocitopênica Idiopática/metabolismo , Pirazóis/farmacologia , Receptores de Trombopoetina/antagonistas & inibidores , Benzoatos/química , Benzoatos/uso terapêutico , Biomarcadores , Gerenciamento Clínico , Suscetibilidade a Doenças , Humanos , Hidrazinas/química , Hidrazinas/uso terapêutico , Modelos Biológicos , Modelos Moleculares , Terapia de Alvo Molecular/métodos , Mapeamento de Interação de Proteínas , Mapas de Interação de Proteínas , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Pirazóis/química , Pirazóis/uso terapêutico , Receptores de Trombopoetina/química , Receptores de Trombopoetina/metabolismo , Transdução de Sinais/efeitos dos fármacos , Relação Estrutura-Atividade , Linfócitos T/efeitos dos fármacos , Linfócitos T/imunologia , Linfócitos T/metabolismo , Resultado do Tratamento
4.
J Vasc Bras ; 20: e20200024, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34925471

RESUMO

BACKGROUND: Open surgical repair (OSR) and endovascular aneurysm repair (EVAR) surgery are alternative treatments for infrarenal abdominal aortic aneurysm (IRAAA). OBJECTIVES: To compare OSR and EVAR for the treatment of IRAAA. METHODS: 119 patients with IRAAA were electively operated by the same surgeon between January 1, 2006 and December 31, 2015, following selection for OSR or EVAR according to surgical risk. Complications, reinterventions, failures, and early and late mortality were analyzed. RESULTS: 63 OSR and 56 EVAR patients were analyzed. They were similar in terms of age (70 years), gender (92% men), and average diameter of IRAAA (6.5 cm), but with different comorbidities, surgical risk, and anatomy. EVAR was better than OSR regarding time in the operating theatre (177.5 vs. 233.3 minutes), need for transfusion (25 vs. 73%), and length of stay in ICU (1.3 vs. 3.3 days) and hospital (8.1 vs. 11.1 days). OSR allowed more associated procedures to be conducted simultaneously (19.0 vs. 1.8%). There were no significant differences between the groups with respect to complications (25.4 vs. 25.1%), reinterventions (3.2 vs. 5.2%), or early mortality (1.6 vs. 0%). During follow-up, OSR was associated with fewer revisions (3.13 vs. 4.21), angio-CTs (0.22 vs. 3.23), complications (6.4 vs. 37.5%), reinterventions (3.2 vs. 23.2%), and failures (1.6 vs. 10.7%), and had better survival (78.2 vs. 63.2%). CONCLUSIONS: Correct selection of patients achieves excellent results because it avoids OSR in patients at high risk and avoids EVAR in patients with high anatomical complexity, achieving similar results in the perioperative period, but better results for OSR over the course of follow-up.


CONTEXTO: A cirurgia aberta (CA) e o reparo endovascular de aneurisma (REVA) são tratamentos alternativos para o aneurisma da aorta abdominal infrarrenal (AAAIR). OBJETIVOS: Comparar CA e REVA no tratamento do AAAIR. MÉTODOS: Foram incluídos 119 pacientes com AAAIR, operados eletivamente pelo mesmo cirurgião entre 1 de janeiro de 2006 e 31 de dezembro de 2015, após seleção para CA ou REVA de acordo com o risco cirúrgico. Complicações, reintervenções, falhas e mortalidade precoce e tardia foram analisadas. RESULTADOS: Foram analisados 63 pacientes de CA e 56 de REVA, com semelhanças de idade (70 anos), sexo (92% homens) e diâmetro médio do AAAIR (6,5 cm), mas com diferentes comorbidades, riscos cirúrgicos e anatomias. O REVA foi melhor que a CA em relação ao tempo na sala de cirurgia (177,5 vs. 233,3 minutos), necessidade de transfusão (25 vs. 73%) e tempo de permanência na unidade de terapia intensiva (1,3 vs. 3,3 dias) e no hospital (8,1 vs. 11,1 dias). A CA permitiu que mais procedimentos associados fossem realizados simultaneamente (19,0 vs. 1,8%). Não houve diferenças significativas entre os grupos em relação a complicações (25,4 vs. 25,1%), reintervenções (3,2 vs. 5,2%) e mortalidade precoce (1,6 vs. 0%). Durante o acompanhamento, a CA apresentou menos revisões (3,13 vs. 4,21), angiotomografias (0,22 vs. 3,23), complicações (6,4 vs. 37,5%), reintervenções (3,2 vs. 23,2%) e falhas (1,6 vs. 10,7%), além de ter melhor sobrevida (78,2 vs. 63,2%). CONCLUSÕES: A seleção correta dos pacientes proporciona excelentes resultados porque evita pacientes com alto risco para CA e com complexidade anatômica para REVA. Os resultados são semelhantes no período perioperatório, mas melhores para CA durante o acompanhamento.

5.
Haematologica ; 103(1): 148-162, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28983057

RESUMO

Inherited platelet disorders are a heterogeneous group of rare diseases, caused by inherited defects in platelet production and/or function. Their genetic diagnosis would benefit clinical care, prognosis and preventative treatments. Until recently, this diagnosis has usually been performed via Sanger sequencing of a limited number of candidate genes. High-throughput sequencing is revolutionizing the genetic diagnosis of diseases, including bleeding disorders. We have designed a novel high-throughput sequencing platform to investigate the unknown molecular pathology in a cohort of 82 patients with inherited platelet disorders. Thirty-four (41.5%) patients presented with a phenotype strongly indicative of a particular type of platelet disorder. The other patients had clinical bleeding indicative of platelet dysfunction, but with no identifiable features. The high-throughput sequencing test enabled a molecular diagnosis in 70% of these patients. This sensitivity increased to 90% among patients suspected of having a defined platelet disorder. We found 57 different candidate variants in 28 genes, of which 70% had not previously been described. Following consensus guidelines, we qualified 68.4% and 26.3% of the candidate variants as being pathogenic and likely pathogenic, respectively. In addition to establishing definitive diagnoses of well-known inherited platelet disorders, high-throughput sequencing also identified rarer disorders such as sitosterolemia, filamin and actinin deficiencies, and G protein-coupled receptor defects. This included disease-causing variants in DIAPH1 (n=2) and RASGRP2 (n=3). Our study reinforces the feasibility of introducing high-throughput sequencing technology into the mainstream laboratory for the genetic diagnostic practice in inherited platelet disorders.


Assuntos
Transtornos Plaquetários/diagnóstico , Transtornos Plaquetários/genética , Doenças Genéticas Inatas/diagnóstico , Doenças Genéticas Inatas/genética , Testes Genéticos , Sequenciamento de Nucleotídeos em Larga Escala , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Plaquetas/metabolismo , Criança , Pré-Escolar , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Testes Genéticos/métodos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Fenótipo , Reprodutibilidade dos Testes , Análise de Sequência de DNA , Adulto Jovem
6.
Br J Haematol ; 178(6): 959-970, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28573819

RESUMO

Eltrombopag is a second-line treatment in primary immune thrombocytopenia (ITP). However, its role in secondary ITP is unknown. We evaluated the efficacy and safety of eltrombopag in secondary ITP in daily clinical practice. Eighty-seven secondary ITP patients (46 with ITP secondary to autoimmune syndromes, 23 with ITP secondary to a neoplastic disease subtype: lymphoproliferative disorders [LPDs] and 18 with ITP secondary to viral infections) who had been treated with eltrombopag were retrospectively evaluated. Forty-four patients (38%) had a platelet response, including 40 (35%) with complete responses. Median time to platelet response was 15 days (95% confidence interval, 7-28 days), and was longer in the LPD-ITP group. Platelet response rate was significantly lower in the LPD-ITP than in other groups. However, having achieved response, there were no significant differences between the durable response of the groups. Forty-three patients (49·4%) experienced adverse events (mainly grade 1-2), the commonest being hepatobiliary laboratory abnormalities. There were 10 deaths in this case series, all of which were related to pre-existing medical conditions. In routine clinical practice, eltrombopag is effective and well-tolerated in unselected patients with ITP secondary to both immune and infectious disorders. However, the response rate in LPD-ITP is low.


Assuntos
Benzoatos/uso terapêutico , Hidrazinas/uso terapêutico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Pirazóis/uso terapêutico , Adulto , Idoso , Doenças Autoimunes/complicações , Benzoatos/administração & dosagem , Benzoatos/efeitos adversos , Esquema de Medicação , Feminino , Humanos , Hidrazinas/administração & dosagem , Hidrazinas/efeitos adversos , Transtornos Linfoproliferativos/complicações , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/etiologia , Pirazóis/administração & dosagem , Pirazóis/efeitos adversos , Receptores de Trombopoetina/agonistas , Estudos Retrospectivos , Viroses/complicações
7.
Eur J Haematol ; 97(2): 128-36, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26505263

RESUMO

OBJECTIVE: The aim of this study was to assess differences in the gene expression profile of peripheral blood cells between patients with early recurrent thrombosis vs. patients without recurrent events after withdrawal of anticoagulant therapy for a first episode of unprovoked deep vein thrombosis (uDVT), to identify novel predictors of recurrence. METHODS: In the discovery population (N = 32), a microarray RNA assay followed by RT-PCR confirmation were performed. In the validation population (N = 44) a multiple RT-PCR-based strategy was applied to assess genes differentially expressed in the discovery population. RESULTS: The sex-adjusted Linear Model for Microarray Data analysis showed 102 genes differentially expressed (P < 0.01) in the discovery population. Nineteen of them underwent further confirmation in the validation population. The gene encoding for Acyl-CoA Synthetase Family Member 2 (ACSF2) was underexpressed in recurrent DVT patients in both, the discovery (P = 0.007) and validation populations (P = 0.004). In the receiver operator characteristic (ROC) analysis, the areas under the curve of ACSF2 expression were 0.77 and 0.80, respectively. CONCLUSIONS: For the first time an association between ACSF2 expression and the risk of recurrent DVT is suggested. Should this association be confirmed in larger prospective studies, ACSF2 could become useful for the selection of patients requiring extended anticoagulant therapy.


Assuntos
Perfilação da Expressão Gênica , Transcriptoma , Trombose Venosa/genética , Trombose Venosa/patologia , Adulto , Biomarcadores , Análise por Conglomerados , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos Retrospectivos , Fatores de Risco , Sensibilidade e Especificidade , Trombose Venosa/diagnóstico
9.
Pain Pract ; 16(7): 842-9, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26407542

RESUMO

BACKGROUND: Pain control in critical limb ischemia (CLI) varies considerably between individuals. OBJECTIVE: To evaluate pharmacogenetically the response to transdermal buprenorphine (BUP-TTS) in patients with CLI who are awaiting revascularization. METHODS: One hundred and seven patients with CLI were treated with BUP-TTS. The following were analyzed: (1) pain perception (visual analog scale (VAS) before and 4 days after treatment) and (2) genetics: glucuronosyltransferase (UGT2B7), cytochrome (CYP3A4), and µ-opioid receptor (OPRM1) gene polymorphisms. RESULTS: Ninety-three patients completed the study. The VAS score by the fourth day of analgesia dropped from 6.82 to 3.38 (P < 0.05). The analgesic response to BUP-TTS was greater in men than in women (P = 0.019). Patients who were AA homozygotes for the CYP3A4 gene showed the best response to analgesic treatment (P = 0.003). The combination of the CYP3A4 gene with UGT2B7 or OPRM1 was favorable to the effect of the CYP3A4 gene (P = 0.045 and P = 0.026, respectively). The combination of UGT2B7 with OPRM1 was ineffective (P = 0.648). The 3 polymorphisms together had no effect on response to treatment (P = 0.461). CONCLUSIONS: BUP-TTS is efficacious in the control of pain in patients with CLI. The homozygous AA carriers of the CYP3A4 gene respond better to treatment with BUP-TTS.


Assuntos
Analgésicos Opioides/administração & dosagem , Buprenorfina/administração & dosagem , Citocromo P-450 CYP3A/genética , Glucuronosiltransferase/genética , Dor/genética , Receptores Opioides mu/genética , Administração Cutânea , Adulto , Feminino , Humanos , Isquemia/complicações , Extremidade Inferior/irrigação sanguínea , Masculino , Pessoa de Meia-Idade , Dor/tratamento farmacológico , Dor/etiologia , Manejo da Dor/métodos , Medição da Dor , Polimorfismo de Nucleotídeo Único
10.
Br J Haematol ; 169(5): 719-25, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25817436

RESUMO

Bleeding is a frequent complication after allogeneic haematopoietic stem cell transplantation (HSCT) and may affect survival. The purpose of this study was to determine the incidence and risk factors for life-threatening bleeding after HSCT by retrospective evaluation of 491 allogeneic HSCT recipients. With a median follow-up of 33 months, 126 out of 491 allogeneic HSCT recipients experienced a haemorrhagic event (25·7%) and 46 patients developed a life-threatening bleeding episode (9·4%). Pulmonary and gastrointestinal bleeding were the most common sites for life-threatening bleeding, followed by central nervous system. In multivariate analyses, the presence of severe thrombocytopenia after day +28 and the development of grade III-IV acute graft-versus-host disease (GVHD) or thrombotic microangiopathy (TMA) retained their association with life-threatening bleeding events. The overall survival at 3 years among patients without bleeding was 67·1% for only 17·1% for patients with life-threatening bleeding (P < 0·001). In conclusion, life-threatening bleeding is a common complication after allogeneic HSCT. Prolonged severe thrombocytopenia, acute grade III-IV GVHD and TMA were associated with its development.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Hemorragia/epidemiologia , Hemorragia/etiologia , Adulto , Idoso , Feminino , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hemorragia/mortalidade , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados da Assistência ao Paciente , Fatores de Risco , Condicionamento Pré-Transplante/efeitos adversos , Transplante Homólogo
11.
World J Surg ; 38(1): 241-51, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24170151

RESUMO

BACKGROUND: The goal of this article is to present for the first time to the international community the detailed findings and outcomes of the Spanish Vascular Registry (SVR) after 16 years of experience. METHODS: We examined the nationwide registry promoted by the Spanish Society of Angiology and Vascular Surgery (1996-2011). The changes in vascular surgical activity in Spain during the period of study were examined. We evaluated the number of services, medical specialists, consultations, admissions, and operations that occurred in Spain. We also assessed the trends in therapeutic activity and the medical and social impact of vascular pathology. RESULTS: A mean of 60 centers (range = 32-83) participated in the SVR (79.3 % of the total). In the last year of the study period, 94.3 % centers (100 % of teaching centers) participated. The mean number of activities per hospital per year was 5,298 consultations, 2,625 vascular explorations, 630 hospital admissions (61 % elective and 31 % emergency), and 742 surgical procedures. A total of 29,289 carotid stenosis procedures had been registered over 16 years. Both carotid endarterectomy (CEA) and carotid artery stenting (CAS) procedures have increased in frequency over time. In 2011, CAS constituted 19.3 % of all carotid procedures. A total of 31,703 abdominal aortic aneurysm (AAA) operations were registered during the study period. Surgery for ruptured AAA remained stable over time. Since its appearance in the year 2000, endovascular treatment (EVAR) increased steadily over time. Currently, EVAR represents about half of all AAA surgery (50.2 %). The total rate of in-hospital operative deaths was 1.1 %, but in-hospital mortality for open arterial surgery was 4 %. Mortality has decreased of late. CONCLUSIONS: The SVR has enabled us to understand the development and implementation of vascular surgery throughout Spain and to note the increased healthcare activity and the better overall results obtained as a consequence.


Assuntos
Sistema de Registros , Procedimentos Cirúrgicos Vasculares/estatística & dados numéricos , Procedimentos Cirúrgicos Vasculares/tendências , Humanos , Espanha , Fatores de Tempo
12.
Biochim Biophys Acta ; 1823(8): 1366-77, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22659131

RESUMO

We have generated mouse transgenic lineages for C3G (tgC3G) and C3GΔCat (tgC3GΔCat, C3G mutant lacking the GEF domain), where the transgenes are expressed under the control of the megakaryocyte and platelet specific PF4 (platelet factor 4) gene promoter. Transgenic platelet activity has been analyzed through in vivo and in vitro approaches, including bleeding time, aggregation assays and flow cytometry. Both transgenes are expressed (RNA and protein) in purified platelets and megakaryocytes and do not modify the number of platelets in peripheral blood. Transgenic C3G animals showed bleeding times significantly shorter than control animals, while tgC3GΔCat mice presented a remarkable bleeding diathesis as compared to their control siblings. Accordingly, platelets from tgC3G mice showed stronger activation in response to platelet agonists such as thrombin, PMA, ADP or collagen than control platelets, while those from tgC3GΔCat animals had a lower response. In addition, we present data indicating that C3G is a mediator in the PKC pathway leading to Rap1 activation. Remarkably, a significant percentage of tgC3G mice presented a higher level of neutrophils than their control siblings. These results indicate that C3G plays an important role in platelet clotting through a mechanism involving its GEF activity and suggest that it might be also involved in neutrophil development.


Assuntos
Plaquetas/metabolismo , Fator 2 de Liberação do Nucleotídeo Guanina/genética , Ativação Plaquetária , Animais , Plaquetas/efeitos dos fármacos , Plaquetas/fisiologia , Células Cultivadas , Ativação Enzimática , Feminino , Engenharia Genética , Fator 2 de Liberação do Nucleotídeo Guanina/biossíntese , Humanos , Contagem de Leucócitos , Masculino , Megacariócitos/metabolismo , Megacariócitos/fisiologia , Camundongos , Camundongos Transgênicos , Neutrófilos/fisiologia , Contagem de Plaquetas , Fator Plaquetário 4/genética , Regiões Promotoras Genéticas , Proteína Quinase C/metabolismo , Transdução de Sinais , Acetato de Tetradecanoilforbol/farmacologia , Proteínas rap1 de Ligação ao GTP/metabolismo
13.
Eur J Haematol ; 91(3): 236-241, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23679653

RESUMO

AIM: Few studies specifically focus on elderly splenectomized immune thrombocytopenia (ITP) patients. Older patients with ITP and excellent health are often excluded from surgery splenectomy. We aimed to compare the safety and efficacy of splenectomy in elderly and non-elderly ITP patients and to examine the effect of age on therapeutic response. MATERIAL AND METHODS: We carried out a retrospective analysis of a series of 218 patients who had undergone splenectomy for ITP. We compared the data from the elderly group (≥65 yrs, 57 patients) with the young group (<65 yrs, 162 patients). RESULTS: Surgical technique (laparoscopy or open laparotomy splenectomy) was comparable between the two age groups. The adjusted risk of major bleeding following splenectomy for elderly patients was three times that for young patients (OR 3.05, 95% CI: 1.44-6.52). The median duration of postoperative hospital stay was longer for elderly than for young patients (8 d vs. 4 d, P < 0.001). However, we identified a subgroup of elderly ITP patients, those aged between 65 and 70 yrs who had undergone laparoscopic splenectomy, with a low risk of postoperative complications. Of the 218 patients, 89% achieved a favorable response to splenectomy. A favorable response was significantly less common in elderly than in young people (79% vs. 92%, P = 0.005). However, we observed an acceptable long-term control of ITP in the elderly group, in which the probability of maintaining response for 14 yrs after splenectomy was 56%. CONCLUSIONS: Patients aged ≥65 yrs experienced negative effects on safety and efficacy outcomes of splenectomy for ITP, but further studies are needed to identify predictors of postsplenectomy outcomes in this group.


Assuntos
Púrpura Trombocitopênica Idiopática/cirurgia , Esplenectomia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/mortalidade , Estudos Retrospectivos , Fatores de Risco , Esplenectomia/efeitos adversos , Resultado do Tratamento
14.
Vasa ; 42(5): 350-6, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23989070

RESUMO

BACKGROUND: The Walking Impairment Questionnaire (WIQ) is a short, easy to complete, disease-specific questionnaire to assess intermittent claudication. A Spanish version of the WIQ for Hispanic Americans has recently been validated in Texas, but it needs to be validated for European Spanish people. PATIENTS AND METHODS: After translation and cultural adaptation of the WIQ, 920 patients with intermittent claudication (ankle brachial index < 0.9) completed two questionnaires (Spanish version of the WIQ and European Quality of Life 5 Dimension [EQ-5D]). The validity of the WIQ was determined by correlating WIQ and EQ-5D. Test-retest reliability and internal consistency were determined using the intra-class correlation coefficient (ICC) and Cronbach's alpha, respectively. RESULTS: The three domains of the WIQ were moderately correlated with the EQ-5D health outcome (r = 0.54 to 0.60; p < 0.001). Test-retest reliabilities ranged from ICC = 0.89 to 0.91 and internal consistency (Cronbach's alpha = 0.92) was high. CONCLUSIONS: The Spanish version of the WIQ for European Spanish patients was valid and reproducible, suggesting that it could be used in Spanish patients with intermittent claudication.


Assuntos
Avaliação da Deficiência , Claudicação Intermitente/diagnóstico , Doença Arterial Periférica/diagnóstico , Inquéritos e Questionários , Caminhada , Idoso , Índice Tornozelo-Braço , Características Culturais , Feminino , Humanos , Claudicação Intermitente/epidemiologia , Claudicação Intermitente/fisiopatologia , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/epidemiologia , Doença Arterial Periférica/fisiopatologia , Valor Preditivo dos Testes , Psicometria , Reprodutibilidade dos Testes , Espanha/epidemiologia , Tradução
15.
Cancer ; 118(1): 127-33, 2012 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-21717439

RESUMO

BACKGROUND: Abnormalities involving chromosome 7 are frequent in myelodysplastic syndrome (MDS) and suggest a poor prognosis. METHODS: The authors examined the hypothesis that the clinical features and survival associated with isolated deletion (del) of part of the long arm of chromosome 7 (7q) in MDS are different from those associated with isolated monosomy 7 (complete loss of chromosome 7). In total, 133 patients with a diagnosis of de novo MDS (according to the World Health Organization [WHO] classification) and chromosome 7 abnormalities in the Spanish MDS Registry were evaluated retrospectively. Four karyotypic groups were identified: isolated del(7q) (n = 29), isolated monosomy 7 (n = 27), del(7q) with additional abnormalities (n = 24), and monosomy 7 with additional abnormalities (n = 53). RESULTS: Isolated del(7q) was more frequent in patients with less advanced MDS according to the WHO classification or the International Prognostic Scoring System. In addition, isolated del(7q) was associated with fewer blasts in bone marrow than other cytogenetics groups. Survival was significantly superior in patients with isolated del(7) than in those with isolated monosomy 7, del(7q) with additional abnormalities, or monosomy 7 with additional abnormalities. On multivariate analysis, age, the percentage of blasts in bone marrow, and other chromosome 7 abnormalities apart from isolated del(7q) were identified as independent risk factors for survival. CONCLUSIONS: The current results demonstrated that patients who had MDS with isolated del(7q) had some distinct clinical-pathologic characteristics as well as better survival than patients who had MDS with isolated monosomy 7.


Assuntos
Deleção Cromossômica , Cromossomos Humanos Par 7 , Síndromes Mielodisplásicas/genética , Deleção de Sequência , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/mortalidade , Prognóstico
16.
Med Clin (Barc) ; 158(11): 503-508, 2022 06 10.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34399987

RESUMO

PURPOSE: To assess whether subjects with Philadelphia negative myeloproliferative neoplasms (Ph-MPNs) show differences in the presence of vascular, cardiac or renal target organ damage (TOD) and other vascular function parameters as compared to individuals without this condition. METHODS: An observational study was conducted. Fifty-seven subjects diagnosed with Ph-MPNs used as cases and 114 subjects without Ph-MPNs as controls. We matched the subjects with and without Ph-MPNs using the propensity scores in a 1:2 ratio using the variables gender, type 2 diabetes mellitus, high blood pressure, hyperlipidaemia and smoking. Vascular, cardiac and renal TOD were established according to the criteria of the European Society of Hypertension and Cardiology guidelines. Arterial stiffness was also assessed using the cardio-ankle vascular index (CAVI). RESULTS: Mean age was 63.50±11.70 and 62.90±8.32 years in subjects with and without Ph-MPNs, 32 females (56%) in the first group and 62 (54%) in the second. Subjects with Ph-MPNs have a higher percentage of carotid injury than subjects without Ph-MPNs (35.1% vs. 21.1%) and higher albumin/creatinine ratio. In the logistic regression analysis, subjects with Ph-MPNs had an OR=2.382 (IC95% 1.066-5.323) for carotid injury versus those without haematological disease. CONCLUSIONS: Subjects with Ph-MPNs have twice the risk of by carotid injury than those without haematological disease.


Assuntos
Diabetes Mellitus Tipo 2 , Transtornos Mieloproliferativos , Rigidez Vascular , Idoso , Feminino , Humanos , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/complicações , Transtornos Mieloproliferativos/diagnóstico , Pontuação de Propensão
17.
J Surg Case Rep ; 2020(10): rjaa324, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33033614

RESUMO

The axillary-femoral bypass is an extra-anatomical arterial reconstruction technique whose indications and complications have been thoroughly discussed in the literature. Shortening or lengthening of the prosthesis (by axillary artery traction or graft angulation, respectively) as a late postoperative complication of the procedure has been described only exceptionally. Here we report a kinking of the prosthesis with a very illustrative figure.

18.
Head Neck ; 42(12): 3538-3550, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32812684

RESUMO

BACKGROUND: Carotid paragangliomas are rare tumors. They are usually unique, non-secreting, resectable, and benign. However, additional rare cases of complex tumors (bilateral, secretory, nonresectable, or malignant) complicate the management and final outcomes. METHODS: Records of paragangliomas from our hospital are reviewed. Criteria defining complex paragangliomas have been previously defined. These are compared with those of the simple group. RESULTS: Fifty patients, two groups: simple (n = 39) and complex (n = 11). The patients in the complex group were significantly younger (47.7 vs 63.8 years). Postoperative nerve complications (45.4% vs 6.3%) and mortality during follow-up (27.3% vs 0%) were significantly more common in the complex group. Vascular complications (0% vs 3.1%) and early mortality (0%) were similarly in both groups. CONCLUSIONS: Patients with complex carotid paragangliomas are heterogeneous. The former are younger, exhibit a high degree of diagnostic and therapeutic complexity, and have poorer morbidity and mortality. Surgical experience and interdisciplinary collaboration are essential.


Assuntos
Tumor do Corpo Carotídeo , Neoplasias de Cabeça e Pescoço , Paraganglioma Extrassuprarrenal , Paraganglioma , Tumor do Corpo Carotídeo/diagnóstico , Tumor do Corpo Carotídeo/cirurgia , Humanos , Paraganglioma/cirurgia , Complicações Pós-Operatórias
19.
Int Angiol ; 39(3): 241-251, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32057214

RESUMO

BACKGROUND: Splenic artery aneurysms are rare, potentially serious, and usually asymptomatic. Several methods are currently available to treat them, each with their own advantages and drawbacks. Therefore, its therapeutic paradigm has changed. METHODS: We review our database of splenic aneurysms (2009-2019) and undertake an exhaustive literature review. Demographic, clinical, diagnostic, therapeutic, early and follow-up outcome data were examined. Our experience comprised: 15 patients with 19 splenic aneurysms. 11 women (average age, 59.4 years) and 4 men (average age, 61.7 years). All asymptomatic. RESULTS: At diagnosis, aneurysms had a mean cross-sectional diameter of 3.4 cm (3.2 and 3.9 for women and men, respectively), the largest measuring 8.5 cm. Two independent aneurysms were detected in four patients. Diagnoses were always incidental to a CT scan. Treatments consisted of open surgery (2 patients), endovascular surgery (10 patients: 7 embolizations, 3 covered stent) and observation/follow-up (3 patients). The cases of open surgery (with splenectomy) were carried out without postoperative morbidity. One embolization failed (requiring subsequent open surgery) and two suffered localized splenic infarction, but without further complications. In patients treated with a covered stent, the aneurysm was always excluded, without complications. There was no 30-day or follow-up (average 26.2 months) mortality. Splenic aneurysms are diagnosed more frequently and earlier (in the asymptomatic phase), albeit incidentally, than in the past. CONCLUSIONS: The correct indication (identifying patients at risk) and individualization of treatment, in which endovascular techniques are the first-line option, have significantly improved morbidity and mortality outcomes in our hospital.


Assuntos
Aneurisma/terapia , Embolização Terapêutica/efeitos adversos , Procedimentos Endovasculares/métodos , Artéria Esplênica/cirurgia , Stents/efeitos adversos , Adulto , Idoso , Aneurisma/diagnóstico por imagem , Aneurisma/cirurgia , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Laparoscopia/efeitos adversos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Literatura de Revisão como Assunto , Fatores de Risco , Espanha , Esplenectomia , Artéria Esplênica/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento
20.
J Cardiovasc Surg (Torino) ; 61(2): 256-263, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31985187

RESUMO

We report on the case of spontaneous rupture of an On-X-pure pyrolytic carbon mechanical valve prosthesis implanted seven years earlier, in a mitral position, at our hospital. The patient was admitted with valvular dysfunction and acute pulmonary edema requiring emergency surgery (prosthesis replacement); the absence of a leaflet was confirmed intraoperatively. The patient presented severe respiratory failure, which prolonged the postoperative period. A CT scan showed that the migrated leaflet was located in the aortic bifurcation with no apparent arterial lesion. Four months later, once the patient had recovered, laparotomy and aortotomy were performed in order to retrieve the leaflet, which was found to have become included (neoendothelized) in the aortic wall without compromising the latter's integrity or obstructing the blood flow. A subsequent CT scan confirmed the persistence of the leaflet in its initial position. The literature review highlights two singular facts: 1) this is the second published case of the escape of a leaflet from an On-X prosthesis (the first patient died); 2) this is the first case in which a laparotomy was performed to retrieve the leaflet but finally a decision was made to leave it in situ. Seven months later, the patient remained asymptomatic.


Assuntos
Bioprótese/efeitos adversos , Migração de Corpo Estranho/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Insuficiência da Valva Mitral/cirurgia , Falha de Prótese , Doença Aguda , Procedimentos Cirúrgicos Cardíacos/métodos , Serviço Hospitalar de Emergência , Seguimentos , Migração de Corpo Estranho/diagnóstico por imagem , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/diagnóstico por imagem , Edema Pulmonar/diagnóstico , Edema Pulmonar/etiologia , Reoperação/métodos , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
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