RESUMO
Leprosy is a contagious and chronic systemic granulomatous disease caused by the bacillus Mycobacterium leprae. To our knowledge, no case of leprosy in a childhood-onset systemic lupus erythematosus (c-SLE) patient has been reported. For a period of 31 years, 312 c-SLE patients were followed at the Pediatric Rheumatology Unit of our University Hospital. One of them (0.3%) had tuberculoid leprosy skin lesions during the disease course and is here reported. A 10-year-old boy from Northwest of Brazil was diagnosed with c-SLE based on malar rash, photosensitivity, oral ulcers, lymphopenia, proteinuria, positive antinuclear antibodies, anti-double-stranded DNA, anti-Sm and anti-Ro/SSA autoantibodies. He was treated with prednisone, hydroxychloroquine and intravenous cyclophosphamide, followed by mycophenolate mofetil. At 12-years-old, he presented asymmetric skin lesions characterized by erythematous plaques with elevated external borders and hypochromic center with sensory loss. Peripheral nerve involvement was not evidenced. No history of familial cases of leprosy was reported, although the region where the patient resides is considered to be endemic for leprosy. Skin biopsy revealed a well-defined tuberculoid form. A marked thickening of nerves was observed, often destroyed by granulomas, without evidence of Mycobacterium leprae bacilli. At that time, the SLEDAI-2K score was 4 and he had been receiving prednisone 15 mg/day, hydroxychloroquine 200 mg/day and mycophenolate mofetil 3 g/day. Paucibacillary treatment for leprosy with dapsone and rifampicine was also introduced. In conclusion, we have reported a rare case of leprosy in the course of c-SLE. Leprosy should always be considered in children and adolescents with lupus who present skin abnormalities, particularly with hypoesthesic or anesthesic cutaneous lesions.
Assuntos
Hanseníase Paucibacilar/diagnóstico , Hanseníase Paucibacilar/microbiologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/microbiologia , Adolescente , Autoanticorpos/análise , Criança , Dapsona/uso terapêutico , Humanos , Hansenostáticos/uso terapêutico , Hanseníase Paucibacilar/tratamento farmacológico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Mycobacterium leprae/isolamento & purificação , Doenças Raras , Rifampina/uso terapêuticoRESUMO
Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childrens Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.
Assuntos
Vesícula/patologia , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/patologia , Vesícula/etiologia , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulinas/análise , Lúpus Eritematoso Sistêmico/complicações , Masculino , PrevalênciaRESUMO
Schistosomiasis mansoni is a non-cirrhotic liver disease. In cirrhosis patients with portal hypertension, a decreased number of reticulated platelets associated with increased thrombopoietin serum levels were reported. We previously reported a 120/nl platelet cutoff level as a marker of clinically significant portal hypertension in schistosomiasis patients. To evaluate reticulated platelet counts and thrombopoietin serum levels (TPO) in schistosomiasis patients and correlate them with portal hypertension markers. Thirty-three schistosomiasis patients without co-morbidities were endoscopically classified as those with (n = 19) or without (n = 14) clinically significant portal hypertension. Flow cytometric determination of reticulated platelets was performed using CD41 antibody and thiazol orange. Ultrasonographic examinations were performed according to the Niamey protocol. TPO and hyaluronic acid serum levels were determined in duplicate using ELISA methods. The platelet number of 120/nl discriminates the two groups with 100% accuracy and 100% positive and negative predictive values, and correlates with spleen length and portal and splenic vein diameters. Differences in reticulated platelets and hyaluronic acid serum levels between both groups were significant (P = 0.025 and 0.012, respectively), but thrombopoietin serum levels were not (P = 0.769). Schistosomiasis patients with portal hypertension have increased reticulated platelets associated with normal TPO serum levels.
Assuntos
Plaquetas/citologia , Esquistossomose/sangue , Trombopoetina/sangue , Plaquetas/metabolismo , Citometria de Fluxo , Humanos , Ácido Hialurônico/sangue , Padrões de Referência , Esquistossomose/diagnóstico por imagem , UltrassonografiaRESUMO
OBJECTIVE: Only few large families with multiple endocrine neoplasia type 1 (MEN1) have been documented. Here, we aimed to investigate the clinical features of a seven-generation Brazilian pedigree, which included 715 at-risk family members. DESIGN: Genealogical and geographic analysis was used to identify the MEN1 pedigree. Clinical and genetic approach was applied to characterize the phenotypic and genotypic features of the family members. RESULTS: Our genetic data indicated that a founding mutation in the MEN1 gene has occurred in this extended Brazilian family. Fifty family members were diagnosed with MEN1. Very high frequencies of functioning and non-functioning MEN1-related tumors were documented and the prevalence of prolactinoma (29.6%) was similar to that previously described in prolactinoma-variant Burin (32%). In addition, bone mineral density analysis revealed severe osteoporosis (T, -2.87+/-0.32) of compact bone (distal radius) in hyperparathyroidism (HPT)/MEN1 patients, while marked bone mineral loss in the lumbar spine (T, -1.95+/-0.39), with most cancellous bone, and femoral neck (mixed composition; T, -1.48+/-0.27) were also present. CONCLUSIONS: In this study, we described clinically and genetically the fifth largest MEN1 family in the literature. Our data confirm previous findings suggesting that prevalence of MEN1-related tumors in large families may differ from reports combining cumulative data of small families. Furthermore, we were able to evaluate the bone status in HPT/MEN1 cases, a subject that has been incompletely approached in the literature. We discussed the bone loss pattern found in our MEN1 patients comparing with that of patients with sporadic primary HPT.
Assuntos
Densidade Óssea , Efeito Fundador , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/fisiopatologia , Proteínas Proto-Oncogênicas/genética , Adolescente , Adulto , Densidade Óssea/genética , Brasil , Análise Mutacional de DNA , Família , Feminino , Geografia , Mutação em Linhagem Germinativa/fisiologia , Haplótipos , Humanos , Hipertireoidismo/complicações , Hipertireoidismo/genética , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , LinhagemRESUMO
Sickle cell disease (SCD) is one of the most common inherited diseases in the world and the patients present notorious clinical heterogeneity. It is known that patients with SCD present activation of the blood coagulation and fibrinolytic systems, especially during vaso-occlusive crises, but also during the steady state of the disease. We determined if the presence of the factor V gene G1691A mutation (factor V Leiden), the prothrombin gene G20210A variant, and methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism may be risk factors for vascular complications in individuals with SCD. We studied 53 patients with SCD (60 percent being women), 29 with SS (sickle cell anemia; 28 years, range: 13-52 years) and 24 with SC (sickle-hemoglobin C disease; 38.5 years, range: 17-72 years) hemoglobinopathy. Factor V Leiden, MTHFR C677T polymorphism, and prothrombin G20210A variant were identified by PCR followed by further digestion of the PCR product with specific endonucleases. The following vascular complications were recorded: stroke, retinopathy, acute thoracic syndrome, and X-ray-documented avascular necrosis. Only one patient was heterozygous for factor V Leiden (1.8 percent) and there was no prothrombin G20210A variant. MTHFR 677TT polymorphism was detected in 1 patient (1.8 percent) and the heterozygous form 677TC was observed in 18 patients (34 percent, 9 with SS and 9 with SC disease), a prevalence similar to that reported by others. No association was detected between the presence of the MTHFR 677T allele and other genetic modulation factors, such as alpha-thalassemia, ß-globin gene haplotype and fetal hemoglobin. The presence of the MTHFR 677T allele was associated with the occurrence of vascular complications in SCD, although this association was not significant when each complication was considered separately. In conclusion, MTHFR C677T polymorphism might be a risk factor for vascular complications in SCD.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Anemia Falciforme/genética , Fator V/genética , /genética , Polimorfismo Genético , Doenças Vasculares Periféricas/etiologia , Protrombina/genética , Alelos , Anemia Falciforme/complicações , Marcadores Genéticos , Reação em Cadeia da Polimerase , Fatores de RiscoRESUMO
Sickle cell disease (SCD) is one of the most common inherited diseases in the world and the patients present notorious clinical heterogeneity. It is known that patients with SCD present activation of the blood coagulation and fibrinolytic systems, especially during vaso-occlusive crises, but also during the steady state of the disease. We determined if the presence of the factor V gene G1691A mutation (factor V Leiden), the prothrombin gene G20210A variant, and methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism may be risk factors for vascular complications in individuals with SCD. We studied 53 patients with SCD (60% being women), 29 with SS (sickle cell anemia; 28 years, range: 13-52 years) and 24 with SC (sickle-hemoglobin C disease; 38.5 years, range: 17-72 years) hemoglobinopathy. Factor V Leiden, MTHFR C677T polymorphism, and prothrombin G20210A variant were identified by PCR followed by further digestion of the PCR product with specific endonucleases. The following vascular complications were recorded: stroke, retinopathy, acute thoracic syndrome, and X-ray-documented avascular necrosis. Only one patient was heterozygous for factor V Leiden (1.8%) and there was no prothrombin G20210A variant. MTHFR 677TT polymorphism was detected in 1 patient (1.8%) and the heterozygous form 677TC was observed in 18 patients (34%, 9 with SS and 9 with SC disease), a prevalence similar to that reported by others. No association was detected between the presence of the MTHFR 677T allele and other genetic modulation factors, such as alpha-thalassemia, beta-globin gene haplotype and fetal hemoglobin. The presence of the MTHFR 677T allele was associated with the occurrence of vascular complications in SCD, although this association was not significant when each complication was considered separately. In conclusion, MTHFR C677T polymorphism might be a risk factor for vascular complications in SCD.
Assuntos
Anemia Falciforme/genética , Fator V/genética , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Doenças Vasculares Periféricas/etiologia , Polimorfismo Genético , Protrombina/genética , Adolescente , Adulto , Idoso , Alelos , Anemia Falciforme/complicações , Feminino , Marcadores Genéticos , Humanos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Reação em Cadeia da Polimerase , Fatores de RiscoRESUMO
Several infectious etiologies are related to cerebral venous thrombosis (CVT), but a review of literature showed only few cases related to tuberculosis (TB), and only one with neurological manifestations.We report an unusual case of CVT related to TB and mutation in prothrombin gene. A 38-man black presented abrupt right hemiparestesis, and hemiparesis. Investigations revealed CVT. Cerebral spinal fluid (CSF) examination evidenced an infection by Mycobacterium. He was heterozygous for G20210A prothrombin mutation. Probably, hypercoagulability mechanisms of TB, added to mutation of prothrombin gene increase the risk of CVT.
As mais variadas etiologias infecciosas estão relacionadas a trombose venosa cerebral (TVC), mas revisando-se a literatura há apenas poucos relatos de casos que se devem à tuberculose (TB), sendo que em apenas um deles havia manifestações no sistema nervoso central.Relatamos um caso de TVC associado a TB e a mutação do gene da protrombina. Homem 38 anos, negro, apresentou hemiparestesia de instalação súbita à direita, evoluindo com hemiparesia homolateral. Durante a internação, foi coletado líquor que evidenciou infecção por micobactéria. A pesquisa de trombofilias mostrou positividade somente para mutação do gene da protrombina(G20210A). Provavelmente os mecanismos de hipercoagulabilidade intrínsecos à tuberculose somados à mutação do gene da protrombina, potencializam o risco de TVC.
Assuntos
Adulto , Humanos , Masculino , Trombose Intracraniana/microbiologia , Trombose Venosa/microbiologia , Tuberculose do Sistema Nervoso Central/complicações , Imageamento por Ressonância Magnética , Mutação Puntual , Protrombina/genéticaAssuntos
Trombose Intracraniana/genética , Mutação Puntual , Protrombina/genética , Adulto , Brasil/epidemiologia , Estudos de Casos e Controles , Fator V , Feminino , Humanos , Trombose Intracraniana/epidemiologia , Trombose Intracraniana/etiologia , Masculino , Epidemiologia Molecular , Fatores de Risco , Trombose Venosa/epidemiologia , Trombose Venosa/etiologia , Trombose Venosa/genéticaRESUMO
Fasting total homocysteine (tHcy) and the methylenetetrahydrofolate reductase (MTHFR) C677T mutation were evaluated in 91 patients with venous thromboembolism and without acquired thrombophilia, and in 91 age-matched and sex-matched controls. Hyperhomocysteinemia was detected in 11 patients (12.1%) and in two controls (2.2%), yielding an odds ratio (OR) for venous thrombosis of 6.1 [95% confidence interval (CI), 1.3-28.4]. After excluding 21 patients and four controls with other known genetic risk factors for venous thrombosis, the OR was not substantially changed (7.0; 95% CI, 1.5-33.1). The prevalence of the MTHFR 677TT genotype was not significantly different in patients (9.9%) and in controls (5.5%), with an OR for venous thrombosis of 1.8 (95% CI, 0.6-5.8). Subjects with the MTHFR 677TT genotype showed higher levels of tHcy compared with the 677CC genotype in patients (P = 0.010) and in controls (P = 0.030). In conclusion, we found that fasting hyperhomocysteinemia is a risk factor for venous thrombosis in patients without known acquired thrombophilia and other genetic risk factors for venous thrombosis. Although tHcy levels are significantly higher in those homozygous for the MTHFR C677T mutation, this genotype does not increase the thrombotic risk in our study population.
Assuntos
Substituição de Aminoácidos , Hiper-Homocisteinemia/epidemiologia , Mutação de Sentido Incorreto , Oxirredutases atuantes sobre Doadores de Grupo CH-NH/genética , Mutação Puntual , Trombofilia/epidemiologia , Trombose Venosa/epidemiologia , Regiões 3' não Traduzidas/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Brasil/epidemiologia , Criança , Jejum/sangue , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2) , Pessoa de Meia-Idade , Razão de Chances , Polimorfismo Genético , Regiões Promotoras Genéticas/genética , Protrombina/genética , Fatores de Risco , Trombofilia/sangue , Trombofilia/genética , Trombose Venosa/etiologiaRESUMO
PURPOSE: To evaluate the response of 73 patients with antivitamin K (AVK) overdose to 3 different therapeutic regimens. METHODS: Seventy three patients were evaluated in 94 occasions: group A (N = 32), consisted of drug withdrawal for 2 days followed by reduced dosage; group B (N = 37), drug withdrawal and reassessment within 4 days; group C (N = 25), oral administration of vitamin K. Therapeutic range was set between INR-values of 2 and 4. RESULTS: Reversal regimens did not result in differences among 61 patients who had initial INR < 8 (chi 2 = 2.352, p = 0.671). There were more patients bellow therapeutic range in group C (N = 14) than group B (N = 19) (chi 2 = 9.998, p = 0.007). After intervention, 7 patients in group B still had INR > 4, but 5 of them were bellow 4.5, without increased bleeding risk. There were 10 patients in group C bellow therapeutic range, 6 of them with INR < 1.6, with risk of thromboembolism. Thirteen patients bled, but none required transfusion. CONCLUSION: Reversal of excessive oral anticoagulation can be safely performed by initial withdrawal of the drug, followed by lower doses. Vitamin K administration may lead to INR bellow the therapeutic range. This should be reserved for patients with high INR or in the presence of bleeding.
Assuntos
Anticoagulantes/efeitos adversos , Hemorragia/prevenção & controle , Tromboembolia/tratamento farmacológico , Vitamina K/antagonistas & inibidores , Administração Oral , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Overdose de Drogas , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tromboembolia/prevenção & controleRESUMO
PURPOSE: We evaluated hemostasic changes in children undergoing open heart surgery with cardiopulmonary bypass (CPB). METHODS: We studied 17 children before, during surgery, in the immediate, first and between the 4th and 7th postoperative days, measuring hematocrit, prothrombin time, activated partial thromboplastin time, fibrinogen, platelet count, factor V and euglobulin lysis time. Children were divided in those with and without excessive bleeding in the postoperative period. RESULTS: We observed significant prolongation of prothrombin time and activated partial thromboplastin time, reduction of fibrinogen and factor V, and shortening of euglobulin lysis time. Six (35%) children bled excessively. Platelet count reduction was greater in the intra operative period in these cases and the duration of CPB was longer in this group. CONCLUSION: Changes in hemostasis during open heart surgery are due to coagulation cascade disorders as well as fibrinolysis. The incidence of excessive bleeding is higher in the pediatric group. Prolonged CPB time and greater reduction in platelet count differentiated both groups.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Circulação Extracorpórea , Hemostasia , Criança , Pré-Escolar , Feminino , Testes Hematológicos , Hemorragia , Humanos , Lactente , Período Intraoperatório , Masculino , Período Pós-Operatório , Estudos ProspectivosRESUMO
BACKGROUND: This study was performed to evaluate antithrombin III levels in postmenopausal women receiving hormonal replacement treatment. METHODS: It is a prospective randomized study concerning 19 postmenopausal patients, aged 40 to 65 years, who received either continuous daily oral equine conjugated estrogen 0.625 mg (group A, N=10) or daily transdermal 17beta-estradiol 50 microg (group B, N=9). Medroxyprogesterone acetate (5 mg/day, 14 days monthly) was given to all patients. Blood samples were obtained before and after 3, 6, 9 and 12 months of treatment. Coagulation tests included Antithrombin III (functional method), prothrombin time, partial activated prothrombin time, thrombin time, factor V, fibrinogen, platelet count and euglobulin lysis time. Friedman analysis of variance and Mann-Whitney test were used for statistical analysis. RESULTS: Antithrombin III level was reduced (p<0.05) in group A but not in group B, although it remained within normal range. No changes were detected in the other coagulation tests. CONCLUSIONS: These data suggest that oral conjugated estrogen replacement reduces functional ATIII, whereas transdermal estradiol replacement therapy does not modify it.
Assuntos
Antitrombina III/análise , Estradiol/farmacologia , Terapia de Reposição de Estrogênios/métodos , Estrogênios Conjugados (USP)/farmacologia , Pós-Menopausa/sangue , Administração Cutânea , Administração Oral , Adulto , Antitrombina III/efeitos dos fármacos , Estudos de Coortes , Estradiol/administração & dosagem , Estrogênios Conjugados (USP)/administração & dosagem , Feminino , Humanos , Medroxiprogesterona/administração & dosagem , Medroxiprogesterona/farmacologia , Pessoa de Meia-Idade , Pós-Menopausa/efeitos dos fármacos , Congêneres da Progesterona/administração & dosagem , Congêneres da Progesterona/farmacologia , Estudos ProspectivosRESUMO
OBJETIVO - Avaliar as alteraçöes de hemostasia encontradas em crianças submentidas a cirurgia cardíaca com circulaçäo extracopórea (CEC). MÉTODOS - Estudamos 17 crianças no pré e pós-operatório (PO) imediato, no 1§ PO e entre o 4§ e 7§ PO, analisando o hematócrito, tempo de protrombina (TP), tempo de tromboplastina parcial ativado, fibrinogênio, contagem de plaquetas, fator V e tempo de lise de euglobulina (TLE). Os pacientes foram divididos em grupos com e sem sangramento execessivo no PO. RESULTADOS - Houve alteraçöes significantes no intraoperatório com aumento do TP e tempo de tromboplastina parcial ativado e reduçäo do fibrinogênio, fator V e do TLE. Seis (35 por cento) crianças sangraram execessivamente. A contagem de plaquetas foi significantemente menor no intra-operatório; neste grupo o tempo de CEC foi menor. CONCLUSÄO - Alteraçöes de hemostasia no intra e PO ocorrem como conseqüência da ativaçÝo da cascata de coagulaçäo e fibrinólise. A incidência de sangramento execessivo é alta nesta faixa etária. Os grupos com e sem sangramento execessivo diferenciam-se pelo maior tempo de CEC e contagem reduzida de plaquetas.
Assuntos
Humanos , Masculino , Feminino , Criança , Lactente , Pré-Escolar , Procedimentos Cirúrgicos Cardíacos , Circulação Extracorpórea , Hemostasia , Período Pós-Operatório , Estudos ProspectivosRESUMO
OBJETIVO - Verificar a resposta de 73 pacientes com superdosagem de droga anti-vitamina K (AVK) a 3 esquemas de tratamento. MÉTODOS - Os 73 pacientes foram avaliadois em 94 ocasiöes e divididos em 3 grupos: grupo A (N=32), suspensäo por dois dias e introduçäo de dose menor; grupo B (N=37), suspensÝo do AVK e reavaliaçäo em 4 dias; grupo C (N=25), vitamina K por via oral. A razäo normalizada internacional (RNI) final foi considerada adequada quando entre 2,0 e 4,0. RESULTADOS - Näo houve diferença entre os tratamentos (x2=2,352, p=0,671) para 61 pacientes com RNI inicial menor que 8. Houve mais pacientes com RNI menor que 2 no grupo C (x2=9,998, p=0,007) entre 33 pacientes com RNI maior que 8. Cinco dos 7 pacientes do grupo B que continuaram com superdosagem tinham RNI menor que 4,5 e pequeno risco de hemorragia. Entretanto 6, dos 10 pacientes do grupo C com anticoagulaçäo insuficiente tinham RNI menor que 1,6 e risco de trombose. Treze pacientes sangraram, mas sem necessidade de transfusäo. Conclusäo - A reversäo da superdosagem de AVK pode ser feita pela suspensäo de transfusÝo da droga Administraçäo de vitamina K, por via oral, deve ser restringir a pacientes com RNI mais elevado para se evitar anticoagulaçäo insuficiente.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Idoso , Adulto , Pessoa de Meia-Idade , Anticoagulantes/efeitos adversos , Hemorragia , Tromboembolia/tratamento farmacológico , Vitamina K/antagonistas & inibidores , Idoso de 80 Anos ou mais , Overdose de Drogas , Estudos Retrospectivos , Fatores de Risco , Tromboembolia , Fatores de TempoRESUMO
A deficiência de antitrombina III (ATIII) é observada na hepatopatia grave e pode ser decorrente da reduçäo de síntese ou de consumo aumentado, o que poderia ser compensado com o uso de concentrado de ATIII. OBJETIVO. Avaliar a eficiência da administraçäo de uma dose fixa de concentrado de ATIII, em pacientes com hepatopatia descompensada com distúrbio de hemostasia. CASUISTICA E MÉTODO. Foram avaliados seis pacientes, com idade média de 44 anos, variando de 14 a 63 anos, portadores de cirrose (quatro de etiologia alcoólica, um viral e um doença de Wilson), com alteraçäo de pelo menos dois dos parâmetros da hemostasia (TP> 1,40, TTPA> 1,25, fibrinogênio < 1,5g/L, plaquetas < 80.000/mm3). A média do nível de albumina foi de 2,6g/dL (1,9 a 3,8g/dL). O concentrado de ATIII (Kybernin) foi administrado na dose de 50U/kg, em dias alternados. Foi colhido sangue antes da primeira infusäo, 4 horas após e, depois, diariamente, antes da infusäo do dia, para medida da ATIII plasmática (amidolítico). Nenhum paciente recebeu hemoderivados. RESULTADOS. As médias da dosagem de ATIII foram: inicial = 35,8 por cento, 4 horas = 56,2 por cento*, 2 dias = 48,7 por cento*, 4 dias = 45,7 por cento* e 8 dias = 42,3 por cento*. Após a infusäo houve elevaçäo significante dos níveis de ATIII (* = p < 0,02, teste de Friedman), que se manteve até o 4§ dia. Näo houve alteraçäo dos demais parâmetros de coagulaçäo. CONCLUSÕES. O uso de concentrado de ATIII na dose utilizada é suficiente para elevar os níveis desse inibidor na hepatopatia; entretanto, com essa dose näo se obteve normalizaçäo de seus níveis. Esses dados sugerem que doses mais elevadas devem ser usadas em pacientes com hepatopatias graves, que apresentam näo apenas reduçäo de síntese, mas aumento de consumo dos fatores da coagulaçäo e de seus inibidores.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Transtornos da Coagulação Sanguínea/terapia , Inibidores de Serina Proteinase/uso terapêutico , Antitrombina III/uso terapêutico , Cirrose Hepática , Tempo de Tromboplastina Parcial , Contagem de Plaquetas , Tempo de Protrombina , Fibrinogênio , Antitrombina III , Hepatite Viral Humana/complicações , Degeneração Hepatolenticular/complicações , Cirrose Hepática/etiologia , Hepatopatias Alcoólicas/complicaçõesRESUMO
BACKGROUND: Patients with severe hepatic failure present acquired deficiency of antithrombin III (ATIII) owing to reduced synthesis associated with intravascular activation of blood coagulation, which may be corrected by ATIII infusion. OBJECTIVE: The aim of this uncontrolled trial was to verify the effect of a standard dose of ATIII concentrate (Kybernin), that is, 50 U/kg of body weight per day, every 2 days, on ATIII levels in patients with severe hepatic failure and hemostatic imbalance. PATIENTS AND METHODS: Six cirrhotic patients were studied: mean age of 44 years (14 to 63 years), who presented at least 2 abnormal coagulation tests (PT > 1.40, APTT > 1.25, Fibrinogen < 1.5 g/dL, Platelet count < 80,000/mm3). Mean serum albumin was 2.6 g/dL (1.9 to 3.8 g/dL). Blood was drawn before infusion, 4 h after the first infusion, and just before the next infusion. ATIII levels were measured by amidolytic method. RESULTS: Mean ATIII levels were: initial = 35.8%, 4th h = 56.2%*, 2nd d = 48.7%*, 4th d = 45.7%*, and 8th d = 42.3%. ATIII levels increased significantly after infusion of this standard dose in all patients, although they have not been fully corrected (Friedman test, * p < 0.02), which has been sustained till the 4th day. There was no improvement on the clinical outcome. CONCLUSIONS: These findings suggest that doses of ATIII concentrate higher than 50 U/kg/infusion must be administered to patients with severe hepatic failure, to guarantee normal levels of the inhibitor, in order to verify its influence on the hemostatic mechanism.
Assuntos
Antitrombina III/uso terapêutico , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Cirrose Hepática/complicações , Inibidores de Serina Proteinase/uso terapêutico , Adolescente , Adulto , Transtornos da Coagulação Sanguínea/complicações , Feminino , Hepatite Viral Humana/complicações , Degeneração Hepatolenticular/complicações , Humanos , Hepatopatias Alcoólicas/complicações , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Tempo de ProtrombinaRESUMO
PURPOSE: To evaluate the clinical and laboratory management of patients taking anti-vitamin K drugs (AVK). METHODS: We studied retrospectively 952 visits of 100 outpatients taking AVK drugs for 7.6 months. There were 56 men and 44 women, 54 patients had acute arterial occlusion, 34 presented venous thromboembolism and 12 had cardiopathy. Anticoagulation level was estimated by the prothrombin time reported as international normalized ratio (INR). RESULTS: Seventy-three patients were considered stable, as they had one visit every at least 3 weeks, and their INR was within the therapeutic range in 59% of their visits, whereas 27 patients were less stable and had 36% of their visits within the therapeutic range. Insufficient anticoagulation was due to poor compliance (22%), vitamin K rich diet (19%) and underdosage (16%). Four patients presented minor bleedings, and there was no recurrence of thromboembolism. CONCLUSION: Careful clinical and laboratory management, using the INR, are necessary to avoid hemorrhage and thrombotic complications in patients taking oral anticoagulants.
Assuntos
Anticoagulantes/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Tromboembolia/tratamento farmacológico , Vitamina K/antagonistas & inibidores , Administração Oral , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tempo de Protrombina , Estudos RetrospectivosRESUMO
Objetivo - Avaliar o acompanhamento clínico e laboratorial de pacientes em uso de drogas antivitamina K(AVK). MÉTODOS - Foram avaliados, retrospectivamente, 952 consultas ambulatoriais de 100 pacientes em uso de AVK, durante 7,6 meses. Havia 56 homens e 44 mulheres, 54 pacientes com obstruçäo arterial aguda, 34 com troboembolismo venoso e 12 com cardiopatia. O nível de anticoagulaçäo foi medido pelo tempo de protrombina expresso em razäo normalizada internacional (RNI). RESULTADOS - Nível adequado de anticoagulaçäo foi observado em 59 "por cento" das consultas dos 73 pacientes considerados estáveis, com intervalo entre consultas maior do que 3 semanas. Os 27 pacientes instáveis tinham 36 "por cento" das consultas com RNI adequado. Anticoagulaçäo insuficiente ocorreu por uso irregular (22 por cento), dieta rica em vitamina (19 por cento) e dose insuficiente (16 por cento). Quatro pacientes tiveram sangramento sem gravidade e näo houve recorrência da trombose durante o período de observaçäo. CONCLUSÄO - O controle clínico e laboratorial, através do RNI, é fundamental para evitar complicaçöes hemorrágicas ou trombóticas em pacientes que necessitam de anticoagulaçäo oral.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Pacientes Ambulatoriais , Anticoagulantes/uso terapêutico , Coagulação Sanguínea , Doenças Cardiovasculares/tratamento farmacológico , Idoso de 80 Anos ou mais , Estudos RetrospectivosRESUMO
OBJECTIVE: To correlate the incidence of hemorrhage and thrombosis to bleeding time (BT) and platelet aggregation in 27 consecutive patients with myeloproliferative diseases (MPD). DESIGN: Retrospective study. SETTING: Public tertiary referral center. PATIENTS: Eighteen patients with chronic myelogenous leukemia (CML), 5 with polycytemia vera (PV), 2 with essential thrombocytemia (ET) and 2 with idiopathic myelofibrosis (MF). Duke's BT and epinephrine-induced platelet aggregation were performed on the patients and on 10 healthy individuals. RESULTS: Eleven patients presented symptoms (41%):9 with hemorrhage (33%) and 5 with thrombosis (19%). There were less symptomatic patients in the CML group (28%) than in the other MPD (67%), without statistical significance (Fisher, p = 0.06). Duke's BT was longer in symptomatic patients (Mann-Whitney, p < 0.05). Platelet aggregation was abnormal in 7 patients (26%) and 71% of them were symptomatic (Fisher, p = 0.07). CONCLUSIONS: The high incidence of bleeding and thrombosis in patients with MPD was related to prolonged BT, but not to platelet aggregation abnormalities.
Assuntos
Tempo de Sangramento , Epinefrina/farmacologia , Hemorragia/etiologia , Transtornos Mieloproliferativos/complicações , Agregação Plaquetária , Trombose/etiologia , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Agregação Plaquetária/efeitos dos fármacos , Contagem de Plaquetas , Estudos RetrospectivosRESUMO
O uso de método funcional para dosagem de antitrombina III (ATIII) é fundamental para o diagnóstico de deficiência deste inibidor da coagulaçao. OBJETIVO. Padronizar metodologia para dosagem da ATIII no plasma, utilizando-se microplacas, em diferentes situaçoes clínicas. MÉTODOS. A dosagem de ATIII foi feita utilizando-se o substrato cromogênico Tos-Gly-Pro-Arg-NAN, específico para trombina, e sintetizado no Departamento de Biofísica da Escola Paulista de Medicina. RESULTADOS. Dos 21 pacientes com trombose venosa (TV-P), 20 apresentaram valores superiores a 70 por cento (ll3 ñ 22 por cento), dos quais uma paciente de 22 anos apresentava deficiência congênita, com ATIII de 56 por cento e história de TVP recorrente, além de história familiar de TVP. O nível de ATIII em seis pacientes portadores de doença de von Willebrand foi normal (lO9 ñ 28 por cento), como esperado. Em 20 pacientes com insuficiência hepática foi observada reduçao importante do nível de ATIII (42 ñ 19 por cento), pois este inibidor é produzido no hepatócito, sendo bom parâmetro para avaliar a funçao hepática. Os três pacientes portadores de sepse com CIVD apresentaram níveis reduzidos de ATIII (45 + 5 por cento), que é consumida durante processo de ativaçao intravascular da coagulaçao. Os níveis de ATIII mostraram correlaçao significante com o TP e os níveis de fator V, ambos bons parâmetros para avaliaçao da funçao hepática e para monitorizaçao da CIVD. Houve correlaçao significante entre as dosagens realizadas com o substrato Tos-Gly-Pro-Arg-NAN, sintetizado na EPM, e o substrato S-2238 do laboratório Kabi. CONCLUSOES. A medida da ATIII usando substrato cromogênico Gly-Pro-Arg-NAN é de fácil execuçao e é sensível para o diagnóstico da deficiência deste inibidor em pacientes com insuficiência heopática, coagulaçao intravascular disseminada e trombofilia.