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BACKGROUND: Kawasaki disease is a vasculitis that can lead to cardiac complications, including coronary artery disease and cardiogenic shock. Various scoring systems have been developed to determine those that will be refractory to routine intravenous immunoglobulin therapy or develop coronary artery disease. The objective of this study was to determine if the neutrophil-lymphocyte ratio could predict refractory disease and coronary artery lesions in patients with Kawasaki disease. METHODS: A systematic review of the literature was performed to identify manuscripts describing comparisons of neutrophil-lymphocyte ratio between those who had refractory disease and those who did not, and between those who developed coronary artery lesions and those who did not. Mean difference was compared between groups. Areas under the curve were utilised to determine the pooled area under the curve. RESULTS: 12 studies with 5593 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of refractory disease. Neutrophil-lymphocyte ratio before therapy was higher in refractory disease with a mean difference of 2.55 (p < 0.01) and pooled area under the curve of 0.724. Neutrophil-lymphocyte ratio after therapy was higher in refractory disease with a mean difference of 1.42 (p < 0.01) and pooled area under the curve for of 0.803. Five studies with 1690 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of coronary artery lesions. Neutrophil-lymphocyte ratio before therapy was higher in coronary artery lesions with a mean difference of 0.65 (p < 0.01). CONCLUSION: The use of neutrophil-lymphocyte ratio may help physicians in the identification of patients at risk of refractory disease and coronary artery lesions in patients with Kawasaki disease.
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Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Humanos , Lactente , Doença da Artéria Coronariana/etiologia , Doença da Artéria Coronariana/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Linfócitos , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Neutrófilos , Estudos RetrospectivosRESUMO
La enfermedad de Kawasaki (EK) es la principal causa de cardiopatía adquirida en menores de cinco años. Nuestro objetivo fue conocer las características clínicas, el compromiso coronario y la evolución de pacientes atendidos en nuestra institución. Se revisó una serie de casos desde 2001 hasta 2018. Se incluyeron 63 pacientes, 58 % varones; la mediana de edad fue 2,6 años. La mediana de días de fiebre al diagnóstico fue 5,5 días. El 33 % presentó la forma incompleta y se detectó compromiso coronario en el 20 %. El 60 % de los pacientes con afectación coronaria presentaron EK incompleta versus el 28 % de presentación incompleta en los pacientes sin compromiso coronario (p 0,06). No se observaron diferencias en datos de laboratorio entre los grupos según el compromiso coronario. En conclusión, 33 % presentó EK incompleta y el 20 %, afectación coronaria. Hubo una tendencia de mayor riesgo para daño coronario en la forma incompleta.
Kawasaki disease (KD) is considered the leading cause of acquired heart disease in children younger than 5 years. Our objective was to know the clinical characteristics, coronary involvement, and course of patients seen at our facility. A case series from 2001 to 2018 was reviewed. Sixty-three patients were included; their median age was 2.6 years; 58% were males. The median duration of fever at the time of diagnosis was 5.5 days. The incomplete form was observed in 33% and coronary involvement, in 20%. Among patients with coronary involvement, 60% had incomplete KD versus 28% among those without coronary involvement (p: 0.06). No differences were observed between groups in laboratory data based on coronary involvement. To conclude, 33% had incomplete KD and 20%, coronary involvement. There was a trend to a higher risk for coronary artery damage in the incomplete form of KD.
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Humanos , Pré-Escolar , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Estudos Retrospectivos , Imunoglobulinas Intravenosas , Febre , Hospitais GeraisRESUMO
Kawasaki disease (KD) is considered the leading cause of acquired heart disease in children younger than 5 years. Our objective was to know the clinical characteristics, coronary involvement, and course of patients seen at our facility. A case series from 2001 to 2018 was reviewed. Sixty-three patients were included; their median age was 2.6 years; 58% were males. The median duration of fever at the time of diagnosis was 5.5 days. The incomplete form was observed in 33% and coronary involvement, in 20%. Among patients with coronary involvement, 60% had incomplete KD versus 28% among those without coronary involvement (p:0.06). No differences were observed between groups in laboratory data based on coronary involvement. To conclude, 33% had incomplete KD and 20%, coronary involvement. There was a trend to a higher risk for coronary artery damage in the incomplete form of KD.
La enfermedad de Kawasaki (EK) es la principal causa de cardiopatía adquirida en menores de cinco años. Nuestro objetivo fue conocer las características clínicas, el compromiso coronario y la evolución de pacientes atendidos en nuestra institución. Se revisó una serie de casos desde 2001 hasta 2018. Se incluyeron 63 pacientes, 58 % varones; la mediana de edad fue 2,6 años. La mediana de días de fiebre al diagnóstico fue 5,5 días. El 33 % presentó la forma incompleta y se detectó compromiso coronario en el 20 %. El 60 % de los pacientes con afectación coronaria presentaron EK incompleta versus el 28 % de presentación incompleta en los pacientes sin compromiso coronario (p 0,06). No se observaron diferencias en datos de laboratorio entre los grupos según el compromiso coronario. En conclusión, 33 % presentó EK incompleta y el 20 %, afectación coronaria. Hubo una tendencia de mayor riesgo para daño coronario en la forma incompleta.
Assuntos
Síndrome de Linfonodos Mucocutâneos , Criança , Masculino , Humanos , Lactente , Pré-Escolar , Feminino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Hospitais Gerais , Imunoglobulinas Intravenosas , Estudos Retrospectivos , FebreRESUMO
OBJECTIVE: To investigate the incidence of coronary artery abnormalities (CAAs) by fever pattern after intravenous immunoglobulin (IVIG) therapy in patients with Kawasaki disease. STUDY DESIGN: This retrospective cohort study included 172 patients with Kawasaki disease aged ≤12 years who underwent IVIG therapy and had no CAAs before treatment. Resistance to initial IVIG was defined as persistent fever ≥37.5 °C for ≥24 hours after therapy or the recurrence of Kawasaki disease after initial defervescence. The patients were divided into 3 groups: IVIG responders, nonresponders with persistent fever, and nonresponders with recurrent fever. CAAs were evaluated 2 or 4 weeks and 12 months after onset and were defined by a coronary artery z-score ≥2.5. RESULTS: The incidence of CAAs within 12 months after onset was significantly higher in nonresponders with persistent fever (27%) compared with the other 2 groups. On multivariate logistic regression analysis, being a nonresponder with persistent fever was an independent risk factor for having CAAs within 12 months after the onset of Kawasaki disease (OR, 6.48; P = .007). CONCLUSIONS: In patients with Kawasaki disease resistant to IVIG therapy, persistent fever, but not recurrent fever, was found to be a risk factor for the incidence of CAAs. Aggressive additional therapy may be beneficial to prevent CAA formation in patients with Kawasaki disease with persistent fever.
Assuntos
Anomalias dos Vasos Coronários/epidemiologia , Febre/epidemiologia , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Criança , Pré-Escolar , Anomalias dos Vasos Coronários/diagnóstico , Feminino , Febre/diagnóstico , Humanos , Incidência , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Estudos Retrospectivos , Fatores de RiscoRESUMO
Description Infantile botulism is a potentially devastating disease caused by ingestion of Clostridium botulinum spores through food products or dust particles. The toxin produced by the spores can lead to descending paralysis requiring hospitalization for supportive care which sometimes includes mechanical ventilation. Human Botulism Immune Globulin-Intravenous (BIG-IV or BabyBIG) from the Infant Botulism Treatment and Prevention Program (IBTPP) has been shown to greatly improve outcomes. A previously healthy 5-month-old infant was admitted to her regional hospital for poor feeding and lethargy. When the weakness progressed and she had trouble with protecting her airway, she was intubated and transferred to our institution. The primary diagnosis was infantile botulism and the decision was made to treat with BabyBIG. The pharmacy department was able to assist with obtaining BabyBIG, ensuring proper preparation, and coordinating the team for swift administration. In the days following the BabyBIG administration, the patient slowly started to recover her respiratory function and muscle tone. On day five of admission the patient was extubated. After transfer to the general pediatric floor the patient was given a transpyloric feeding tube and worked with gastrointestinal and nutrition services to improve oral feeding. She was discharged on day seventeen with plans to continue working with therapy and nutrition. BIG-IV can have a major impact in the recovery of infantile botulism. Pharmacists are in an optimal position to assist with coordinating the multidisciplinary team regarding its procurement, preparation, and administration.
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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible of the coronavirus disease 2019 (COVID-19) pandemic. Therapeutic options including antimalarials, antivirals, and vaccines are under study. Meanwhile the current pandemic has called attention over old therapeutic tools to treat infectious diseases. Convalescent plasma (CP) constitutes the first option in the current situation, since it has been successfully used in other coronaviruses outbreaks. Herein, we discuss the possible mechanisms of action of CP and their repercussion in COVID-19 pathogenesis, including direct neutralization of the virus, control of an overactive immune system (i.e., cytokine storm, Th1/Th17 ratio, complement activation) and immunomodulation of a hypercoagulable state. All these benefits of CP are expected to be better achieved if used in non-critically hospitalized patients, in the hope of reducing morbidity and mortality.
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Anticorpos Neutralizantes/uso terapêutico , Infecções por Coronavirus/terapia , Pneumonia Viral/terapia , Betacoronavirus , COVID-19 , Ensaios Clínicos como Assunto , Infecções por Coronavirus/imunologia , Humanos , Imunização Passiva , Linfócitos/imunologia , Pandemias , Pneumonia Viral/imunologia , Estudos Retrospectivos , SARS-CoV-2 , Soroterapia para COVID-19RESUMO
Rationale: Kawasaki disease (KD) is an acute vasculitis of small and medium vessels; whereas systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease. Their presentation is varied and not always straightforward, leading to misdiagnosis. There have been case reports of lupus onset mimicking KD and KD presenting as lupus-like. Coexistence of both diseases is also possible. Patient concerns: We present three adolescents, one with fever, rash, arthritis, nephritis, lymphopenia, and coronary aneurysms, a second patient with rash, fever, aseptic meningitis, and seizures, and a third patient with fever, rash, and pleural effusion. Diagnoses: The first patient was finally diagnosed with SLE and KD, the second patient initially diagnosed as KD but eventually SLE and the third patient was diagnosed at onset as lupus but finally diagnosed as KD. Interventions: The first patient was treated with IVIG, corticosteroids, aspirin, coumadin and mycophenolate mofetil. The second patient was treated with IVIG, corticosteroids and methotrexate and the third patient with IVIG, aspirin and corticosteroids. Lessons: Both diseases may mimic each other's clinical presentation. KD in adolescence presents with atypical signs, incomplete presentation, and develop coronary complications more commonly. An adolescent with fever and rash should include KD and SLE in the differential diagnosis.
RESUMO
BACKGROUND: Intravenous immunoglobulin (IVIG) is prepared using purified human plasma. IVIG therapy has immunomodulatory effects on autoimmune diseases, including severe systemic lupus erythematosus (SLE). However, reports of its effects on large cohorts are scarce. METHODS: This single-center retrospective study included SLE patients treated with at least one IVIG cycle for SLE complications. Demographic data, indications, cycle numbers, and clinical improvement with IVIG were evaluated. SLE Disease Activity Index 2000 (SLEDAI-2K) scores were calculated at admission and after IVIG treatment in order to measure clinical improvement. RESULTS: Sixty-three SLE patients treated with IVIG (median age: 29 years; interquartile range 21-36 years; 84.13% female) were included, who received 2 g/kg IVIG for two to five days. Main indications were immune thrombocytopenia, hypogammaglobulinemia, infection during a SLE flare, bicytopenia, and immune hemolytic anemia. Seven patients received more than one IVIG cycle without severe adverse effects. Significant differences were found in SLEDAI-2K scores when the indications were immune thrombocytopenia and hypogammaglobulinemia, with a trend for hemolytic anemia. Patients with concomitant infection, myopathy, and gastrointestinal involvement showed a considerable reduction in their last SLEDAI-2K scores. Fourteen patients died during hospitalization, mainly due to septic shock and active SLE. CONCLUSIONS: IVIG showed adequate tolerance and effectiveness in selected severe SLE manifestations, mainly hematological involvement. It was useful for concomitant infection.
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Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adulto , Estudos de Coortes , Feminino , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Fatores Imunológicos/efeitos adversos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto JovemRESUMO
Kawasaki disease (KD) has features that appear supporting an infectious cause with a secondary deranged inflammatory/autoimmune response. The association of KD in adults with human immunodeficiency virus infection and the presence of KD in patients with immunodeficiency disorders support the infectious theory. We present four KD patients associated with immunodeficiencies: one with X-linked agammaglobulinemia, one with HIV infection, and two with leukemia; one of these patients also had Down syndrome. We did a literature search to find out all reported cases of immunodeficiency with KD in children. In immunodeficiency disorders, the inability of the immune system to eradicate the pathogens coupled to an exaggerated inflammatory response, especially in chronic granulomatous disease, may lead to the development of KD. The study of patients with immunodeficiencies complicated with KD may shed light into the etiopathogenesis of the disease.
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Agamaglobulinemia/imunologia , Doenças Genéticas Ligadas ao Cromossomo X/imunologia , Infecções por HIV/imunologia , Hospedeiro Imunocomprometido , Leucemia/imunologia , Síndrome de Linfonodos Mucocutâneos/imunologia , Corticosteroides/uso terapêutico , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Agamaglobulinemia/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Síndrome de Down/complicações , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Infecções por HIV/complicações , Infecções por HIV/diagnóstico , Infecções por HIV/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Leucemia/complicações , Leucemia/diagnóstico , Leucemia/tratamento farmacológico , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Fatores de Risco , Resultado do TratamentoRESUMO
Previous studies have suggested an association of IgG levels (before and after IVIG infusion) with clinical outcomes in Kawasaki disease. A retrospective analysis was performed that included 418 patients with KD admitted to Tokyo Women's Medical University Yachiyo Medical Center to evaluate pre- and post-IVIG IgG levels and its relation to outcomes. All patients received an initial IVIG infusion and aspirin; IgG levels were measured in 350 patients before IVIG (pre-IVIG IgG levels) and in 373 patients 48 h after starting IVIG infusion (post-IVIG IgG levels). Media and standard deviation of the pre- and post-IVIG IgG levels were reported and classified according to age. Also, IgG z-scores were calculated according to normal values of IgG by age. The number of cases and corresponding percentage of non-responders were reported by age and total patients. The association of pre-IVIG, post-IVIG IgG levels and post-IVIG IgG level/pre-IVIG IgG level ratio with no-response was evaluated by simple logistic regression model based on the IgG z-score, and regression coefficient, X2 value, p, and R2 of Nagelkerke were reported. Pre-IVIG and post-IVIG IgG levels presented an association with non-responders with statistical significance. This association was more evident between post-IVIG IgG levels and non-responders. Regarding coronary alterations, it was not possible to perform an adequate statistical analysis due the small number of patients. Pre- and post-IVIG infusion IgG levels could be an important biomarker in KD as well as in other inflammatory conditions. Higher IgG levels could be associated with a more effective immunomodulatory action and associated with better clinical outcomes.
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Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/terapia , Aspirina/uso terapêutico , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Modelos Logísticos , Masculino , Síndrome de Linfonodos Mucocutâneos/sangue , Inibidores da Agregação Plaquetária/uso terapêutico , Prognóstico , Retratamento , Estudos Retrospectivos , Resultado do TratamentoRESUMO
INTRODUCTION: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Microorganisms such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Haemophilus influenzae and Zika virus have been linked to the disease. The most common clinical variants are acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Plasma exchange and intravenous immunoglobulins are the standard therapy for the disease. AREAS COVERED: Research to elucidate the pathophysiology of Guillain-Barré syndrome has led to the development of drugs directed towards new potential therapeutic targets. This review offers a comprehensive view of the current treatment based upon the physiopathology. EXPERT OPINION: Patients with Guillain-Barré syndrome need a multidisciplinary approach, limitation to walk unaided and disability score are indicators for treatment as well as the presence of autonomic dysfunction and pain. Admission to intensive care units should be considered for those patients presenting with respiratory failure, bulbar involvement and progression of the disease. Research aimed to deciphering the pathophysiology of the disease, discovering new biomarkers and establishing algorithms of prediction of both the disease and its outcomes is warranted.
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Síndrome de Guillain-Barré/terapia , Imunoterapia/métodos , Progressão da Doença , Síndrome de Guillain-Barré/imunologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Imunoterapia/efeitos adversos , Troca Plasmática/métodos , Plasmaferese/métodosRESUMO
Resumen El compromiso gastrointestinal en pacientes con lupus eritematoso sistémico (LES) es muy diverso. Su frecuencia y ubicación a lo largo del tracto digestivo varían ampliamente. Los procesos inflamatorios secundarios a los depósitos de complejos inmunes o eventos vasculares pueden ser los causantes de este compromiso. Una de las manifestaciones gastrointestinales características en los pacientes con LES es la pseudoobstrucción intestinal, que se define como la propulsión intestinal ineficaz que se produce en ausencia de factores mecánicos u obstructivos. Esta es, sin embargo, una complicación rara y poco entendida del LES. En este artículo, reportamos el caso de un paciente masculino con diagnóstico de LES y pseudoobstrucción intestinal, que fue tratado exitosamente con esteroides e inmunoglobulinas intravenosas. Se presenta una revisión completa de la literatura y una propuesta de la fisiopatología de la manifestación.
Abstract Gastrointestinal involvement in patients with systemic lupus erythematosus (SLE) is very diverse, and the frequency of occurrence and location along the digestive tract varies widely. Inflammatory processes secondary to immune complex deposits or vascular events may cause this involvement. One of the most characteristic gastrointestinal manifestations in these patients is the intestinal pseudo-obstruction, which is defined as the ineffective intestinal propulsion that occurs in the absence of mechanical or obstructive factors. This is, however, a rare and poorly understood complication of SLE. The case is presented of a male SLE patient presenting with intestinal pseudo-obstruction, and was successfully treated with steroids and intravenous immunoglobulin. A complete review of the literature and a proposal for the pathophysiology of intestinal pseudo-obstruction are presented.
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Humanos , Masculino , Adulto , Terapêutica , Pseudo-Obstrução Intestinal , Imunoglobulinas Intravenosas , Lúpus Eritematoso Sistêmico , EsteroidesRESUMO
Objetivo: Reportar el caso de una paciente obstétrica que desarrolló un shock séptico secundario a una infección gastrointestinal y el uso de inmunoglobulina endovenosa (IVIG) como terapia coadyuvante y hacer una revisión de la literatura publicada del uso de inmunoglobulinas en shock séptico con énfasis en su uso en el embarazo. Materiales y métodos: Se presenta el caso de una paciente con embarazo de 36 semanas, quien presentó un choque séptico secundario a una infección gastrointestinal y progresó con falla orgánica multisistémica. En vista de la pobre respuesta al tratamiento instaurado se inició terapia coadyuvante con inmunoglobulina enriquecida en IgM con una buena respuesta. Se realizó una búsqueda de la literatura en la bases de datos de Medline vía PubMed, Direct Sciencie, Biblioteca Cochrane y SciELO utilizando las palabras clave de los Descriptores en Ciencias de la Salud (DeCS) como inmunoglobulina endovenosa y sepsis en embarazo. Resultados: Se encontraron 6 artículos relacionados directamente con el uso de IVIG en sepsis en el embarazo, 1 revisión de tema, 2 reportes de caso y 3 guías diagnósticas y de manejo. Las inmunoglobulinas intravenosas se han utilizado con mayor frecuencia en pacientes no obstétricas en shock séptico como terapia coadyuvante especialmente en infección por estreptococos y estafilococos. Conclusión: Las inmunoglobulinas intravenosas podrían ser una alternativa como coadyuvante en manejo de shock séptico en gestantes con pobre respuesta al tratamiento convencional. Se necesitan estudios analíticos que incluyan gestantes para confirmar estos hallazgos.
Objective: To report the case of an obstetric patient who developed septic shock secondary to a gastrointestinal infection, and the use of intravenous immunoglobulin (IVIG) as an adjunct, and to conduct a review of the published literature on the use of immunoglobulins in septic shock with emphasis on its use during pregnancy. Materials and methods: Case report of a patient with 36 weeks of gestation who presented with septic shock secondary to a gastrointestinal infection resulting in multiple organ failure. In view of poor response to treatment, adjunctive therapy with IgM-enriched immunoglobulin therapy was initiated, achieving good response. A search of the literature was conducted in the Medline, Direct Science, Cochrane Library and SciELO databases using Health Science Descriptors (DeCS) such as intravenous immunoglobulin and sepsis in pregnancy. Results: Six articles directly related to the use of IVIG in sepsis during pregnancy were found, including 1 review, 2 case reports and 3 diagnosis and management guidelines. Intravenous immunoglobulins have been used with greater frequency in non-obstetric patients in septic shock as an adjunct especially in streptococcal and staphylococcal in fections. Conclusion: Intravenous immunoglobulins could be an alternative as adjunct therapy in the management of septic shock in pregnant women who respond poorly to conventional treatment. Analytical studies including pregnant women are needed in order to confirm these findings.
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Feminino , Gravidez , Adulto , Imunoglobulinas Intravenosas , GravidezRESUMO
Morvan's syndrome is a rare autoimmune channelopathy. A case of Morvan's syndrome is presented as a paraneoplastic syndrome associated to the recurrence of a well-differentiated thymic carcinoma, which showed a good clinical response to treatment with intravenous immunoglobulin.
El síndrome de Morvan es una canalopatía rara de origen autoinmune. Se presenta el caso de un síndrome de Morvan como una entidad paraneoplásica asociada a la recurrencia de un carcinoma tímico bien diferenciado, el cual presentó una buena respuesta clínica al tratamiento con inmunoglobulina intravenosa.
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Imunoglobulinas Intravenosas/uso terapêutico , Siringomielia/tratamento farmacológico , Timoma/complicações , Neoplasias do Timo/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Siringomielia/etiologia , Resultado do TratamentoRESUMO
Disseminated encephalomyelitis (ADEM) is an infrequent condition with considerable morbidity and mortality in adult patients. It requires a high level of suspicion and diagnosis emerges by gathering clinical information, laboratory exams and images studies. ADEM is related to an immunological phenomena occurring after a bacterial/viral infection or recent vaccination. Glucocorticoids are the first line treatment, reserving immunoglobulins and plasmapheresis to refractory cases. We report a male patient aged 25, with ADEM associated to parainfluenza 3 virus respiratory infection that required mechanical ventilation and that had a complete recovery only after plasmapheresis.
La encefalomielitis aguda diseminada es una enfermedad infrecuente pero de elevada morbi-mortalidad en pacientes adultos. Demanda una sospecha y diagnóstico precoz que requiere el concurso de información clínica, pruebas de laboratorio y estudio de imágenes. De sustrato inmunológico, se puede relacionar a una infección viral, bacteriana o inmunización reciente. Los glucocorticoides son el tratamiento de elección, mientras que la inmunoglobulina intravenosa y la plasmaféresis se reservan para casos refractarios. Se presenta el caso de una encefalomielitis aguda diseminada grave, en un paciente de sexo masculino de 25 años, asociado a una infección respiratoria por virus parainfluenza 3. Requirió conexión a ventilación mecánica y tuvo una respuesta completa con plasmaféresis.
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Adulto , Humanos , Masculino , Encefalomielite Aguda Disseminada/virologia , Infecções por Respirovirus/complicações , Encefalomielite Aguda Disseminada/terapia , Espectroscopia de Ressonância Magnética , Plasmaferese , Respiração Artificial , Infecções por Respirovirus/terapia , Índice de Gravidade de DoençaRESUMO
The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.
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Queimaduras/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Infecção dos Ferimentos/etiologia , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Ativação de Macrófagos , Masculino , Recidiva , Esteroides/administração & dosagem , Síndrome , Infecção dos Ferimentos/tratamento farmacológico , Adulto JovemRESUMO
El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.(AU)
The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.(AU)
Assuntos
Humanos , Masculino , Adulto Jovem , Queimaduras/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Infecção dos Ferimentos/etiologia , Imunoglobulinas Intravenosas/administração & dosagem , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Ativação de Macrófagos , Recidiva , Esteroides/administração & dosagem , Síndrome , Infecção dos Ferimentos/tratamento farmacológicoRESUMO
El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.
The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.
Assuntos
Humanos , Masculino , Adulto Jovem , Queimaduras/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Infecção dos Ferimentos/etiologia , Imunoglobulinas Intravenosas/administração & dosagem , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Ativação de Macrófagos , Recidiva , Síndrome , Esteroides/administração & dosagem , Infecção dos Ferimentos/tratamento farmacológicoRESUMO
O Pênfigo Foliáceo Endêmico é doença bolhosa autoimune crônica da pele. Geralmente, o tratamento com prednisona tem excelente resposta, mas existem formas refratárias, sendo necessária terapêutica alternativa. Apresentamos paciente adolescente masculino, com diagnóstico clínico-patológico de pênfigo foliáceo, com forma clínica eritrodérmica grave e refratária a várias terapêuticas, que apresentou evolução satisfatória com imunoglobulina intravenosa. Destaca-se, neste relato, o fato de tratar-se de um paciente adolescente que obteve melhora clínica substancial com imunoglobulina intravenosa e remissão completa da doença, após o quarto ciclo da medicação, possibilitando redução da dose do corticoide e de seus efeitos colaterais.
Endemic Pemphigus Foliaceous is a chronic autoimmune bullous skin disease. Treatment with prednisone often produces excellent results, but resistant forms exist, requiring alternative therapy. Alternative treatments have been used in cases of corticosteroid-refractory pemphigus, showing favorable results. This case study focuses on an adolescent male with a clinical-pathological diagnosis of pemphigus foliaceous with a severe clinical form of erythrodermis, unresponsive to multiple therapies, but which showed a satisfactory outcome with intravenous immunoglobulin. In this case we highlight the fact that the patient was a teenager who showed substantial clinical improvement as the result of using intravenous immunoglobulin, followed by complete remission after the fourth cycle of medication, allowing reduced doses of steroids and a consequent reduction of side effects.