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1.
Article in Chinese | WPRIM | ID: wpr-928621

ABSTRACT

OBJECTIVES@#To examine the association between duration of fever before intravenous immunoglobulin (IVIG) treatment and IVIG resistance in children with Kawasaki disease (KD).@*METHODS@#A retrospective analysis was performed on the medical data of 317 children with KD who were admitted from January 2018 to December 2020. According to the duration of fever before IVIG treatment, they were divided into two groups: short fever duration group (≤4 days) with 92 children and long fever duration group (>4 days) with 225 children. According to the presence or absence of IVIG resistance, each group was further divided into a drug-resistance group and a non-drug-resistance group. Baseline data and laboratory results were compared between groups. A multivariate logistic regression analysis was used to identify the influencing factors for IVIG resistance.@*RESULTS@#In the short fever duration group, 19 children (20.7%) had IVIG resistance and 5 children (5.4%) had coronary artery aneurysm, and in the long fever duration group, 22 children (9.8%) had IVIG resistance and 19 children (8.4%) had coronary artery aneurysm, suggesting that the short fever duration group had a significantly higher rate of IVIG resistance than the long fever duration group (P<0.05), while there was no significant difference in the incidence rate of coronary artery aneurysm between the two groups (P>0.05). In the short fever duration group, compared with the children without drug resistance, the children with drug resistance had a significantly lower level of blood sodium and significantly higher levels of procalcitonin, C-reactive protein, and N-terminal B-type natriuretic peptide before treatment (P<0.05). In the long fever duration group, the children with drug resistance had significantly lower levels of blood sodium and creatine kinase before treatment than those without drug resistance (P<0.05). The multivariate logistic regression analysis showed that a reduction in blood sodium level was associated with IVIG resistance in the long fever duration group (P<0.05).@*CONCLUSIONS@#IVIG resistance in children with KD varies with the duration of fever before treatment. A reduction in blood sodium is associated with IVIG resistance in KD children with a duration of fever of >4 days before treatment.


Subject(s)
Child , Coronary Aneurysm/drug therapy , Fever/etiology , Humans , Immunoglobulins, Intravenous/therapeutic use , Infant , Mucocutaneous Lymph Node Syndrome/drug therapy , Retrospective Studies , Sodium/therapeutic use
3.
Arch. argent. pediatr ; 119(4): S198-S211, agosto 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1281043

ABSTRACT

La pandemia ocasionada por el nuevo coronavirus (SARS-CoV-2), declarada por la Organización Mundial de la Salud OMS) en marzo de 2020, afecta a un reducido número de pacientes pediátricos, quienes presentan, en su mayoría, compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos, comenzó a observarse un aumento de casos definidos como síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por el nuevo coronavirus (COVID-19) (KL-C) que evolucionan al shock y requieren internación en la unidad de cuidados intensivos.Los cuadros de SIM-C y los KL-C se caracterizan por fiebre, signos de inflamación, síntomas gastrointestinales y disfunción cardiovascular; las formas graves de presentación tienen mayor incidencia de hipotensión y/o shock. En el laboratorio se observan marcadores de inflamación, hipercoagulabilidad y daño miocárdico. El tratamiento farmacológico de primera línea consiste en la administración de inmunoglobulina por vía intravenosa más ácido acetilsalicílico por vía oral.Se recomienda un abordaje multidisciplinario para un diagnóstico certero y un tratamiento temprano y eficaz para disminuir la morbimortalidad.


The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution.However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KL-C) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit.MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. First-line drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid.A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Systemic Inflammatory Response Syndrome/therapy , COVID-19/therapy , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Aspirin/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Systemic Inflammatory Response Syndrome/complications , Systemic Inflammatory Response Syndrome/diagnosis , Critical Care , Diagnosis, Differential , COVID-19/complications , COVID-19/diagnosis , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy
5.
Arq. bras. cardiol ; 116(3): 485-491, Mar. 2021. tab
Article in English, Portuguese | LILACS | ID: biblio-1248884

ABSTRACT

Resumo Fundamento: A doença de Kawasaki (DK) é a principal causa de cardiopatia adquirida em idade pediátrica nos países desenvolvidos. Objetivos: Identificar fatores preditores de resistência à imunoglobulina intravenosa (IGIV), calcular a eficácia dos modelos preditores japoneses e caracterizar as complicações cardíacas. Métodos: Análise retrospectiva dos casos de DK entre janeiro de 2006 e julho de 2018 em um hospital pediátrico português. Foram construídas curvas ROC para encontrar fatores preditores de resistência e utilizada regressão logística multivariada para elaborar o modelo preditor. O nível de significância utilizado foi de 5%. Resultados: Foram incluídos 48 pacientes com mediana de idade de 36 meses. Verificou-se resistência à IGIV em 21%. Ocorreram alterações ecocardiográficas em 46%, com envolvimento coronário em 25%. Como variáveis preditoras de resistência, a proteína C-reativa (PC-R) apresentou uma AUC ROC = 0,789, ponto de corte = 15,1 mg/dL, sensibilidade (S) = 77,8% e especificidade (E) = 78,9%. A velocidade de sedimentação (VS) apresentou uma AUC ROC = 0,781, ponto de corte = 90,5 mm/h, S = 66,7% e E = 85,7%. O modelo com as duas variáveis apresentou valor p = 0,042 e AUC ROC = 0,790. O modelo Kobayashi apresentou S = 63,6% e E = 77,3%; Egami, S = 66,7% e E = 73,1%; e Sano, S = 28,6% e E = 94,1%. Conclusão: A PC-R e a VS são variáveis independentes que mostraram tendência preditora de resistência à IGIV com pontos de corte ótimos de 15,1 mg/dL e 90,5 mm/h, respectivamente. Cerca de metade dos pacientes teve algum tipo de envolvimento cardíaco. Os modelos japoneses não têm utilidade nessa população. (Arq Bras Cardiol. 2021; 116(3):485-491)


Abstract Background: Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children, in developed countries. Objectives: To identify predictive factors for resistance to intravenous immunoglobulin (IVIG), calculate the effectiveness of Japanese predictive models and characterize cardiac complications. Methods: Retrospective analysis of KD cases admitted in a Portuguese paediatric hospital between january 2006 and july 2018. ROC curves were used to determine predictive factors for resistance and the multivariate logistic regression analysis was used to develop the predictive model. A significance level of 5% was used. Results: 48 patients with a median age of 36 months were included. The IVIG resistance was 21%. Echocardiographic anomalies were noted in 46%, with coronary involvement in 25% of the sample population. As predictive variable of resistance, the C-reactive protein (CRP) presented an AUC ROC = 0.789, optimal cut-off value 15.1 mg/dL, sensitivity (Sn) 77.8% and specificity (Sp) 78.9%. The erythrocyte sedimentation rate (ESR) presented an AUC ROC = 0.781, optimal cut-off value 90.5 mm/h, Sn 66.7% and Sp 85.7%. The model with the two variables showed p = 0.042 and AUC ROC = 0.790. Predictive strength of Japanese models were: Kobayashi (Sn 63.6%, Sp 77.3%), Egami (Sn 66.7%, Sp 73.1%), Sano (Sn 28.6%, Sp 94.1%). Conclusion: CRP and ESR are independent variables that were related to IVIG resistance, with optimal cut-off points of 15.1 mg/dL and 90.5 mm/h, respectively. About half of the patients had some form of cardiac involvement. The Japanese models appeared to be inadequate in our population. (Arq Bras Cardiol. 2021; 116(3):485-491)


Subject(s)
Humans , Infant, Newborn , Child, Preschool , Child , Heart Diseases , Mucocutaneous Lymph Node Syndrome/drug therapy , Drug Resistance , Retrospective Studies , Risk Factors , Immunoglobulins, Intravenous/therapeutic use
7.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 39: e2019338, 2021. tab, graf
Article in English | LILACS, SES-SP | ID: biblio-1136764

ABSTRACT

ABSTRACT Objective: To describe the case of an infant - diagnosed with incomplete Kawasaki disease - who developed BCG scar reactivation. Case description: A 6-month-old patient was admitted to hospital with fever associated with ocular hyperemia, cervical lymphadenopathy, and hyperemic lips, and remained hospitalized for 12 days. The physical examination revealed an inflammatory reaction at the site of the BCG scar, leading to the diagnosis of incomplete Kawasaki disease. The patient was treated with venous immunoglobulin, but presented recurrence of Kawasaki disease, with subsequent onset of coronary artery disease. Comments: BCG scar reactivation is an important finding in countries where the vaccine is routinely given and may be a useful marker for early diagnosis of Kawasaki disease, especially in its incomplete form.


RESUMO Objetivo: Descrever o caso de um lactente - com diagnóstico de Doença de Kawasaki incompleta - que desenvolveu reativação da cicatriz da vacina BCG. Descrição do caso: Um paciente de 6 meses de idade foi admitido no hospital com febre, associada à hiperemia ocular, linfadenomegalia cervical e fissuras labiais, permanecendo hospitalizado por 12 dias. Apresentava, no exame físico, reação inflamatória no local da cicatriz da vacina BCG, tendo sido feito o diagnóstico de Kawasaki incompleto. O paciente foi tratado com imunoglobulina venosa, mas apresentou recorrência da doença, com posterior surgimento de coronariopatia. Comentários: A reativação da BCG é um achado importante na doença de Kawasaki em países onde a vacina é aplicada de forma rotineira e pode ser um marcador útil para o diagnóstico precoce da doença de Kawasaki, principalmente em sua forma incompleta.


Subject(s)
Humans , Male , Infant , BCG Vaccine/immunology , Mucocutaneous Lymph Node Syndrome/diagnosis , Brazil , BCG Vaccine/adverse effects , Biomarkers , Cicatrix/immunology , Cicatrix/pathology , Immunoglobulins, Intravenous/therapeutic use , Mucocutaneous Lymph Node Syndrome/immunology , Mucocutaneous Lymph Node Syndrome/drug therapy
8.
Article in Chinese | WPRIM | ID: wpr-879854

ABSTRACT

Intravenous immunoglobulin (IVIG) has been widely used in chemotherapy for hematological malignancies, targeted therapy, and hematopoietic stem cell transplantation; however, there are still no available guidelines or consensus statements on the application of IVIG in pediatric hematological/neoplastic diseases at present in China and overseas. This consensus is developed based on the research advances in the application of IVIG in pediatric hematological/neoplastic diseases across the world and provides detailed recommendations for the clinical application of IVIG in pediatric hematological/neoplastic diseases and the prevention and treatment of related adverse reactions.


Subject(s)
Child , China , Consensus , Hematologic Neoplasms/drug therapy , Hematopoietic Stem Cell Transplantation , Humans , Immunoglobulins, Intravenous/therapeutic use
10.
Ciudad de México; CENETEC; 19 jun. 2020.
Non-conventional in Spanish | LILACS, BRISA | ID: biblio-1104209

ABSTRACT

CONTEXTO: Debido a la contingencia por COVID-19 provocada por el nuevo coronavirus, SARS-CoV-2, en la actualidad hay una intensa investigación de alternativas terapéuticas que sean seguras y eficaces. (Hay registrados 2208 protocolos de estudios en ClinicalTrials.gov1). Con el propósito de conocer el panorama terapéutico actual contra COVID-19, se realizó una búsqueda exhaustiva de las alternativas que han demostrado cierta eficacia en esta infección, concluyendo que los estudios que se han realizado tienen limitaciones metodológicas. Se trata de estudios no controlados, con alta probabilidad de sesgos que comprometen la validez interna y externa, consideran evidencia indirecta o la experiencia de expertos ante esa emergencia sanitaria, por lo que toda recomendación derivada de estos documentos debe de tomarse con extrema cautela. El uso de esas alternativas debe considerar los riesgos y los beneficios en casos individuales, en una decisión compartida entre médicos, pacientes y familiares ya que la mayoría de la evidencia se considera de baja o muy baja calidad. A la fecha no existe tratamiento específico en contra de este virus. BÚSQUEDA REALIZADA: Inmunoglobulinas intravenosas: Las inmunoglobulinas intravenosas (IgIV) es un grupo de IgG obtenido de donantes sanos, expuestos a enfermedades infecciosas endémicas, vacunas y microorganismos ubicuos que participan en la producción de anticuerpos IgG contra diferentes microorganismos y sus productos. El uso de inmunoglobulina intravenosa se ha


Subject(s)
Humans , Pneumonia, Viral/drug therapy , Ribavirin/therapeutic use , Thymosin/therapeutic use , Ivermectin/therapeutic use , Dexamethasone/therapeutic use , Chloroquine/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Coronavirus Infections/drug therapy , Ritonavir/therapeutic use , Lopinavir/therapeutic use , Darunavir/therapeutic use , Cobicistat/therapeutic use , Sofosbuvir/therapeutic use , Hydroxychloroquine/therapeutic use , Technology Assessment, Biomedical , Health Evaluation
11.
Brasília; s.n; 23 abr. 2020.
Non-conventional in Portuguese | LILACS, BRISA, PIE | ID: biblio-1097412

ABSTRACT

Essa é uma produção do Departamento de Ciência e Tecnologia (Decit) da Secretaria de Ciência, Tecnologia, Inovação e Insumos Estratégicos em Saúde (SCTIE) do Ministério da Saúde (Decit/SCTIE/MS), que tem como missão promover a ciência e tecnologia e o uso de evidências científicas para a tomada de decisão do SUS, tendo como principal atribuição o incentivo ao desenvolvimento de pesquisas em saúde no Brasil, de modo a direcionar os investimentos realizados em pesquisa pelo Governo Federal às necessidades de saúde pública. Informar sobre as principais evidências científicas descritas na literatura internacional sobre tratamento farmacológico para a COVID-19. Além de resumir cada estudo identificado, o informe apresenta também uma avaliação da qualidade metodológica e a quantidade de artigos publicados, de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, entre outros). Foram encontrados 14 artigos.


Subject(s)
Humans , Pneumonia, Viral/drug therapy , Coronavirus Infections/drug therapy , Betacoronavirus/drug effects , Chloroquine/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Tissue Plasminogen Activator/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Disease Progression , Drug Combinations , Hydroxychloroquine/therapeutic use
12.
Clinics ; 75: e2209, 2020. tab
Article in English | LILACS | ID: biblio-1133484

ABSTRACT

OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p<0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p<0.001), vasoactive agent use (83% vs. 3%, p<0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p<0.001), and death (67% vs. 3%, p<0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p<0.001), aspirin therapy (50% vs. 0%, p<0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39-526.79; p<0.0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.


Subject(s)
Humans , Male , Child , Pneumonia, Viral/complications , Pneumonia, Viral/mortality , Coronavirus Infections/complications , Coronavirus Infections/mortality , Coronavirus , Pandemics , Respiration, Artificial , Vomiting/etiology , Abdominal Pain/etiology , Cross-Sectional Studies , Immunoglobulins, Intravenous/therapeutic use , Coronavirus Infections/diagnosis , Coronavirus Infections/therapy , Systemic Inflammatory Response Syndrome/epidemiology , Diarrhea/etiology , Fever/etiology , Betacoronavirus , SARS-CoV-2 , COVID-19 , Glucocorticoids/therapeutic use , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/virology
13.
Rev. méd. Maule ; 34(2): 30-40, dic. 2019. tab
Article in Spanish | LILACS | ID: biblio-1371248

ABSTRACT

Myasthenia gravis is an acquired autoimmune disorder of the neuromuscular junction characterized by fluctuating weakness and fatigability of skeletal muscles. The diagnosis can be established by clinical and serologic testing, with predominance of autoantibodies against the acetylcholine receptor, and Muscle-specific kinase antibodies. We report two cases of Myasthenia gravis, the first one is a 31 year old patient with a debut of the disease, mainly with bulbar symptoms, and the second one is a 29 year old patient diagnosed with generalized Miasthenia Gravis also mainly with bulbar symptoms with worsening of symptomatology. In this report treatments alternatives and management approaches are discused


Subject(s)
Humans , Female , Adult , Myasthenia Gravis/immunology , Myasthenia Gravis/drug therapy , Pyridostigmine Bromide/therapeutic use , Thymectomy , Immunoglobulins, Intravenous/therapeutic use , Glucocorticoids/therapeutic use , Immunosuppressive Agents/therapeutic use , Immunotherapy , Myasthenia Gravis/surgery , Myasthenia Gravis/classification
14.
Rev. chil. infectol ; 36(5): 636-641, oct. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1058090

ABSTRACT

Resumen Introducción: La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda con riesgo de desarrollar aneurismas coronarios. Objetivos: Describir características clínico-epidemiológicas en niños con diagnóstico de EK en Argentina. Analizar factores de riesgo para el desarrollo de complicaciones coronarias (CC). Población y Métodos: Estudio multicéntrico, retrospectivo, transversal, observacional y analítico. Incluyó pacientes bajo 18 años de edad, con diagnóstico de EK en hospitales de Argentina, entre el 1 de enero de 2010 y el 31 de diciembre de 2013. Resultados: N = 193 sujetos. Edad: mediana: 29 meses. Tasa promedio total país 5 casos/10.000 egresos hospitalarios. Presentaron CC 15,5%. Mayor riesgo de CC: Mayor cantidad de días de fiebre al momento de colocación del tratamiento (p = 0,0033); Aumento de: frecuencia cardíaca (p = 0,0021), eritrosedimentación (VSG) (p = 0,005), proteína C reactiva (PCR) (p < 0,0001), leucocitosis (p = 0,0006), neutrofilia (p = 0,0021); Disminución de hematocrito (p = 0,0007) y hemoglobina (p < 0,0001). Asociación con CC: alteraciones cardiológicas no coronarias (ORv10.818); PCR mayor de 68 mg/L (OR = 11.596); leucocitos mayores a 20.000/mm3 (OR= 4.316); y VSG mayor de 64 mm/1° hora (OR = 4.267). Conclusión: La forma de presentación más frecuente fue EK completa, el riesgo de CC fue mayor en varones, menores de 5 años de edad, los factores de riesgo (clínicos y de laboratorio) fueron semejantes a los descritos en la bibliografía.


Background: Kawasaki disease (EK) is an acute systemic vasculitis with a risk of developing coronary aneurysms. Aim: To describe the clinical and epidemiological characteristics of children with EK in Argentina and to analyse the risk factors for the development of coronary's complications (CC). Methods: Multicenter, retrospective, cross-sectional, observational and analytical study. It included patients younger than 18 years of age diagnosed with EK in hospitals in Argentina, between January the 1st, 2010 and December the 31th, 2013. Results: N = 193 subjects. Age: medium: 29 months. Total incidence 5 cases / 10,000 hospital discharges. CC was observed in 15.5% of patients. Increased risk factors for CC: Elevated number of days with fever at the time of treatment placement (p = 0.0033); Increased of: heart frequency (p = 0.0021), erythrosedimentation (ESR) (p = 0.005), C-reactive protein (CRP) (p < 0.0001), leukocytes (p = 0.0006), neutrophils (p = 0.0021); Decreased of hematocrit (p = 0.0007) and hemoglobin (p < 0.0001).Association with CC: non-coronary cardiological alterations (OR = 10,818); PCR greater than 68 mg /L (OR = 11,596); leukocytes greater than 20,000 / mm3 (OR = 4.316); and ESR greater than 64 mm / 1 hour (OR = 4.267). Conclusion: The most frequent form of presentation was complete EK, the risk of CC was higher in males, younger than 5 years old, the risk factors (clinical and laboratory) were similar to those described in the literature.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Coronary Disease/etiology , Coronary Disease/epidemiology , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/epidemiology , Argentina/epidemiology , Seasons , Blood Sedimentation , C-Reactive Protein/analysis , Incidence , Cross-Sectional Studies , Retrospective Studies , Risk Factors , Immunoglobulins, Intravenous/therapeutic use , Statistics, Nonparametric , Risk Assessment , Mucocutaneous Lymph Node Syndrome/therapy
15.
An. bras. dermatol ; 94(3): 264-278, May-June 2019. tab, graf
Article in English | LILACS | ID: biblio-1011108

ABSTRACT

Abstract: Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Pemphigus/diagnosis , Skin/pathology , Autoantibodies/immunology , Surveys and Questionnaires , Pemphigus/classification , Pemphigus/therapy , Pemphigus/epidemiology , Immunoglobulins, Intravenous/therapeutic use , Desmosomes/immunology , Diagnosis, Differential , Immunosuppressive Agents/classification , Immunosuppressive Agents/therapeutic use , Immunotherapy/methods
16.
Yonsei Medical Journal ; : 113-118, 2018.
Article in English | WPRIM | ID: wpr-742496

ABSTRACT

PURPOSE: Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease (KD). However, there is still no standard treatment for IVIG-resistant KD. This study aimed to evaluate the efficacy of low-dose methotrexate (MTX) as a treatment for IVIG-resistant KD. MATERIALS AND METHODS: We retrospectively analyzed 10-year data for patients with IVIG-resistant KD who were administered MTX at Severance Children's Hospital. RESULTS: The subjects included 75 patients with KD aged 5 months to 9.2 years who had been administered MTX. Their maximum body temperatures decreased significantly within 24 h of therapy. The patients' C-reactive protein levels were significantly lower 1 week after administering the first dose of MTX than those before treatment. No adverse effect for MTX was observed. CONCLUSION: MTX treatment of IVIG-resistant KD resulted in rapid defervescence, improvement of clinical symptoms, and normalization of acute-phase reactants in all patients. Thus, MTX could be a candidate treatment for IVIG-resistant KD.


Subject(s)
C-Reactive Protein/analysis , Child , Child, Preschool , Coronary Vessels/pathology , Demography , Dose-Response Relationship, Drug , Drug Therapy, Combination , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Infant , Male , Methotrexate/administration & dosage , Methotrexate/therapeutic use , Mucocutaneous Lymph Node Syndrome/blood , Mucocutaneous Lymph Node Syndrome/drug therapy , Retrospective Studies , Steroids/therapeutic use , Treatment Outcome
17.
Rev. Col. Méd. Cir. Guatem ; 156(1): 7-10, 2017 jul. Tab
Article in Spanish | LILACS | ID: biblio-908630

ABSTRACT

Antecedentes: La enfermedad de Kawasaki es una vasculitis sistémica aguda que involucra principalmente vasos de mediano calibre. Se ha convertido en la primera causa de enfermedad cardiaca adquirida en niños. Su frecuencia es mayor en <5 años. No se conoce agente etiológico. En Guatemala no ha sido descrita previamente. Objetivo: Determinar la presentación clínica, diagnóstico, tratamiento y resultados en papeletas de los niños de 0 a 12 años diagnosticados con enfermedad de Kawasaki en Guatemala. Resultados: Durante el período enero 2003 a diciembre 2013 se identificaron 26 casos diagnosticados como enfermedad de Kawasaki en el grupo de estudio. Todos los casos a excepción de 1 fueron en menores de 5 años. Todos los pacientes tenían historia de fiebre, aunque solo 11 la presentaron al ingreso al hospital.


Subject(s)
Humans , Infant , Child, Preschool , Child , Aspirin/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/drug therapy , Epidemiology, Descriptive , Cross-Sectional Studies , Guatemala/epidemiology
18.
Arq. neuropsiquiatr ; 75(3): 142-146, Mar. 2017. tab, graf
Article in English | LILACS | ID: biblio-838885

ABSTRACT

ABSTRACT The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia.


RESUMO A enzima ácido glutâmico descarboxilase (GAD), presente nos neurônios GABAérgicos e células beta do pâncreas, catalisa a conversão do ácido gama-aminobutírico (GABA). O cerebelo é altamente susceptível a mecanismos imunomediados, sendo a ataxia cerebelar associada ao anticorpo anti-GAD (CA-GAD) uma doença potencialmente tratável. Embora rara, sua frequência é crescente, com poucos casos descritos. Métodos Estudo retrospectivo e descritivo avaliando características clínicas e desfechos da CA-GAD. Resultados Três pacientes com CA-GAD, altos títulos de anti-GAD e doença endócrina autoimune foram identificados. Os pacientes 1 e 2 tinham síndrome da pessoa rígida em forma clássica e apresentação insidiosa da ataxia cerebelar, enquanto o paciente 3 tinha ataxia cerebelar pura e apresentação subaguda. Os pacientes 1 e 3 não melhoraram com imunoglobulina intravenosa e o paciente 2 teve recuperação parcial. Conclusão A CA-GAD é rara e pode ter apresentação clínica desafiadora. Os médicos devem ser capazes de reconhecer essa forma potencialmente tratável de ataxia cerebelar autoimune.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Autoantibodies/blood , Cerebellar Ataxia/complications , Glutamate Decarboxylase/blood , Magnetic Resonance Imaging , Cerebellar Ataxia/diagnosis , Cerebellar Ataxia/immunology , Cerebellar Ataxia/drug therapy , Retrospective Studies , Treatment Outcome , Immunoglobulins, Intravenous/therapeutic use , Glutamate Decarboxylase/immunology
19.
Santiago; Chile. Ministerio de Salud; 2017. tab.
Monography in Spanish | LILACS, BRISA | ID: biblio-882744

ABSTRACT

INTRODUCCIÓN: Las inmunodeficiencias primarias conforman un grupo de distintas patologías, que comprometen la inmunidad humoral o celular, resultando en un aumento de la susceptibilidad a infecciones. En general, no existe una terapia curativa, no obstante se administra tratamiento antibiótico profiláctico, y el uso de inmunoglobulinas. Dentro de los 7 grandes grupos de inmunodeficiencias, son de particular importancia los pacientes diagnosticados con inmunodeficiencia común variable (ICV) y con agammaglobulinemia autosómica recesiva o ligada al cromosoma X (ALX), por ser las patologías más prevalentes dentro de este grupo. TECNOLOGÍAS SANITARIAS ANALISADAS: Inmunoglobulina Humana subcutánea. EFICACIA DE LOS TRATAMIENTOS: No se encontró evidencia sobre la eficacia de la Inmunoglobulina humana (IgG) en pacientes con ICV o ALX, en comparación a placebo u otro tratamiento. Sin embargo, se utilizaron 3 revisiones sistemáticas, las cuales incluyeron evidencia de 6 estudios de cohorte, comparando el uso de IgG subcutánea (IGSC) contra su uso intravenoso (IGIV) en niños y adultos con ICV y ALX. Los resultados de estos estudios muestran que es incierto si la IGSC produzca un cambio en la IgG en suero inferior y en la tasa de infecciones severas, en comparación a la IGIV, en niños y adultos con ICV y ALX. Esto debido a que la certeza en la evidencia es muy baja. Además, no se encontró evidencia que comparara IGIV con IGSC en el uso de antibióticos y la hospitalización, en pacientes con ICV y ALX, mientras que tampoco se encontró evidencia en la tasa de infecciones severas en pacientes con ICV. No se encontraron Ensayos Controlados Aleatorizados (ECAs) que evaluaran la eficacia de la IgG (ni su forma de administración) en población con ICV o ALX. ANÁLISIS ECONÓMICO: No se evaluó esta dimensión, en conformidad con el Título III De las Evaluaciones Favorables de la Norma Técnica N° 0192 del Ministerio de Salud, sobre el proceso de evaluación científica de la Evidencia establecido en el artículo 7° de la ley N°20.850.. CONCLUSIÓN: Para dar cumplimiento al artículo 28° del Reglamento que establece el proceso destinado a determinar los diagnósticos y tratamientos de alto costo con Sistema de Protección Financiera, según lo establecido en los artículos 7°y 8° de la ley N°20.850, aprobado por el decreto N°13 del Ministerio de Salud, se concluye que el presente informe de evaluación se considera no favorable, dado que la evidencia presentada es de certeza muy baja, de acuerdo a lo establecido en el Título III. de las Evaluaciones Favorables de la Norma Técnica N° 0192 de este mismo Ministerio.


Subject(s)
Humans , Agammaglobulinemia/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Immunologic Deficiency Syndromes/drug therapy , Technology Assessment, Biomedical
20.
Medicina (B.Aires) ; 76(1): 36-39, feb. 2016. ilus, graf, tab
Article in Spanish | LILACS | ID: biblio-841537

ABSTRACT

La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad adquirida que puede afectar a raíces, plexos y nervios periféricos. A pesar de su baja incidencia, su diagnóstico cobra especial relevancia dado que actualmente existen tratamientos efectivos para la misma. La gammaglobulina humana endovenosa (IVIgG) es, junto con los esteroides y la plasmaféresis, uno de los tratamientos de primera elección. La vía de administración subcutánea se ha propuesto como una alternativa novedosa frente a la administración endovenosa con una eficacia similar. Presentamos tres casos de CIDP definitiva, clasificados según los criterios de la European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) en los cuales se utilizó tratamiento crónico con inmunoglobulina subcutánea (IgSC). Todos ellos habían recibido tratamiento previo con IVIgG. Se obtuvo mejoría de la fuerza evaluada por Overall Neuropathy Limitations Scale (ONLS) y los tres pacientes manifestaron una mejor adaptación a sus actividades de la vida diaria.


Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disease that may affect nerve roots and peripheral nerves. Despite its low incidence, diagnosis is particularly important because there are different effective treatments. Human immunoglobulin is one of the mainstays of the treatment. Although there are few studies up to date, subcutaneous immunoglobulin (IgSC) has been proposed as an alternative to intravenous administration with similar efficacy. We present three cases with definite CIDP, classified according to the European Federation of Neurological Societies / Peripheral Nerve, Society (EFNS /PNS) criteria in which was used SCIgG as a treatment after success with the intravenous route. The Overall Neuropathy Limitations Scale (ONLS) was used to estimate the changes in the muscular strength before and after treatment.


Subject(s)
Humans , Male , Adult , Aged , Immunoglobulins/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy , Immunoglobulins/administration & dosage , Magnetic Resonance Imaging , Treatment Outcome , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Injections, Subcutaneous
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