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1.
Rev. colomb. obstet. ginecol ; 72(2): 210-218, Apr.-June 2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1289318

ABSTRACT

Resumen Objetivo: Reportar el caso de una paciente gestante con síndrome de Guillain-Barré (SGB) presentado en la variante denominada síndrome de Miller Fisher (SMF), y realizar una revisión en torno al diagnóstico, tratamiento y pronóstico de esta variedad de SGB durante la gestación. Materiales y métodos: Se presenta el caso de una gestante de 27 semanas con síndrome de Miller Fisher, quien fue tratada con plasmaféresis en un hospital militar de referencia, con evolución satisfactoria a los 15 días y continuación normal del embarazo, parto a las 38 semanas con recién nacido sano. Se realizó una búsqueda bibliográfica en bases de datos electrónicas: Medline vía PubMed, Lilacs, SciELO, ScienceDirect, Ovid, con los términos "Embarazo", "Síndrome de Miller Fisher", "Síndrome de Guillain-Barré". Se incluyeron cohortes, series y reportes de casos de mujeres gestantes con síndrome de Miller Fisher; se extrajo información sobre los métodos diagnósticos, el tratamiento utilizado y el pronóstico materno y perinatal. La búsqueda se hizo en junio de 2020, sin restricción por fecha, pero sí por tipo de idioma (español e inglés). Resultados: Se identificaron 423 títulos, tres estudios cumplieron los criterios de inclusión, los tres correspondieron a reportes de caso. Todos los casos mostraron seropositividad para antigangliósidos GQ1b positivos; en ningún caso hubo alteración imagenológica. Dos pacientes recibieron inmunoglobulina intravenosa y la tercera paciente se dejó en observación. Hasta el momento no se documentan complicaciones obstétricas. Conclusión: Existen pocos casos reportados de SMF durante la gestación, el diagnóstico se basa en el examen clínico; el tratamiento con inmunoglobulina IV representa la alternativa utilizada con mayor frecuencia. En el caso presentado se utilizó la plasmaféresis. Se desconoce el impacto de la variedad del síndrome de Miller Fisher sobre el curso normal de la gestación y sobre los resultados perinatales a largo plazo. Se requieren más estudios que aborden el diagnóstico, el tratamiento y el pronóstico de esta entidad.


Abstract Objective: To report the case of pregnant woman with Guillain-Barré syndrome (GBS) presenting as the Miller Fisher variant, and to review the literature on the diagnosis, treatment and prognosis of this GBS variant during gestation. Materials and Methods: Pregnant woman presenting at 27 weeks of gestation with Miller Fisher syndrome (MFS), treated in a military referral hospital with a satisfactory course after 15 days, continuation of normal pregnancy and delivery of a healthy neonate at 38 weeks. A search of the literature was conducted in the Medline via PubMed, Lilacs, SciELO, ScienceDirect and Ovid databases using the terms "Pregnancy," "Miller Fisher syndrome," "Guillain-Barré syndrome". Cohorts, case series and case reports of pregnant women with MFS were included. Data on diagnostic methods, treatment and maternal and perinatal prognosis were extracted. The search was made on June 2020, with no restriction by date, but restriction by language (Spanish and English). Results: Overall, 423 titles were identified, three studies met the inclusion criteria, the three of them corresponding to case reports. All cases were found to be seropositive for anti-GQ1b ganglioside antibodies. No imaging abnormalities were found in any of the cases. Two patients received IV immunoglobulin and the third patient was kept under observation. No obstetric complications have be documented so far. Conclusion: There are few cases of MFS reported during pregnancy. Intravenous immunoglobulin is the most frequently used treatment option. Plasmapheresis was used in the case presented here. The impact of the Miller Fisher variant on the normal course of gestation and on long-term perinatal outcomes is unknown. Further studies that look into the diagnosis, treatment and prognosis of this condition are required.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Miller Fisher Syndrome , Guillain-Barre Syndrome , Pregnancy , Plasmapheresis
2.
Rev. cuba. hematol. inmunol. hemoter ; 37(2): e1235, 2021. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1289431

ABSTRACT

Introducción: La púrpura trombocitopénica trombótica puede presentarse en menos del 2 por ciento de los pacientes con lupus eritematoso sistémico. Esta asociación implica un aumento de la mortalidad y un periodo de remisión más prolongado. Objetivo: Se presenta el caso de paciente peruana que desarrolló esta asociación y presentó complicaciones relacionadas con shock séptico. Caso clínico: Paciente femenina, con antecedente de púrpura trombocitopénica inmunológica y lupus eritematoso sistémico, acudió a emergencia por presentar palidez cutánea generalizada, petequias en miembros inferiores y hematuria. Posteriormente, su estado de salud se complicó con un shock séptico y deterioro del nivel de conciencia. Por todo esto, es referida a un hospital de mayor complejidad y hace su ingreso a la unidad de cuidados intensivos. La clínica y los exámenes de laboratorio revelaron hallazgos compatibles con púrpura trombocitopénica trombótica (anemia grave, plaquetopenia, esquistositosis) y lupus eritematoso sistémico activo grave. Antes de ser referida, recibió pulsos de metilprednisona y prednisona. Ya en unidad de cuidados intensivos, se cambió a soporte ventilatorio y tratamiento antibiótico. Con el diagnóstico presuntivo de púrpura trombocitopénica trombótica, asociada a lupus eritematoso sistémico activo grave, se inició tratamiento oportuno con plasmaféresis, corticoterapia y ciclofosfamida. La paciente recuperó los niveles plaquetarios y el nivel óptimo de conciencia. Actualmente acude a controles. Conclusiones: La púrpura trombocitopénica trombótica es una emergencia hematológica con alta mortalidad en ausencia de tratamiento. Su reconocimiento oportuno, sin dosificación de la proteína ADAMTS13, en esta asociación poco frecuente con lupus eritematoso sistémico es importante en el buen pronóstico del paciente(AU)


Introduction: Thrombotic thrombocytopenic purpura may occur in less than 2 percent of patients with systemic lupus erythematosus. This association implies an increase in mortality and a longer remission period. Objective: We present the case of a Peruvian woman who developed this association, and complicating herself with septic shock. Clinical case: A female patient, with a history of immunological thrombocytopenic purpura and systemic lupus erythematosus, comes to the emergency room due to generalized skin pallor, lower limb petechiae and hematuria. Subsequently, her state of health gets complicated with a septic shock and deterioration of the level of consciousness. For all of this, she was referred to a hospital of greater complexity and makes admission to an intensive care unit. Clinical and laboratory tests revealed findings compatible with thrombotic thrombocytopenic purpura (severe anemia, platelet disease, schistositosis) and severe active systemic lupus erythematosus. Before being referred, she received pulses of methylprednisone and prednisone. When already in the intensive care unit, it was changed to ventilatory support andantibiotic treatment. With the presumptive diagnosis of thrombotic thrombocytopenic purpura, associated with severe active systemic lupus erythematosus, a timely treatment was initiated with plasmapheresis, corticosteroids and cyclophosphamide. The patient recovered platelet levels and optimal level of consciousness. She is currently going to controls. Conclusions: Thrombotic thrombocytopenic purpura is a hematological emergency with high mortality in the absence of treatment. Its timely recognition, without dosing of ADAMTS13 protein, in this rare association with systemic lupus erythematosus is important in the good prognosis of the patient(AU)


Subject(s)
Humans , Female , Purpura, Thrombocytopenic/complications , Plasmapheresis/methods , Intensive Care Units , Lupus Erythematosus, Systemic/complications , Purpura, Thrombocytopenic/drug therapy
3.
Arq. bras. med. vet. zootec. (Online) ; 72(6): 2017-2026, Nov.-Dec. 2020. tab, graf
Article in English | LILACS, VETINDEX | ID: biblio-1142308

ABSTRACT

Separation techniques of seminal plasma [centrifugation (SC) and Sperm Filter® (SF)] and sperm selection [Androcoll-E (SCA) and filtration glass wool (GW)] were used in 24 ejaculates from 6 stallions. In experiment 1, the ejaculates were allocated into control (no spin), centrifugation at 600 g x 10min, SF and GW. In experiment 2, semen was submitted to SC, SGA and filtered through GW. Following the treatments in both experiments, samples were kept chilled at 5°C to 50 x 106 sperm/ml for 48h. The variables measured on fresh and cooling semen were pH, motility, membrane viability function by 6-carboxyfluorescein diacetate and propidium iodide (CFDA / PI), viability or vitality (eosin / nigrosine) and mitochondrial activity. In experiment 1, centrifugation to remove seminal plasma resulted in greater damage to sperm than separation by sperm filter, and selection by glass wool was more efficient in separating viable cells and maintaining viability during cooling. In experiment 2 Androcoll-E and glass wool treatments resulted in higher (P <0.0001) motility, membrane function, mitochondrial activity, and viability than centrifuged semen. Both selection by Androcoll- E and glass wool improved the quality of semen pony stallions for preservation for up to 48h to 5ºC.(AU)


As técnicas de separação do plasma seminal (centrifugação, SpermFilter) e de seleção espermática (Androcoll-E e filtração por lã de vidro) foram aplicadas em 24 ejaculados de seis garanhões da raça Pônei Brasileiro. Após coleta e separação da fração gel, os ejaculados foram diluídos 1:1 com diluente à base de leite em pó. No experimento 1, os ejaculados foram distribuídos em controle (sem centrifugação), centrifugação a 600g x 10min, SpermFilter e filtração por lã de vidro. No experimento 2, o sêmen foi submetido aos procedimentos: centrifugado (SC), centrifugado com Androcoll-E e filtrado por lã de vidro. Após os procedimentos de ambos os experimentos, as amostras foram mantidas refrigeradas a 5ºC, com 50 x 106 espermatozoides/mL, por 48h. As variáveis mensuradas a fresco, 24h e 48h foram: pH, motilidade, funcionalidade de membrana, viabilidade por diacetato de carboxifluoresceína e iodeto de propídio (CFDA/PI, vitalidade (eosina/nigrosina) e atividade mitocondrial. Já osmolaridade e morfologia espermática foram avaliadas somente imediatamente após a coleta. No experimento 1, a centrifugação para retirada do plasma seminal resultou em maiores danos aos espermatozoides do que a separação por SpermFilter. A filtração por lã de vidro mostrou-se mais eficiente em separar células viáveis e manter a viabilidade durante o resfriamento. No experimento 2, os tratamentos com Androcoll-E e filtrado por lã de vidro foram superiores (P<0,0001) ao sêmen centrifugado quanto à motilidade, à funcionalidade de membrana, à atividade mitocondrial e à viabilidade, tanto nas amostras de sêmen fresco como de sêmen refrigerado. O Androcoll-E e a lã de vidro permitiram manter por 48h, a 5ºC, o sêmen de garanhões pôneis utilizando-se diluente à base de leite.(AU)


Subject(s)
Animals , Male , Semen/cytology , Plasmapheresis/methods , Plasmapheresis/veterinary , Horses , Osmolar Concentration , Centrifugation/veterinary
5.
San Salvador; Ministerio de Salud; Primera; 27/07/2020. 32 p.
Non-conventional in Spanish | LILACS, BISSAL | ID: biblio-1095306

ABSTRACT

A finales del 2019 y principios de 2020, la humanidad se enfrenta una pandemia por el virus SARS-CoV2 que causa un síndrome respiratorio agudo grave (SARS por sus siglas en inglés) en el contexto de la enfermedad por coronavirus, (COVID-19). Al momento el SARS-CoV-2 se ha extendido en todo el mundo, se ha catalogado como pandemia, con una cifra de infectados que sobrepasa los 2 millones de personas según cifras oficiales. Actualmente no hay vacunas, anticuerpos monoclonales, o medicamentos disponibles para el SARSCoV-2, por tal razón el plasma humano convaleciente es una opción para la prevención y tratamiento del COVID-19, que podría estar rápidamente disponible cuando haya un número suficiente de personas recuperadas y puedan donar plasma que contiene inmunoglobulinas. La obtención de "Plasma de paciente convaleciente" el cual es obtenida a través del procedimiento plasmaféresis, el cual es un procedimiento extracorpóreo, en el cual a partir de la sangre extraída del paciente se procede a separarla en sus componentes plasma y elementos celulares. Constituye una variedad de aféresis, y su objetivo principal es remover elementos específicos del plasma, los cuales se consideran que son mediadores de procesos patológicos.


Subject(s)
Humans , Plasmapheresis , Coronavirus
6.
San Salvador; MINSAL; 2 ed; jul. 21, 2020. 32 p. ilus.
Non-conventional in Spanish | LILACS, BISSAL | ID: biblio-1293345

ABSTRACT

Los presentes lineamientos establecen las disposiciones para la utilización de plasma convaleciente en pacientes con COVID-19, en los hospitales del Sistema Nacional Integrado de Salud (SNIS), para disminuir las complicaciones y mortalidad en pacientes que se encuentran en estado de severidad, de moderado a grave por COVID-19. Y así definir los mecanismos a implementar en la red de captación de plasma convaleciente, directrices para la evaluación clínica en el banco de sangre, el proceso de plamaferesis, así como la utilización clínica del plasma convaleciente en dichos pacientes


These guidelines establish the provisions for the use of convalescent plasma in patients with COVID-19, in the hospitals of the Integrated National Health System (SNIS), to reduce complications and mortality in patients who are in a state of severity, moderate severe from COVID-19. And thus define the mechanisms to be implemented in the convalescent plasma uptake network, guidelines for clinical evaluation in the blood bank, the plasmapheresis process, as well as the clinical use of convalescent plasma in said patients


Subject(s)
Patients , Plasma , COVID-19 , Blood , Plasmapheresis
7.
Rev. colomb. gastroenterol ; 35(2): 226-231, abr.-jun. 2020. tab
Article in Spanish | LILACS | ID: biblio-1126313

ABSTRACT

Resumen Introducción: la pancreatitis aguda es una entidad de alta incidencia e impacto a nivel mundial. Presenta múltiples causas dentro de las cuales las más frecuentes son la obstrucción de la vía biliar, el consumo de alcohol y, en tercer orden, la hipertrigliceridemia. Esta última se entiende como aquellos niveles séricos de triglicéridos >1000 mg/dL. Dicho escenario representa entre el 1 y el 7 % del total de los casos. Metodología: presentamos un caso de pancreatitis aguda secundaria a hipertrigliceridemia severa, manejada con plasmaféresis. Se realiza una revisión de la literatura sobre las condiciones, indicaciones y ventajas de esta estrategia terapéutica. Conclusiones: en casos escogidos, la plasmaféresis es una estrategia de manejo segura y efectiva en el tratamiento de pacientes con pancreatitis aguda secundaria a hipertrigliceridemia severa.


Abstract Introduction: Globally, acute pancreatitis has a high incidence and a large. Among its numerous causes, the most frequent are obstructions of the bile duct, alcohol consumption and hypertriglyceridemia (triglyceride serum levels higher than 1000 mg/dL). Hypertriglyceridemia accounts for 1% to 7% of the total cases. Methodology: We present a case of acute pancreatitis secondary to severe hypertriglyceridemia which was managed with plasmapheresis. We include a review of the literature on the conditions, indications and advantages of this therapeutic strategy. Conclusions: In selected cases, plasmapheresis is a safe and effective management strategy for patients with acute pancreatitis secondary to severe hypertriglyceridemia.


Subject(s)
Humans , Male , Adult , Pancreatitis , Therapeutics , Hypertriglyceridemia , Plasmapheresis , Incidence , Literature
8.
s.l; s.n; 4 maio 2020. ilus.
Non-conventional in Portuguese | LILACS, BRISA | ID: biblio-1099490

ABSTRACT

CONTEXTO: Conforme classificação da OMS, a COVID-19 é uma pandemia de risco muito alto a nível global. Até o momento não existem terapias específicas para a doença, embora diferentes alternativas, incluindo o uso de plasma de pacientes recuperados, estejam em investigação. OBJETIVOS: Identificar, avaliar sistematicamente e sumarizar as melhores evidências científicas disponíveis sobre a eficácia e a segurança do uso de plasma de pacientes recuperados para COVID-19. MÉTODOS: Revisão sistemática rápida (rapid review methodology). RESULTADOS: Após o processo de seleção, 55 estudos foram incluídos: 15 estudos com resultados e 40 estudos clínicos em andamento. Os estudos evidenciaram que o plasma de paciente recuperado para tratamento de SARS-CoV-2 resultou em redução de mortalidade, carga viral e tempo de internação hospitalar. Entretanto, estes estudos têm risco de viés moderado a alto e os estudos clínicos têm amostra pequena. Estes fatores contribuíram para que a certeza na evidência fosse muito baixa. CONCLUSÃO: Esta revisão sistemática rápida identificou 15 estudos com qualidade metodológica baixa a moderada, que avaliaram os efeitos da terapia com plasma de pacientes recuperados para COVID-19. Com base nos achados destes estudos, a eficácia e a segurança do plasma de pacientes recuperados em pacientes com COVID-19 ainda são incertas e seu uso de rotina, para esta situação, não pode ser recomendado, até que resultados de ensaios clínicos em andamento possam ser avaliados.(AU)


Subject(s)
Humans , Pneumonia, Viral/drug therapy , Plasmapheresis/instrumentation , Coronaviridae Infections/drug therapy , Betacoronavirus/drug effects , Technology Assessment, Biomedical , Cost-Benefit Analysis
9.
Brasília; s.n; 13 maio 2020.
Non-conventional in Portuguese | PIE, LILACS, BRISA, PIE | ID: biblio-1097393

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 15 artigos e 10 protocolos.


Subject(s)
Pneumonia, Viral/drug therapy , Coronavirus Infections/drug therapy , Betacoronavirus/drug effects , Renin-Angiotensin System , Ribavirin/therapeutic use , Steroids/therapeutic use , Technology Assessment, Biomedical , Methylprednisolone/therapeutic use , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Sulbactam/therapeutic use , Cefoperazone/therapeutic use , Chloroquine/therapeutic use , Plasmapheresis/instrumentation , Adrenal Cortex Hormones/therapeutic use , Azithromycin/therapeutic use , Disease Progression , Ritonavir/therapeutic use , Lopinavir/therapeutic use , Glucocorticoids/therapeutic use , Hydroxychloroquine/therapeutic use , Anticoagulants/therapeutic use , Antimalarials/therapeutic use
10.
Med. lab ; 24(1): 24-57, 2020.
Article in Spanish | LILACS, COLNAL | ID: biblio-1097020

ABSTRACT

El trasplante renal constituye la mejor opción de tratamiento para los pacientes con enfermedad renal crónica terminal. La supervivencia del injerto es de gran importancia y puede ser afectada por factores inmunológicos o no inmunológicos; esto unido al número de pacientes en las listas de espera, hace necesario definir estrategias de manejo que permitan tener mejores resultados a largo plazo. Objetivo. Determinar las características clínicas y humorales, y los desenlaces en receptores de trasplante renal o combinado hígado-riñón, altamente sensibilizados, que recibieron profilaxis combinada con inmunoglobulina intravenosa y plasmaféresis en el Hospital San Vicente Fundación, en Colombia. Materiales y métodos. Se realizó un estudio retrospectivo, observacional, descriptivo, que incluyó los pacientes trasplantados entre el 4 de julio de 2010 y el 19 de abril de 2017. Como variables se incluyeron, entre otras, la etiología de la enfermedad renal crónica, el tipo de terapia recibida, y el tiempo en lista de espera en días. Como desenlace se evaluó la presencia de rechazo, el tipo de rechazo, la pérdida del injerto, las complicaciones y la muerte. Resultados. Del total de 25 pacientes, el 100% recibió inmunoglobulina intravenosa y el 84% plasmaféresis. El 12% presentó rechazo del injerto, todos de tipo humoral, y el 20% perdió el injerto. Discusión. A pesar de la gran variedad de protocolos propuestos en la literatura, en esta población especial no se ha establecido un protocolo óptimo de inmunosupresión. El protocolo en nuestra pequeña cohorte no tuvo un impacto negativo en el porcentaje de infecciones postrasplante ni en la pérdida del injerto renal, pero sí redujo el tiempo en las listas de espera; por lo tanto, se requieren estudios adicionales para confirmar los hallazgos encontrados en este estudio


Kidney transplantation is the best treatment option for patients with terminal chronic kidney disease, regardless of the etiology, making graft survival an important feature, which may be affected by immunological or non-immunological factors. This, added to the increasing number of patients on waiting lists, makes it necessary to define management strategies for these patients that allow better long-term results. Objectives. To determine the clinical, humoral and outcome characteristics in highly sensitized recipients of kidney and simultaneous kidneyliver transplant who received combined prophylaxis with intravenous immunoglobulin and plasmapheresis therapy in a Colombian medical center. Materials and methods. A retrospective, observational, descriptive study was carried out that included the transplanted patients between July 4, 2010 and April 19, 2017. Variables included the etiology of chronic kidney disease, the type of therapy received, and waiting time in days, among others. As outcomes, the presence of rejection, type of rejection, graft loss, complications and death were evaluated. Results. From a total of 25 patients, 100% received intravenous immunoglobulin and 84% plasmapheresis. Twelve percent presented graft rejection, all humoral, and 20% lost the graft. Discussion. Despite the great variety of protocols proposed in the literature, an optimal immunosuppression protocol has not been established for this particular population. The protocol in our small cohort did not have a negative impact on the percentage of post-transplant infections nor in the loss of the renal graft, but it did reduce waiting time; therefore, additional studies are required to confirm the findings in this study


Subject(s)
Kidney Transplantation , Plasmapheresis , Complement Activation , Graft Rejection
11.
In. Verga, Federico; Burghi, Gastón. Encares de paciente crítico. Montevideo, Oficina del Libro FEFMUR, 2020. p.119-133, tab.
Monography in Spanish | LILACS, BNUY, UY-BNMED | ID: biblio-1342643
12.
J. bras. nefrol ; 41(2): 296-299, Apr.-June 2019. graf
Article in English | LILACS | ID: biblio-1012529

ABSTRACT

ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.


RESUMO Introdução: Purpura Fulminans (PF) é uma doença trombótica de rápida progressão, com infarto hemorrágico da pele e coagulação intravascular disseminada (CIVD). É potencialmente causadora de injúria renal aguda (IRA). Porém, não há descrição na literatura médica dos achados histológicos renais causados por PF. Relato de caso: Mulher, 20 anos, previamente hígida, hospitalizada por odinofagia, febre, mialgia generalizada e anúria, evoluiu com aparecimento de placas purpúricas em face e membros. Necessitou de hemodiálise (HD) já na admissão. Exames laboratoriais mostravam anemia, leucocitose, plaquetopenia e elevação de desidrogenase lática. As lesões purpúricas tornaram-se bolhosas com rompimento e progressão para necrose, se aprofundaram, atingindo derme, subcutâneo e musculatura, até a exposição óssea. Não houve melhora com antibioticoterapia inicial voltada para tratamento de meningococemia. Suspeitou-se, então, de microangiopatia trombótica (MAT) e PF. A paciente permaneceu em HD diária e necessitou também de plasmaférese, após melhora sustentada da plaquetopenia, foi submetida à biópsia renal, que não foi compatível com MAT, possivelmente caracterizando PF. Houve recuperação completa da função renal e as sequelas cutâneas foram tratadas com enxerto. Conclusão: Em casos nos quais os fenômenos trombóticos e hemorrágicos se sobrepõem, a obtenção da biópsia renal se torna difícil. Neste caso, a biópsia permitiu excluir IRA causada por MAT e mostrar, pela primeira vez, achados compatíveis com PF.


Subject(s)
Humans , Female , Young Adult , Purpura Fulminans/complications , Purpura Fulminans/diagnosis , Thrombotic Microangiopathies/complications , Thrombotic Microangiopathies/diagnosis , Acute Kidney Injury/complications , Acute Kidney Injury/pathology , Kidney/pathology , Biopsy , Renal Dialysis , Plasmapheresis , Skin Transplantation , Treatment Outcome , Acute Kidney Injury/therapy , Length of Stay
13.
Rev. med. Rosario ; 85(1): 17-19, ene.-abr. 2019. graf
Article in Spanish | LILACS | ID: biblio-1052376

ABSTRACT

El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)


Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Glomerulonephritis/diagnosis , Glomerulonephritis/therapy , Granulomatosis with Polyangiitis , Immunosuppression , Plasmapheresis , Anti-Glomerular Basement Membrane Disease/diagnosis , Renal Insufficiency, Chronic , Lupus Erythematosus, Systemic
14.
Article in Korean | WPRIM | ID: wpr-766753

ABSTRACT

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.


Subject(s)
Antibodies , Asian Continental Ancestry Group , Axons , Complement System Proteins , Gangliosides , Guillain-Barre Syndrome , Hope , Humans , Immunoglobulins , Immunotherapy , Miller Fisher Syndrome , Models, Animal , Molecular Mimicry , Mortality , Plasmapheresis , Polyneuropathies , Prognosis
15.
Article in Chinese | WPRIM | ID: wpr-775075

ABSTRACT

OBJECTIVE@#To study the clinical effect and safety of double filtration plasmapheresis (DFPP) combined with double pulse therapy with methylprednisolone (MP) and cyclophosphamide (CTX) in the treatment of children with severe Henoch-Schönlein purpura nephritis (HSPN).@*METHODS@#A total of 60 children with severe HSPN who were admitted to the hospital from January 2014 to March 2018 were enrolled and were randomly divided into an observation group and a control group (n=30 each). In addition to routine treatment, the children in the control group were given MP+CTX pulse therapy. Those in the observation group were given DFPP treatment in addition to the treatment in the control group, with three courses of treatment in total. After three courses of treatment, the two groups were compared in terms of 24-hour urinary protein, urinary microproteins, renal function parameters, adverse reactions, and clinical outcome.@*RESULTS@#After three courses of treatment, the observation group had significantly greater reductions in 24-hour urinary protein, urinary albumin, urinary immunoglobulin G, urinary β2-microglobulin, serum creatinine, and blood urea nitrogen than the control group (P0.05).@*CONCLUSIONS@#Compared with MP+CTX pulse therapy alone in the treatment of severe HSPN in children, DFPP combined with MP+CTX pulse therapy can further alleviate renal injury and improve clinical outcome and does not increase the incidence rate of adverse reactions.


Subject(s)
Child , Glucocorticoids , Humans , Immunosuppressive Agents , Nephritis , Plasmapheresis , Purpura, Schoenlein-Henoch
16.
Article in Korean | WPRIM | ID: wpr-759585

ABSTRACT

In terminally ill patients, organ transplantation could be recommended as the treatment of choice. In Korea, living donor liver or kidney transplantation is much more frequent than deceased donor transplantation due to organ shortages from deceased donors. ABO or HLA incompatibility in transplantation can be a major barrier in living donor transplantation. Currently, the rate of ABO incompatible organ transplantation accompanied by desensitization is 20~25% of living donor transplantation, and the blood bank laboratory plays an active role by plasmapheresis. The desensitization of HLA incompatible transplantation in highly sensitized patients is more difficult than that of ABO incompatible transplantation. The HLA antibody is not easy to remove and it is difficult to prevent sensitization. In addition, setting the target treatment goals and predicting the treatment outcomes based on the HLA antibody results are problematic. Therefore, a range of desensitization protocols have been attempted and various therapeutic goals have been introduced. This article reviews the various desensitization methods for antibody removal focusing on HLA incompatible kidney transplantation, and discusses the prognosis of desensitization methods for antibody removal based on the literature.


Subject(s)
Blood Banks , Humans , Kidney Transplantation , Korea , Liver , Living Donors , Organ Transplantation , Plasmapheresis , Prognosis , Terminally Ill , Tissue Donors , Transplantation , Transplants
17.
Article in English | WPRIM | ID: wpr-765002

ABSTRACT

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.


Subject(s)
Adolescent , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Antibodies, Antineutrophil Cytoplasmic , Biopsy , Child , Cyclophosphamide , Diagnosis , Dyspnea , Emergency Service, Hospital , Female , Glomerulonephritis , Hematuria , Hemoptysis , Hemorrhage , Hepatolenticular Degeneration , Humans , Penicillamine , Peroxidase , Plasmapheresis , Proteinuria , Trientine , Vasculitis
18.
Article in English | WPRIM | ID: wpr-719458

ABSTRACT

The catastrophic variant of antiphospholipid syndrome (APS) is a very rare and life-threatening condition of APS. This condition is characterized by thrombosis in multiple organs within a short period of time in the presence of positive antiphospholipid antibodies (aPL). Over the past few decades, considerable progress has been made in the treatment of patients with catastrophic APS; however, the mortality rate still remains very high. Although some cases of rituximab treatment in patients with catastrophic APS have been reported, there is no clear treatment protocol. A 14-year-old girl with systemic lupus erythematosus was diagnosed with catastrophic APS. She received several medications: corticosteroids, intravenous immunoglobulin, and plasmapheresis with anticoagulants. Unfortunately, she did not improve, and rituximab was started with four courses. After the rituximab treatment, she did not experience further thrombotic events during the follow up. This paper reports a pediatric case of catastrophic APS treated successfully with rituximab in Korea.


Subject(s)
Adolescent , Adrenal Cortex Hormones , Antibodies, Antiphospholipid , Anticoagulants , Antiphospholipid Syndrome , Clinical Protocols , Female , Follow-Up Studies , Humans , Immunoglobulins , Korea , Lupus Erythematosus, Systemic , Mortality , Plasmapheresis , Rituximab , Thrombosis
19.
Article in Korean | WPRIM | ID: wpr-741127

ABSTRACT

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.


Subject(s)
Acute Kidney Injury , Anemia, Hemolytic , Comorbidity , Diagnosis , Diagnosis, Differential , Disseminated Intravascular Coagulation , Enterohemorrhagic Escherichia coli , Hemolytic-Uremic Syndrome , Humans , Hypertension, Malignant , Mortality , Plasma Exchange , Plasmapheresis , Polymerase Chain Reaction , Purpura, Thrombotic Thrombocytopenic , Thrombocytopenia , Thrombospondins , Thrombotic Microangiopathies
20.
Rev. chil. enferm. respir ; 35(3): 199-206, 2019. graf
Article in Spanish | LILACS | ID: biblio-1058075

ABSTRACT

El patrón llamado 'crazy paving' en tomografia computada de tórax (TAC) puede deberse a diferentes condiciones siendo una de ellas la Proteinosis Alveolar Pulmonar (PAP), rara condición que puede llevar a insuficiencia respiratoria y a menudo, a la muerte. Presentamos el caso de una mujer joven con una historia de un año de evolución de disnea progresiva y tos seca que consultó por un cuadro de aparición brusca de fiebre, calofríos, malestar general y falla respiratoria hipoxémica severa (PaO2 = 51,9 mmHg con FiO2 = 0,50) en la cual la TAC de tórax mostraba un patrón de empedrado o 'crazy paving' que significó un desafío diagnóstico resuelto finalmente con una biopsia pulmonar quirúrgica que mostró una PAP. Ante el fracaso del tratamiento tradicional de Lavado Pulmonar Total (LPT) se usó una aproximación terapéutica novedosa consistente en una serie de 4 lavados lobares con un perfluorocarbono, Perflubron (PFC) bajo anestesia local seguido por 5 sesiones de Plasmaféresis. Casi inmediatamente después de este tratamiento la paciente evidenció mejoría radiológica y funcional. La PaO2 fue de 89,9 mmHg respirando aire ambiental y la CVF y el VEF1 aumentaron alcanzado respectivamente el 77 y el 75% de sus valores normales de referencia. Dadas las características químicas y físicas del PFC, pensamos que es una alternativa válida al LPT en estos casos.


Crazy paving computed tomography pattern may be due to a number of causes, one of them being Pulmonary Alveolar Proteinosis, a rare condition leading to respiratory failure and often to death. We present the case of a young woman with a one-year history of progressive dyspnea and dry cough, who consulted for an acute onset of fever, chills, malaise and severe hypoxemic respiratory failure (PaO2 = 51.9 mmHg; FiO2 = 0.50) with a 'crazy paving' pattern on chest CT. This diagnostic challenge was resolved by a surgical lung biopsy that showed a pulmonary alveolar proteinosis. Taking into account that the traditional treatment using whole lung lavage had already failed in this patient, a novel therapeutic approach was settled. A series of 4 lobar lavages with a perfluorocarbon (Perflubron) under local anesthesia followed by 5 plasmapheresis sessions were carried out. The patient showed radiographic and functional improvement almost immediately after this treatment. PaO2 was 89.9 mmHg breathing room air and FVC and FEV1 increased to reach 77 and 75% respectively of their normal reference values. Because of its chemical and physical properties we think this novel therapeutic approach should be a valuable alternative to saline solution for whole lung lavage in these cases.


Subject(s)
Humans , Female , Adult , Pulmonary Alveolar Proteinosis/therapy , Pulmonary Alveolar Proteinosis/diagnostic imaging , Pulmonary Alveolar Proteinosis/surgery , Pulmonary Alveolar Proteinosis/complications , Respiratory Insufficiency/prevention & control , Tomography, X-Ray Computed/methods , Plasmapheresis , Bronchoalveolar Lavage/methods , Fluorocarbons
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