COVID-19 associated transverse myelitis: case report.

BACKGROUND: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients. CLINICAL CASE: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution. CONCLUSION: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out.

INTRODUCCIÓN: La mielitis transversa (MT) es una enfermedad inflamatoria desmielinizante que se presenta con disfunción motora, sensitiva y autonómica, de forma aguda o subaguda. La MT asociada al COVID-19 se ha escrito en un escaso número de pacientes. CASO CLÍNICO: Se presenta el caso de un masculino de 15 años previamente sano, quien cursaba con un cuadro respiratorio y que desarrollo un deterioro neurológico súbito que involucro un síndrome medular completo localizado en el nivel cérvico dorsal, con paraparesia simétrica que progreso a la paraplejia, con disfunción sensitiva desde el nivel medular de T3, disfunción de esfínteres y deterioro ventilatorio que requirió manejo avanzado de la vía aérea. Su resonancia magnética fue compatible con mielitis transversa aguda. Se descartaron causas inflamatorias y no inflamatorias de la patología. Además, se obtuvo un resultado positivo de SARS-COV-2. Se inició tratamiento con pulsos de metilprednisolona y plasmaféresis, con una evolución insidiosa. CONCLUSIÓN: El COVID-19 es una causa infrecuente de MT y debe sospecharse cuando otras causas han sido descartadas.

Divide et Vinces, Therapeutic Apheresis in Nephrological Clinical Practice.

Therapeutic plasma exchange (TPE) or plasmapheresis has been used in various life-threatening diseases as a primary treatment or in combination with other therapies. It was first successfully employed in the 1960s for diseases like Waldenström's disease and myeloma. Since then, TPE techniques using apheresis membranes have been introduced. Apheresis therapies separate plasma components from blood using membrane screening or centrifugation methods. TPE aims to remove substances involved in the pathophysiology of diseases. It selectively removes high-molecular-weight molecules, substances with prolonged half-life, and those associated with disease pathogenesis. TPE can be performed using membranes or centrifugation, with replacement of extracted plasma volume using albumin or fresh frozen plasma. TPE requires specific competencies in nephrology and can be prescribed and monitored by nephrologists and performed by dialysis nursing staff. TPE can be combined with adsorption-based therapies to enhance its effect, and this approach is called plasma filtration adsorption. Another variation is double plasma filtration, which selectively removes substances based on molecular size. TPE can also be combined with lipoprotein removal strategies for managing familial hypercholesterolemia. TPE is an affordable extracorporeal therapy that benefits patients with life-threatening diseases. It requires collaboration between nephrologists and other specialists, and our results demonstrate successful TPE without anticoagulation in general hospitalization or outpatient settings.

Treatment of post-transplant recurrent FSGS in children using plasmapheresis and augmentation of immunosuppression.

BACKGROUND: Up to 60% of pediatric renal transplant recipients with end-stage renal disease due to primary focal and segmental glomerulosclerosis (FSGS) may develop recurrent disease. Such recurrence is associated with poor prognosis if no remission is achieved. We report a single center experience with a protocol based on plasmapheresis and increased immunosuppression that resulted in a high long-lived remission rate. METHODS: This retrospective cohort study included consecutive pediatric renal transplant patients with recurrent FSGS treated with a standardized protocol using plasmapheresis and cyclophosphamide to supplement usual post-transplant immunosuppression with calcineurin inhibitors and steroids. Relapse was defined as urinary protein/creatinine ratio > 1.0 g/g and remission as < 0.5 g/g. RESULTS: Seventeen patients with FSGS recurrence post-transplant were treated. All had therapy resistant FSGS in native kidneys and had been on dialysis from 4 to 10 years. Of the 17, one died perioperatively from a pulmonary thromboembolism. Fifteen others achieved a complete remission within 3 months of treatment for FSGS recurrence. After a median follow-up period of 4 years, there were no recurrences of significant proteinuria. One patient achieved remission with rituximab. CONCLUSION: The addition of plasmapheresis and cyclophosphamide to a calcineurin- and steroid-based immunosuppression regime was highly successful in inducing high remission rates with recurrent FSGS. Prospective trials are needed to evaluate further the efficacy of increased immunosuppression along with plasmapheresis in this setting.

Hemostatic effects of therapeutic plasma exchange: A concise review.

Therapeutic plasma exchange (TPE) alters the hemostatic balance. Contributing to TPE's hemostatic effects is the mechanical processing of blood in the extracorporeal circuit, circuit anticoagulant, type of replacement fluid, TPE schedule and number of procedures, TPE timing relative to invasive procedures, and removal of nontargeted components such as platelets, coagulation proteins, and cytokines. Although TPE's hemostatic effects are well established, how it impacts the bleeding risk is not clearly understood. In this concise review, we describe the effects of the above TPE-related factors on hemostatic balance, present data on the effects of TPE on blood hemostasis, including its effects on platelet counts and clotting assays, and review the literature on the impact of TPE-induced hemostatic changes on TPE-associated bleeding events. Finally, we discuss risk factors associated with bleeding during TPE and review the literature on TPE-associated hemostatic effects in the pediatric population.

Therapeutic plasmapheresis for the treatment of thyrotoxicosis: A retrospective multi-center study.

BACKGROUND AND AIMS: Thyroid storm and severe thyrotoxicosis remain among the most frequent endocrine emergencies, and first-line hyperthyroidism treatment is not always an option. Since the first report in 1970, plasmapheresis is a second-line treatment for severe or otherwise untreatable thyrotoxicosis when rapid euthyroidism is desired. METHODS: We present a retrospective study of the experience in treating thyrotoxicosis with plasmapheresis between 2012 and 2020 in two specialized centers in Colombia. We register the demographic and clinical characteristic and compare the thyroid hormones and other biochemical measurements before and after treatment. RESULTS: Data from 19 patients was obtained, 58% female with a median age of 35 years (IQR 23.5), and most of them with Graves' disease. The most frequent indication for plasmapheresis was thyroid storm. A median of 4 (IQR 2) sessions lead to a significant reduction in FT4 (P .0001) and TT3 (P < .0003) with a nonsignificant decrease in beta-blocker (P .7353) dose, no change in hepatic enzymes, and no adverse events. After plasmapheresis, thyroidectomy was performed in 10 patients. CONCLUSIONS: Plasmapheresis is an effective and safe treatment option for reducing circulating thyroid hormones in severe thyrotoxicosis when other forms of treatment are contraindicated or in case of urgent thyroid and non-thyroid surgery. It is limited by its cost and the need for highly specialized resources.

Manejo oportuno de púrpura trombocitopénica trombótica en paciente con lupus eritematoso sistémico: reporte de caso / Timely management of thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus: case report

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)

Shrinking lung syndrome in pediatric systemic lupus erythematosus.

OBJECTIVE: To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. MATERIAL AND METHODS: This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. RESULTS: Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. CONCLUSIONS: SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.

Long-term plasmapheresis therapy in the management of focal segmental glomerulosclerosis recurrence after kidney transplantation.

The recurrence of primary focal segmental glomerulosclerosis (FSGS) after kidney transplantation (KT) appears in 30 % of the recipients. Sometimes it can cause the loss of the allograft. Although many treatments for this condition have been reported, 20 %-40 % of the affected patients are refractory or presents frequents relapses. In this paper we describe the evolution of three recipients treated with long-term plasmapheresis therapy after a recurrence of FSGS with a bad or incomplete response to other treatments. Although our findings require confirmation, long-term plasmapheresis could be a therapeutic option for this condition.

Effect of plasma separation techniques and sperm selection on sperm recovery and viability of cooled pony stallion semen for 48h at 5°C / [Efeito das técnicas de separação de plasma e de seleção de esperma na recuperação e viabilidade do sêmen de garanhão pônei resfriado por 48h a 5°C]

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)

The immunotherapy of Guillain-Barré syndrome.

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.

Estudio prospectivo de la concentración de hierro sérico en donantes de plasma durante un año / Prospective study during a year of the iron concentration in plasma donors

Introducción: los donantes regulares de plasmaféresis, tienen pérdidas de masa eritrocitaria que pueden afectar, en dependencia de las individualidades, sus reservas de hierro. Objetivo: determinar comportamiento evolutivo durante un año de la sideremia en donantes de plasmaféresis. Método: se realizó un estudio observacional descriptivo en 200 donantes de plasma del Banco de Sangre Provincial de Cienfuegos. Se cuantificó de forma seriada la concentración de hierro sérico. Se relacionó la cantidad de individuos con valores bajos del mineral y variables sexo, edad, tiempo donando plasma, frecuencia de donaciones y concentración de hemoglobina. Resultados: los valores grupales promedio de hierro sérico mostraron tendencia a disminuir dentro de la normalidad, aunque se constató en un pequeño grupo déficit de hierro latente y en otro ligera anemia, más frecuente en mujeres, y mayores de 44 años, relacionado con mayor intensidad en cada ciclo de donación y tiempo de permanencia como donante de plasma. Conclusiones: sin llegar a establecer relación causal directa, los resultados de la investigación apuntan hacia el desarrollo de déficit progresivo de hierro en los donantes regulares de plasma,por balance negativo del mineral. Es preciso observar con mayor acercamiento la donación de plasma, que como proceso ético e inocuo, evite efectos no deseados en los donantes(AU)

Introduction: Regular plasmapheresis donors have erythrocytemas losses that can affect, depending on individualities, their iron stores. Aim: To determine evolutionary behavior during one year of serum iron in plasmapheresis donors. Method: A descriptive observational study was carried out on 200 plasma donors from the Provincial Blood Bank of Cienfuegos. The serum iron concentration was serially quantified. The number of individuals with low values of the mineral and variables gender, age, time donating plasma, frequency of donations and hemoglobin concentration were related. Results: The serum iron average showed a tendency to decrease within normal range, although it was found in a small group of latent iron deficiency and in another slight anemia, more frequent in women, and over 44 years old, related to greater intensity in each donation cycle and time of permanence as a plasma donor. Conclusions: Without establishing a direct causal relationship, the results of the research point to the development of progressive iron deficiency in regular plasma donors, due to the negative balance of the mineral. The donation of plasma, as an ethical and harmless process, should be monitored more closely, avoiding undesirable effects on donors(AU)

Novas abordagens terapêuticas na Doença de Chagas: terapia celular, anticorpos, aférese / New therapheutic approaches in Chagas Disease stem cell antibodies apheresis

A doença de Chagas é considerada, atualmente, pela Organização Mundial da Saúde uma das doenças tropicais negligenciadas, com estimativa de mais de 8 milhões de pessoas infectadas em todo o mundo. Recentemente tem havido crescente interesse na doença de Chagas, importante etiologia de miocardiopatia na América Latina, devido, em grande parte, ao aumento da incidência dessa doença em países desenvolvidos. A despeito do amplo emprego de drogas antiparasitárias na forma aguda da doença de Chagas, o tratamento etiológico da miocardiopatia chagásica crônica permanece incerto, sendo o benefício para o prognóstico dos pacientes ainda indeterminado. No presente artigo realizamos revisão sistemática a respeito do emprego de terapia celular, anticorpos, vacinas e plasmaferese em pacientes com doença de Chagas. Os dados levantados indicam que, a despeito de a terapia baseada em células-tronco mostrar potencial benéfico em modelos experimentais, em seres humanos, a evidência até o momento disponível não nos autoriza a utilizar tal modalidade na rotina assistencial. Vacinas baseadas em antígenos genéticos têm potencial a ser explorado, mas sem aplicabilidade momentânea em seres humanos. Novos estudos são necessários para elucidar o uso real dessas modalidades alternativas no contexto da miocardiopatia chagásica.

ABSTRACT The World Health Organization, with an estimate of over 8 million people infected worldwide, currently considers the Chagas disease as one of the neglected tropical diseases. Recently there has been increasing interest in Chagas disease, an important etiology of cardiomyopathy in Latin America, due in large part to the increased incidence in developed countries. Despite the widespread use of antiparasitic drugs in the acute infected form of Chagas disease, etiological treatment of chronic Chagas cardiomyopathy remains uncertain, and the benefit for the prognosis of patients still undetermined. In the present article, we present a systematic review on the use of cell therapy, antibodies, vaccines and plasmapheresis in patients with Chagas disease. The data collected indicate that, despite the stem cell-based therapy showing beneficial potential in experimental models, in humans, the evidence so far available does not allows us to use this modality as a routine treatment. Vaccines based on genetic antigens have potential to be explored, but without immediate applicability in humans. Further studies are needed to elucidate the actual use of these alternative methods in the context of Chagas cardiomyopathy.

Eficacia y seguridad de rituximab para el tratamiento de pacientes menores de 18 años con encefalitis autoinmune refractario al tratamiento de primera línea (inmunoglobulina, pulsos corticoides y plasmaféresis) / Efficacy and safety of rituximab for the treatment of patients younger than 18 years with autoimmune encephalitis refractory to first-line therapy (immunoglobulin, corticoid pulses and plasmapheresis)

INTRODUCCIÓN: Antecedentes: El presente dictamen expone la evaluación de la eficacia y seguridad del medicamento rituximab para el tratamiento de pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarios a la inmunoterapia de primera línea. Aspectos Generales: La Encefalitis se refiere a un trastorno inflamatorio del cerebro que resulta en un estado mental alterado, convulciones, o problemas en el funcionamento del cerebro. Es de progresión rápida (menos de 6 semanas) y su incidencia estimada en paises industrializados de occidente (Finlandia, EEUU, Jutlandia, Inglaterra, Francia, Grecia, Canadá, Eslovenia) es de 6.3 a 7.4 casos por 100,000 habitantes (adultos y niños) por año y aproximadamente 10.5 a 13.8 casos por 100,000 niños por ano. Las causas de encefalitis mayormente reconocidas son las de origen infeccioso; no obstante, recientemente se ha descubierto que un 4% de las encefalitis son el resultado de la producción de anticuerpos que atacan los receptores neuronales y proteínas de superfície de las células involucradas en la transmisión sináptica, plasticidad, o excitabilidad neuronal. Tecnología Sanitaria de Interés: Rituximab: Rituximab es un anticuerpo monoclonal quimérico murino/humano que se une al antígeno CD20 localizado en la superficie de los linfocitos pre-B y B maduros. Este antígeno se encuentra tanto en células B normales como malignas. Tras la unión a CD20, rituximab destruye las células B. Los posibles mecanismos de lisis celular incluyen la citotoxidad dependiente del complemento (CDC) y la citotoxidad mediada por células dependientes de anticuerpos (ADCC); debido a ello es usado para el tratamiento de sídromes linfoproliferativoscrónicos de estirpe B, en enfermedades autoinmunes y en otras entidades donde hay proliferación de linfocitos B. METODOLOGÍA: Estrategia de Búsqueda: Se realizó una búsqueda sistemática de la evidencia, especialmente la proveniente de ensayos clínicos, con respecto a la eficacia y seguridad de rituximab en pacientes pediátricos con diagnóstico de encefalitis autoinmune en las bases de datos MEDLINE, TRIPDATABASE, ScienceDirect y LILACS. Una vez identificados los artículos que respondían a la pregunta PICO, se pasó a revisar la bibliografia incluida en dichos artículos seleccionados, con la finalidad de identificar evidencia adicional. Asimismo, se realizó una búsqueda dentro de bases de datos pertenecientes a grupos que realizan revisiones sistemáticas, evaluación de tecnologías sanitarias y guías de práctica clínica, tales como The National Guideline for Clearinghouse (NGC), Scottish Intercollegiate Guidelines Network (SIGN), The National Institute for Health and Care Excellence (NICE), The Canadian Agency for Drugs and Technologies in Health (CADTH), The Agency for Healthcare Research and Quality (AHQR) y The Cochrane Collaboration. Se hizo una búsqueda adicional en www.clinicaltrials.gov, para poder identificar ensayos clínicos en curso o que no hayan sido publicados. RESULTADOS: Sinopsis de la Evidencia: Se realizó una búsqueda de la literatura con respecto a a eficacia y seguridad de rituximab, en comparación a la terapia de primera línea (inmunoglobulina intravenosa, pulsos de corticoides y plasmaféresis) o placebo, como tratamiento de segunda línea en pacientes pediátricos con diagnóstico de encefalitis autonmune refractarios a la inmunoterapia de primera línea.No se encontratón ensayos clínicos aleatorizados por lo que se incluyeron resultados de estudios observacionlaes y series de casos que aportaran información relevante. CONCLUSIONES: A la fecha, no existe evidencia suficiente sobre la eficacia científica de rituximab, con respecto a la inmunoterapia de primera línea (corticoesteroides, inmunoglobulina intravenosa y plasmaféresis) o placebo, en pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarioa a la inmunoterapia de primera línea en términos de mayor calidad de vida y disminución de secuelas neurológicas. No se encontraron ensayos clínicos que hayan evaluado el uso de rituximab en la población de la presente evaluación de tecnologia sanitaria. La GPC clínica encontrada para el tratamiento de este tipo de pacientes no incluyen a rituximab dentro de sus recomendaciones. Del mismo modo, la única revisión sistemática identificada concluye que existe un aparente beneficio en el uso de la inmunoterapia de segunda línea con respecto al no uso de esta, pero que estos resultados se ven afectados por sesgos de selección y de reporte. El Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI) aprueba el uso de rituximab como alternativa de tratamiento para pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarios a la inmunoterapia de primera línea. El periodo de vigencia de este dictamen es de un año y la continuación de dicha aprobación estará sujeta a los resultados obtenidos de los pacientes que se beneficien con dicho tratamiento y a nueva evidencia que pueda surgir en el tiempo.

Acute Antibody-Mediated Rejection in Kidney Transplant Based on the 2013 Banff Criteria: Single-Center Experience in Uruguay.

BACKGROUND: Acute antibody-mediated rejection (AMR) diagnosis criteria have changed in recent consensus of Banff, with current evidence of C4d-negative AMR. Our objective was to evaluate incidence of AMR in renal transplantation according to Banff 2013 criteria and to examine the histological features and outcome. METHODS: This retrospective study involved all kidney transplants with histological diagnosis of acute rejection (AR) at our center between 2000 and 2014. All the biopsies with AR were re-assessed by a nephro-pathologist and classified by use of the Banff 2013 criteria. RESULTS: Of 205 kidney transplants, biopsy-proven AR was diagnosed in 25 cases (12%). Re-assessing them according to Banff 2013 criteria, AMR was diagnosed in 17 (8.3%) and represented 68% of the confirmed rejections. AMR diagnosis was performed on day 23 ± 26, with median of 11 days. From the 17 cases, 7 had concomitant T-cell-mediated rejection. All cases presented endothelial edema and acute tubular necrosis. Glomerulitis was found in 12 cases and capillaritis in 14. In 3, associated thrombotic micro-angiopathy (TMA) was found. Intimal and transmural arteritis was evidenced in 5 and 1 patient. In 2, transplant glomerulopathy was present. Seven of the 10 biopsies with C4d staining in the peri-tubular capillaries were positive. Twelve cases received plasmapheresis, 6 received gamma-globulin, and 6 received rituximab. After administration of anti-AMR therapy, 16 cases recovered renal function, reaching a serum creatinine level of 1.5 ± 0.6 mg %. Graft survival at 1 year was lower in the AMR group versus patients without AMR (81.9% vs 98.9%, log-rank test, P < .001). Risk factors for AMR were re-transplant (30% vs 7%, P = .02), HLA-DR mismatch (1.06 ± 0.65 vs 0.7 ± 0.6, P = .03), panel-reactive antibody (28% ± 33 vs 6.2 ± 13, P = .00), and delayed graft function (82% vs 30%, P = .00). CONCLUSIONS: Adapting the new Banff 2013 criteria increased the sensitivity of the diagnosis of ARM. Regarding our data, despite an adequate response to the therapy, it resulted in a worse graft survival by the first year of renal transplant.

[Clinical features of Guillain-Barré syndrome in 41 patients admitted to a public hospital]. / Características epidemiológicas del síndrome de Guillain-Barré en población chilena: estudio hospitalario en un período de 7 años.

BACKGROUND: Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis worldwide, with an incidence of 0.6-4 per 100.000 inhabitants per year. It affects all age groups and carries an incapacity burden of up to 20%. AIM: To describe the features of GBS in adult Chilean patients admitted to a tertiary care hospital. MATERIAL AND METHODS: Review of medical records of 41 patients aged 17 to 81 years (30 males) admitted to a public hospital with the diagnosis of GBS between 2003 and 2009. According to clinical and electrophysiological criteria, the patients were classified into different varieties of GBS. RESULTS: The incidence of GBS was higher in males (2.7:1) and the demyelinated GBS variety was found in 66% of cases. According to the Hughes’ disability score, patients treated with plasmapheresis, showed non-statistically significant better outcomes than those treated with intravenous immunoglobulin. CONCLUSIONS: In this group of patients the demyelinated variety of GBS was more common than the axonal type. Although not statistically significant, the better response to plasmapheresis is encouraging and should prompt a controlled study.

[Use of plasmapheresis in hypertriglyceridemic pancreatitis]. / Uso de plasmaféresis en pancreatitis hipertrigliceridémica.

Hypertriglyceridemia is reported as cause of 1 to 4% of the episodes of acute pancreatitis. We report the case of a 42-year-old woman with a history of obesity, type 2 diabetes mellitus, hypertriglyceridemia and hypercholesterolemia, with triglycerides of 9365 mg/dl, total cholesterol of 1822 mg/dl, one month prior to the consultation. She presented at the emergency unit with a 5 day history of abdominal pain, which progressed in intensity in the last 48 hours. Abdominal computed tomography revealed pancreatic and peripancreatic inflammation. Thirty-six hours after admission, a first session of plasmapheresis was conducted with a plasma triglyceride and cholesterol reduction of 25 and 30%, respectively. A second session was performed the next day, with a further reduction of triglycerides to 996 mg/dl and cholesterol to 238 mg/dl. During hospitalization the patient presented fever and Klebsiella pneumoniae bacteremia with no pancreatic collection or necrosis in tomography and, later on, nosocomial pneumonia, both infections with adequate response to antibiotic therapy. At the time of discharge, triglycerides and cholesterol levels were 652 mg/dl and 167 mg/dl respectively, no abdominal pain was present and the patient resumed oral nutrition. We observed a 90% reduction of triglycerides and 87% of cholesterol after 2 sessions of plasmapheresis, compared to 70% in average of reduction in most of the studies reviewed. We did not find the presence of bacteremia or nosocomial pneumonia as complications in the reported cases.

Uso de plasmaféresis en pancreatitis hipertrigliceridémica / Use of plasmapheresis in hypertriglyceridemic pancreatitis

La hipertrigliceridemia es causa de 1-4% de las pancreatitis agudas. Presentamos el caso de una mujer de 42 años con antecedentes de obesidad, diabetes mellitus tipo 2, hipertrigliceridemia e hipercolesterolemia (9365 mg/dl y 1822 mg/dl, respectivamente, 1 mes previo a la consulta). Concurrió a nuestro hospital por cuadro de dolor abdominal de 5 días de evolución de tipo cólico con progresión continua en las últimas 48 horas. Se realizó tomografía de abdomen que informó imágenes compatibles con pancreatitis. A las 36 horas de su ingreso se inició la primera sesión de plasmaféresis con una reducción de triglicéridos y colesterol del 25 y 30% respectivamente y una segunda sesión al día siguiente con descenso de triglicéridos a 996 mg/dl y colesterol a 238 mg/dl. Durante su internación presentó bacteriemia por Klebsiella pneumoniae, sin colección ni necrosis pancreática detectables por tomografía de abdomen, y luego neumonía intrahospitalaria, ambas infecciones con buena respuesta a antibioticoterapia. Al alta, los triglicéridos habían descendido a 652 mg/dl, el colesterol a 167 mg/dl, el dolor abdominal había cedido y la paciente presentaba buena tolerancia por vía oral. Observamos una reducción del 90% de triglicéridos y 87% de colesterol luego de dos sesiones de plasmaféresis, comparado con 70% de reducción en promedio en la mayoría de los estudios consultados. En los mismos, no hemos encontrado la presencia de bacteriemia ni neumonía hospitalaria como complicaciones.

Hypertriglyceridemia is reported as cause of 1 to 4% of the episodes of acute pancreatitis. We report the case of a 42-year-old woman with a history of obesity, type 2 diabetes mellitus, hypertriglyceridemia and hypercholesterolemia, with triglycerides of 9365 mg/dl, total cholesterol of 1822 mg/dl, one month prior to the consultation. She presented at the emergency unit with a 5 day history of abdominal pain, which progressed in intensity in the last 48 hours. Abdominal computed tomography revealed pancreatic and peripancreatic inflammation. Thirty-six hours after admission, a first session of plasmapheresis was conducted with a plasma triglyceride and cholesterol reduction of 25 and 30%, respectively. A second session was performed the next day, with a further reduction of triglycerides to 996 mg/dl and cholesterol to 238 mg/dl. During hospitalization the patient presented fever and Klebsiella pneumoniae bacteremia with no pancreatic collection or necrosis in tomography and, later on, nosocomial pneumonia, both infections with adequate response to antibiotic therapy. At the time of discharge, triglycerides and cholesterol levels were 652 mg/dl and 167 mg/dl respectively, no abdominal pain was present and the patient resumed oral nutrition. We observed a 90% reduction of triglycerides and 87% of cholesterol after 2 sessions of plasmapheresis, compared to 70% in average of reduction in most of the studies reviewed. We did not find the presence of bacteremia or nosocomial pneumonia as complications in the reported cases.

Uso de plasmaféresis en pancreatitis hipertrigliceridémica / Use of plasmapheresis in hypertriglyceridemic pancreatitis

La hipertrigliceridemia es causa de 1-4% de las pancreatitis agudas. Presentamos el caso de una mujer de 42 años con antecedentes de obesidad, diabetes mellitus tipo 2, hipertrigliceridemia e hipercolesterolemia (9365 mg/dl y 1822 mg/dl, respectivamente, 1 mes previo a la consulta). Concurrió a nuestro hospital por cuadro de dolor abdominal de 5 días de evolución de tipo cólico con progresión continua en las últimas 48 horas. Se realizó tomografía de abdomen que informó imágenes compatibles con pancreatitis. A las 36 horas de su ingreso se inició la primera sesión de plasmaféresis con una reducción de triglicéridos y colesterol del 25 y 30% respectivamente y una segunda sesión al día siguiente con descenso de triglicéridos a 996 mg/dl y colesterol a 238 mg/dl. Durante su internación presentó bacteriemia por Klebsiella pneumoniae, sin colección ni necrosis pancreática detectables por tomografía de abdomen, y luego neumonía intrahospitalaria, ambas infecciones con buena respuesta a antibioticoterapia. Al alta, los triglicéridos habían descendido a 652 mg/dl, el colesterol a 167 mg/dl, el dolor abdominal había cedido y la paciente presentaba buena tolerancia por vía oral. Observamos una reducción del 90% de triglicéridos y 87% de colesterol luego de dos sesiones de plasmaféresis, comparado con 70% de reducción en promedio en la mayoría de los estudios consultados. En los mismos, no hemos encontrado la presencia de bacteriemia ni neumonía hospitalaria como complicaciones.(AU)

Hypertriglyceridemia is reported as cause of 1 to 4% of the episodes of acute pancreatitis. We report the case of a 42-year-old woman with a history of obesity, type 2 diabetes mellitus, hypertriglyceridemia and hypercholesterolemia, with triglycerides of 9365 mg/dl, total cholesterol of 1822 mg/dl, one month prior to the consultation. She presented at the emergency unit with a 5 day history of abdominal pain, which progressed in intensity in the last 48 hours. Abdominal computed tomography revealed pancreatic and peripancreatic inflammation. Thirty-six hours after admission, a first session of plasmapheresis was conducted with a plasma triglyceride and cholesterol reduction of 25 and 30%, respectively. A second session was performed the next day, with a further reduction of triglycerides to 996 mg/dl and cholesterol to 238 mg/dl. During hospitalization the patient presented fever and Klebsiella pneumoniae bacteremia with no pancreatic collection or necrosis in tomography and, later on, nosocomial pneumonia, both infections with adequate response to antibiotic therapy. At the time of discharge, triglycerides and cholesterol levels were 652 mg/dl and 167 mg/dl respectively, no abdominal pain was present and the patient resumed oral nutrition. We observed a 90% reduction of triglycerides and 87% of cholesterol after 2 sessions of plasmapheresis, compared to 70% in average of reduction in most of the studies reviewed. We did not find the presence of bacteremia or nosocomial pneumonia as complications in the reported cases.(AU)