Quality indicators in prolonged hemodialysis with regional citrate anticoagulation with the genius system: retrospective cohort of critical patients with acute kidney injury.

BACKGROUND: Prolonged hemodialysis (HD) is performed from 6 to 12 h and can last up to 24 h. To prevent system clotting some studies suggest that Regional Citrate Anticoagulation (RCA) use reduces bleeding rates relative to systemic heparin. However, there may be difficulties in the patient's clinical management and completing the prescribed HD with Genius system using RCA. OBJECTIVE: To analyze safety Quality Indicators (IQs) and follow up on prolonged HD with 4% sodium citrate solution in a Genius® hybrid system. METHODS: This is a retrospective cohort conducted in an intensive care unit. RESULTS: 53 random sessions of prolonged HD with 4% sodium citrate solution of critically ill patients with AKI assessed. Evaluated safety indicators were dysnatremia and metabolic alkalosis, observed in 15% and 9.4% of the sessions, respectively. Indicators of effectiveness were system clotting which occurred in 17.3%, and the minimum completion of the prescribed HD time, which was 75.5%. CONCLUSION: The assessment of the indicators showed that the use of RCA with a 4% sodium citrate solution in prolonged HD with the Genius system in critically ill patients with AKI can be performed in a simple, safe, and effective way.

Neurological syndromes and potential triggers associated with antibodies to neuronal surface antigens.

BACKGROUND: Autoantibodies against surface neuronal antigens have been associated with specific neurological presentations including autoimmune encephalitis (AE), with variable association with neoplasia and infections. METHODS: We described the phenotype and environmental associations of patients with neurological syndromes associated with antibodies against neuronal surface antigens who were referred to a tertiary center in the South of Brazil. All patients were tested for neuronal autoantibodies using cell-based assays. Clinical, radiological, and laboratory findings were retrospectively reviewed. RESULTS: We identified 16 patients, 15 had subacute, and one had a progressive disease course. Among patients with subacute onset, 11 (73 %) were N-Methyl-d-Aspartate receptor (NMDAr-IgG)+, 3 (20 %) were Leucine-rich Glioma-Inactivated-1 (LGI1-IgG)+, and 1 (6 %) was positive for Glycine receptor-IgG. The patient with a progressive disease course had antibodies against IgLON5. Most patients had disease onset in spring and summer suggesting environmental factors for the development of AE. Also, we observed a different pattern of brain lesions when NMDAr-IgG encephalitis followed herpes encephalitis and a previously unreported association with Rosai-Dorfman-Destombe disease. All patients with encephalopathy met criteria for possible AE and all proven NMDAr-IgG+ met criteria for NMDAr-IgG encephalitis. However, only one LGI1-IgG+ patient fulfilled clinical criteria for limbic encephalitis. All but one received high-dose intravenous methylprednisolone, 11 also had intravenous human immunoglobulin, and 4 plasma exchange. Furthermore, all patients received second-line immunotherapy. Importantly, most patients improved with immunotherapy, even when initiated later in the disease course. CONCLUSION: We identified seasonal variability associated with neuronal surface antibodies suggesting environmental triggers. Also, we described the coexistence of NMDAr-IgG encephalitis with histiocytosis. In our series, most patients received second-line immunotherapy. We observed neurologic improvement after treatment even in cases of delayed diagnosis. Increasing the recognition and availability of tests and treatments for these conditions is of paramount importance in low- and middle-income countries.

Síndrome inflamatória multissistêmica (SIMS) pós-COVID-19: um conceito em evolução / Post-COVID-19 multisystem inflammatory syndrome: an evolving concept

INTRODUÇÃO: A potencial associação da COVID-19 com fenômenos inflamatórios e autoimunes abre um novo capítulo na prática clínica. Entre várias condições inflamatórias descritas no pós-COVID-19, destacam-se a doença de Kawasaki e uma nova afecção denominada síndrome inflamatória multissistêmica. OBJETIVOS: Revisar, de forma prática e concisa, conceito e critérios diagnósticos da síndrome inflamatória multisistêmica, as sobreposições com a doença de Kawasaki, assim como a imunopatogênese e o tratamento desta nova e intrigante enfermidade. MÉTODOS: Revisão da literatura disponível na base de dados Pubmed, com ênfase em revisões sistemáticas com metaanálises. RESULTADOS: A síndrome inflamatória multisistêmica se configura como uma condição hiperinflamatória multiorgânica pós-viral. A condição é primordialmente pediátrica, e os primeiros casos foram descritos na Inglaterra em maio de 2020. Os critérios diagnósticos são ainda imprecisos, e incluem algumas manifestações doença de Kawasaki-símiles. A síndrome inflamatória multisistêmica difere da doença de Kawasaki, entretanto, por geralmente acometer crianças acima cinco anos e de raças negras ou hispânicas; em termos clínicos, se distingue pela alta frequência de gastroenteropatia, miocardiopatia e choque. O diagnóstico diferencial inclui sepse bacteriana, síndrome de ativação macrofágica e formas sistêmicas de artrite reumatoide. Uma hiperexpressão de interferons e de outras citocinas inflamatórias caracteriza patogenicamente a síndrome inflamatória mulsistêmica. A enfermidade é, via de regra, responsiva a cuidados de terapia intensiva, corticóides, imunoglobulina intravenosa e imunobiológicos. CONCLUSÕES: A síndrome inflamatória multisistêmica é uma nova e complexa afecção hiperinflamatória associada à exposição prévia ao SARS-CoV-2. Apresenta instigantes interfaces com a doença de Kawasaki. Apesar da descrição recente, a literatura já é quantitativamente robusta, e algumas pendências de imunopatogênese, critérios diagnósticos e terapêutica deverão ser esclarecidas em breve.

INTRODUCTION: A potential association of COVID-19 with inflammatory and autoimmune phenomena opens a new chapter in clinical practice. Among several inflammatory conditions described in the post-COVID-19 context, Kawasaki disease and a new condition named multisystem inflammatory syndrome stand out. AIMS: To review, in a practical and concise way, the concept and diagnostic criteria of multisystem inflammatory syndrome, the overlaps with Kawasaki disease, as well as the immunopathogenesis and treatment of this new and intriguing condition. METHODS: Literature review available in the Pubmed database, with emphasis on systematic reviews with meta-analyses. RESULTS: The multisystem inflammatory syndrome is a post-viral, multiorgan hyperinflammatory disorder. The condition is primarily pediatric, and the first cases were reported in England in May 2020. Diagnostic criteria are still imprecise, and include some Kawasaki disease-like manifestations. However, multisystem inflammatory syndrome differs from Kawasaki disease by usually affecting children above five years of age and of black or hispanic races; in clinical terms, it is distinguished by the high frequency of gastroenteropathy, cardiomyopathy and shock. Differential diagnosis includes bacterial sepsis, macrophage activation syndrome, and systemic forms of rheumatoid arthritis. An overexpression of interferons and other inflammatory cytokines pathogenically characterize the multisystem inflammatory syndrome. The disease is, as a rule, responsive to intensive care, steroids, intravenous immunoglobulin, and immunobiologics. CONCLUSIONS: Multisystem inflammatory syndrome is a new and complex hyperinflammatory condition associated with previous exposure to SARS-CoV-2. It shows interesting interfaces with Kawasaki disease. Despite the recent description, the literature is already quantitatively robust, and some pending issues in immunopathogenesis, diagnostic criteria and therapy should be shortly clarified.

A brief history of antiphospholipid antibodies and antiphospholipid syndrome / Uma breve história dos anticorpos antifosfolípides e da síndrome antifosfolípide

AIMS: To review the historical reports on antiphospholipid antibodies (aPL) from the early years of the 20th century; to outline the cardinal features of the antiphospholipid syndrome (APS) from 1983 on, including clinical criteria, etiopathogenesis and current therapy. METHODS: Literature review using PubMed. Articles on the history of aPL and APS were selected. RESULTS: The original aPL were described in patients with syphilis yet in 1906 by Wassermann. A first definition of lupus anticoagulant was proposed in 1963,while the anticardiolipin antibody (aCL) test was depicted twenty years later. The APS, initially reported by Hughes in 1985as the "aCL syndrome", is one of the most prevalent acquired thrombophilia. Venous and arterial thrombosis, associated or not to pregnancy morbidity, comprise the main features. It is a novel disorder firstly associated to systemic lupus erythematosus. A primary form of APS was put forward in 1989, and many APS variants are currently known. Lifelong, full-dose anticoagulation is the mainstream for treatment of thrombotic APS. In obstetric APS, the combination of acetil-salicilic acid and enoxoparin has been a mostly effective therapy. CONCLUSIONS: The sequential characterization of aPL since Wassermann in 1906, and later of the APS in the 1980-thies, is a rather interesting example of how a new entity is sketched step by step. APS is an intriguing novel cause of autoimmune thrombophilia, with a complex pathogenesis and a plethora of clinical and laboratory abnormalities. Treatment is based on life-long anticoagulation.

OBJETIVOS: Revisar os relatos históricos sobre anticorpos antifosfolípides (aAF) dos primeiros anos do século XX; delinear as características cardinais da síndrome antifosfolípide (SAF) a partir de 1983, incluindo critérios clínicos, etiopatogênese e terapia atual. MÉTODOS: Revisão de literatura utilizando o PubMed. Foram selecionados artigos com foco na história dos aAF e da SAF. RESULTADOS: Os aAF foram originalmente descritos em pacientes com sífilis ainda em 1906 por Wassermann. Uma primeira definição do anticoagulante lúpico foi proposta em 1963, enquanto o anticorpo anticardiolipina (aCL) foi descrito 20 anos mais tarde. A SAF, inicialmente reportada por Hughes em 1985 como "síndrome do aCL" é uma das mais prevalentes trombofilias adquiridas. Tromboses arteriais e venosas, associadas ou não à morbidade gestacional, compreendem os achados principais. É uma nova entidade, tendo sido primeiramente associada ao lupus eritematoso sistêmico. Uma forma primária de SAF foi reconhecida em1989, e muitas variantes de SAF são modernamente conhecidas. A terapia-padrão para a SAF trombótica é a anticoagulação plena e ininterrupta. Na SAF obstétrica, a combinação de ácido acetil-salicílico com enoxaparina tem-se mostrado altamente efetiva. CONCLUSÕES: A caracterização sequencial dos aAF desde Wasserman em 1906, e mais tarde da SAF nos anos 1980, é um interessante exemplo de como uma nova entidade é concebida passo a passo. A SAF é uma nova e intrigante causa de trombofilia autoimune, com uma complexa patogênese e uma pletora de manifestações clínicas e laboratoriais. O tratamento é baseado em anticoagulação contínua.