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    Tofacitinib: uma Opção na Colite Ulcerosa Aguda Grave?

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    Tofacitinib has emerged as a new option for ulcerative colitis. Its rapid absorption, metabolism, and clinical improvement make it an interesting option for rescue therapy in acute severe ulcerative colitis (ASUC), a situation with limited therapeutic options in patients with a long-term disease course and multiple drug failure. The management of ASUC in this setting becomes challenging, underlying the need for new drugs and data on their efficacy and safety. We describe 2 cases of acute episodes in which tofacitinib was used as a rescue therapy.O tofacitinib surgiu como uma nova terapêutica para a colite ulcerosa. A rapidez da sua absorção, metabolismo e melhoria clínica tornam-no uma opção apelativa como terapêutica de resgate na colite ulcerosa aguda grave (termo em inglês – ASUC), entidade esta com opções terapêuticas limitadas em doentes com longo curso de doença e falência prévia a múltiplos fármacos. A abordagem da ASUC neste contexto é desafiante, salientando-se a necessidade de novos fármacos e dados reais sobre a sua eficácia e segurança. Descrevemos dois casos de episódios agudos nos quais o tofacitinib foi utilizado como terapêutica de resgate.info:eu-repo/semantics/publishedVersio

    Risk Factors for Mortality in Patients With COVID-19 Needing Extracorporeal Respiratory Support

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    When indicating ECMO in patients with COVID-19, centre case volume, age, driving pressure and the duration of symptoms (not the length of MV) should be taken into account. Large drainage cannula and high PEEP levels during the first days are recommended.info:eu-repo/semantics/publishedVersio

    Anterior Slope Correction-Flexion Osteotomy in Traumatic Genu Recurvatum

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    A decreased posterior tibial slope has been associated with an increased risk of posterior cruciate ligament failure, anterior knee pain, and premature knee osteoarthritis. Trauma is a common cause of osseous genu recurvatum. Surgical management is recommended to correct the tibial slope and prevent knee pain and osteoarthritis progression. This article discusses our preferred treatment using a proximal tibial opening-wedge osteotomy for surgical management of genu recurvatum secondary to significant anterior tibial slope.info:eu-repo/semantics/publishedVersio

    Hysteroscopic Myomectomy: the Guidelines of the International Society for Gynecologic Endoscopy (ISGE)

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    Objective: With this publication, the International Society for Gynecologic Endoscopy (ISGE) aims to provide the clinicians with the recommendations arising from the best evidence currently available on hysteroscopic myomectomy (HM). Study design: The ISGE Task Force for HM defined key clinical questions, which led the search of Medline/PubMed and the Cochrane Database. We selected and analyzed relevant English-language articles, published from January 2005 to June 2021, including original works, reviews and the guidelines previously published by the European Society for Gynecological Endoscopy (ESGE) and the American Association of Gynecologic Laparoscopists (AAGL), in which bibliographies were also checked in order to identify additional references, using the medical subject heading (MeSH) term "Uterine Myomectomy" (MeSH Unique ID: D063186) in combination with ''Myoma" (MeSH Unique ID: D009214) and ''Hysteroscopy" (MeSH Unique ID: D015907). We developed the recommendations through multiple cycles of literature analysis and expert discussion. Results: The ISGE Task Force did develop 10 grade 1A-C and 4 grade 2A-C recommendations. For planning HM, evaluation of the uterus with saline infusion sonohysterography (SIS) or combined assessment by transvaginal ultrasound (TVUS) and diagnostic hysteroscopy is recommended (Grade 1A). The use of STEPW (Size, Topography, Extension of the base, Penetration and lateral Wall position) classification system of submucosal leiomyoma (LM) is recommended to predict the complex surgeries, incomplete removal of the LM, long operative time, fluid overload and other major complications (grade 1B). For type 0 LMs, in addition to resectoscopy (slicing technique), morcellation is recommended, being faster and having a shorter learning curve with respect to resectoscopy (grade 1C). For type 1-2 LMs, slicing technique is currently recommended (grade 1C). A fluid deficit of 1000 mL also in case of bipolar myomectomy with saline solution, in healthy women of reproductive age, contains low risk for major complications. Lower thresholds (750 mL) for fluid deficit should be considered in the elderly and in women with cardiovascular, renal or other co-morbidities (Grade 1B). Conclusion: HM is the most effective conservative minimally invasive gynecologic intervention for submucous LM. The set of 14 ISGE recommendations can significantly contribute to the success of HM and the safety of patients for whom the choice of appropriate surgical technique, as well as the surgeon's awareness and measures to prevent complications are of the utmost importance.info:eu-repo/semantics/publishedVersio

    Presence of Multilobular Necrosis on Liver Biopsy Identifies Corticosteroid Responsiveness in Acute Indeterminate Hepatitis

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    Background and aims: Treatment of patients with severe indeterminate hepatitis (IAH) is an unmet need. Corticosteroids are often used in the management of these patients but criteria for the selection of patients for this intervention are arbitrary. The aims of this study were to analyse the clinical and pathological features of patients with IAH to define predictors of corticosteroid responsiveness. Methods: This study included consecutive patients with acute indeterminate hepatitis admitted to a single hospital and underwent a liver biopsy. The clinical manifestation and histopathological features of steroid and non-steroid groups were compared and their relationship with corticosteroids response was evaluated. Results: Forty-eight patients were included, 24 (50%) recovered and the other half underwent liver transplantation or died within 3-months. Of the 48 cases, 24 received corticosteroids (initial dose of 45 ± 12 mg prednisolone). Corticosteroids were initiated 2.7 ± 3.8 days after admission. Liver biopsy was performed 2-days (median, IQR 1-3) after admission. Fifteen (62.5%) patients receiving corticosteroids survived without transplantation compared with 9 (37.5%) that did not receive steroids (P = .149). In those with multilobular necrosis, 50% reduction in the death/transplantation rate was observed after steroid treatment (P = .018). In patients without multilobular necrosis and with or without perivenulitis, corticosteroids did not impact the outcome. Response to corticosteroids was independent of the MELD score. Conclusions: The presence of multilobular necrosis on liver biopsy helps identify a subgroup of IAH cases who may benefit from the administration of corticosteroids.info:eu-repo/semantics/publishedVersio

    Reviewing Treatment Options for Advanced Renal Cell Carcinoma: Is There Still a Place for Tyrosine Kinase Inhibitor (TKI) Monotherapy?

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    Renal cell carcinoma (RCC) comprises a highly heterogeneous group of kidney tumours built upon distinct genetic- and epigenetic-driven mechanisms and molecular pathways. Therefore, responsiveness to treatment is considerably variable across patients, adding an extra layer of complexity to the already challenging therapeutic decision process. The last decade brought an unprecedented shift in the medical approach to advanced or metastatic RCC; in fact, immunotherapy-based combinations have significantly transformed the therapeutic arsenal and clinical outcomes of these patients. These strategies were quickly adopted by international guidelines committees as the new standards of care. However, this enhanced efficacy comes at the expense of tolerability, with a predictable negative impact on patients' quality of life. Moreover, subgroup and post hoc analyses of the major clinical trials have shown that not all patients benefit equally from these innovative approaches. In this context, a group of experts on kidney cancer met and discussed the state of the art in the field, with a special emphasis on the appropriateness of using monotherapy with an anti-angiogenesis tyrosine kinase inhibitor (TKI) to treat specific subgroups of patients with RCC. This article reviews the main topics that were considered to be pertinent for that discussion and establishes the profile of patients for whom TKI monotherapy remains a sensible frontline option by avoiding overtreatment and an unnecessary exposure to treatment-related toxicity.info:eu-repo/semantics/publishedVersio

    Late Gadolinium Enhancement in the Left Ventricular Wall Is Associated with Atrial Fibrillation in Patients with Hypertrophic Cardiomyopathy

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    Purpose: Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the relation between AF and left ventricular (LV) late gadolinium enhancement (LGE). Methods: 55 patients with HCM were retrospectively included. Patients were divided in HCM with AF and HCM without AF. Baseline clinical, echocardiographic and cardiovascular magnetic resonance (CMR) characteristics were collected and compared between groups. Results: In univariable analysis, the factors related to AF development were HCM risk score for sudden cardiac death (SCD) > 2.29% (p = 0.002), left atrium (LA) diameter > 42.5 mm (p = 0.014) and LGE in the mid anterior interventricular septum (IVS) (p = 0.021), basal inferior IVS (p = 0.012) and mid inferior IVS (p = 0.012). There were no differences in LV diastolic function and LA strain between groups. Independent predictors of AF were LA diameter (p = 0.022, HR 5.933) and LGE in mid inferior IVS (p = 0.45, HR 3.280). Combining LA diameter (> 42.5 mm or < 42.5 mm) and LGE in mid inferior IVS (present or absent) in a model with four groups showed a statistically significant difference between groups (p = 0.013 for the model). Conclusions: LGE in mid inferior IVS is an independent predictor for AF occurrence in patients with HCM. Combining both LGE in mid inferior IVS and enlarged LA improves prediction of AF and may be preferred for risk stratification.info:eu-repo/semantics/publishedVersio

    Capacidade Preditiva dos Parâmetros do Teste de Esforço Cardiopulmonar em Pacientes com Insuficiência Cardíaca em Terapia de Ressincronização Cardíaca

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    Background: There is evidence suggesting that a peak oxygen uptake (pVO2) cut-off of 10ml/kg/min provides a more precise risk stratification in cardiac resynchronization therapy (CRT) patients. Objective: To compare the prognostic power of several cardiopulmonary exercise testing (CPET) parameters in this population and assess the discriminative ability of the guideline-recommended pVO2cut-off values. Methods: Prospective evaluation of consecutive heart failure (HF) patients with left ventricular ejection fraction ≤40%. The primary endpoint was a composite of cardiac death and urgent heart transplantation (HT) in the first 24 follow-up months, and was analysed by several CPET parameters for the highest area under the curve (AUC) in the CRT group. A survival analysis was performed to evaluate the risk stratification provided by several different cut-offs. p values <0.05 were considered significant. Results: A total of 450 HF patients, of which 114 had a CRT device. These patients had a higher baseline risk profile, but there was no difference regarding the primary outcome (13.2% vs 11.6%, p =0.660). End-tidal carbon dioxide pressure at anaerobic threshold (PETCO2AT)had the highest AUC value, which was significantly higher than that of pVO2in the CRT group (0.951 vs 0.778, p =0.046). The currently recommended pVO2cut-off provided accurate risk stratification in this setting (p <0.001), and the suggested cut-off value of 10 ml/min/kg did not improve risk discrimination in device patients (p =0.772). Conclusion: PETCO2ATmay outperform pVO2's prognostic power for adverse events in CRT patients. The current guideline-recommended pVO2 cut-off can precisely risk-stratify this population.Fundamento: Há evidências sugerindo que um corte do pico de consumo de oxigênio (pVO2) de 10ml/kg/min fornece uma estratificação de risco mais precisa em pacientes com Terapia de Ressincronização Cardíaca (TRC). Objetivo: Comparar o poder prognóstico de vários parâmetros do teste cardiopulmonar de exercício (TCPE) nesta população e avaliar a capacidade discriminativa dos valores de corte de pVO2 recomendados pelas diretrizes. Métodos: Avaliação prospectiva de uma série consecutiva de pacientes com insuficiência cardíaca (IC) com fração de ejeção do ventrículo esquerdo ≤40%. O desfecho primário foi um composto de morte cardíaca e transplante cardíaco urgente (TC) nos primeiros 24 meses de acompanhamento, e foi analisado por vários parâmetros do TCPE para a maior área sob a curva (AUC) no grupo TRC. Uma análise de sobrevida foi realizada para avaliar a estratificação de risco fornecida por vários pontos de corte diferentes. Valores de p < 0,05 foram considerados significativos. Resultados: Um total de 450 pacientes com IC, dos quais 114 possuíam aparelho de TRC. Esses pacientes apresentaram um perfil de risco basal mais alto, mas não houve diferença em relação ao desfecho primário (13,2% vs 11,6%, p = 0,660). A pressão expiratória de dióxido de carbono no limiar anaeróbico (PETCO2AT) teve o maior valor de AUC, que foi significativamente maior do que o de pVO2 no grupo TRC (0,951 vs 0,778, p = 0,046). O valor de corte de pVO2 atualmente recomendado forneceu uma estratificação de risco precisa nesse cenário (p <0,001), e o valor de corte sugerido de 10 ml/min/kg não melhorou a discriminação de risco em pacientes com dispositivos (p = 0,772). Conclusão: A PETCO2AT pode superar o poder prognóstico do pVO2 para eventos adversos em pacientes com TRC. O ponto de corte de pVO2 recomendado pelas diretrizes atuais pode estratificar precisamente o risco dessa população.info:eu-repo/semantics/publishedVersio

    Familial Amyloid Polyneuropathy Misdiagnosed as Systemic Sclerosis

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    We present the case of a 53-year-old woman of Portuguese ancestry with a diagnosis of progressive systemic sclerosis (SSc), proposed for haematopoietic stem cell transplantation (HSCT). Clinical re-evaluation when assessing eligibility for the procedure led to the alternative diagnosis of familial amyloid polyneuropathy (FAP). We discuss the clinical presentations of FAP and SSc, focusing on their overlapping and distinguishing features. We emphasize the need for a high level of suspicion in order to establish an early diagnosis of FAP in the absence of a family history, and provide prognostic and genetic counselling. Learning points: It is important to review diagnoses, especially when the clinical course is atypical.Cutaneous involvement is a commonly unrecognized feature of familial amyloid polyneuropathy.Hereditary conditions should be included in the differential diagnosis of multisystemic diseases, even in the absence of a family history.info:eu-repo/semantics/publishedVersio

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