Anorectal Function and Clinical Characteristics Associated with Faecal Incontinence in Patients with Crohn's Disease.

BACKGROUND AND AIMS: Faecal incontinence is an important complaint reported by patients with Crohn's disease [CD] and it is associated with several disease-related mechanisms, including anorectal functional disorders. This study aimed to assess the anorectal function and clinical characteristics to identify parameters associated with faecal incontinence in CD patients. METHODS: This is a cross-sectional study of 104 patients with CD, aged 18 years or older, from a referral centre between August 2019 and May 2021. Patients responded to a specific questionnaire, and underwent medical record review, proctological examination and anorectal functional assessment with anorectal manometry. RESULTS: Of the 104 patients, 49% were incontinent. Patients with incontinence had a lower mean resting pressure [43.5 vs 53.1 mmHg; p = 0.038], lower mean squeeze pressure [62.1 vs 94.1 mmHg; p = 0.036] and lower maximum rectal capacity [140 vs 180 mL; p < 0.001]. Faecal incontinence was also associated with disease activity [p < 0.001], loose stools [p = 0.02], perianal disease [p = 0.006], previous anoperineal surgery [p = 0.048] and number of anorectal surgeries [p = 0.036]. CONCLUSIONS: This is the largest reported study describing manometric findings of Crohn's disease patients with and without faecal incontinence. Our results identified an association between faecal incontinence and functional disorders, in addition to clinical features in these patients. Functional assessment with anorectal manometry may help choose the best treatment for faecal incontinence in patients with CD.

Causes of renal failure in patients with decompensated cirrhosis and its impact in hospital mortality.

BACKGROUND: Renal failure (RF) is reported to occur in 11-49% of the patients with decompensated end-stage liver disease (ESLD) and has been associated with increased mortality, particularly in the occurrence of hepatorenal syndrome (HRS) type 1. AIMS: To evaluate the frequency and outcome of RF in patients admitted to the hospital due to decompensated ESLD and to assess the impact of the underlying cause of RF on survival. MATERIAL AND METHODS: Four hundred and six patients (65% males, mean age 62 ± 12 years) with decompensated ESLD were evaluated for the occurrence of RF (defined as serum creatinine ³ 1.5 mg/mL). The underlying cause of RF was reckoned in each subject and compared to outcome. RESULTS: Renal failure was observed in 39% of the patients at admission and in 10% of the subjects during hospitalization. Mortality was significantly higher in subjects with RF (26 vs. 1%, p < 0.000001). Hypovolemia, bacterial infections, parenchymal kidney diseases and HRS were identified as causes of RF in, respectively, 40, 32, 15 and 12% of the cases. Mortality was significantly higher in those subjects with HRS type 1 and bacterial infections, when compared to other causes of RF. CONCLUSIONS: Renal failure occurs in nearly half of the patients with decompensated ESLD. It is most commonly caused by hypovolemia and bacterial infections. Occurrence of RF has an adverse impact in patient survival, particularly in those subjects with bacterial infections and HRS type 1, prone to develop progressive renal dysfunction despite intensive medical care.

Variables associated with progression of moderate-to-severe Crohn's disease.

OBJECTIVE: Determine the variables associated with hospitalisations in patients with Crohn's disease and those associated with surgery, intestinal resection, hospital readmission, need for multiple operations and immunobiological agent use. DESIGN: A cross-sectional study was conducted from 2019 to 2021, using two centres for inflammatory bowel diseases in the Brazilian Public Health System. RESULTS: This study included 220 patients. Only perianal disease was associated with hospitalisation (31.6% vs 13.0%, p=0.012). Stricturing or penetrating behaviour (35.8% vs 12.6%, p<0.001) and perianal disease (45.9% vs 9.9%, p<0.001) were associated with surgery. Ileal or ileocolonic location (80.0% vs 46.5%, p=0.044) and stricturing or penetrating behaviour (68.0% vs 11.2%, p<0.001) were associated with intestinal resection. Steroids use at first Crohn's disease occurrence and postoperative complications were associated with hospital readmission and need for multiple operations, respectively. Age below 40 years at diagnosis (81.3% vs 62.0%, p=0.004), upper gastrointestinal tract involvement (21.8% vs 10.3%, p=0.040) and perianal disease (35.9% vs 16.3%, p<0.001) were associated with immunobiological agent use. CONCLUSION: Perianal disease and stricturing or penetrating behaviour were associated with more than one significant outcome. Other variables related to Crohn's disease progression were age below 40 years at diagnosis, an ileal or ileocolonic disease localisation, an upper gastrointestinal tract involvement, the use of steroids at the first Crohn's disease occurrence and history of postoperative complications. These findings are similar to those in the countries with a high prevalence of Crohn's disease.

BRAZILIAN IBD STUDY GROUP POSITION STATEMENT ON SARS-COV2 VACCINATION.

Mass vaccination offers the best strategy to fight against COVID-19 pandemic, and SARS-CoV2 vaccines are being approved in several countries for emergency use. In Brazil, vaccine approval is expected in the next few days, however potential concerns exist regarding vaccine recommendations for specific populations, such as patients with inflammatory bowel disease (IBD). To address these questions, the Brazilian IBD Study Group (GEDIIB) provides this practical advice with key recommendations about the COVID-19 vaccines in IBD population.

Risk stratification and geographical mapping of Brazilian inflammatory bowel disease patients during the COVID-19 outbreak: Results from a nationwide survey.

BACKGROUND: Coronavirus disease 2019 (COVID-19) pandemic is still evolving globally, and Brazil is currently one of the most affected countries. It is still debated whether patients with inflammatory bowel disease (IBD) are at a higher risk for developing COVID-19 or its complications. AIM: To assess geographical distribution of IBD patients at the highest risk and correlate these data with COVID-19 mortality rates in Brazil. METHODS: The Brazilian IBD Study Group (Grupo de Estudos da Doença Inflamatória Intestinal do Brasil) developed a web-based survey adapted from the British Society of Gastroenterology guidelines. The included categories were demographic data and inquiries related to risk factors for complications from COVID-19. Patients were categorized as highest, moderate or lowest individual risk. The Spearman correlation test was used to identify any association between highest risk and mortality rates for each state of the country. RESULTS: A total of 3568 patients (65.3% females) were included. Most participants were from the southeastern and southern regions of Brazil, and 84.1% were using immunomodulators and/or biologics. Most patients (55.1%) were at moderate risk, 23.4% were at highest risk and 21.5% were at lowest risk of COVID-19 complications. No association between the proportion of IBD patients at highest risk for COVID-19 complications and higher mortality rates was identified in different Brazilian states (r = 0.146, P = 0.467). CONCLUSION: This study indicates a distinct geographical distribution of IBD patients at highest risk for COVID-19 complications in different states of the country, which may reflect contrasting socioeconomic, educational and healthcare aspects. No association between high risk of IBD and COVID-related mortality rates was identified.

Grupo de Estudos da Doença Inflamatória Intestinal do Brasil sobre a vacinação para SARS-CoV2 / Brazilian ibd study group position statement on sars-cov2 vaccination

ABSTRACT Mass vaccination offers the best strategy to fight against COVID-19 pandemic, and SARS-CoV2 vaccines are being approved in several countries for emergency use. In Brazil, vaccine approval is expected in the next few days, however potential concerns exist regarding vaccine recommendations for specific populations, such as patients with inflammatory bowel disease (IBD). To address these questions, the Brazilian IBD Study Group (GEDIIB) provides this practical advice with key recommendations about the COVID-19 vaccines in IBD population.

RESUMO A vacinação em massa oferece a melhor estratégia para enfrentamento da pandemia de COVID-19, e as vacinas contra SARS-CoV2 estão sendo aprovadas em vários países para uso emergencial. No Brasil, a aprovação da vacina é esperada em breve, no entanto, existem potenciais preocupações em relação às recomendações de vacinas para populações específicas, como pacientes com doença inflamatória intestinal (DII). Para responder essas questões, o Grupo Brasileiro de Estudos IBD (GEDIIB) fornece conselhos práticos com recomendações importantes sobre as vacinas para COVID-19 na população com DII.

Immune thrombocytopenic purpura induced by intestinal tuberculosis in a liver transplant recipient.

A variety of clinical manifestations are associated directly or indirectly with tuberculosis. Among them, haematological abnormalities can be found in both the pulmonary and extrapulmonary forms of the disease. We report a case of immune thrombocytopenic purpura (ITP) associated with intestinal tuberculosis in a liver transplant recipient. The initial management of thrombocytopenia, with steroids and intravenous immunoglobulin, was not successful, and the lack of tuberculosis symptoms hampered a proper diagnostic evaluation. After the diagnosis of intestinal tuberculosis and the initiation of specific treatment, a progressive increase in the platelet count was observed. The mechanism of ITP associated with tuberculosis has not yet been well elucidated, but this condition should be considered in cases of ITP that are unresponsive to steroids and intravenous immunoglobulin, especially in immunocompromised patients and those from endemic areas.

Poor outcomes with treatment of hepatorenal syndrome type 1 with splanchnic vasoconstrictors and albumin: report of seven cases and review of the literature.

CONTEXT: Treatment of hepatorenal syndrome type 1 (HRS-1) with splanchnic vasoconstrictors and high-dose albumin has been associated with reversal of renal failure in approximately 60% to 80% of the cases in pilot or uncontrolled studies. OBJECTIVE: To evaluate the results of treatment of HRS-1 with terlipressin and high-dose albumin. METHODS: All patients with HRS-1 that underwent treatment with terlipressin and high-dose albumin at our unit were retrospectively reviewed. Outcomes including reversal of renal failure and death were recorded and compared to baseline clinical and laboratory parameters. RESULTS: Seven subjects (median age 64 [47-69] years, 5 males) with median Child-Pugh and MELD scores of 12 [10-15] and 22 [17-38], respectively, hospitalized with decompensated chronic liver disease secondary to tense ascitis and infections, who exhibited criteria for HRS-1, were submitted to therapy with terlipressin and high-dose albumin according to a predefined standard protocol. Baseline creatinine levels were 2.9 [2.3-4.0] mg/mL. None of the patients achieved reversal of HRS-1 and five subjects died on-treatment due to sudden-death (n = 1), multiple organ dysfunction associated with end-stage liver failure (n = 2) and sepsis (n = 2). CONCLUSIONS: Treatment of HRS-1 with terlipressin and high-dose albumin was not associated with reversal of renal failure, but most of the treated subjects had severe end-stage liver disease with high MELD scores as well as high baseline creatinine values, parameters previously associated with bad outcomes.

Soft tissue Rosai-Dorfman disease of the posterior mediastinum.

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

Poor outcomes with treatment of hepatorenal syndrome type 1 with splancnic vasoconstrictors and albumin: report of seven cases and review of the literature / Resultados adversos com o emprego de vasoconstritores esplâncnicos e albumina para tratamento da síndrome hepatorrenal do tipo 1: relato de sete casos e revisão da literatura

CONTEXT: Treatment of hepatorenal syndrome type 1 (HRS-1) with splancnic vasoconstrictors and high-dose albumin has been associated with reversal of renal failure in approximately 60 percent to 80 percent of the cases in pilot or uncontrolled studies. OBJECTIVE: To evaluate the results of treatment of HRS-1 with terlipressin and high-dose albumin. METHODS: All patients with HRS-1 that underwent treatment with terlipressin and high-dose albumin at our unit were retrospectively reviewed. Outcomes including reversal of renal failure and death were recorded and compared to baseline clinical and laboratory parameters. RESULTS: Seven subjects (median age 64 [47-69] years, 5 males) with median Child-Pugh and MELD scores of 12 [10-15] and 22 [17-38], respectively, hospitalized with decompensated chronic liver disease secondary to tense ascitis and infections, who exhibited criteria for HRS-1, were submitted to therapy with terlipressin and high-dose albumin according to a predefined standard protocol. Baseline creatinine levels were 2.9 [2.3-4.0] mg/mL. None of the patients achieved reversal of HRS-1 and five subjects died on-treatment due to sudden-death (n = 1), multiple organ dysfunction associated with end-stage liver failure (n = 2) and sepsis (n = 2). CONCLUSIONS: Treatment of HRS-1 with terlipressin and high-dose albumin was not associated with reversal of renal failure, but most of the treated subjects had severe end-stage liver disease with high MELD scores as well as high baseline creatinine values, parameters previously associated with bad outcomes.

CONTEXTO: O tratamento da síndrome hepatorrenal do tipo 1 (SHR-1) com vasoconstritores esplâncnicos e albumina intravenosa tem se associado, em relatos de caso e estudos piloto não-controlados, à reversão da insuficiência renal em 60 por cento-80 por cento dos pacientes tratados. OBJETIVO: Avaliar os resultados do tratamento da SHR-1 com terlipressina e albumina. MÉTODOS: Foram avaliados, retrospectivamente, todos os pacientes hospitalizados com o diagnóstico de SHR-1 que se submeteram a tratamento com terlipressina associada à albumina em altas doses. As frequências de reversão de insuficiência renal e óbito foram comparados com parâmetros clínicos e laboratoriais pré-tratamento. RESULTADOS: Sete pacientes (5 homens, idade mediana 64 [47-69] anos) com mediana de pontuação Child-Pugh e MELD respectivamente de 12 [10-15] e 22 [17-38], admitidos na unidade de terapia intensiva por desconforto respiratório secundário à ascite tensa ou por infecções, que apresentaram critérios para SHR-1 e eligibilidade para o transplante de fígado foram submetidos a tratamento com terlipressina e albumina, de acordo com protocolo pré-definido. Níveis de creatinina prévios ao tratamento foram de 2.9 [2.3-4.0] mg/mL. Nenhum paciente apresentou reversão da SHR-1 e cinco faleceram por morte súbita (n = 1), disfunção de múltiplos órgãos associada a falência hepática (n = 2) e sepse (n = 2), a maioria antes de completar o tratamento. CONCLUSÕES: O tratamento da SHR-1 com terlipressina e albumina, em altas doses, não foi associado à reversão da insuficiência renal em nenhum dos pacientes tratados, mas a maioria dos pacientes apresentava doença hepática em fase avançada, com altos valores de MELD, e níveis elevados de creatinina pré-tratamento, parâmetros previamente associados com pior resposta e prognóstico mais reservado.

Soft tissue Rosai-Dorfman disease of the posterior mediastinum / Doença de Rosai-Dorfman de partes moles no mediastino posterior

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

A doença de Rosai-Dorfman (DRD) consiste em histiocitose sinusal com linfadenopatia maciça. O envolvimento extranodal ocorre em até 43% dos casos. Entretanto, a DRD de partes moles de forma isolada é rara. A forma isolada de DRD mediastinal é muito rara, com apenas três relatos prévios. O envolvimento do mediastino posterior só foi descrito no contexto da DRD disseminada. Relatamos o caso de uma paciente de 49 anos de idade com história de dor e linfadenomegalia cervical há dois anos, com resolução espontânea. A TC revelou uma massa paravertebral à esquerda medindo 6 cm de diâmetro. A paciente foi submetida à ressecção cirúrgica da massa. O exame microscópico e estudo do imunofenótipo da peça cirúrgica definiram o diagnóstico de DRD. A paciente foi acompanhada por 12 meses, queixando-se de tosse discreta e dor torácica. O acompanhamento por imagem não detectou recorrência, e nenhuma linfadenomegalia cervical foi observada após a cirurgia.