Acute-on-chronic liver failure syndrome - clinical results from an intensive care unit in a liver transplant center. / Síndrome da doença hepática crônica agudizada - resultados clínicos de uma unidade de terapia intensiva em centro de transplante hepático.

OBJECTIVE: To characterize a cohort of acute-on-chronic liver failure patients in Intensive Care and to analyze the all-cause 28-day mortality risk factors assessed at ICU admission and day 3. METHODS: This was a retrospective cohort study of consecutive patients admitted to the intensive care unit between March 2013 and December 2016. RESULTS: Seventy-one patients were included. The median age was 59 (51 - 64) years, and 81.7% of patients were male. Alcohol consumption alone (53.5%) was the most frequent etiology of cirrhosis and infection (53.5%) was the most common acute-on-chronic liver failure precipitating event. At intensive care unit admission, the clinical severity scores were APACHE II 21 (16 - 23), CLIF-SOFA 13 (11 - 15), Child-Pugh 12 (10 - 13) and MELD 27 (20 - 32). The acute-on-chronic liver failure scores were no-acute-on-chronic liver failure: 11.3%; one: 14.1%; two: 28.2% and three: 46.5%; and the number of organ failures was one: 4.2%; two: 42.3%; three: 32.4%; four: 16.9%; and five: 4.2%. Liver transplantation was performed in 15.5% of patients. The twenty-eight-day mortality rate was 56.3%, and the in-ICU mortality rate was 49.3%. Organ failure at intensive care unit admission (p = 0.02; OR 2.1; 95%CI 1.2 - 3.9), lactate concentration on day 3 (p = 0.02; OR 6.3; 95%CI 1.4 - 28.6) and the international normalized ratio on day 3 (p = 0.03; OR 10.2; 95%CI 1.3 - 82.8) were independent risk factors. CONCLUSION: Acute-on-chronic liver failure patients presented with high clinical severity and mortality rates. The number of organ failures at intensive care unit admission and the lactate and international normalized ratio on day 3 were independent risk factors for 28-day mortality. We consider intensive care essential for acute-on-chronic liver failure patients and timely liver transplant was vital for selected patients.

Síndrome da doença hepática crônica agudizada - resultados clínicos de uma unidade de terapia intensiva em centro de transplante hepático / Acute-on-chronic liver failure syndrome - clinical results from an intensive care unit in a liver transplant center

RESUMO Objetivo: Caracterizar uma coorte de pacientes com insuficiência hepática crônica agudizada em unidade de terapia intensiva e analisar os fatores de risco da mortalidade global aos 28 dias presentes no dia da admissão e no dia 3. Métodos: Estudo de coorte retrospectiva de pacientes consecutivamente admitidos à unidade de terapia intensiva entre março de 2013 e dezembro de 2016. Resultados: Incluímos 71 pacientes com idade mediana de 59 (51 - 64) anos, sendo 81,7% do sexo masculino. Consumo de álcool isoladamente (53,5%) foi a etiologia mais frequente de cirrose, e infecção (53,5%) foi o evento que mais frequentemente precipitou a agudização da insuficiência hepática crônica. No momento da admissão à unidade de terapia intensiva, os escores de severidade foram: APACHE II 21 (16 - 23), CLIF-SOFA 13 (11 - 15), Child-Pugh 12 (10 - 13) e MELD 27 (20 - 32). Dentre os indivíduos, 14,1% tiveram escore 1 de insuficiência hepática crônica agudizada, 28,2% pontuaram 2, 64,5% pontuaram 3, e 11,3% se apresentaram sem insuficiência hepática crônica agudizada. Sobre o número de falências de órgãos, 4,2% tiveram um órgão; 42,3%, dois órgãos; 32,4%, três órgãos; 16,9%, quatro órgãos; e 4,2% cinco órgãos. Foi realizado transplante hepático em 15,5% dos pacientes. A taxa de mortalidade aos 28 dias foi de 56,3%, e a taxa de mortalidade na unidade de terapia intensiva foi de 49,3%. Foram fatores independentes de risco: falências do órgãos quando da admissão à unidade de terapia intensiva (p = 0,02; RC 2,1; IC95% 1,2 - 3,9), concentração de lactato em sangue arterial no dia 3 (p = 0,02; RC 6,3; IC95% 1,4 - 28,6) e RC internacional no dia 3 (p = 0,03; RC 10,2; IC95% 1,3 - 82,8). Conclusão: Pacientes com insuficiência hepática crônica agudizada apresentaram elevados níveis de severidade e taxa de mortalidade. O número de falências de órgãos no momento da admissão à unidade de terapia intensiva e o nível de lactato, assim como a razão normalizada internacional no dia 3 de permanência na unidade foram fatores independentes de risco para a mortalidade ao 28º dia. Consideramos que a terapia intensiva é essencial para pacientes com insuficiência hepática crônica agudizada, e a realização de um transplante hepático em tempo adequado foi vital para os pacientes selecionados.

ABSTRACT Objective: To characterize a cohort of acute-on-chronic liver failure patients in Intensive Care and to analyze the all-cause 28-day mortality risk factors assessed at ICU admission and day 3. Methods: This was a retrospective cohort study of consecutive patients admitted to the intensive care unit between March 2013 and December 2016. Results: Seventy-one patients were included. The median age was 59 (51 - 64) years, and 81.7% of patients were male. Alcohol consumption alone (53.5%) was the most frequent etiology of cirrhosis and infection (53.5%) was the most common acute-on-chronic liver failure precipitating event. At intensive care unit admission, the clinical severity scores were APACHE II 21 (16 - 23), CLIF-SOFA 13 (11 - 15), Child-Pugh 12 (10 - 13) and MELD 27 (20 - 32). The acute-on-chronic liver failure scores were no-acute-on-chronic liver failure: 11.3%; one: 14.1%; two: 28.2% and three: 46.5%; and the number of organ failures was one: 4.2%; two: 42.3%; three: 32.4%; four: 16.9%; and five: 4.2%. Liver transplantation was performed in 15.5% of patients. The twenty-eight-day mortality rate was 56.3%, and the in-ICU mortality rate was 49.3%. Organ failure at intensive care unit admission (p = 0.02; OR 2.1; 95%CI 1.2 - 3.9), lactate concentration on day 3 (p = 0.02; OR 6.3; 95%CI 1.4 - 28.6) and the international normalized ratio on day 3 (p = 0.03; OR 10.2; 95%CI 1.3 - 82.8) were independent risk factors. Conclusion: Acute-on-chronic liver failure patients presented with high clinical severity and mortality rates. The number of organ failures at intensive care unit admission and the lactate and international normalized ratio on day 3 were independent risk factors for 28-day mortality. We consider intensive care essential for acute-on-chronic liver failure patients and timely liver transplant was vital for selected patients.

Can Red Cell Distribution Width Be Used as a Marker of Crohn's Disease Activity?

INTRODUCTION: Recently, it has been suggested an association between red cell distribution width (RDW) and Crohn's disease activity index (CDAI), but its use is not yet performed in daily clinical practice. OBJECTIVES: To determine whether RDW can be used as a marker of Crohn's disease (CD) activity. METHODS: This was a cross-sectional study including patients with CD, observed consecutively in an outpatient setting between January 1st and September 30th 2013. Blood cell indices, erythrocyte sedimentation rate (ESR), and C-reactive protein were measured. CD activity was determined by CDAI (active disease if CDAI ≥ 150). Associations were analyzed using logistic regression (SPSS version 20). RESULTS: 119 patients (56% female) were included in the study with a mean age of 47 years (SD 15.2). Twenty patients (17%) had active disease. The median RDW was 14.0 (13-15). There was an association between RDW and disease activity (p = 0.044). After adjustment for age and gender, this association remained consistent (OR 1.20, 95% CI 1.03-1.39, p = 0.016). It was also found that the association between RDW and disease activity was independent of hemoglobin and ESR (OR 1.36, 95% CI 1.08-1.72, p = 0.01) and of biologic therapy (OR 1.19, 95% CI 1.03-1.37, p = 0.017). A RDW cutoff of 16% had a specificity and negative predictive value for CDAI ≥ 150 of 88% and 86%, respectively. CONCLUSION: In this study, RDW proved to be an independent and relatively specific marker of CD activity. These results may contribute to the implementation of this simple parameter, in clinical practice, aiming to help therapeutic decisions.

INTRODUÇÃO: Recentemente, tem vindo a ser sugerida uma associação entre o valor de RDW e a atividade da doença de Crohn (DC), mas a sua utilização não está ainda implementada na prática clínica diária. OBJETIVOS: Determinar se o RDW pode ser utilizado como marcador de atividade da DC. MÉTODOS: Estudo transversal, em doentes com DC, observados consecutivamente em consulta de Doença Inflamatória Intestinal, entre 1 de janeiro e 30 de setembro de 2013. Analisaram-se índices do hemograma, proteína C reativa e velocidade de sedimentação. A gravidade da doença foi avaliada pelo Crohn's disease activity index (doença ativa se CDAI≥150). As associações foram estudadas usando a regressão logística (SPSS Statistics V20). RESULTADOS: Incluídos 119 doentes (56% do sexo feminino), com idade média de 47 anos (DP 15,2 anos). Vinte doentes (17%) tinham doença ativa. O valor do RDW mediano foi 14,0% (13-15). Verificou-se uma associação entre RDW e atividade da doença (p = 0,044). Após ajuste para a idade e o sexo, esta associação manteve-se consistente (OR 1,20; 95% CI 1,03-1,39; p = 0,016). Verificou-se ainda que a associação do valor do RDW com a atividade da doença foi independente do valor da hemoglobina e da velocidade de sedimentação (OR 1,36; 95% CI 1,08-1,72; p = 0,01) e da terapêutica biológica (OR 1,19; 95% CI 1,03-1,37; p = 0,017). Para um valor de corte de RDW de 16%, a especificidade e o valor preditivo negativo de CDAI≥ 150 foram de 88% e 86%, respetivamente. CONCLUSÃO: Neste estudo, o valor do RDW demonstrou ser um marcador independente e relativamente específico da atividade da doença de Crohn. Estes resultados poderão contribuir para a aplicação deste parâmetro simples, na prática clinica diária, visando auxiliar decisões terapêuticas.

Hepatic nodule: a case of primary myelofibrosis.

Primary myelofibrosis is one of the entities that may manifest with lesions of extramedullary haematopoiesis, especially in spleen and liver. The authors report a case of primary myelofibrosis presenting incidentally as an intrahepatic focal lesion of extramedullary haematopoiesis, a rare occurrence that highlights the challenge of hepatic nodule differential diagnosis, and allows reflection about the diagnostic criteria and prognostic factors of this myeloproliferative disease.