Diarrhea: a missed D in the 4D glucagonoma syndrome

Glucagonoma is a rare and slow-growing pancreatic tumor that usually manifests as glucagonoma syndrome. It is mainly characterized by a typical Dermatosis named necrolytic migratory erythema (NME), Diabetes and glucagon oversecretion. Deep vein thrombosis and Depression complete this set. We report the case of an advanced glucagonoma with liver spread, where all these 4D symptoms occurred but a chronic secretory Diarrhea was the most relevant feature. A 65-year-old man was referred to our center to investigate multiple hepatic nodules evidenced by abdominal tomography. He had a recent diagnosis of diabetes and complained of significant weight loss (25 kg), crusted skin lesions and episodes of a large amount of liquid diarrhea during the past 6 months. On admission, there were erythematous plaques and crusted erosions on his face, back and limbs, plus angular cheilitis and atrophic glossitis. The typical skin manifestation promptly led dermatologists to suspect glucagonoma as the source of our patient’s symptoms. A contrast-enhanced abdominal computed tomography showed a hypervascularized pancreatic lesion and multiple hepatic nodules also hypervascularized in the arterial phase. Despite initial improvement of diarrhea after subcutaneous octreotide, the patient’s impaired nutritional status limited other therapeutic approaches and he died of respiratory failure due to sepsis. His high levels of serum glucagon were not yet available so we performed an autopsy, confirming the diagnosis of metastatic glucagonoma with NME on histology. Chronic diarrhea is not a common feature in glucagonoma syndrome; however, its severity can lead to serious nutritional impairment and set a poor outcome

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson’s capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcom

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson’s capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcom

Early liver function improvement following successful treatment of chronic hepatitis C in patients with decompensated cirrhosis: a real-life study

OBJECTIVES: Despite higher rates of sustained virologic response (SVR), important concerns remain when patients with decompensated cirrhosis due to hepatitis C virus (HCV) are treated with direct-acting antiviral agents (DAA). Questions include efficacy, safety, and the magnitude of liver function improvement. Here, we aimed to evaluate HCV treatment data in this specific population in Brazil. METHODS: We included 85 patients with decompensated cirrhosis submitted to HCV therapy with DAA followed at two academic tertiary centers in the southeastern region of Brazil. RESULTS: Seventy-nine patients (92.9%) were Child-Pugh (CP) score B, and six (7.1%) were CP score C. The mean MELD score was 12.86. The most common treatment was sofosbuvir plus daclatasvir±ribavirin for 24 weeks. The overall intention-to-treat (ITT) SVR rate was 87.4% (74/85) and modified-ITT 96.1% (74/77). ITT SVR was associated with lower baseline INR values (p=0.029). Adverse events (AE) occurred in 57.9% (44/76) of patients. Serious AE were reported in 12.8% (10/78), and were related to the presence of hepatic encephalopathy (p=0.027). SVR was associated with improvement in CP (p<0.0001) and MELD scores (p=0.021). Among baseline CP score B patients with SVR, 46% (29/63) regressed to CP score A. Ascites was independently associated with no improvement in liver function in patients who achieved SVR (p=0.001; OR:39.285; 95% CI:4.301-258.832). CONCLUSIONS: Patients with decompensated HCV cirrhosis showed a high SVR rate with interferon-free therapy. Early liver function improvement occurred after successful HCV eradication. However, long-term follow-up of these patients after SVR remains strongly advised. &nbsp

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson’s capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcom

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson’s capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcom

Bacterial infection after liver transplantation : epidemiological characteristics, multidrug-resistant bacteria and risk factors

Orientadores: Raquel Silveira Bello Stucchi, Ilka de Fátima Santana Ferreira BoinTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciências MédicasResumo: Introdução: O transplante de fígado vem sendo realizado rotineiramente como ferramenta terapêutica de rotina há quase 30 anos. A evolução clínica dos pacientes após o transplante depende de múltiplos fatores, entre os quais a presença de infecções, que é uma de suasdas maiores complicações mais frequentes e está associada a grande parte das mortes no primeiro ano pós-transplante de fígado. Estas infecções podem estar associadas a fatores relacionados aos pacientes com cirrose hepática, especialmente em casos de insuficiência hepática avançada, que por sua vez frequentemente são acometidos por infecções bacterianas e utilizam antibióticos de amplo espectro. Ultimamente, tem-se descrito aumento importante de infecções por agentes multirresistentes (MR), cujos principais fatores de risco são as infecções adquiridas em ambiente hospitalar, uso prévio de antibióticos e infecção prévia por bactéria MR. Estes fatores de risco, ao ocorrerems ocorrendo em candidatos ao transplante poderiam afetar também sua evolução após o transplante. Objetivos: Avaliar a prevalência e as características das infecções bacterianas, particularmente por agentes MR, no período pósem pacientes transplantados de-transplante de fígado na realidade brasileirana Unicamp. Além disso, definir fatores de risco para infecções pós-transplante, em especial os relacionados ao paciente cirrótico em sua fase pré-transplante, e avaliar a influência das infecções na evolução clínica na mortalidade após o transplante. Métodos: Fforam avaliados retrospectivamente todos os pacientes adultos submetidos a um primeiro transplante hepático, com sobrevida ? 48 h, entre setembro de 2010 e agosto de 2013 no Hospital de Clínicas da Unicamp. Os desfechos estudados foram as infecções bacterianas, as infecções por bactérias MR e a sobrevida. Foram analisadas variáveis clínicas e laboratoriais dos receptores no períodos pré-transplante, intraoperatório e pós-transplante. Os fatores de risco foram avaliados por análise multivariada de regressão de Cox, e a sobrevida pelo método de Kaplan-Meier. Resultados: Eentre os 98 pacientes estudados, 51 (52%) apresentaram infecção bacteriana, dos quais 37 (72%) tiveram cultura positiva, e em 24 (47%) destes pacientes foi identificada bactéria MR. Os fatores de risco independentes para infecção bacteriana foram síndrome hepatorrenal prévia (razão de risco [HR]: 2.,65; p = 0.,015) e níveis de albumina sérica pré-transplante (HR: 0.,65; p = 0.,044). Uso profilático de norfloxacino (HR: 2.,78; p = 0.,033) e a relação normatizada internacional (HR: 1.,69; p = 0.,021) foram fatores de risco para infecções MR. A sobrevida foi significativamente menor em pacientes que apresentaram infecção após o transplante (log-rank p < 0.,001), no entanto a presença de infecção MR não foi associada a uma piora adicional da sobrevida (log-rank p = 0.,712). A pPresença de infecção bacteriana após o transplante de fígado (HR: 4.,11; p = 0.,001) e maior o volume de transfusão de concentrado de hemácias (HR: 1.,08; p = 0.,02) foram fatores independentes de risco para mortalidade. Conclusões: Aas infecções bacterianas após o transplante hepático estão associadas a maior mortalidade, e os fatores de risco para infecções são a presença de síndrome hepatorrenal e níveis baixos de albumina antes do transplante. O uso profilático de norfloxacino pode aumentar o risco para infecção MR após o transplanteAbstract: Background: Infection is a frequent and severe complication of liver transplantation. However, widespread antibiotic use and frequent hospitalizations have led to the emergence of multidrug-resistant (MDR) bacteria that affects cirrhotic patients and may complicate post-transplantation care. Methods: We retrospectively reviewed all adults who underwent their first liver transplantation, and survived for ? 48 h, at a single Brazilian center (September 2010¿August 2013). The study outcomes included bacterial infection, multidrug-resistant (MDR) infection, complications, and outcomes. Patients were followed for ?1 year, until death, or until re-transplantation. We used the Kaplan-Meier method to evaluate survival and multivariate Cox regression analysis to identify infection risk factors. Results: Among 98 patients, bacterial infections were detected in 51 (52%) patients. Thirty-seven (72%) patients had positive cultures, and 24 (47%) patients had MDR infections. The risk factors for infection were previous hepatorenal syndrome (hazard ratio [HR]: 2.65; p = 0.015) and low pre-transplantation albumin levels (HR: 0.65; p = 0.044). Higher pre-transplantation international normalization ratio (HR: 1.69; p = 0.021) and prophylactic use of norfloxacin (HR: 2.78; p = 0.033) were risk factors for MDR infection. Significantly lower survival was associated with bacterial infection (log-rank p < 0.001), although MDR infection did not further affect survival (log-rank p = 0.712). Infection (HR: 4.11; p = 0.001) and larger red blood cell transfusions (HR: 1.08; p = 0.02) were independent mortality risk factors. Conclusions: Bacterial infections after liver transplantation are affected by pre-transplantation status, and are associated with mortality. Long-term norfloxacin prophylaxis can increase the risk of MDR infectionDoutoradoFisiopatologia CirúrgicaDoutor em Ciência

Evaluation of the Hepatocellular Carcinoma Predictive Scores PAGE-B and mPAGE-B among Brazilian Patients with Chronic Hepatitis B Virus Infection

Hepatitis B virus (HBV) is intrinsically oncogenic and related to hepatocellular carcinoma (HCC). Predictive scores of HCC have been developed but have been poorly studied in admixed populations. Therefore, we aimed to evaluate the performance of PAGE-B and mPAGE-B scores for HCC prediction in HBV Brazilian patients and factors related to HCC occurrence. This is a retrospective study that evaluated patients followed at a tertiary university center. A total of 224 patients were included, with a median follow-up period of 9 years. The mean age at HBV diagnosis was 38.71 ± 14.19 years, predominantly males (66.1%). The cumulative incidence of HCC at 3, 5, and 7 years was 0.993%, 2.70%, and 5.25%, respectively, being related in the univariate logistic regression analysis to male sex (p = 0.0461), older age (p = 0.0001), cirrhosis at HBV diagnosis (p p = 0.0002 and p p = 0.9767). In conclusion, both PAGE-B and mPAGE-B showed a correct prediction of HCC above 70% in this cohort

Weight Loss And Resting Energy Expenditure In Patients With Chronic Hepatitis C Before And During Standard Treatment.

Infection with hepatitis C virus (HCV) is a serious public health problem worldwide. In clinical studies, weight loss has been reported in 11% to 29% of patients treated with pegylated interferon-α-2a/2b. Few reports have tried to explain such a weight loss. The aim of this study was to evaluate nutritional status, body composition, and resting energy expenditure (REE) in patients with chronic hepatitis C before and during treatment with pegylated interferon and ribavirin. This was a prospective study with the evaluation of patients with hepatitis C virus before and after 12 wk of treatment with pegylated interferon and ribavirin. The evaluation consisted of anthropometry (weight, height, body mass index, and waist circumference), and body composition was determined by bioelectrical impedance analysis. The REE of each individual was obtained by indirect calorimetry. To compare the two phases of treatment, the Wilcoxon test was used. The significance level was 5%. Subjects had significant weight loss during treatment with a consequent decrease in body mass index. This weight decrease was accompanied by a significant decrease in body fat and no decrease in fat-free mass. There was a significant decrease in energy intake as assessed by 24-h recall. However, there was no change in REE and in REE corrected for fat-free mass. Our study of patients with hepatitis C treatment showed that these patients had significant weight loss and this was not associated with changes in energy expenditure. However, we observed a significant decrease in energy intake, pointing to a possible need for intervention measures to decrease the damage.28630-