Sensory neuronopathy is a specific and disabling neurological manifestation of autoimmune hepatitis.

BACKGROUND AND PURPOSE: Neurological manifestations have been identified in the context of autoimmune hepatitis (AIH). Previous case reports highlighted the association between AIH and sensory neuronopathy (SN). Despite that, little is known about the frequency of AIH-related SN and its clinical/neurophysiological profile. Moreover, it is not clear whether SN is an AIH-specific manifestation or related to chronic liver damage. METHODS: Seventy consecutive AIH patients were enrolled and their characteristics were compared with 52 consecutive patients with chronic active hepatitis B. All subjects underwent clinical and neurophysiological evaluation. Further comparisons were performed between AIH SN and AIH non-SN patients. RESULTS: Mean ages and male:female proportions in the AIH and chronic active hepatitis B groups were 42.2 ± 16.3/51.7 ± 13.6 years and 14:56/29:23, respectively. The frequencies of carpal tunnel syndrome, radiculopathy and polyneuropathy were similar between groups. In contrast, SN was identified only in AIH patients (5/70 vs. 0/52, P = 0.04); the overall prevalence of AIH-related SN was 7% with an average profile of a woman in her 40s with asymmetric onset of sensory deficits that chronically evolved to disabling proprioceptive ataxia associated with marked dysautonomia. Neurological disability and hepatocellular damage did not follow in parallel. Anti-fibroblast growth factor receptor type 3 antibodies were found in 3/5 (60%) of the patients with AIH-related SN. Clinical or demographic predictors of SN in the context of AIH could not be identified. CONCLUSION: Sensory neuronopathy, but not other peripheral nervous system diseases, is a specific AIH neurological manifestation. It is often disabling and, in contrast to hepatocellular injury, does not respond to immunosuppression.

Predicting survival after liver transplantation using up-to-seven criteria in patients with hepatocellular carcinoma.

BACKGROUND: Liver transplantation is a controversial issue in the initial management of hepatocellular carcinoma (HCC). The aim of this study was to demonstrate that up-to-seven could be useful as a model in evaluating potential candidates for liver transplantation for HCC. METHODS: Between January 1997 and December 2010, 84 patients undergoing liver transplantation were analyzed for HCC. Pathologic variables included the number of lesions, maximal tumor diameter, vascular invasion, and tumor grade. The following variables were recorded: Age, gender, recurrence of HCC, survival, donor age, incidental, Milan criteria, Model for End-Stage Liver Disease score, and alpha-fetoprotein (AFP) levels. To evaluate variables we used the Kaplan-Meier method, Cox regression and Kolmogorov-Smirnov test. RESULTS: There were 68 (80.9%) patients within up-to-seven criteria and 16 (19.1%) beyond this criteria. AFP levels were 96.4 versus 464.3 ng/dL in patients within versus beyond up-to-seven criteria. The median diameter of the largest nodule was 22.2 versus 32.0 mm in patients within versus beyond up-to-seven criteria, respectively. The median sum of nodules was 1.8 and 5.6 nodules in patients within versus beyond up-to-seven criteria. Vascular invasion was present in 11 (13.0%) patients within versus 7 (8.3%) beyond. Recurrence was observed in 9 (10.7%) patients and only 3 were beyond up-to-seven criteria, 2 had vascular invasion, and another showed poor differentiation. Post liver transplantation survival rates were 87.7%, 74.5%, and 65.3% at 1, 3, and 5 years in patients within up-to-seven versus 80.7%, 51.7%, and 32.1% beyond (P = .03). Similar results were observed when we applied Milan criteria or expanded Milan criteria. The largest nodule was the only predictive factor after proportional hazard Cox regression application (Beta = 0.037; exponent = 1.0377; Wald = 4.542; P = .03). In the present study, up-to-seven criteria could be useful to evaluate potential liver transplant candidates due to HCC.

Prognostic factors for hepatocellular carcinoma recurrence: experience with 83 liver transplantation patients.

INTRODUCTION: Orthotopic liver transplantation (OLT) is a rational therapeutic option for early-stage hepatocellular carcinoma (HCC) providing a potential cure and improving survival. METHODS: This retrospective study of a longitudinal cohort used an electronic database collected prospectively from September 1997 to May 2010. The variables were gender, age (years), and alpha-fetoprotein (AFP) level (ng/mL). In explanted livers we observed: microvascular or macrovascular invasion, number of nodules and their largest size, Edmondson-Steiner histological differentiation, incidental tumor transarterial chemoembolization (TACE), Milan criteria, and previous down-staging. RESULTS: Five of 83 (6.0%) subjects including 68 (82%) males with a mean time to diagnosis of 9 months experienced tumor relapses. Mean patient age at HCC recurrence was 55.3 years for male and 44.6 years for female subjects. Vascular invasion was detected in 17/83 (20.5%) subjects, namely 2% of macrovascular invasion, and 52.5% with expanded Milan criteria due to an increased number and size of nodules in the explanted livers. An incidental tumor was observed in 29.5% of cases. Preoperative TACE treatment was performed in 13 (15.6%) patients. None of the patients who had a HCC recurrence had undergone TACE. AFP level at the time of recurrence was around 1,900 ng/mL. The predictive factor for mortality was nodule size (P=.04; hazard ratio=0.0269; confidence interval [CI], 95% 0.0094-0.299). CONCLUSION: Patients with relapses showed the worst survival and tumor size was a predictive factor for recurrence.

Large benign hepatocellular nodules in cirrhosis due to chronic venous outflow obstruction: diagnostic confusion with hepatocellular carcinoma.

Budd-Chiari syndrome (BCS) in patients progressing to cirrhosis is an indication for liver transplantation. At this stage of disease, it is common to find large benign hepatocellular nodules (LBHNs) of undetermined cause that may be confused with hepatocellular carcinoma (HCC). Patients with indications for liver transplantation are currently classified according to the MELD (Model for End-Stage Liver Disease) severity score. When they fit Barcelona and Milan eligibility criteria for HCC, they receive 20 points. Thus, misdiagnosis of HCC leads to a privileged position on the waiting list. Herein, we have reported three BCS cases of cirrhotic patients who underwent liver transplantation; the pathologic results of their explanted livers showed LBHN. We analyzed three of 489 OLT who had chronic venous outflow obstruction (CVOO) the first case: was a 19-year-old man, with BCS of undetermined cause. The second 20-year-old female patients displayed BCS due to antiphospholipid syndrome the third, 45-year-old man had CVOO diagnosed preliminarily due to cryptogenic cirrhosis in the explanted liver. In the three cases, the nodules in the explant measured 0.5 to 2.4 cm. In the first case, the diagnosis was not in doubt; in the second case, 23 nodules were confused with HCC histologic evaluation, and in the third case three larger hypervascular nodules were misdiagnosed as HCC in the preoperative period despite low alpha-fetoprotein levels. In conclusion it is fundamental to recognize these benign lesions so as to avoid misdiagnosis, thereby allowing the proper selection of candidates for liver transplantation.

Survival before and after model for end-stage liver disease score introduction on the Brazilian liver transplant waiting list.

INTRODUCTION: To examine whether the official adoption of Model for End-Stage Liver Disease (MELD) as a criterion for organ allocation was effective, we studied risk factors for patient deaths and the accuracy of the MELD score to predict mortality. METHODS: Patients on the waiting list for liver transplantation were divided into two periods depending on whether they were on the waiting list before (period 1) or after (period 2) the MELD introduction in Brazil. The Kaplan-Meier method with log-rank tests were used to study patient survivals. Predictive factors were identified using the Cox regression method. A receiver operating characteristic (ROC) curve was used to analyze Child-Turcotte-Pugh (CTP) and MELD accuracy. RESULTS: We analyzed 295 patients in period 1 and 240 in period 2. The survivals after 3, 6, 9, and 12 months in periods 1 and 2, were 95.6%, 90.5%, 84.9%, and 69.6% vs 95.7%, 92.1%, 85.3%, and 83.3%, respectively (P = NS). Multivariate analysis showed CTP, MELD-Na, and albumin levels, besides spontaneous bacterial peritonitis (SBP), to be independent factors related to survival in period 1. In period 2, CTP, creatinine levels, international normalized ratio, besides spontaneous bacterial peritonitis, were the independent factors. The ROC curve for CTP was 0.676 and for MELD, 0.644 (P = .4) in period 1. In period 2, the ROC curve for CTP was 0.680 and for MELD, 0.718 (P = .4). CONCLUSION: Patient survival on the waiting list for liver transplantation did not change at 1 year after the introduction of the MELD.

Portal vein thrombosis and liver transplantation: long term.

Obstruction of the portal vein may be related to constriction by malignant tumors or thrombosis associated with liver disease. We herein have reported our experience with patients undergoing liver transplantation with portal vein thrombosis (PVT) whose diagnosis was made intraoperatively. From September 1991 to May 2009, we studied 27/419 (6.4%) patients with PVT who were evaluated according to the presence of esophagogastric varices, underlying disease, malignancy, and if there was previous surgery, review of medical records on data collected prospectively. We observed 24 (88.9%) patients with PVT grade 1, 2 (7.4%) with grade 2, and 1 (3.7%) with grade 3. The average age of the PVT patients was 47.5 years; the average model for End-Stage Liver Discase score was 18.3, and the predominant diagnosis, hepatitis C cirrhosis. Eighteen underwent a sclerotherapy/ligature. The sensitivity of ultrasound for grade 1 thrombosis was 39.1%; for grade 2, 50%; and for grade 3, 100%. Portal vein thrombectomy was performed in 24 patients. In other patients (grade 2), we performed an anastomosis of the donor portal vein to the recipient gastric vein or to a greater splanchnic collateral vein. In only 1 patient was the graft performed using the donor portal vein-donor iliac vein-recipient superior mesenteric vein. None of the patients displayed PVT in the immediate postoperative period. Actuarial survivals at the years 1, 3, and 5 were 85%, 74%, and 63%, respectively. We concluded that PVT cannot be considered to be a contraindication for liver transplantation.

Gastrite crônica associada ao Helicobacter pylori em pacientes com dispepsia näo ulcerosa e com úlcera duodenal / Chronic gastritis associated to Helicobacter pylori in patients with non-ulcerating dyspepsia and with duodenal ulcer

Prevalência da gastrite crônica e da infecçäo da mucosa gástrica por Helicobacter pylori em pacientes com dispepsia näo ulcerosa e com úlcera duodenal. Tipo de estudo, local, pacientes: Foram estudados, prospectivamente, 48 pacientes consecutivos com dispepsia näo ulcerosa (DNU) do tipo dismotilidade e 13 pacientes consecutivos com úlcera duodenal (UD) em atividade, selecionados no ambulatório de Gastrenterologia do Hospital das Clínicas da Universidade Estadual de Campinas (Unicamp). Intervençöes: Em cada um dos pacientes, foram realizadas oito biópsias endoscópicas (quatro do corpo e quatro do antro gástrico), para identificaçäo do H. pylori, utilizando-se três testes: urease, gram e exaqme histopatológico. Medidas e resultados: Nos 48 pacientes com DNU, o teste de urease foi postivo em 89,6% no antro e 81,2% no corpo; o gram foi positivo em 81,2% no antro e em 77,1% no corpo; e o H. pylori foi identificado no exame histopatológico em 79,2% no antro e em 70,8% no corpo gástrico. Na mucosa do antro gástrico de todos os 48 pacientes com DNU, pelo menos um dos três testes empregados foi positivo. Em todos os 13 pacientes com UD, os três testes foram positivos no antro gástrico, todos com gastrite crônica do antro no exame histopatológico. Gastrite crônica do corpo gástrico, com presença do HY. pylori no exame histopatológico, foi encontrada em 10 dos 13 pacientes. O teste de urease foi positivo na mucosa do corpo gástrico em todos os casos de UD. Conclusöes: Os autores acreditam que a alta prevalência dos testes empregados para identificaçäo fo H. pylori nos pacientes com DNU possa ser explicada pelo grupo selecionado de pacientes pertencentes a uma classe socioeconômica menos favorecida. A presença do H. pyloru na mucosa gástrica de todos os pacientes com UD está de acordo com algums trabalhos já publicados