ABSTRACT Polycystic liver disease, a hereditary pathology, usually manifests as autosomal dominant polycystic kidney disease. The many cysts in the liver cause massive hepatomegaly, majorly affecting the patient’s quality of life. In cases of refractory symptoms, liver transplantation is the only treatment choice. A 43-year-old woman was followed up as a hepatology outpatient in August 2020, with a progressive increase in abdominal volume, lower limb edema, and cachexia. The patient was diagnosed with polycystic renal and liver disease with massive hepatomegaly in March 2021, a combined kidney-liver transplant. Liver size represented 13% of the patient’s corporal composition, weighing 8.6kg. The patient was discharged on the 7th postoperative day with no complications. Only 10-20% of patients with polycystic liver disease have clinical manifestations, most of which result from hepatomegaly. An increase in liver volume deteriorates liver function until the condition becomes end-stage liver disease, as kidney function is already compromised; liver-kidney transplantation remains the only treatment choice. The case described drew significant attention to the massive hepatomegaly presented in the patient, with the liver representing over 10% of the patient’s body weight, approximately five to six times larger than a normal-sized liver.

ABSTRACT Schistosomiasis affects approximately 207 million people in 76 countries. The association between hepatocellular carcinoma and Schistosoma mansoni infection has been investigated. Studies using animal models suggest that the parasite may accelerate the oncogenic process when combined with other factors, such as hepatitis C virus infection or exposure to a carcinogen. Herein, we report a case series of six hepatocellular carcinoma patients from Northeast Brazil, with negative serology for both hepatitis B and C virus, submitted to liver transplantation, whose explant showed evidence of schistosomal liver fibrosis. Since all patients enrolled in this study were submitted to liver transplantation, we were able to access the whole explanted liver and perform histopathological analysis, which is often not possible in other situations. Although 50% of them showed signs of liver failure, no cirrhosis or any liver disease other than schistosomal fibrosis had been detected. These uncommon findings suggest that Schistosoma mansoni infection might predispose to hepatocellular carcinoma development, regardless of the absence of other risk factors.

Contexto Dados em relação às alterações vasculares em pacientes com esquistossomose hepatoesplênica e colopatia hipertensiva portal e suas modificações após cirurgia para atenuação da hipertensão portal são restritos. Objetivo Analisar as alterações da colopatia hipertensiva portal antes e seis a 12 meses após a esplenectomia e desvascularização gástrica. Métodos Foram estudados prospectivamente 12 pacientes com esquistossomose hepatoesplênica e antecedente de hemorragia digestiva alta. Os achados colonoscópicos antes e após 6 a 12 meses após a cirurgia foram analisados. Nesses períodos, biopsias da mucosa do cólon ascendente, sigmóide e reto foram encaminhadas para análise histológica e histomorfométrica. Foi utilizado um grupo controle pela falta de padrão de normalidade das medidas histomorfométricas das vênulas do cólon e reto em indivíduos sem hipertensão portal. O nível de significância crítica adotado em todos os testes foi de probabilidade máxima de erro de 5%. Resultados Não foram encontradas diferenças significantes na intensidade das alterações endoscópicas e histológicas nos vasos da mucosa do cólon e reto após a cirurgia. Entretanto, houve decréscimo estatisticamente significante nas áreas, diâmetros e espessuras dos vasos estudados através da histomorfometria. Conclusão Cirurgia para descompressão da hipertensão portal esquistossomótica tem efeito benéfico na colopatia associada, sendo bem indicada nos pacientes com hemorragia digestive alta e varizes esofágicas.

Context Data on vascular alterations in patients with hepatosplenic schistosomiasis and portal hypertensive colopathy and changes in these after surgery to decrease portal hypertension are limited. Objective The purpose of this study was to analyse the alterations of portal hypertensive colopathy previously and 6-12 months after splenectomy and gastric devascularization. Methods Twelve patients with hepatosplenic schistosomiasis who also had upper gastrointestinal bleeding were studied prospectively. Their endoscopic findings before and 6-12 months after the surgery were analysed. In addition, mucosal biopsies from ascending colon, sigmoid colon and rectum at these time points were subjected to histological and histomorphometric assessment. It was used a control group due to lack of normal pattern of the histomorphometric measures of vessels in individuals without portal hypertension. The critical level of significance adopted in all tests was of a maximum probability error of 5%. Results Surgery did not lead to significant improvement in histological and endoscopic findings. However, on histomorphometry, there was a significant decrease in the area, diameter and thickness of the vessels in mucosa at all colonic sites. Conclusion Surgery for decompression of schistosomal portal hypertension has a beneficial effect on the associated colopathy, being best indicated in patients with gastrointestinal bleeding and esophageal varices.

Liver biopsy is the gold-standard method to stage fibrosis; however, it is an invasive procedure and is potentially dangerous. The main objective of this study was to evaluate biological markers, such as cytokines IL-13, IFN-γ, TNF-α and TGF-β, platelets, bilirubins (Bil), alanine aminotransferase (ALT) and aspartate aminotransferase (AST), total proteins, γ-glutamil transferase (γ-GT) and alkaline phosphatase (AP), that could be used to predict the severity of hepatic fibrosis in schistosomiasis and hepatitis C (HC) as isolated diseases or co-infections. The following patient groups were selected: HC (n = 39), HC/hepatosplenic schistosomiasis (HSS) (n = 19), HSS (n = 22) and a control group (n = 13). ANOVA and ROC curves were used for statistical analysis. P < 0.05 was considered significant. With HC patients we showed that TNF-α (p = 0.020) and AP (p = 0.005) could differentiate mild and severe fibrosis. With regard to necroinflammatory activity, AST (p = 0.002), γ-GT (p = 0.034) and AP (p = 0.001) were the best markers to differentiate mild and severe activity. In HC + HSS patients, total Bil (p = 0.008) was capable of differentiating between mild and severe fibrosis. In conclusion, our study was able to suggest biological markers that are non-invasive candidates to evaluate fibrosis and necroinflammatory activity in HC and HC + HSS.

CONTEXTO: O benefício da terapia de erradicação do H. pylori como parte do tratamento da dispepsia funcional ainda é uma questão em aberto. Diferenças na distribuição geográfica, a ampla variabilidade genética e o fato de que a expressão clínica da infecção está fortemente relacionada com a virulência das cepas infectantes, são fatores que provavelmente guiam as controvérsias. OBJETIVO: Estudar a correlação entre histologia gástrica e sorologia para H. pylori em doentes com dispepsia. MÉTODOS: Estudo descritivo-transversal com 40 pacientes consecutivos com sintomas dispépticos (28 mulheres e 12 homens, média de idade de 48,5 anos) e achado endoscópico de estômago normal, selecionados a partir da sala de endoscopia (Hospital das Clínicas da Universidade Federal de Pernambuco, Recife, PE.) entre março de 1998 e julho de 1999. Todos foram submetidos a biopsias gástricas e testes sorológicos (anti-Hp e anti-CagA). As biopsias foram analisadas pelos métodos de H-E e Giemsa e os achados de gastrite classificados de acordo com o Sistema Sydney atualizado. RESULTADOS: A histologia dos 40 pacientes revelou que cerca de ¼ apresentava gastrite moderada (25%) ou severa (2,5%). Esse grupo também apresentava maior frequência de positividade anti-Hp (100% vs 41%; P = 0,0005) e anti-CagA (91% vs 58%; P = 0,09) quando comparado com os casos com histologia normal (27,5%) ou gastrite leve (45%). CONCLUSÃO: Considerando que a endoscopia digestiva alta é parte da rotina de investigação da dispepsia funcional e que a sorologia anti-CagA não está disponível na prática clínica diária, através da histologia pode-se selecionar e aplicar terapia de erradicação do H. pylori apenas para os casos que muito provavelmente estão associados a cepas patogênicas de H. pylori (doentes com gastrite moderada ou severa) - cerca de ¼ da presente amostra.

CONTEXT: It still remains an open debate whether Helicobacter pylori eradication is beneficial or not for the improvement of symptoms in functional dyspepsia. Differences in geographic distribution, the worldwide H. pylori genetic variability and the fact that the outcome of infection is strongly related to the virulence of the infecting strain are factors that might be driving ongoing controversies. OBJECTIVE: To study the correlation between gastric histology and H. pylori serology status in patients with dyspepsia. METHODS: This is a cross-sectional study where 40 consecutive dyspeptic patients (28 women and 12 men, mean age 48.5 years) with endoscopically normal stomachs were selected from the endoscopy unit at a university hospital in Recife, PE, Northeast of Brazil, between March 1998 and July 1999. Patients underwent gastric mucosal biopsy and serological tests (anti-Hp and anti-CagA antibodies). Gastric biopsies were examined using H-E and Giemsa stains and gastritis was classified and graded (mild, moderate or severe) according to "the updated Sydney System - Houston, 1994". RESULTS: Among 40 patients with dyspepsia the gastric histology revealed that about ¼ had moderate (25%) or severe (2.5%) gastritis. This subgroup of patients also had a greater positive frequency of anti-Hp (100% vs 41%; P = 0.0005) and anti-CagA (91% vs 58%; P = 0.09) antibodies when compared with those with normal histology (27.5%) or mild gastritis (45%). CONCLUSION: Since upper gastrointestinal endoscopy is part of the functional dyspepsia investigation and serology for anti-CagA antibody is not available in daily clinical practice, by biopsying gastric mucosa we would only be able to selectively apply H. pylori eradication therapy for those with histology that best correlate with virulent infecting strains (moderate or severe gastritis) - around ¼ of our study patients with dyspepsia.

Evaluation of hepatic fibrosis is usually performed by histopathological examination of biopsies. However, this is an invasive and potentially dangerous procedure. Several studies have proposed serum biological markers of hepatic fibrosis. This communication evaluates the use of serum cytokines as markers of hepatic fibrosis in hepatitis C, schistosomiasis, and co-infection.

Portal hypertension is a frequent complication of chronic liver disease, detected not only in schistosomiasis, but also in cirrhosis of any etiology. Vascular alterations in the colonic mucosa are a potential source for acute or chronic bleeding and have been observed in patients with portal hypertension. The purpose of this prospective study was to describe and propose a classification for the vascular alterations of portal hypertension in the colonic mucosa among patients with hepatosplenic schistosomiasis mansoni. One or more alterations of portal colopathy were observed in all patients and they were classified according to their intensity, obeying the classification proposed by the authors. Portal colopathy is an important finding in hepatosplenic schistosomiasis and might be the cause of lower gastrointestinal bleeding in patients with severe portal hypertension.

Geographical differences in the prevalence of Helicobacter pylori genes and their association with disease severity have been identified. This study analyzes the prevalences of the cagA gene and alleles of the vacA gene in H. pylori-associated gastroduodenal diseases in isolates from Recife, PE, Brazil. Gastric biopsy of 61 H. pylori-positive patients were submitted to DNA extraction and gene amplification by polymerase chain reaction. Among the 61 patients, 21 suffered from duodenal ulcer (DU) and 40 from gastritis (GT). The prevalence of H. pylori strains harbouring the cagA gene was higher in the DU group (90.5%) than in the GT group (60%) (p = 0.02). The vacA gene was amplified in 56 out of 61 biopsies, of which 43 (76.8%) contained bacteria carrying the s1 allele and 13 (23.2%) the s2. However, the prevalence of the vacA s1 genotying was the same in either DU or GT group. The majority of the s1-typed strains, 39 (90.7%) out of 43, were subtype s1b. In resume there was a strong association between the H. pylori cagA+ gene and DU. However, there were no differences between the DU and GT groups in relation to the vacA s1 and s2 alleles distribution, albeit the subtype s1b was predominat.

Objetivando melhor compreender a fisiopatologia da esquistossomose grave em adultos e crianças, avaliaram-se diferenças histopatológicas do fígado e do padrão de endoscopia digestiva entre esses pacientes. Vinte e um adultos e igual número de crianças foram incluídos no estudo. Todos foram submetidos a esplenectomia e ligadura da veia gástrica esquerda. Os achados histopatológicos foram avaliados em secções de biópsia hepática. Utilizou-se videoendoscópio para endoscopia digestiva. Nos adultos foram encontradas: 1 - fibrose de Symmers, grau I, em um paciente; grau II, em sete; e grau III, em treze; 2 - atividade inflamatória mínima em cinco; leve, em sete; moderada, em seis; e intensa, em um; ausência de atividade inflamatória, em dois; 3 - mínima granulomatose, em quatro pacientes; moderada, em dois; ausência de granulomas, em quinze; 4 - pigmento esquistossomótico mínimo, em quatro pacientes; moderada, em um; ausência, em dezesseis. Nas crianças, foram encontradas: 1 - fibrose de Symmers, grau I, em um paciente; grau II, em quatro e grau III, em dezesseis; 2 - atividade inflamatória mínima, em sete pacientes; leve, em quatorze; 3 - mínima granulomatose, em seis pacientes; leve, em cinco; moderada, em dois; ausência, em oito; 4 - pigmento esquistossomótico mínimo, em seis pacientes; leve, em três; moderada, em seis; ausência, em seis. Na endoscopia digestiva alta encontrou-se nos adultos: oito (38%) com varizes de médio calibre e treze (62%), de grosso calibre. Oito pacientes (38%) tiveram varizes de fórnix gástrico e cinco (24%), da cárdia. Gastropatia da hipertensão porta foi diagnosticada em dezesseis pacientes (76%). Com relação às crianças, quatro (19%) apresentaram varizes de fino calibre, oito (38%) de médio; e nove (43%) de grosso calibre. Três (14%) tiveram varizes gástricas, sendo duas no fórnix e uma no cárdia. Nove (43%) das crianças apresentavam gastropatia de hipertensão porta. Os achados evidenciaram uma histopatologia hepática similar nos dois grupos; entretanto, as repercussões hemodinâmicas da hipertensão porta foram mais importantes nos adultos, sem, no entanto, alcançar significação estatística.

Aiming to get a better understanding on the pathogenesis of severe schistosomiasis mansoni in adults and children, the differences on the liver histopathology and the pattern of upper digestive endoscopy among these patients were studied, Twenty-one adults and equal number of children were included in this investigation. All patients underwent splenectomy and ligature of the left gastric vein. The pathological findings were assessed on the sections of wedge liver biopsy. The pathological findings in adults were Symmer’s fibrosis grades - I, in one; II, in seven; and III, in thirteen. Minimal inflammatory activity was observed in five, fair in seven, moderate in six and severe in one. Two patients showed no inflammatory activity. Minimal granuloma reaction was observed in four patients and moderare in two; fifteen had no such reaction. Minimal schistosomotic pigmentation was observed in four patients and moderare in one, howeve1; in sixteen patients, such pigmentation was not observed. in children, Symmer's fibrosis grade I was observed in one patient, grade II in four and grade III in sixteen. Minimal inflammatory reaction was observed in seven patients; and fair reaction in fourteen. Minimal granuloma reaction in six patients, fair in five, and moderate in two; in eight patients such reaction was not observed. Minimal schistosomotic pigmentation was observed in six patients, fair in three, moderare in six, and not observed in six patients. Upper digestive endoscopy showed the following results. Adults: medium size esophageal varices in eight (38%) patients, and large size in thirteen (62%). Varices in the pyloric part of the stomach were found in eight patients (38%), and five (24%) in the body. Portal hypertension gastropathy was seen in sixteen patients (76%). Children: four (19%) showed thin esophageal varices, eight (38%) medium size varices, and nine (43%) large size varices. Three (14%) patients showed gastric varices, two in the pyloric region and one in the body. Nine children (43%) showed portal hypertension gastropathy. From these results, the following conclusions can be drawn although the differences were not statistically significant: liver histopathology tended to be more evident in children, while hemodynamic portal hypertension repercussions were more severe in adults.