Combined liver-kidney transplant in polycystic diseases: a case report.

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.

Combined liver-kidney transplant in polycystic diseases: a case report

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.

Influence of LGALS3 and PNPLA3 genes in non-alcoholic steatohepatitis (NASH) in patients undergone bariatric surgery.

AIM: This study evaluated the genesPNPLA3 and LGALS3 in patients who have undergone bariatric surgery. METHODS: Individuals with NAFLD and NASH were evaluated, the DNA was extracted from total blood for genotyping of rs4644, rs4652 from LGALS3 and rs738409 from PNPLA3 genes, the total RNA was obtained from liver biopsy. For the detection of the molecular targets, real-time PCR through Taqman probes was used. RESULTS: From a total of 46 collected patients, of those 21 (456%) were included as NASH and 25 (544%) as steatosis group. This groups showed significant difference to aspartate aminotransferase (AST), alanine aminotransferase (ALT) and Glutamyl transpeptidase (GGT) (p = 0.0108, p = 0.0090 and p = 0.0044). Regarding to gene expression in studied groups, hepatic steatosis vs NASH, we observed a higher expression of the LGALS3 gene in NASH (p = 0.0273). In addition, patients with C allele in homozygous for rs4644 and rs4652 of LGALS3 gene had higher expression, in NASH group (p = 0.0500 and p = 0.0242, respectively), furthermore for rs4644 both alleles in homozygous showed higher expression (AA/CC vs AC) (p = 0.0500), when analyzed PNPLA3 rs738409, NASH patients with G allele in homozygous had higher expression (p = 0.0494). CONCLUSIONS: Therefore, an increased expression of the LGALS3 gene in patients with NASH may be important in the etiopathogenesis of the disease, as well as the presence of rs4652 and rs4644 SNPs in the regulation of transcriptional levels of the gene in patients with NAFLD and NASH.

Schistosomal liver fibrosis and hepatocellular carcinoma - case series of patients submitted to liver transplantation

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.

Schistosomal liver fibrosis and hepatocellular carcinoma - case series of patients submitted to liver transplantation.

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.

Proton nuclear magnetic resonance-based metabonomic models for non-invasive diagnosis of liver fibrosis in chronic hepatitis C: Optimizing the classification of intermediate fibrosis.

AIM: To develop metabonomic models (MMs), using 1H nuclear magnetic resonance (NMR) spectra of serum, to predict significant liver fibrosis (SF: Metavir ≥ F2), advanced liver fibrosis (AF: METAVIR ≥ F3) and cirrhosis (C: METAVIR = F4 or clinical cirrhosis) in chronic hepatitis C (CHC) patients. Additionally, to compare the accuracy of the MMs with the aspartate aminotransferase to platelet ratio index (APRI) and fibrosis index based on four factors (FIB-4). METHODS: Sixty-nine patients who had undergone biopsy in the previous 12 mo or had clinical cirrhosis were included. The presence of any other liver disease was a criterion for exclusion. The MMs, constructed using partial least squares discriminant analysis and linear discriminant analysis formalisms, were tested by cross-validation, considering SF, AF and C. RESULTS: Results showed that forty-two patients (61%) presented SF, 28 (40%) AF and 18 (26%) C. The MMs showed sensitivity and specificity of 97.6% and 92.6% to predict SF; 96.4% and 95.1% to predict AF; and 100% and 98.0% to predict C. Besides that, the MMs correctly classified all 27 (39.7%) and 25 (38.8%) patients with intermediate values of APRI and FIB-4, respectively. CONCLUSION: The metabonomic strategy performed excellently in predicting significant and advanced liver fibrosis in CHC patients, including those in the gray zone of APRI and FIB-4, which may contribute to reducing the need for these patients to undergo liver biopsy.

Transplante de fígado em paciente com hepatocarcinoma fibrolamelar - relato de caso / Liver transplantation in patient with fibromellar hepatocarcinoma - case report

O hepatocarcinoma fibrolamelar (HCC-FL), variante do hepatocarcinoma (HCC), é uma neoplasia rara, responsável por 0,6-8,6% das neoplasias de origem no hepatócito. O diagnóstico é feito através de exames de imagem e confirmado pelo exame anatomopatológico. O transplante de fígado (TF) apresenta-se como tratamento curativo do HCC-FL. Neste relato, documentou-se um caso de hepatocarcinoma fibrolamelar irressecável tratado, de maneira curativa, com o transplante de fígado.

Fibrolamellar hepatocelullar carcinoma (FHCC), a variant of conventional hepatocelullar neoplasms originated in the carcinoma, is a rare neoplasm, responsible for 0.8-8.6% of all hepatocarcinomas. Diagnose is performed through image scans and confirmed through the anatomopatological examination. Liver ressection and liver transplantation are shown as a curative treatment for the FHCC. In this report, we documented a case of unresectable fibrolamellar hepatocellular carcinoma curatively treated with liver transplantation.

Estudo de gene da lectina ligadora de manose em doador falecido e transplante de fígado - relato de caso / Gene study of mannose-binding lectin in deceased donor and liver transplantation - case report

A hepatite autoimune é uma doença de curso crônico e etiologia ainda não esclarecida, que em alguns casos evolui para falência hepática aguda, condição em que o tratamento definitivo é o transplante hepático. Assumidos todos os riscos da cirurgia, restam ainda outras variáveis que concorrem para o êxito do tratamento, como a influência do sistema imune. Dentre essas, uma alteração na formação da lectina ligadora de manose (MBL), presente no fígado do doador, está relacionada a uma maior predisposição do receptor a eventos infecciosos. Relata-se o caso de uma paciente de 18 anos, submetida ao transplante hepático por falência hepática aguda, que, no pós-operatório, apresentou choque séptico de origem desconhecida, evoluindo a óbito. O doador foi genotipado com polimorfismo da MBL relacionado a níveis intermediários ou baixos de MBL sérica. Complicações infecciosas pós-transplantes continuam sendo frequentes nos receptores de órgãos. Assim, a utilização de parâmetros modificadores de resposta imune, como a MBL, poderá ajudar a melhorar o prognóstico desses pacientes.

Autoimmune hepatitisis mostly a chronic disease, which etiology remains unclear, progressing in some cases to acute liver failure, a condition whose definitive treatmentis liver transplantation. Assume dallrisks of surgery, there are still other variables that contribute to the success of the treatment, as the influence of the immune system. Among these, a change in the formation of mannose bindin glectin (MBL), present in the do nor liver, is related to increased predisposition to infectious events in the receptor. It is reported a case of a 18 year old patient who under went liver transplantation for acute liver failure, which evolved post operatively to septics hockofunk now no riginand death. The liver donor was genotyped with polymorphis mof MBL, related to intermediate or low level sof sérum MBL. Post-operative infectious complications remain common in organ recipients. Thus, the use of modified parameters in immune response, such as MBL, may help to improve the prognosis of these patients.

Could gastric histology be a useful marker for making decision on Helicobacter pylori eradication therapy in patients with dyspepsia?

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 (1/4) 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 (1/4) of our study patients with dyspepsia.

Could gastric histology be a useful marker for making decision on Helicobacter pylori eradication therapy in patients with dyspepsia? / É a histologia gástrica um marcador útil na decisão de erradicar o Helicobacter pylori nos pacientes com dispepsia?

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 » had moderate (25 percent) or severe (2.5 percent) gastritis. This subgroup of patients also had a greater positive frequency of anti-Hp (100 percent vs 41 percent; P = 0.0005) and anti-CagA (91 percent vs 58 percent; P = 0.09) antibodies when compared with those with normal histology (27.5 percent) or mild gastritis (45 percent). 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 » of our study patients with dyspepsia.

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 » apresentava gastrite moderada (25 por cento) ou severa (2,5 por cento). Esse grupo também apresentava maior frequência de positividade anti-Hp (100 por cento vs 41 por cento; P = 0,0005) e anti-CagA (91 por cento vs 58 por cento; P = 0,09) quando comparado com os casos com histologia normal (27,5 por cento) ou gastrite leve (45 por cento). 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 » da presente amostra.

Transplante de figado em Hospital Publico de Pernambuco / Liver transplantation in a public Hospital, recife-Pernambuco -Brasil

Os autores apresentam os oito primeiros casos de transplante ortotopico de figado em adultos, com doador cadaver, realizados em Hospital Universitario do Recife, capital de uma regi'ao pobre e populosa do Brasil. As intervencoes foram realizadas em condicoes estruturais adequadas, mas ainda sub-otimas. Mesmo assim todos os pacientes obtiveram alta hospitalar e passaram a viver normalmente. Os autores concluem que o transplante hepatico deve ser estimulado no Nordeste, onde existe uma demanda estimada de 900 transplate por ano