RESUMO
Chronic fatigue and an impairment of general health-related quality of life (HRQoL) are frequently reported by patients with primary sclerosing cholangitis (PSC). Studies on patients with primary biliary cholangitis (PBC) suggest that, unlike pruritus, fatigue may not be ameliorated by liver transplantation (LT). However, there are few data regarding the assessment of fatigue before and after transplantation in PSC. To investigate the effect of LT on fatigue and HRQoL in patients with PSC, 81 patients with PSC (median age 33 years; 69% men) were prospectively enrolled in this study. The PBC-40 and Short Form 36 (SF-36) questionnaires were used for assessment before and twice after LT. A total of 26 patients who received a transplant for PBC were included as controls. The potential impact of the clinical and laboratory parameters was evaluated by univariate and multivariate analyses. Although in addition to other well-being indexes the median fatigue score improved after LT (P < 0.001), a detailed analysis demonstrated that fatigue persists in one-third of patients. A significant fatigue reduction was seen in men (P < 0.001) but not women (P = 0.25). Posttransplant fatigue did not depend on concomitant inflammatory bowel disease, laboratory indexes of cholestasis, or disease recurrence. In the multivariate regression model, female sex was the only independent covariate associated with persistent fatigue. In terms of other measures of HRQoL, LT caused a substantial improvement in the majority of SF-36 and PBC-40 domains. Recurrent PSC and unemployment negatively affected the well-being of patients. Patients who received a transplant for PSC had significantly better HRQoL than those patients with PBC. LT improves various measures of HRQoL, but it does not ameliorate fatigue in female patients with PSC.
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Colangite Esclerosante , Síndrome de Fadiga Crônica , Cirrose Hepática Biliar , Transplante de Fígado , Adulto , Colangite Esclerosante/complicações , Colangite Esclerosante/cirurgia , Feminino , Humanos , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/cirurgia , Transplante de Fígado/efeitos adversos , Masculino , Qualidade de VidaRESUMO
BACKGROUND Orthotopic liver transplantation (OLT) is the standard of care for end-stage liver disease. The Charlson Comorbidity Index (CCI) was originally created to assess the survival rate of patients with chronic diseases, although it was modified and adopted in OLT recipients as CCI-OLT. MATERIAL AND METHODS In total of 248 consecutive liver transplant recipients with viral cirrhosis in 98 (39.5%) patients were included. CCI-OLT was calculated assigning a weight of 3 to chronic obstructive pulmonary disease; weight of 2 to coronary artery disease, connective tissue disease, and renal insufficiency; and a weight of 1 to diabetes mellitus. RESULTS CCI-OLT was significantly correlated with recipient age (p<0.001; R=0.333) and was a significant risk factor for early post-transplant mortality (p=0.004). The presence of diabetes mellitus significantly increased the odds of early mortality (p=0.010). The optimal cut-off for CCI-OLT in prediction of mortality during the first 90 days after transplantation was ≥1, with an AUROC of 0.780 (95% CI: 0.670-0.891; p<0.001). Increasing CCI-OLT was a significant risk factor for worse 5-year post-transplant survival (p=0.001), along with coronary artery disease (p=0.008) and diabetes mellitus (p=0.021). The optimal cut-off for prediction of 5-year mortality for CCI-OLT was ≥1, with the AUROC of 0.638 (95% CI: 0.544-0.733; p=0.004). CONCLUSIONS CCI-OLT is a useful tool for measuring the effect of pretransplant comorbidities and to stratify the effect of risk on both short- and long-term outcomes after OLT. Recipient age and diabetes strongly affected short-term survival after OLT, and metabolic and vascular complications were the leading causes of death at 5 years after OLT.
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Doença Hepática Terminal/mortalidade , Transplante de Fígado/mortalidade , Adulto , Idoso , Doença Crônica , Comorbidade , Doenças do Tecido Conjuntivo/complicações , Doença das Coronárias/complicações , Diabetes Mellitus , Doença Hepática Terminal/complicações , Feminino , Sobrevivência de Enxerto , Humanos , Cirrose Hepática/complicações , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Polônia , Doença Pulmonar Obstrutiva Crônica , Insuficiência Renal/complicações , Fatores de Risco , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Gyromitra esculenta, also known as "false morel" is one of the most poisonous mushrooms. This species is found all over the world, growing in coniferous forest in early spring time. Common manifestation of poisoning includes gastrointestinal symptoms which include varied degrees of liver impairment. We describe three cases: acute liver injury, acute liver failure and acute-on-chronic liver failure due to G. esculenta poisoning. At admission patients presented with encephalopathy and features of liver failure. Two of them recovered completely following supportive management while the remaining patient who also had preexisting liver disease developed multiorgan failure and subsequently died. Although a rare occurrence, G. esculenta poisoning should be considered in the differential diagnosis of acute liver failure.
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Insuficiência Hepática Crônica Agudizada/etiologia , Fígado/efeitos dos fármacos , Manihot/intoxicação , Intoxicação Alimentar por Cogumelos/complicações , Insuficiência Hepática Crônica Agudizada/diagnóstico , Idoso , Diagnóstico Diferencial , Evolução Fatal , Humanos , Fígado/diagnóstico por imagem , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/etiologia , Masculino , Pessoa de Meia-Idade , Intoxicação Alimentar por Cogumelos/diagnósticoRESUMO
BACKGROUND: Iatrogenic bile duct injuries (BDIs) are mostly associated with laparoscopic cholecystectomy but may also occur following gastroduodenal surgery or liver resection. Delayed diagnosis of type of injury with an ongoing biliary leak as well as the management in a non-specialized general surgical units are still the main factors affecting the outcome. CASE PRESENTATION: Herein we present three types of BDIs (Bismuth type I, IV and V) following three different types of upper abdominal surgery, ie. Billroth II gastric resection, laparoscopic cholecystectomy and left hepatectomy. All of them were complex injuries with complete bile duct transections necessitating surgical treatment. All were also very difficult to treat mainly because of a delayed diagnosis of type of injury, associated biliary leak and as a consequence severe inflammatory changes within the liver hilum. The treatment was carried out in our specialist hepatobiliary unit and first focused on infection and inflammation control with adequate biliary drainage. This was followed by a delayed surgical repair with the technique which had to be tailored to the type of injury in each case. CONCLUSION: We emphasize that staged and individualized treatment strategy is often necessary in case of a delayed diagnosis of complex BDIs presenting with a biliary leak, inflammatory intraabdominal changes and infection. Referral of such patients to expert hepatobiliary centres is crucial for the outcome.
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Doenças dos Ductos Biliares/etiologia , Ductos Biliares/lesões , Complicações Pós-Operatórias/cirurgia , Adulto , Colecistectomia Laparoscópica/efeitos adversos , Drenagem , Feminino , Hepatectomia/efeitos adversos , Humanos , Doença Iatrogênica , Fígado/patologia , Masculino , Pessoa de Meia-IdadeAssuntos
Adenocarcinoma/secundário , Colangite Esclerosante/cirurgia , Colectomia , Neoplasias do Colo/patologia , Transplante de Fígado , Neoplasias Esplênicas/secundário , Adenocarcinoma/diagnóstico por imagem , Adulto , Colangite Esclerosante/complicações , Colite Ulcerativa/complicações , Colite Ulcerativa/cirurgia , Neoplasias do Colo/complicações , Neoplasias do Colo/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Esplênicas/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Mini-Mental State Examination (MMSE) is one of the most commonly used methods in the assessment of cognitive mental status. MMSE has been used in hepatology but its usefulness in the evaluation of hepatic encephalopathy (HE) has never been properly assessed. The aim of the study was to investigate the value of MMSE in detection of HE in patients with cirrhosis. METHODS: One hundred and one consecutive patients with liver cirrhosis underwent neurological examination, MMSE and electroencephalography (EEG). Spectral analysis of EEG was done with calculation of mean dominant frequency (MDF) and relative power of delta, theta, alpha and beta rhythms. Minimal HE was diagnosed in patients with normal neurological status and alterations in spectral EEG. Statistical analysis included Fisher's exact and Anova analysis. Categorical data were compared using Levene's test for equality of variances. Correlation-coefficient analysis was performed by the Pearson's r or Z-test, as needed. Tests performance was assessed by the calculating the area under the ROC curve (AUC) and evaluating its difference from reference area (AUC=0.5). A p value <0.05 was considered statistically significant. RESULTS: Overt HE was identified in 49 (48.5%) and minimal HE in 22 (21.8%) patients. Although there were significant correlations between both severity of liver disease (Child-Pugh classification), overt HE (West-Haven criteria) and various MMSE items, MDF showed no correlation with any of MMSE items as well as MMSE summary score. MMSE (score and items) did not discriminate patients without HE and minimal HE. The only significant differences between patients without HE and with overt HE were seen in respect of MMSE score (p<0.02), orientation to place (p<0.003), repetition (p<0.01) and complex commands-understanding (p<0.02). Test performance analysis has shown that MMSE has no value as a prediction method in determining minimal HE and in respect of overt HE has a sensitivity of 63% and specificity of 52% by a cut-off level at 27.5 points to diagnose overt HE. CONCLUSIONS: In conclusion, although MMSE score and single items are altered in patients with overt HE, MMSE has no value in the assessment of minimal HE. Because MMSE could be impaired in several cognitive dysfunctions, more specific test should be used for measuring HE.
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Transtornos Cognitivos/diagnóstico , Encefalopatia Hepática/diagnóstico , Cirrose Hepática/complicações , Inquéritos e Questionários , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Área Sob a Curva , Eletroencefalografia , Feminino , Encefalopatia Hepática/etiologia , Encefalopatia Hepática/psicologia , Humanos , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Curva ROC , Índice de Gravidade de Doença , Adulto JovemRESUMO
Spontaneous portosystemic shunts can steal the blood away from the portal system. This may result in graft dysfunction or even loss following liver transplantation and can be sorted by shunt occlusion based on intraoperative flow measurements. Herein, we present an alternative technique with cavoportal hemitransposition performed for unrecognized spontaneous mesocaval shunt with 'portal steal' syndrome and primary graft nonfunction diagnosed first day following the transplant. This was chosen as a rescue strategy because an attempt to locate the shunt during relaparotomy was unsuccessful. As there was no improvement, emergency liver retransplantation with preservation of the cavoportal hemitransposition was performed on the fourth day after the primary transplant with good long-term outcome. We conclude that cavoportal hemitransposition during or after liver transplantation can be used to provide an adequate inflow into the donor portal vein if the shunting vessels responsible for the steal cannot be located and dealt with at surgery.
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Transplante de Fígado/efeitos adversos , Derivação Portocava Cirúrgica/métodos , Veia Porta/cirurgia , Veia Cava Inferior/cirurgia , Adulto , Anastomose Cirúrgica , Velocidade do Fluxo Sanguíneo , Feminino , Humanos , Cirrose Hepática/cirurgia , Transplante de Fígado/métodos , ReoperaçãoRESUMO
Splenic artery "steal" syndrome after orthotopic liver transplantation (OLT) is an important cause of graft dysfunction. Direct pressure measurement in the hepatic (HA) and radial artery (RA) may identify patients at risk allowing its prevention. This observational study compared radial and hepatic mean arterial pressures (MAP) measured during 100 OLTs performed in 99 recipients, in whom the HA was considered suitable for the anastomosis. A difference of ≥5 mmHg between the radial and hepatic MAP was arbitrarily chosen as the criterion for inflow modulation. Seven patients fulfilled this criterion showing a MAP gradient that was significantly different compared to the others (-10.8±3.3 vs. 2.6±5.0; p<0.0001). They underwent splenic artery ligation (n=5), arcuate ligament division (n=1) and aortohepatic bypass grafting (n=1) that all resulted in immediate normalization of the arterial inflow pressure to the graft. The splenic artery "steal" syndrome occurred in one patient (day 2 after OLT) in whom the mean HA pressure normalized during OLT following arcuate ligament division, suggesting pathology within the graft as the most likely etiology. Our results indicate that radial MAP can reflect the hepatic MAP during OLT. If a substantial pressure gradient is found, it can be corrected by intraoperative splenic artery ligation or arcuate ligament division.
Assuntos
Pressão Sanguínea , Artéria Hepática/fisiologia , Circulação Hepática , Transplante de Fígado/efeitos adversos , Adolescente , Adulto , Idoso , Feminino , Artéria Hepática/cirurgia , Humanos , Período Intraoperatório , Ligadura , Masculino , Pessoa de Meia-Idade , Disfunção Primária do Enxerto/etiologia , Artéria Radial/fisiologia , Artéria Esplênica/cirurgia , Síndrome , Adulto JovemRESUMO
CONTEXT: Fibrolamellar hepatocellular carcinoma is a rare liver tumor with the propensity to metastasize to the lymph nodes months or years after initial surgery. However, its metastatic spread to the pancreas was previously reported only in a child. CASE REPORT: We present an unusual case of a young female patient who was repeatedly treated by surgical excision of abdominal and mediastinal lymph node recurrences between 2 and 6 years after left hepatic lobectomy for fibrolamellar hepatocellular carcinoma. At 8 years following her initial surgery, the patient was diagnosed with pancreatic head metastasis and a pancreaticoduodenectomy was performed. Postoperative course was uneventful and the patient did not experience recurrence within the last 18 months. CONCLUSION: The metastasis of fibrolamellar hepatocellular carcinoma to the pancreas is highly exceptional but possible and its excision appears warranted as well.
Assuntos
Carcinoma Hepatocelular/patologia , Neoplasias Hepáticas/patologia , Linfonodos/patologia , Neoplasias Pancreáticas/secundário , Adulto , Carcinoma Hepatocelular/cirurgia , Feminino , Hepatectomia , Humanos , Neoplasias Hepáticas/cirurgia , Metástase Linfática , Recidiva Local de Neoplasia , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia , Resultado do TratamentoRESUMO
BACKGROUND/AIMS: Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011. METHODOLOGY: The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound. RESULTS: BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis. CONCLUSIONS: Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.
Assuntos
Anticoagulantes/administração & dosagem , Síndrome de Budd-Chiari/terapia , Transplante de Fígado , Derivação Portossistêmica Transjugular Intra-Hepática , Adolescente , Adulto , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/mortalidade , Substituição de Medicamentos , Feminino , Fidelidade a Diretrizes , Heparina de Baixo Peso Molecular/administração & dosagem , Humanos , Transplante de Fígado/efeitos adversos , Transplante de Fígado/mortalidade , Masculino , Pessoa de Meia-Idade , Polônia , Derivação Portossistêmica Transjugular Intra-Hepática/efeitos adversos , Derivação Portossistêmica Transjugular Intra-Hepática/mortalidade , Guias de Prática Clínica como Assunto , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia Doppler , Vitamina K/antagonistas & inibidores , Adulto JovemRESUMO
BACKGROUND/AIMS: The aim of the study was to analyze in-hospital morbidity and mortality after pancreatoduodenectomy (PD) with a modified duct-to-mucosa pancreaticojejunostomy. METHODOLOGY: We retrospectively analyzed 101 consecutive patients who underwent PD at our center between January 2002 and December 2010. Two-layered duct-to-mucosa pancreaticojejunostomy was performed over an internal transanastomotic stent in all patients. RESULTS: The overall in-hospital morbidity and mortality rate was 48% and 6%, respectively. Three patients died as a consequence of local complications including mesenteric ischemia in two and acute necrotizing pancreatitis in one case. Pancreatic fistula occurred in one (1%) patient and was treated conservatively with good outcome. The wound infection was the most common surgical complication (20/101; 20%) and occurred more often in patients who had a biliary stent inserted endoscopically prior to surgery (15/38; 39%), as compared to those without the stent (5/63; 8%; p=0.0003). CONCLUSIONS: The results of the present study suggest that a two-layered duct-to-mucosa pancreaticojejunostomy with internal transanastomotic stent is a safe anastomosis, associated with a very low risk of pancreatic fistula. The presence of a biliary stent at the time of surgery represents a risk factor for the development of postoperative wound infection.
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Neoplasias dos Ductos Biliares/cirurgia , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia/efeitos adversos , Pancreaticoduodenectomia/métodos , Pancreaticojejunostomia/efeitos adversos , Pancreaticojejunostomia/métodos , Adulto , Idoso , Fístula Anastomótica/etiologia , Feminino , Mortalidade Hospitalar , Humanos , Mucosa Intestinal/cirurgia , Masculino , Pessoa de Meia-Idade , Ductos Pancreáticos/cirurgia , Hemorragia Pós-Operatória/etiologia , Estudos Retrospectivos , Sepse/etiologia , Stents , Infecção da Ferida Cirúrgica/etiologia , Adulto JovemRESUMO
Fatigue is the most commonly encountered symptom in patients with chronic liver disease (CLD). The resulting decrease in quality of life contributes markedly to the societal costs of fatigue. Moreover, fatigue is associated with social dysfunction, increased daytime somnolence, impaired working ability, and increased risk of mortality. Fatigue is not related to the severity of the underlying liver fibrosis or dysfunction. In CLD patients, fatigue manifests with both central symptoms, characterised by cognitive impairment, sleep disturbance, apathy, and autonomic dysfunction, and peripheral symptoms, characterised by decreased exercise tolerance and reduced physical activity levels. The pathogenesis of fatigue in CLD is multifactorial and involves changes in the brain-liver axis resulting from changes in inflammatory cytokines or the gut microbiome. Numerous interventions have attempted to alleviate fatigue in CLD by improving its central and peripheral manifestations or the underlying liver disease. Currently, however, there are no widely accepted or effective treatments for fatigue in CLD patients. In this review, we highlight the problem of fatigue in CLD, the current theories regarding its pathogenesis, and current approaches to its treatment.
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INTRODUCTION: The effectiveness of SARSCoV2 vaccination in liver transplant (LT) recipients varies between reports. OBJECTIVES: In this study, we analyzed the immune response to the SARSCoV2 vaccine, factors affecting the response, and reasons for the vaccine refusal. PATIENTS AND METHODS: Among 300 consecutive LT recipients, 75% were vaccinated. The humoralresponse was assessed by the quantitative determination of antitrimeric spike proteinspecific IgG antibodies to SARSCoV2. Thirtyfour vaccinated patients with prior SARSCoV2 infection were analyzed separately. RESULTS: Among 192 LT recipients vaccinated without past natural infection, 69% developed the immune response (median time of 125 days after the second dose). Older age, worse kidney function, and dual immunosuppression negatively affected the humoral response. Mycophenolate mofetil increased the risk of nonresponse (odds ratio [OR], 2.99; 95% CI, 1.45-6.19). The antibody concentration was higher in the first 90 days from the second dose and stable as compared with 90-150 days and over 150 days. LT recipients with prior COVID19 presented with a robust immune response (100%). The female sex, living in a rural area, lower body mass index, and younger age (all P <0.05) were associated with the refusal of the vaccine. CONCLUSIONS: The lower immune response in the vaccinated LT recipients than in the general population justifies administering the third dose of the vaccine. However, more data are needed to recommend any therapy modification before the vaccination.
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COVID-19 , Transplante de Fígado , Vacinas , Anticorpos Antivirais , COVID-19/prevenção & controle , Vacinas contra COVID-19 , Feminino , Humanos , Imunidade , SARS-CoV-2 , VacinaçãoRESUMO
PURPOSE: Biliary injury is a severe complication of cholecystectomy. The Hepp-Couinaud reconstruction with the hepatic duct confluence and the left duct may offer best long-term outcome as long as the confluence remains intact (Bismuth I-III). Complex liver surgery is usually indicated in most proximal (Bismuth IV) injuries in non-cirrhotic patients. The aim of this study was to evaluate the surgical treatment and outcome of bile duct injuries managed in a referral hepatobiliary unit. METHODS: We retrospectively analyzed surgical management and outcome of biliary injuries following cholecystectomy in 35 patients (27 laparoscopic) referred to our center between June 2001 and December 2009. There was no liver cirrhosis diagnosed in any patient. High injuries (Bismuth III-IV) were found in 14 patients. Management after referral included the Hepp-Couinaud hepaticojejunostomy in 32 patients with Bismuth I-III injuries, which in four cases with biliary peritonitis was preceded by abdominal lavage and prolonged external biliary drainage. Liver transplantation was performed in two patients with Bismuth IV injuries. RESULTS: After median follow-up of 59 months (range, 6-102), 34 (97%) patients are alive and 32 (92%) remain in good general condition with normal liver function. One patient who had combined biliary and colonic injury died of sepsis before repair. Recurrent strictures following the Hepp-Couinaud repair developed in two (6%) patients with high injuries combined with right hepatic arterial injury. CONCLUSION: The Hepp-Couinaud hepaticojejunostomy offers durable results, even after previous interventions have failed. In case of diffuse biliary peritonitis, delayed biliary reconstruction following external biliary drainage may be the best option.
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Colecistectomia Laparoscópica/efeitos adversos , Colecistectomia/efeitos adversos , Ducto Hepático Comum/lesões , Complicações Intraoperatórias/cirurgia , Complicações Pós-Operatórias/cirurgia , Adulto , Idoso , Feminino , Ducto Hepático Comum/cirurgia , Humanos , Jejunostomia/métodos , Masculino , Pessoa de Meia-Idade , Encaminhamento e Consulta , Reoperação , Estudos RetrospectivosRESUMO
BACKGROUND: Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary neoplasm of the bile duct (IPN-B) are two different types of mucin-producing bile duct tumour that may complicate the course of pregnancy. To the best of our knowledge, we describe herein the first case of MCN-L with spontaneous rupture during pregnancy necessitating complex surgical treatment. CASE SUMMARY: A 24-year-old woman was initially admitted to another hospital in October 2018 with signs of jaundice (serum bilirubin level 12 mg/dL) and upper abdominal pain radiating to the left shoulder. Initial magnetic resonance imaging (MRI) of the abdominal cavity revealed a multilocular cystic tumour of the liver hilum (37 mm × 40 mm in diameter) located between segments 3 and 4 of the left liver lobe. Six weeks later (December 2018), the patient was found to be 12 wk pregnant and was referred to our institution for further diagnostics and treatment. At admission, a soft, palpable, and tender mass in the left upper abdomen was found. It was determined via MRI (with no intravenous contrast in view of the first-trimester pregnancy) to be a large collection of fluid (19 cm × 17 cm × 10 cm) located close to the liver hilum and below the left liver lobe. The patient did not undergo any diagnostic or therapeutic procedures nor did they have any abdominal trauma in the preceding weeks. The fluid collection proved to be of biliary origin following percutaneous drainage. Therefore, we concluded this was a spontaneous rupture of an MCN-L with the formation of a biloma. The MRI study also revealed the previously found cystic tumour of the liver hilum communicating with the left hepatic duct, which, together with left hepatic duct dilatation, suggested the diagnosis of IPN-B. The follow-up MRI with intravenous gadolinium contrast performed in the second trimester of pregnancy (week 14) showed, in turn, some features of MCN-L, including enhancement of the internal septations within the cystic liver mass. A precise preoperative differential diagnosis between IPN-B and MCN-L was therefore not possible. The patient was submitted to surgery in the second trimester of pregnancy (week 18). Surgery included a cholecystectomy, left hepatectomy, and concomitant resection of the extrahepatic bile ducts followed by anastomosis of the right hepatic duct with the Roux limb of the jejunum. The post-operative period was uneventful and the patient was discharged 8 days after surgery. The histopathological examination of the resected specimen revealed a final diagnosis of MCN-L with low-grade dysplasia and epithelium surrounded by ovarian-type stromal tissue. The patient delivered a healthy baby girl and both remain well at present, after 2 years of follow-up since surgery. CONCLUSION: The differential diagnosis and management of MCN-L and IPN-B may be very challenging, particularly in the setting of pregnancy. When indications for surgery are obvious, the final diagnosis is based on histopathological examination, with ovarian-type stroma being pathognomonic for MCN-L. We believe that the growth of this subepithelial stroma secondary to the high levels of sex hormones produced during pregnancy might have been the main causative factor leading to the tumour rupture with the formation of a biloma in our patient.
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INTRODUCTION: Impaired elimination of toxic compounds via inadequate sulfation may contribute to the pathogenesis of primary sclerosing cholangitis (PSC). Dehydroepiandrosterone (DHEA), which is metabolized into its sulfated form (DHEA-S) in the liver, has been linked with health-related quality of life (HRQoL) in various conditions. OBJECTIVES: We aimed to assess the sulfation capacity of the liver in PSC using DHEA-S as a surrogate marker. PATIENTS AND METHODS: We assessed serum levels of DHEA-S in 233 patients with PSC and in 201 patients with other liver conditions serving as controls. We also evaluated the effect of low levels of DHEA-S on the course of PSC and HRQoL assessed using the 36-Item Short Form Health Survey (SF-36) and the PBC-40. RESULTS: The proportion of patients with low DHEA-S in the PSC group was 7-fold higher than in the control group (21% vs 3%; P <â 0.001). Patients with decreased levels of DHEA-S were younger at the time of PSC diagnosis (median age, 23 vs 29 years; P = 0.007) and presented with lower HRQoL scores, particularly regarding the physical domains of the SF-36. Patients with low DHEA-S also complained of more severe fatigue (31 vs 23; P = 0.006) assessed with the PBC-40. CONCLUSIONS: Our findings support the role of impaired liver sulfation capacity in the development of PSC. Low levels of DHEA-S are associated with increased fatigue, a devastating symptom significantly affecting HRQoL. Thus, the effects of DHEA administration on chronic fatigue and other measures of HRQoL in patients with PSC warrant further attention.
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Colangite Esclerosante , Qualidade de Vida , Biomarcadores , Sulfato de Desidroepiandrosterona , Humanos , FígadoRESUMO
Primary sclerosing cholangitis (PSC) is commonly accompanied by ulcerative colitis (UC). MicroRNA-506 modulates expression of genes which are essential for sphingosine-mediated signaling pathway and intestinal mucosa protection. We investigated whether miR-506 and its target genes are involved in phenotypic presentations of colonic inflammation and/or neoplasia. We analyzed serum and colon tissue samples collected from patients with PSC, PSC with concurrent UC (PSC + UC), UC alone, and healthy controls (n = 10 each). MiR-506 was substantially upregulated in ascending colons of PSC and PSC + UC patients, in contrast to sigmoid colons of PSC and UC patients. Upregulation of miR-506 was associated with inhibition of SPHK1, AE2, InsP3R3, and p53. Colonic suppression of miR-506 presented in UC was accompanied by substantially increased DNMT1, SPHK1, and S1P lyase expressions. A functional in vitro analysis in Caco-2 cells showed that the induction of miR-506 activity by miR-506 mimic or GDCDA bile acid suppressed, whereas inhibition of miR-506 by miR-506 inhibitor or lipopolysaccharide (LPS) upregulated the expression of the examined target genes. A different phenotypic presentation of colitis may be related to miR-506 expression. In ascending colons with PSC + UC, upregulation of miR-506 may result in failure of bicarbonate secretion and inhibition of p53, which predisposes to pro-tumorigenic transformation. In contrast, downregulation of miR-506 enhances S1P production, leading to pro-inflammatory signaling.
Assuntos
Colangite Esclerosante/genética , Colite Ulcerativa/genética , Suscetibilidade a Doenças , MicroRNAs/genética , Adulto , Biomarcadores , Células CACO-2 , Colangite Esclerosante/complicações , Colangite Esclerosante/diagnóstico , Colite Ulcerativa/complicações , Colite Ulcerativa/diagnóstico , Perfilação da Expressão Gênica , Regulação da Expressão Gênica , Predisposição Genética para Doença , Humanos , Pessoa de Meia-Idade , Modelos Biológicos , Adulto JovemRESUMO
BACKGROUND Renal dysfunction in the peri-transplant period appears to complicate both short- and long-term outcome of liver transplantation (LT). The aim of this study was to analyze the impact of selected clinical features in the peri-liver transplant period, as well calcineurin inhibitor, particularly tacrolimus given after LT, on kidney function in a single liver transplant center's experience. MATERIAL AND METHODS A total 125 consecutive liver-grafted individuals (82 M, 43 F), mean age 50±13 y (with alcohol-related liver disease in 48 (38%) patients) were included into the study. Their clinical data were collected in the database until 46 months of follow-up, and the Python packages Pandas (version 0.22.0) and scikit-learn (version 0.21.3) were used for data analysis. RESULTS More advanced liver disease as judged by Child-Pugh class and MELD score differed significantly patients with preserved (serum creatinine SCr <1.5 mg/dL) and impaired (SCr ≥1.5 mg/dL) kidney function before LT. Older age and higher SCr pre-LT were associated with higher levels of SCr after LT in 2 time-points. SCr before LT was correlated with delta SCr for the highest and last recorded value (P<0.0001). Higher amounts of transfused colloids during surgery were associated with increased delta SCr for the highest value (P=0.019) after grafting in logistic regression analysis. There were no associations between SCr after LT and duration of anhepatic phase, urine output ≤100 mL/h, or post-reperfusion syndrome during transplantation (all P>0.05). There were no associations between SCr after LT and tacrolimus trough levels in analyses of correlations and linear regression analyses (all P>0.05). CONCLUSIONS We found that pretransplant serum creatinine was the only factor affecting kidney function after LT in our liver transplant center. The restricted fluid policy was safe and effective in terms of long-term renal function. The role of kidney-saving immunosuppressive protocols in preserving renal function long-term after LT was also confirmed.
Assuntos
Rim/fisiologia , Transplante de Fígado , Adulto , Inibidores de Calcineurina/uso terapêutico , Creatinina/sangue , Feminino , Humanos , Testes de Função Renal , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Tacrolimo/uso terapêuticoRESUMO
BACKGROUND/AIMS: Arterial complications continue to be a major source of morbidity, graft loss and mortality after liver transplantation (OLT). In this study we analyzed the incidence, treatment and outcome of arterial complications in patients who underwent OLT in our center. METHODOLOGY: Between February 2002 and May 2009, 210 whole-organ OLTs were performed in 199 adults. Analyzed patients were divided into group I (the first 100 OLTs) and group II (subsequent 110 OLTs). Factors that could contribute to the development of arterial complications were analyzed. RESULTS: Fourteen (6.5%) arterial complications occurred in 13 patients resulting in graft loss in 4 (31%) and mortality in 5 (38%) cases. There were two (1%) serious intraoperative bleedings requiring major arterial reconstruction. The most frequent arterial complication was hepatic artery thrombosis (3.3%; 7/210), requiring re-OLT in 5 cases and resulting in death in 4 patients. Hepatic artery kinking was found in 3 (1.4%) patients while the splenic artery steal syndrome and hepatic artery stenosis coexistent with portal vein stenosis occurred in one patient each. The incidence of arterial complications (9% vs. 4.6%; p=NS), related graft loss (3% vs. 0.9%; p=NS) and mortality (4% vs. 0.9%; p=NS) were comparable in both groups. CONCLUSIONS: Arterial complications remain a major source of graft loss and mortality after OLT. Their occurrence and related graft loss and mortality were not associated with a significant learning curve in our series. Hepatic artery thrombosis although rare, is a devastating complication requiring re-OLT in majority of cases. Early diagnosis and prompt therapy are crucial to improve outcome.
Assuntos
Artéria Hepática/cirurgia , Transplante de Fígado/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/terapia , Adolescente , Adulto , Idoso , Anastomose Cirúrgica/métodos , Distribuição de Qui-Quadrado , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
In view of growing disproportion between the number of organ donors and recipients awaiting transplantation the issue of an appropriate allocation of organs is becoming more and more important. The Child-Turcotte-Pugh Scale (CTP), which has been used for many years, has proved limited in many aspects. Hence, MELD Score (Model for End-Stage Liver Disease) was first introduced in the US in 2002 and later on in numerous other countries. This has led to a decrease in mortality among patients waiting for a liver transplant. Research into constructing new prognostic scales or improving the old ones are being continued. Various modifications of both MELD and CTP score have been evaluated. In this article we review recently tested scores and discuss their potential applications.