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1.
Rev Esp Enferm Dig ; 2024 Jul 05.
Artigo em Inglês | MEDLINE | ID: mdl-38967282

RESUMO

Abdominal tuberculosis (TB), a potential differential diagnosis for acute and chronic abdomen, should be considered in specific situations, such as immunosuppressant or biological drug use, HIV, or in patients residing in endemic areas. Although the presence of thoracic tuberculosis may indicate abdominal TB, only 15% of patients with abdominal TB have evidence of pulmonary disease. Involvement of the liver and spleen is a common autopsy finding in patients with disseminated TB. However, the most common pattern is of fine miliary lesions. Hepatic involvement with a macronodular pattern, a rare occurrence, is linked to dissemination through the portal vein. The splenic macronodular form, an extremely rare presentation, can manifest as solitary or multiple nodules, oval or round, with a variable appearance reflecting different stages of the disease, adding to the uniqueness of this case.

2.
J Thromb Thrombolysis ; 53(4): 887-897, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-34800258

RESUMO

The relationship between acidosis and coagulopathy has long been described in vitro and in trauma patients, but not yet in orthotopic liver transplantation (OLT). The association of metabolic acidosis with coagulopathy and with transfusion requirements was evaluated in patients submitted to OLT. Changes in acid-base and coagulation parameters were analyzed by repeated measures. Regression analyses [adjusted for sex, age, model for end stage liver disease (MELD) score, and baseline values of hemoglobin, fibrinogen, international normalized ratio, platelets] determined the association of acid-base parameters with coagulation markers and transfusion requirement. We included 95 patients, 66% were male, 49.5% of the patients had hepatocellular carcinoma and the mean MELD score was 20.4 (SD 8.9). The values of all the coagulation and acid-base parameters significantly changed during OLT, particularly in the reperfusion phase. After adjustments for baseline parameters, the decrease in pH and base excess (BE) values were associated with a decrease in fibrinogen levels (mean decrease of fibrinogen level = 14.88 mg/dL per 0.1 unit reduction of pH values and 3.6 mg/dL per 1 mmol/L reduction of BE levels) and an increase in red blood cells transfusion (2.16 units of RBC per 0.1 unit reduction of pH and 0.38 units of RBC per 1 mmol/L reduction of BE levels). Among multiple factors potentially associated with adverse outcomes, decreasing pH levels were independently associated with the length of hospitalization but not with in-hospital mortality. Metabolic acidosis is independently associated with decreased fibrinogen levels and increased intraoperative transfusion requirement during OLT. Awareness of that association may improve treatment strategies to reduce intraoperative bleeding risk in OLT.


Assuntos
Acidose , Transtornos da Coagulação Sanguínea , Doença Hepática Terminal , Transplante de Fígado , Acidose/complicações , Transtornos da Coagulação Sanguínea/etiologia , Transtornos da Coagulação Sanguínea/terapia , Doença Hepática Terminal/complicações , Feminino , Fibrinogênio , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Índice de Gravidade de Doença
3.
Transpl Int ; 34(1): 97-109, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33040420

RESUMO

This study aimed to compare liver transplantation (LT) outcomes and evaluate the potential rise in numbers of LT candidates with hepatocellular carcinoma (HCC) of different allocation policies in a high waitlist mortality region. Three policies were applied in two Latin American cohorts (1085 HCC transplanted patients and 917 listed patients for HCC): (i) Milan criteria with expansion according to UCSF downstaging (UCSF-DS), (ii) the AFP score, and (iii) restrictive policy or Double Eligibility Criteria (DEC; within Milan + AFP score ≤2). Increase in HCC patient numbers was evaluated in an Argentinian prospective validation set (INCUCAI; NCT03775863). Expansion criteria in policy A showed that UCSF-DS [28.4% (CI 12.8-56.2)] or "all-comers" [32.9% (CI 11.9-71.3)] had higher 5-year recurrence rates compared to Milan, with 10.9% increase in HCC patients for LT. The policy B showed lower recurrence rates for AFP scores ≤2 points, even expanding beyond Milan criteria, with a 3.3% increase. Patients within DEC had lower 5-year recurrence rates compared with those beyond DEC [13.3% (CI 10.1-17.3) vs 24.2% (CI 17.4-33.1; P = 0.0006], without significant HCC expansion. In conclusion, although the application of a stricter policy may optimize the selection process, this restrictive policy may lead to ethical concerns in organ allocation (NCT03775863).


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Transplante de Fígado , Carcinoma Hepatocelular/cirurgia , Estudos de Coortes , Humanos , Neoplasias Hepáticas/cirurgia , Recidiva Local de Neoplasia , Seleção de Pacientes , Estudos Prospectivos , Estudos Retrospectivos
4.
Transpl Infect Dis ; 23(4): e13583, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33583111

RESUMO

Cryptococcus neoformans is rarely associated with peritonitis in cirrhotic patients; nevertheless, it has a high mortality rate. Early diagnosis and prompt treatment may be the determining prognostic factors. This is a report of two patients awaiting a liver transplant who had opposite outcomes after the diagnosis of spontaneous cryptococcal peritonitis. In Patient 1, the fungal culture was positive in the blood and ascites. She had a poor evolution and died, which was likely caused by the delayed diagnosis and concomitant bacterial infections. In Patient 2, the fungus was found in the ascites, urine, and cerebrospinal fluid cultures. Antifungal treatment was effective. He underwent a liver transplant on the 83rd day of antifungal therapy and is still alive 1 year later. It is important to suspect fungal etiology when there is a lack of response to antibiotics in patients with decompensated cirrhosis and spontaneous peritonitis, and physicians must be aware of leukocyte count in the ascitic fluid, which is not so high in these cases. This report also emphasizes the need for the routine use of blood culture bottles for microbiological analysis of the ascitic fluid, as it was helpful to diagnose fungal peritonitis in both cases.


Assuntos
Infecções Bacterianas , Transplante de Fígado , Peritonite , Ascite , Líquido Ascítico , Feminino , Humanos , Cirrose Hepática/complicações , Transplante de Fígado/efeitos adversos , Masculino , Peritonite/diagnóstico , Peritonite/tratamento farmacológico , Peritonite/etiologia
5.
Transpl Infect Dis ; 21(5): e13151, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31344763

RESUMO

Yellow fever (YF) is a viral disease, with clinical presentation among immunosuppressed patients not fully understood. YF vaccination (YFV), a live vaccine, is contraindicated in patients receiving immunosuppressive treatment due to the risk of developing the disease after vaccination. We report a case of a 50-year-old male recipient who presented wild-type YF five years after a deceased donor kidney transplant. He lived in a YF endemic area and inadvertently received YFV. One day after YFV, the patient presented nausea, vomiting, fever, diarrhea, polyarthralgia, thrombocytopenia, and increased levels of liver function enzymes. The serological test was compatible with YF disease, and quantitative viral load confirmed the diagnosis of wild-type YF. The patient received supportive care for twelve days, with hospital discharge in good clinical condition and stable renal function. One month after discharge, the patient developed de novo donor-specific anti-HLA antibodies (DSA) and histological evidence of endothelial lesion, with a diagnosis of acute antibody-mediated rejection (AMR), treated with plasmapheresis and human IVIg therapy. Six months after therapy, he presented normal renal function with a reduction of DSA MFI. In the reported case, we observed a clinical wild-type YF diagnosed even after YF vaccine administration, with good clinical outcome. De novo DSA and AMR occurred after the recovering of disease, with an adequate response to therapy and preserved allograft function. We reviewed the published literature on YF and YFV in solid organ transplantation.


Assuntos
Transplante de Rim/efeitos adversos , Febre Amarela/diagnóstico , Febre Amarela/etiologia , Rejeição de Enxerto/etiologia , Antígenos HLA/imunologia , Humanos , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-Idade , Transplantados , Transplante Homólogo
6.
Liver Int ; 36(11): 1657-1667, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27169841

RESUMO

BACKGROUND & AIMS: The French alpha-fetoprotein (AFP) model has recently shown superior results compared to Milan criteria (MC) for prediction of hepatocellular carcinoma (HCC) recurrence after liver transplantation (LT) in European populations. The aim of this study was to explore the predictive capacity of the AFP model for HCC recurrence in a Latin-American cohort. METHODS: Three hundred twenty-seven patients with HCC were included from a total of 2018 patients transplanted at 15 centres. Serum AFP and imaging data were both recorded at listing. Predictability was assessed by the Net Reclassification Improvement (NRI) method. RESULTS: Overall, 82 and 79% of the patients were within MC and the AFP model respectively. NRI showed a superior predictability of the AFP model against MC. Patients with an AFP score >2 points had higher risk of recurrence at 5 years Hazard Ratio (HR) of 3.15 (P = 0.0001) and lower patient survival (HR = 1.51; P = 0.03). Among patients exceeding MC, a score ≤2 points identified a subgroup of patients with lower recurrence (5% vs 42%; P = 0.013) and higher survival rates (84% vs 45%; P = 0.038). In cases treated with bridging procedures, following restaging, a score >2 points identified a higher recurrence (HR 2.2, P = 0.12) and lower survival rate (HR 2.25, P = 0.03). A comparative analysis between HBV and non-HBV patients showed that the AFP model performed better in non-HBV patients. CONCLUSIONS: The AFP model could be useful in Latin-American countries to better select patients for LT in subgroups presenting with extended criteria. However, particular attention should be focused on patients with HBV.


Assuntos
Carcinoma Hepatocelular/sangue , Neoplasias Hepáticas/sangue , Transplante de Fígado , Recidiva Local de Neoplasia/diagnóstico , alfa-Fetoproteínas/análise , Idoso , Carcinoma Hepatocelular/cirurgia , Feminino , Humanos , América Latina , Neoplasias Hepáticas/cirurgia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Recidiva Local de Neoplasia/mortalidade , Estudos Retrospectivos , Análise de Sobrevida
7.
Gastroenterology Res ; 17(1): 23-31, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38463146

RESUMO

Background: Tyrosine kinase inhibitors have been used to treat hepatocellular carcinoma (HCC), but the outcomes of patients under treatment vary. Since the roles of clinicopathological aspects and markers of chronic inflammation/immune homeostasis in the outcome of HCC patients treated with sorafenib are still unclear, these were the aims of this study. Methods: Patients with alcohol-induced and/or hepatitis C virus (HCV)-induced HCC (n = 182) uniformly treated with sorafenib were included in the study. Baseline clinicopathological aspects of patients were computed from the medical records. The neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic inflammation response index (SIRI), and systemic immune-inflammation index (SII) were obtained from the hematological exam performed before the administration of sorafenib. Overall survival (OS) was analyzed using Kaplan-Meier probabilities, log-rank test, and univariate and multivariate Cox proportional hazard ratio (HR) analyses. Results: In multivariate analysis, alpha-foetoprotein (AFP) level and Child-Pugh score were predictors of OS. Patients with AFP levels higher than 157 ng/mL and Child-Pugh B or C had 1.40 (95% confidence interval (CI): 1.03 - 1.91, P = 0.03) and 1.64 (95% CI: 1.07 - 2.52, P = 0.02) more chances of evolving to death than the remaining patients, respectively. NLR, PLR, LMR, SIRI, and SII did not alter the OS of HCC patients. Conclusions: AFP level and Child-Pugh score act as independent prognostic factors in patients with alcohol and/or HCV-induced HCC treated with sorafenib, but markers of chronic inflammation/immune homeostasis seem not to alter the outcome of patients with HCC induced by alcohol and/or HCV.

8.
Autops Case Rep ; 14: e2024485, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38628284

RESUMO

Giant hepatic hemangiomas are occasional in patients with cirrhosis. It remains a challenge to decide on the need for treatment and choose the most appropriate intervention. A 62-year-old woman was recently diagnosed with cirrhosis and complained of upper abdominal fullness, reduction in oral food intake, and weight loss of 6 kg over the last three years. Upper digestive endoscopy evidenced thin-caliber esophageal varices and significant extrinsic compression of the lesser gastric curvature. Abdominal computed tomography revealed an exophytic tumor in the left hepatic lobe, measuring 11.5 cm, which had progressive centripetal contrast enhancement from the arterial phase, compatible with hepatic hemangioma. Serum tumor markers were negative, and her liver function was unimpaired. The patient underwent surgical resection (non-anatomical hepatectomy of segments II and III) which had no immediate complications, and the histopathological evaluation confirmed cavernous hepatic hemangioma. Two weeks later, she was admitted to the emergency room with jaundice, signs of hepatic encephalopathy, and moderate ascites, and was further diagnosed with secondary bacterial peritonitis. As no perforations, abscesses, or fistulas were observed on subsequent imaging tests, clinical management was successfully carried out. This case highlights that giant hepatic hemangiomas may be symptomatic and warrant treatment. In the setting of cirrhosis and portal hypertension, physicians should be aware of the risk of hepatic decompensation following surgical resection, even in patients with Child-Pugh class A.

9.
Transplant Proc ; 56(5): 1077-1079, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38908955

RESUMO

In some specific cases, the surgical technique involved in liver transplantation can be challenging, as, for example, in cases of Budd-Chiari syndrome. This case describes a case of liver transplantation in a patient diagnosed with Budd-Chiari syndrome due to a previous surgery that required retrohepatic vena cava resection. During the liver transplantation procedure, a cavo-atrial anastomosis was performed through transdiaphragmatic access, with a good postoperative result, showing that, although challenging, this technique is a feasible option in specific cases.


Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Veia Cava Inferior , Humanos , Síndrome de Budd-Chiari/cirurgia , Síndrome de Budd-Chiari/etiologia , Veia Cava Inferior/cirurgia , Masculino , Anastomose Cirúrgica , Pessoa de Meia-Idade
10.
Transplant Proc ; 56(5): 1096-1097, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38969610

RESUMO

INTRODUCTION: The use of natural products for therapeutic purposes is a common practice throughout the world, in part, due to the global obesity epidemic and the search for products with appetite suppression and weight loss properties, which include nutritional supplements, vitamins and minerals to herbal products. It is known that such products may be associated with various adverse health effects. Thus, the objective of this study is to report a series of cases of patients, who presented fulminant liver failure (HFI) requiring liver transplantation (LT), related to the consumption of products used for weight loss. MATERIAL AND METHODS: This is a retrospective cohort based on the evaluation of patients listed for LT due to IHF at the Hospital das Clínicas of the Universidade Estadual de Campinas, between 1991 and 2022, with patients who had confirmed consumption of products with the aim of loss being selected. RESULTS: During the studied period, 92 patients were listed for HT due to IHF according to the Kings College criteria, with 5 cases being selected with proven consumption of herbal products for weight loss, and other causes that could explain the IHF were excluded. Four (80%) of the patients were female, with a mean age of 40.5 years, and 40% of the cases died. DISCUSSION AND CONCLUSIONS: Unlike traditional pharmaceutical medicines, in most countries, the commercialization of these products is not conditioned on clinical and safety evidence or prior approval by regulatory bodies. Hepatoxicity can be related to several factors, such as the presence of toxins naturally found in plants, the presence of heavy metals, contamination during obtaining or processing and the addition of substances omitted from the labels. The use of weight loss products can evolve with IHF, a fact that deserves attention, due to ease of access and growing demand, and it is important to regulate the trade of these products and raise public awareness about the risks of use without professional supervision and guidance.


Assuntos
Fármacos Antiobesidade , Falência Hepática Aguda , Transplante de Fígado , Humanos , Feminino , Estudos Retrospectivos , Adulto , Masculino , Falência Hepática Aguda/cirurgia , Fármacos Antiobesidade/efeitos adversos , Fármacos Antiobesidade/uso terapêutico , Pessoa de Meia-Idade , Redução de Peso
11.
Transplant Proc ; 55(6): 1454-1456, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37183065

RESUMO

Drug-induced liver injury has become the main cause of acute liver failure (ALF). Injury may be dose-dependent or occur idiosyncratically. We report the case of a 37-year-old female patient admitted with grade 4 hepatic encephalopathy. Morosil use was initiated 30 days prior, and after 2 weeks, the case evolved with jaundice and diarrhea interspersed with constipation when the medication was discontinued. Morosil is a dry extract of the juice of red Moro oranges (Citrus species), grown in eastern Sicily (Italy). It is an important source of anthocyanidin pigments, which act as antioxidants and are used in the care and prevention of obesity, insulin resistance, hepatic steatosis, and cardiovascular diseases. According to the clinical picture and tests performed, the diagnosis of ALF was confirmed, by the criteria of Clichy and King's College. The patient was listed for liver transplantation as a priority, with the model for end stage liver disease (MELD) score: 41. Orthotopic liver transplantation was uneventfully performed using the Piggy-back technique. There are few reports in the literature of acute liver injury by other Citrus species, and we found no case reports of ALF related to the use of Morosil.


Assuntos
Doença Hepática Terminal , Falência Hepática Aguda , Feminino , Humanos , Adulto , Prognóstico , Antocianinas/efeitos adversos , Doença Hepática Terminal/complicações , Índice de Gravidade de Doença , Falência Hepática Aguda/induzido quimicamente , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/cirurgia
12.
Transplant Proc ; 55(8): 1815-1821, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37330340

RESUMO

BACKGROUND: Infections by SARS-CoV-2 in liver transplant recipients (LT) patients are of particular concern, notably due to perceived added risks related to immunosuppression and comorbidity burden. Current literature on this topic often relies on small, non-standardized, and geographically limited studies. This manuscript describes COVID-19 presentations and causes for elevated mortality in a large cohort of LT recipients. METHODS: This study was designed as a multicentric historical cohort, including LT recipient patients with COVID-19 in 25 study centers, with the primary endpoint being COVID-related death. We also collected demographic, clinical, and laboratory data regarding presentation and disease progression. RESULTS: Two hundred and thirty-four cases were included. The study population was predominantly male and White and had a median age of 60 years. The median time from transplantation was 2.6 years (IQR 1-6). Most patients had at least one comorbidity (189, 80.8%). Patient age (P = .04), dyspnea (P < .001), intensive care unit admission (P < .001), and mechanical ventilation (P < .001) were associated with increased mortality. Modifications of immunosuppressive therapy (P < .001), specifically the suspension of tacrolimus, maintained significance in multivariable analysis. CONCLUSIONS: Attention to risk factors and the individualization of patient care, especially regarding immunosuppression management, is crucial for delivering more precise interventions to these individuals.


Assuntos
COVID-19 , Transplante de Fígado , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , COVID-19/epidemiologia , SARS-CoV-2 , Transplante de Fígado/efeitos adversos , Brasil/epidemiologia , Terapia de Imunossupressão/efeitos adversos , Transplantados
13.
Transplant Proc ; 54(5): 1341-1344, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35787311

RESUMO

BACKGROUND: Current literature reports diverge on the impact of COVID-19 in liver transplant (LT) recipients. Literature findings often report conflicting results, relying on small sample sizes, limited ethnic variability, and nonstandardized methodologies. Notably, there are no studies on this topic regarding Latin American populations. This study seeks to report the impact of COVID-19, disease characteristics, and progression in LT recipients in a Latin American academic center environment. METHODS: The study design was a historic cohort, including adult LT recipient patients with suspected or confirmed COVID-19 who sought care between December 2019 to October 2021. The primary end point was defined as COVID-19-related death. Demographic, clinical, and laboratory data was also collected. RESULTS: Twenty-seven patients were included, representing a 3.5% incidence within 752 patients in the follow-up. The mean age and years from transplantation were 54 (SD ± 11) and 6.3 years (SD ± 5.4), respectively. Most patients were white (23 - 85.2%) and male (21 - 25.2%). The hospitalization rate was 55.6%, and 5 patients (18.5%), all of whom subsequently died, were admitted to the intensive care unit. Neither the presence of comorbidities nor advanced age were related to lethality. Patients with immunosuppression modifications (P = 0.039) or isolated tacrolimus suspension (P = 0.006) were associated with increased mortality. CONCLUSIONS: This study described COVID-19 infections in LT recipients in Latin American populations. This group was not affected by common factors associated with higher lethality, and displayed a tendency toward lower hospitalization rates. Our study concurred with previously reported evidence of a protective association of tacrolimus maintenance during treatment in LT recipients affected by COVID-19.


Assuntos
COVID-19 , Transplante de Fígado , Adulto , Brasil/epidemiologia , COVID-19/epidemiologia , Humanos , Incidência , Transplante de Fígado/efeitos adversos , Masculino , Tacrolimo , Transplantados
14.
Transplant Proc ; 54(5): 1300-1303, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35810016

RESUMO

Liver transplant is the main treatment for hepatocellular carcinoma and there is currently an important demand from patients waiting in transplant queues. Thus, it is extremely important to improve the criteria for selecting patients who will undergo transplant to mitigate graft loss and reduce cases of recurrence. Thus, it becomes necessary to use models, such as the New York/California (NYCA), that include alpha fetoprotein as a marker of recurrence and prognosis. The aim of this study was to assess whether the NYCA score correlated with the presence of tumor recurrence after transplant in patients undergoing orthotopic liver transplant at the Clinics Hospital of the University of Campinas. We had 214 patients undergoing liver transplant who met the inclusion Milan criteria. The age of the patients ranged from 34 to 77 years, with a median age of 61 years. The mean waiting time on the transplant list was 6.12 months. After calculating the NYCA score, it was possible to stratify 13 patients (6.1%) as high risk, 64 patients (29.9%) as medium risk, and 137 patients (64%) as low risk. Patients with recurrence had higher scores with a mean of 4 points in relapse and 2 points in the absence of relapse (P = .0011). Patients with recurrence had statistically higher high- and medium-risk scores (P = .0010). Therefore, the NYCA score was higher in patients with recurrence. Therefore, in this study, our findings suggest the possibility of using the NYCA score as an aid to detect patients with a higher risk of tumor recurrence.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Transplante de Fígado , Adulto , Idoso , Carcinoma Hepatocelular/patologia , Hospitais , Humanos , Neoplasias Hepáticas/patologia , Transplante de Fígado/efeitos adversos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , New York , Período Pré-Operatório , Estudos Retrospectivos , Fatores de Risco
15.
Medicine (Baltimore) ; 101(35): e30315, 2022 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-36107601

RESUMO

RATIONALE: Lysosomal acid lipase deficiency (LAL-D) is a poorly diagnosed genetic disorder characterized by the accumulation of cholesteryl esters and triglycerides in many tissues, leading to dyslipidemia and cardiovascular complications. In the liver, deposits are found within hepatocytes and Kupffer cells, generating microvesicular steatosis, progressive fibrosis, and cirrhosis. Sebelipase alfa is the target therapy which can improve laboratory changes and reduce the progression of liver damage, but this is not yet widely available. PATIENT CONCERNS: We are reporting a 15-year follow-up of a Brazilian man who was diagnosed with cirrhosis at age 43 and with LAL-D at age 53, but he has never been treated with sebelipase alfa for economic reasons. During the coronavirus disease 2019 (COVID-19) pandemic, he lost follow-up and missed three 6-month ultrasound exams for liver cancer screening. DIAGNOSIS: At age 58, a remarkable deterioration in liver function was observed and he was diagnosed with hepatocellular carcinoma (HCC) outside the Milan Criteria (two nodules measuring 48mm and 25mm). Three other individuals with LAL-D and progression to liver cancer have been reported so far and none of them underwent enzyme replacement therapy: an 11-year-old girl with HCC, a 51-year-old male with cholangiocarcinoma, and a 21-year-old male with hepatocellular-cholangiocarcinoma. The latter had the same mutation in the gene LIPA as our patient, but a relationship between this variant and malignancies has not yet been established. LESSONS: We emphasize how important is to treat LAL-D patients after diagnosis in order to avoid worsening liver function and progression to neoplasms. Untreated individuals should be considered at a higher risk but the most appropriate liver cancer screening program for this subgroup is still unknown.


Assuntos
COVID-19 , Carcinoma Hepatocelular , Colangiocarcinoma , Neoplasias Hepáticas , Adulto , Carcinoma Hepatocelular/etiologia , Criança , Ésteres do Colesterol , Feminino , Humanos , Cirrose Hepática , Neoplasias Hepáticas/etiologia , Masculino , Pessoa de Meia-Idade , Triglicerídeos , Doença de Wolman , Adulto Jovem , Doença de Wolman
16.
Transplant Proc ; 54(5): 1310-1312, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35537877

RESUMO

Hepatocellular carcinoma (HCC) is the most common primary neoplasm of the liver, mainly secondary to cirrhosis caused by hepatitis C virus. Liver transplant (LT) is considered the best treatment because, in addition to removing the tumor, it also removes the underlying cirrhotic liver. The Milan criteria for LT have limitations because they do not consider the biological characteristics of the tumor. Thus, our objective was to evaluate the association of α-fetoprotein (AFP) levels before LT performed for HCC with recurrence of this tumor, and, based on the results, a new predictive model that combines the AFP values at the list entry with the usual criteria of tumor size and number of nodules was validated. In present study, the Score AFP model, we were able to correlate a greater occurrence of relapse with scores of 3 and 4 (P = .0001), indicating the usefulness of using AFP as a predictor of recurrence.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Transplante de Fígado , alfa-Fetoproteínas , Carcinoma Hepatocelular/patologia , Carcinoma Hepatocelular/cirurgia , Humanos , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Recidiva Local de Neoplasia/etiologia , Estudos Retrospectivos , alfa-Fetoproteínas/análise
17.
Transplant Proc ; 54(5): 1308-1309, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35534281

RESUMO

Hepatocellular carcinoma (HCC) is the sixth leading cause of cancer in the world, and liver transplant (LT) is a good therapeutic option in selected cases because it treats the neoplasm and the underlying disease. Recurrence after LT is usually aggressive and has low survival; thus, an adequate selection of recipients is ideal. The new models aim to assess the individual risk of HCC recurrence in patients undergoing LT and to improve post-LT survival. In this study, our aim was to assess the applicability of the "Metroticket" score, correlating it with our rates of recurrence and survival after LT. Overall survival at 5 years in our study differed from that in Metroticket 2.0 because that study did not consider only recurrence as the cause of death; our study evaluated only patients with recurrence, so we were able to validate the score as a predictor of greater tumor aggressiveness after LT.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Transplante de Fígado , Carcinoma Hepatocelular/patologia , Humanos , Neoplasias Hepáticas/patologia , Transplante de Fígado/efeitos adversos , Recidiva Local de Neoplasia/etiologia , Período Pré-Operatório , Estudos Retrospectivos , Fatores de Risco
18.
World J Hepatol ; 14(7): 1459-1469, 2022 Jul 27.
Artigo em Inglês | MEDLINE | ID: mdl-36158916

RESUMO

BACKGROUND: Challenging lesions, difficult to diagnose through non-invasive methods, constitute an important emotional burden for each patient regarding a still uncertain diagnosis (malignant x benign). In addition, from a therapeutic and prognostic point of view, delay in a definitive diagnosis can lead to worse outcomes. One of the main innovative trends currently is the use of molecular and functional methods to diagnosis. Numerous liver-specific contrast agents have been developed and studied in recent years to improve the performance of liver magnetic resonance imaging (MRI). More recently, one of the contrast agents introduced in clinical practice is gadoxetic acid (gadoxetate disodium). AIM: To demonstrate the value of the hepatobiliary phases using gadoxetic acid in MRI for the characterization of focal liver lesions (FLL) in clinical practice. METHODS: Overall, 302 Lesions were studied in 136 patients who underwent MRI exams using gadoxetic acid for the assessment of FLL. Two radiologists independently reviewed the MRI exams using four stages, and categorized them on a 6-point scale, from 0 (lesion not detected) to 5 (definitely malignant). The stages were: stage 1- images without contrast, stage 2- addition of dynamic phases after contrast (analogous to usual extracellular contrasts), stage 3- addition of hepatobiliary phase after 10 min (HBP 10'), stage 4- hepatobiliary phase after 20 min (HBP 20') in addition to stage 2. RESULTS: The interobserver agreement was high (weighted Kappa coefficient: 0.81- 1) at all stages in the characterization of benign and malignant FLL. The diagnostic weighted accuracy (Az) was 0.80 in stage 1 and was increased to 0.90 in stage 2. Addition of the hepatobiliary phase increased Az to 0.98 in stage 3, which was also 0.98 in stage 4. CONCLUSION: The hepatobiliary sequences improve diagnostic accuracy. With growing potential in the era of precision medicine, the improvement and dissemination of the method among medical specialties can bring benefits in the management of patients with FLL that are difficult to diagnose.

19.
Rev Col Bras Cir ; 48: e20212997, 2021.
Artigo em Inglês, Português | MEDLINE | ID: mdl-34932735

RESUMO

OBJECTIVE: to correlate clinical and epidemiological data with the pathological analysis of liver explants from patients undergoing liver transplantation for hetapocarcinoma in the UNICAMP HC and to verify whether the MELD and MELD-Na scores are reliable factors to predict a worse post-transplant prognosis. METHODS: we studied liver transplants carried out between May 2010 and November 2017. After excluding 38 patients, we included 87, analyzing clinical and laboratory data for correlation with the outcome Microvascular Invasion (MVI). Subsequently, we computed the MELD and MELD-Na scores and performed a descriptive analysis of clinical and laboratory data and, finally, calculated ROC curves to assess the association between these laboratory parameters and mortality in these patients. RESULTS: most patients were male (78.30%), with an average age of 58.53 years. Most liver diseases were caused by HCV (53.26%). We found no predictors for MVI among the laboratory parameters. The ROC curves for death identified the MELD score as the cutoff point with the highest combined sensitivity (90.91%) and specificity (37.50%), with a value of 10 points, whereas in the MELD-Na the cutoff point was 7 points, with a sensitivity of 90.91% and a specificity of 33.33%, both scores being significant. CONCLUSIONS: there were no reliable predictors of MVI between clinical, laboratory, and epidemiological variables. The MELD-Na score is more sensitive than the MELD one for predicting mortality in patients undergoing liver transplantation.


Assuntos
Doença Hepática Terminal , Hepatopatias , Transplante de Fígado , Doença Hepática Terminal/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Índice de Gravidade de Doença
20.
Rev Assoc Med Bras (1992) ; 66(7): 908-912, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32844950

RESUMO

Hepatocellular carcinoma in patients with hepatitis C in the absence of cirrhosis is uncommon. We demonstrate the importance of morphofunctional magnetic resonance imaging (MRI) with a hepatospecific contrast agent by describing an asymptomatic female patient with HCV, who presented with a nodule detected on ultrasound. She underwent inconclusive computed tomography, presenting no signs of chronic liver disease. MRI with hepatospecific contrast providing functional information combined with the superior tissue contrast inherent to this method stands out for its greater accuracy with the possibility of not resorting to invasive diagnostic methods. With increasing experience and the dissemination of this new diagnostic modality in the medical field, its use and other potential benefits of morphofunctional MRI with hepatospecific contrast agents may be established, benefiting patients with challenging focal liver lesions.


Assuntos
Carcinoma Hepatocelular , Hepatite C , Neoplasias Hepáticas , Carcinoma Hepatocelular/diagnóstico por imagem , Carcinoma Hepatocelular/virologia , Meios de Contraste , Feminino , Hepatite C/complicações , Humanos , Cirrose Hepática , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/virologia , Imageamento por Ressonância Magnética
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