RESUMO

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare autosomal recessive disorder caused by mutations in the ABCB11 gene. Clinical manifestations include cholestasis with low γ-glutamyltransferase (GGT), hepatosplenomegaly, and severe pruritus. Liver transplantation is required for individuals with progressive liver disease or failure of the bypass procedure and has been considered curative. However, in the case of PFIC2, although bile salt excretory pump (BSEP) deficiency is a liver-specific condition rather than a systemic disease, evidence of recurrent BSEP disease has been shown in a small proportion of allografts. We describe an unusual case of a 21-year-old individual with PFIC2 and evidence of recurrent BSEP disease after liver transplantation, with clinical and laboratory improvement after pulse therapy with methylprednisolone for 3 days and adjustment of oral immunosuppression. This case report highlights the recurrence of PFIC2 in patients post liver transplant. It also emphasizes the importance of clinical suspicion, which should be considered in cases of posttransplant cholestasis in PFIC2 patients, especially those with low γ-glutamyltransferase (GGT) and without signs of acute graft rejection. Having knowledge of the condition favors a targeted diagnostic approach and contributes to early therapeutic management and a higher success rate.

Assuntos

RESUMO

BACKGROUND: Liver transplantation is a unique treatment opportunity for patients with chronic liver disease and hepatocellular carcinoma (HCC). Selection of HCC patients for transplantation was revolutionized by Milan-based criteria, but tumor recurrence and shortage of organs are still a major concern. Nowadays, additional preoperative tumor parameters can help to refine the graft allocation process. The objective of this study was to evaluate the prognostic value and cut-off points of pretransplant serum alpha-fetoprotein (AFP) levels and radiological tumor parameters on liver transplantation outcomes. METHODS: This is a single-team retrospective cohort of 162 consecutive deceased donor liver transplants (DDLT) with pathologically confirmed HCC. Pretransplant serum AFP levels and radiological tumor parameters were retrieved from a preoperative follow-up. Receiver-operating characteristics (ROC) curves were used to evaluate cut-off points for each outcome. Multivariate Cox regression model was used to assess the predictors of HCC relapse and recipient mortality. RESULTS: Twelve recipients (7.4%) had HCC recurrence after transplantation, with median survival time of 5.8 months. Pretransplant AFP ≥30 ng/mL (hazard ratio [HR]: 13.84, P = .003) and radiological total tumor diameter (TTD) ≥5 cm (HR: 12.89, P = .005) were independent predictors for HCC relapse. Moreover, pretransplant AFP ≥150 ng/mL was independently associated with recipient mortality (HR: 4.45, P = .003). CONCLUSIONS: Pretransplant AFP levels and radiological TTD were independently associated with HCC relapse and recipient mortality after DDLT, with different cut-off points predicting different outcomes. These findings may contribute to improving decision-making in the context of liver transplantation for HCC patients.

Assuntos

RESUMO

Liver transplantation is the only potentially curative treatment for patients with end-stage liver disease. After the procedure, histopathologic analysis of the liver explant may reveal neoplasms that were not previously diagnosed in preoperative imaging examinations. This incidental finding of primary liver neoplasms in the explant is not an uncommon situation in liver transplant, and hepatocarcinomas and cholangiocarcinomas are the types of tumors most frequently encountered in this scenario. These are the most common primary neoplasms of the liver, and liver transplantation is often a curative treatment for these types of tumors when they are in their earlier stages. In contrast, liver plasmacytoma is a rare type of plasma cell neoplasm, consisting of a single mass of monoclonal plasma cells, which is treated primarily by radiotherapy and is seldom encountered in the setting of liver transplant. We report the case of a patient who underwent liver transplantation for the treatment of cryptogenic cirrhosis, with no preoperative diagnosis of liver tumors. Analysis of the liver explant revealed the presence of three synchronous neoplasms with different histologic origins: a 27-mm hepatocellular carcinoma, a 17-mm intrahepatic cholangiocarcinoma, and a 25-mm solitary hepatic plasmacytoma. The patient received no further adjuvant treatment and remained well and with no signs of disease recurrence over an observation period of 44 months. We found no previous report in the literature of the synchronous presence of these three types of liver neoplasms.

Assuntos

RESUMO

BACKGROUND: Portal vein thrombosis is a relatively frequent complication in patients with liver cirrhosis. Its detection and management are essential to avoid worsening portal hypertension or liver function complications. This complication can also negatively impact or even preclude liver transplant. CASE PRESENTATION: We report the case of a patient who presented with acute portal vein thrombosis, which allowed the diagnosis of liver cirrhosis and hepatocarcinoma within the Milan criteria. Chemical thrombolysis was performed with a mechanical aspiration of the thrombus, and in a second moment, the patient was submitted to a liver transplant. CONCLUSIONS: Advances in the therapeutic approach to portal vein thrombosis and surgical techniques have allowed the condition to no longer be an absolute contraindication to liver transplantation. Diagnosis in the acute phase is associated with greater therapeutic success, aiming to avoid the extension of thrombosis and achieve portal vein recanalization.

Assuntos

RESUMO

BACKGROUND: Tacrolimus and mycophenolate mofetil are immunosuppressive agents widely used on the postoperative period of the transplants. AIM: To evaluate the influence of the association of them on the abdominal wall healing in rats. METHODS: Thirty-six Wistar rats were randomly assigned in three groups of 12. On the early postoperative period, four of the control group and three of the experimental groups died. The three groups were nominated as follow: control group (GC, n=8); group I (GI, n=11, standard operation, mycophenolate mofetil and tacrolimus); group II (GII, n=10, standard operation, mycophenolate mofetil and tacrolimus). The standard operation consisted of right total nephrectomy and 20 min ischemia of the left kidney followed by reperfusion. Both NaCl 0.9% and the immunosuppressive agents were administered starting on the first postoperative day and continuing daily until the day of death on the 14th day. On the day of their deaths, two strips of the anterior abdominal wall were collected and submitted to breaking strength measurement and histological examination. RESULTS: There were no significant differences in wound infection rates (p=0,175), in the breaking strength measurement and in the histological examination among the three groups. CONCLUSION: The combination of the immunosuppressive agents used in the study associated with renal ischemia and reperfusion does not interfere in the abdominal wall healing of rats.

Assuntos

RESUMO

INTRODUCTION: Liver transplantation is the standard treatment for end-stage liver disease. Brazil holds the third highest number of liver transplants performed per year, but center maldistribution results in high discrepancies in accessing this treatment. In 2012, an interstate partnership successfully implemented a new liver transplantation program in the middle west of Brazil. Here, we report the results of the first 500 liver transplants performed in this new program and discuss the impacts of a new transplant center in regional transplantation dynamics. METHODS: We reviewed data from the first 500 consecutive deceased donor liver transplants performed in the new program during an 8-year period. We analyzed data on patients' clinical and demographic profiles, postoperative outcomes, and graft and recipient survival rates. Univariate survival analysis was conducted using log-rank tests to compare the groups. RESULTS: Almost half (48%) of the procured organs and 40% of the recipients transplanted in our center were from outside our state. Recipient 30-day mortality was 9%. Overall recipient survival at 1 year and 5 years was 85% and 80%, respectively. Mortality was significantly associated with higher Model for End-Stage Liver Disease (P < .001) but not with the presence of hepatocellular carcinoma (P = .795). DISCUSSION: The new transplantation program treated patients from different regions of Brazil and became the reference center in liver transplantation for the middle west region. Despite the recent implementation, our outcomes are comparable to experienced centers around the world. This model can inspire the creation of new transplantation programs aiming to democratize access to liver transplantation nationwide.

Assuntos

RESUMO

BACKGROUND: Cryptococcosis is a fungal infection caused by the yeast-like encapsulated basidiomycetous fungus of the Cryptococcus neoformans (C. neoformans) species complex. These fungi are ubiquitous in soil and bird droppings, and infection by them is an important global health concern, particularly in immunosuppressed patients, such as organ transplant recipients and those infected by the human immunodeficiency virus. The fungus usually enters the body through the respiratory tract, but extremely rare cases of infection acquired by transplantation of solid organs have been reported. CASE SUMMARY: We report a case of disseminated cryptococcosis in a liver transplant recipient, diagnosed 2 wk after the procedure. The patient initially presented with fever, hyponatremia and elevated transaminase levels, manifesting intense headache after a few days. Blood cultures were positive for C. neoformans. Liver biopsy showed numerous fungal elements surrounded by gelatinous matrix and sparse granulomatous formations. Magnetic resonance imaging of the brain showed multiple small lesions with low signal in T2, peripheric enhancement and edematous halo, diffuse through the parenchyma but more concentrated in the subcortical regions. Treatment with amphotericin B for 3 wk, followed by maintenance therapy with fluconazole, led to complete resolution of the symptoms. The recipients of both kidneys from the same donor also developed disseminated cryptococcosis, confirming the transplant as the source of infection. The organ donor lived in a rural area, surrounded by tropical rainforest, and had negative blood cultures prior to organ procurement. CONCLUSION: This case highlights the risk of transmission of fungal diseases, specifically of C. neoformans, through liver graft during liver transplantation.

RESUMO

ABSTRACT Background: Tacrolimus and mycophenolate mofetil are immunosuppressive agents widely used on the postoperative period of the transplants. Aim: To evaluate the influence of the association of them on the abdominal wall healing in rats. Methods: Thirty-six Wistar rats were randomly assigned in three groups of 12. On the early postoperative period, four of the control group and three of the experimental groups died. The three groups were nominated as follow: control group (GC, n=8); group I (GI, n=11, standard operation, mycophenolate mofetil and tacrolimus); group II (GII, n=10, standard operation, mycophenolate mofetil and tacrolimus). The standard operation consisted of right total nephrectomy and 20 min ischemia of the left kidney followed by reperfusion. Both NaCl 0.9% and the immunosuppressive agents were administered starting on the first postoperative day and continuing daily until the day of death on the 14th day. On the day of their deaths, two strips of the anterior abdominal wall were collected and submitted to breaking strength measurement and histological examination. Results: There were no significant differences in wound infection rates (p=0,175), in the breaking strength measurement and in the histological examination among the three groups. Conclusion: The combination of the immunosuppressive agents used in the study associated with renal ischemia and reperfusion does not interfere in the abdominal wall healing of rats.

RESUMO Racional: O tacrolimus e o micofenolato mofetil são imunossupressores amplamente utilizados no pós-operatório dos transplantes de órgãos. Objetivo: Avaliar os efeitos deles sobre a cicatrização da parede abdominal em ratos. Métodos: Foram utilizados 36 ratos Wistar, distribuídos aleatoriamente em três grupos de 12. No pós-operatório imediato, quatro do grupo controle e três do grupo experimentação morreram. Os três grupos receberam as seguintes denominações: grupo controle (GC, n=8); grupo I (GI, n=11, operação-padrão, micofenolato mofetil e tacrolimus); grupo II (GII, n=10, operação-padrão, micofenolato mofetil e tacrolimus). A operação-padrão consistiu de nefrectomia total à direita, isquemia durante 20 min seguida de reperfusão do rim esquerdo. Solução de NaCl 0,9% e micofenolato mofetil + tracolimus foram administradas a partir do 1° dia do pós-operatório e mantidas até o dia do sacrifício dos animais, no 14° dia. Na data do sacrifício, foram retirados dois fragmentos da parede abdominal para análise da resistência à ruptura e exame histológico. Resultados: Não houve diferença estatisticamente significativa no índice de infecção de ferida operatória (p=0,175), nos valores de resistência de ruptura e nos achados histopatológicos entre os três grupos de animais. Conclusão: Os esquemas de imunossupressão empregados associados ao fenômeno da isquemia-reperfusão renal não induzem fraqueza significativa da cicatriz da parede abdominal em ratos no 14° dia de pós-operatório.

Assuntos

RESUMO

BACKGROUND: Infection by the hepatitis C virus (HCV) is currently considered to be a global health issue, with a high worldwide prevalence and causing chronic disease in afflicted individuals. The disease largely involves the liver but it can affect other organs, including the skin. While leukocytoclastic vasculitis has been reported as one of the dermatologic manifestations of HCV infection, there are no reports of this condition as the first symptom of HCV recurrence after liver transplantation. CASE SUMMARY: We report here a case of leukocytoclastic vasculitis in a liver transplant recipient on maintenance immunosuppression. The condition presented as a palpable purpura in both lower extremities. Blood and urine cultures were negative and all biochemical tests were normal, excepting evidence of anemia and hypocomplementemia. Imaging examination by computed tomography showed a small volume of ascites, diffuse thickening of bowel walls, and a small bilateral pleural effusion. Skin biopsy showed leukocytoclasia and fibrinoid necrosis. Liver biopsy was suggestive of HCV recurrence in the graft, and HCV polymerase chain reaction yielded 11460 copies/mL and identified the genotype as 1A. Treatment of the virus with a 12-wk direct-acting antiviral regimen of ribavirin, sofosbuvir and daclatasvir led to regression of the symptoms within the first 10 d and subsequent complete resolution of the symptoms. CONCLUSION: This case highlights the difficulties of diagnosing skin lesions caused by HCV infection in immunosuppressed patients.

RESUMO

We report the case of a female patient, 58 years of age, without known heart disease, who underwent liver transplantation without complications. On the second postoperative day, the patient developed cardiogenic shock secondary to stress-induced cardiomyopathy (Takotsubo-like syndrome). The patient was successfully managed with veno-arterial peripheral extracorporeal membrane oxygenation for 6 days, with complete recovery of cardiac function and of the hepatic graft. Coronary syndrome and acute myocarditis were excluded as the causes of the shock. The use of extracorporeal membrane oxygenation in this scenario is possible and safe, considering its specialized protocols and treatment.

Reportamos o caso de paciente do sexo feminino, 58 anos, sem doença cardíaca conhecida, submetida a transplante hepático sem intercorrências. No segundo dia do pós-operatório desenvolveu choque cardiogênico secundário à miocardiopatia induzida pelo estresse (síndrome de Takotsubo-like). A paciente foi manejada com sucesso com oxigenação por membrana extracorpórea venoarterial periférica, por 6 dias, com recuperação completa da função cardíaca, bem como do enxerto hepático. Síndrome coronariana e miocardite aguda foram excluídas como causas do choque. O uso de oxigenação por membrana extracorpórea neste cenário é possível e seguro, considerando protocolos e tratamento especializado neste tipo de suporte.

Assuntos

RESUMO

RESUMO Reportamos o caso de paciente do sexo feminino, 58 anos, sem doença cardíaca conhecida, submetida a transplante hepático sem intercorrências. No segundo dia do pós-operatório desenvolveu choque cardiogênico secundário à miocardiopatia induzida pelo estresse (síndrome de Takotsubo-like). A paciente foi manejada com sucesso com oxigenação por membrana extracorpórea venoarterial periférica, por 6 dias, com recuperação completa da função cardíaca, bem como do enxerto hepático. Síndrome coronariana e miocardite aguda foram excluídas como causas do choque. O uso de oxigenação por membrana extracorpórea neste cenário é possível e seguro, considerando protocolos e tratamento especializado neste tipo de suporte.

ABSTRACT We report the case of a female patient, 58 years of age, without known heart disease, who underwent liver transplantation without complications. On the second postoperative day, the patient developed cardiogenic shock secondary to stress-induced cardiomyopathy (Takotsubo-like syndrome). The patient was successfully managed with veno-arterial peripheral extracorporeal membrane oxygenation for 6 days, with complete recovery of cardiac function and of the hepatic graft. Coronary syndrome and acute myocarditis were excluded as the causes of the shock. The use of extracorporeal membrane oxygenation in this scenario is possible and safe, considering its specialized protocols and treatment.

Assuntos

RESUMO

PURPOSE: The aim of this study was to investigate the effects of untreated and treated hypertension on abdominal wall healing. METHODS: Thirty-two spontaneously hypertensive rats (SHR) were randomly allocated into two groups: H (n = 16), untreated and E (n = 16), treated with enalapril (40 mg/kg per day). Group C (n = 16) was a nonhypertensive control group. The animals of each group were submitted to a midline laparotomy and randomly divided, according to the day on which they were killed (7th or 14th postoperative day), into subgroups of 8 animals, as follows: H-7, H-14, E-7, E-14, C-7 and C-14. On the day of their deaths, two strips of the anterior abdominal wall were collected. One strip was submitted to breaking strength measurement and the other to hydroxyproline determination. RESULTS: No mortalities or complications were observed in the six subgroups. The breaking strength in E-7 subgroup was significantly lower than in C-7 (P < 0.05). The tissue hydroxyproline levels were similar in all six subgroups (P > 0.05). CONCLUSIONS: Untreated hypertension had no effect on the abdominal wall healing of rats. Hypertensive animals treated with enalapril showed a significant decrease in abdominal wound-breaking strength on the 7th postoperative day.

Assuntos

RESUMO

PURPOSE: To analyse the effect of tenoxicam, a nonsteroidal anti-inflammatory, on the abdominal wall healing in rats. METHODS: It was used 40 rats, submitted to longitudinal laparotomies, and allocated, randomly, in one control group (C), consisted of 20 rats treated with saline solution; and one test group (T), consisted of 20 rats treated with tenoxicam. The animals of each group were divided, according to their sacrifice day, into subgroups of 10 animals, named as C7, C14, T7 and T14. The numbers 7 and 14 indicated that the animal would be sacrificed on the 7th and 14th postoperative day, respectively. The tenoxicam (1 mg/ml) and saline solution (NaCl 0.9%) were administrated by intramuscular injections, at the dose of 0.6 ml/kg/day, immediately after surgery and continued for 4 days. In the sacrifice day, two segments of the abdominal wall (1 cm x 3 cm) were prepared and submitted to breaking strength measurement and hydroxyproline determination. RESULTS: No complications were observed in the four subgroups, including infection or dehiscence. There were no significant differences in the breaking strength measurement (p=0.262) and the hydroxyproline levels (p=0.392) among the four subgroups. CONCLUSION: The tenoxicam, administered intramuscularly, does not interfere on the abdominal wall healing of rats.

Assuntos

RESUMO

OBJETIVO: Analisar os efeitos do tenoxicam, um antiinflamatório não hormonal, na cicatrização da parede abdominal de ratos. MÉTODOS: Foram utilizados 40 ratos adultos, submetidos à laparotomia mediana e distribuídos, aleatoriamente, em um grupo controle (C), constituído de 20 animais que receberam solução de NaCl a 0,9 por cento; e um grupo tratado (T), constituído de 20 animais que receberam o tenoxicam. Os animais de cada grupo foram divididos, conforme a data de sacrifício, em subgrupos de 10 animais, denominados C7, C14, T7 e T14. As inscrições 7 e 14 determinaram o sacrifício dos animais no sétimo e décimo quarto dia pós-operatório, respectivamente. As soluções de tenoxicam (1mg/ml) e de NaCl a 0,9 por cento foram administradas no pós-operatório imediato e nos quatro dias seguintes, por via intramuscular, na dose volume de 0,6 ml/kg/dia. No dia do sacrifício, realizou-se a ressecção de dois fragmentos da parede abdominal (1cm x 3cm), que foram utilizados para determinação da concentração de hidroxiprolina e avaliação da força de ruptura. RESULTADOS: Não foram observadas complicações da ferida operatória, incluindo infecção ou deiscência, nos quatro subgrupos de animais. Na análise comparativa dos quatro subgrupos de animais, não foi evidenciada diferença estatisticamente significante no estudo da força de ruptura (p=0,262) e na concentração de hidroxiprolina (p=0,392). CONCLUSÃO: A administração de tenoxicam, por via intramuscular, não interfere na cicatrização da parede abdominal de ratos.

Assuntos

RESUMO

OBJETIVO: Analisar os efeitos do tenoxicam, um antiinflamatório não hormonal, na cicatrização da parede abdominal de ratos. MÉTODOS: Foram utilizados 40 ratos adultos, submetidos à laparotomia mediana e distribuídos, aleatoriamente, em um grupo controle (C), constituído de 20 animais que receberam solução de NaCl a 0,9 por cento; e um grupo tratado (T), constituído de 20 animais que receberam o tenoxicam. Os animais de cada grupo foram divididos, conforme a data de sacrifício, em subgrupos de 10 animais, denominados C7, C14, T7 e T14. As inscrições 7 e 14 determinaram o sacrifício dos animais no sétimo e décimo quarto dia pós-operatório, respectivamente. As soluções de tenoxicam (1mg/ml) e de NaCl a 0,9 por cento foram administradas no pós-operatório imediato e nos quatro dias seguintes, por via intramuscular, na dose volume de 0,6 ml/kg/dia. No dia do sacrifício, realizou-se a ressecção de dois fragmentos da parede abdominal (1cm x 3cm), que foram utilizados para determinação da concentração de hidroxiprolina e avaliação da força de ruptura. RESULTADOS: Não foram observadas complicações da ferida operatória, incluindo infecção ou deiscência, nos quatro subgrupos de animais. Na análise comparativa dos quatro subgrupos de animais, não foi evidenciada diferença estatisticamente significante no estudo da força de ruptura (p=0,262) e na concentração de hidroxiprolina (p=0,392). CONCLUSAO: A administração de tenoxicam, por via intramuscular, não interfere na cicatrização da parede abdominal de ratos. (AU)

Assuntos