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OBJECTIVES: Carbamoyl phosphate synthetase 1 (CPS-1) deficiency is a rare urea cycle disorder with an estimated prevalence of one in 150,000-200,000 live births. Patients often present with hyperammonemia shortly after protein feeding in the early days of life, and early-onset type is associated with high mortality rate. CASE PRESENTATION: We present here a case of a newborn male with a history of two deceased siblings whose ammonium level exceeded 200⯵mol/L on the first day after birth, and who was started on dextrose infusion and ammonia-scavenging therapy after oral feeding was discontinued. Peritoneal dialysis was initiated after the patient's ammonia level exceeded 500⯵mol/L. At the age of five months, the patient underwent hemodialysis due to elevated ammonia levels accompanied by lethargy. The patient's ammonia levels were successfully brought under control, and the patient underwent a liver transplantation at the age of six month, donated by the father. CONCLUSIONS: We present this case to emphasize the efficacy of liver transplantation from a parent carrying a CPS-1 deficiency. The authors believe that, with further support from future studies, the use of carglumic acid can improve the prognosis in the chronic management of CPS-1 deficiency.
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Background and Aim: This study aimed to identify the indications for liver transplantation (LT) based on underlying etiology and to characterize the patients who underwent LT. Materials and Methods: We conducted a multicenter cross-sectional observational study across 11 tertiary centers in Turkiye from 2010 to 2020. The study included 5,080 adult patients. Results: The mean age of patients was 50.3±15.2 years, with a predominance of female patients (70%). Chronic viral hepatitis (46%) was the leading etiological factor, with Hepatitis B virus infection at 35%, followed by cryptogenic cirrhosis (24%), Hepatitis C virus infection (8%), and alcohol-related liver disease (ALD) (6%). Post-2015, there was a significant increase in both the number of liver transplants and the proportion of living donor liver transplants (p<0.001). A comparative analysis of patient characteristics before and after 2015 showed a significant decline in viral hepatitis-related LT (p<0.001), whereas fatty liver disease-related LT significantly increased (p<0.001). Conclusion: Chronic viral hepatitis continues to be the primary indication for LT in Turkiye. However, the proportions of non-alcoholic fatty liver disease (NAFLD) and ALD-related LT have seen an upward trend over the years.
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BACKGROUND: The expanded polytetrafluoroethylene (ePTFE) grafts are used to drain anterior sector veins during the living donor liver transplantation procedure. We aimed to analyze the potentially life-threatening complications, such as the infection and migration of ePTFE grafts. METHODS: A total of 1264 liver transplantations (LTs) were performed for 1097 adult and 167 pediatric liver failure cases. In total, 1169 living and 95 cadaveric liver transplantation procedures were performed between 2011 and 2021. Right liver transplantation was performed in 1016 cases, including 1002 living donors and 14 cadaveric split right livers. Cadaveric LT was performed in 81 cases. RESULTS: For 1002 right living liver grafts, 905 vascular grafts were used during the backtable for anterior sector outflow venoplasty. The most commonly drained segments were 5 and 8 (472 cases); there were isolated (5 or 8) and multiple drained segments. Vascular graft migration was described in 7 of 905 (0.77%) patients. CONCLUSIONS: Although complication rates regarding ePTFE grafts are low, there are serious life-threatening causes of morbidity and mortality. We recommend cushioning the vascular graft with the omentum, which is effective in preventing graft migration.
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Transplante de Fígado , Adulto , Humanos , Criança , Transplante de Fígado/efeitos adversos , Transplante de Fígado/métodos , Politetrafluoretileno/efeitos adversos , Veias Hepáticas/cirurgia , Doadores Vivos , Fígado/irrigação sanguínea , Cadáver , Estudos RetrospectivosRESUMO
BACKGROUND: Liver transplantation is a life-saving treatment for end-stage pediatric liver failure. We aimed to present the results of pediatric liver transplants performed in our center in the last 11 years (between 2012 and March 2022) in association with prognostic factors affecting survival. METHODS: Demographic characteristics, etiologic reasons, previous operations (Kasai procedure), morbidity, mortality, survival, and bilio-vascular complication rates were determined, and outcomes were evaluated. In the postoperative period, the duration of mechanical ventilation and intensive care unit stay and surgical and other complications were evaluated. Graft and patient survival rates were determined, and univariate and multivariate factors affecting these rates were evaluated. RESULTS: In the last 10 years, 229 pediatric liver transplantaion (Pe-LT)/1513 adult liver taransplantation (Ad-LT) (21.35%) were performed in our center. This ratio (Pe-LT/Ad-LT ratio) is 1741/15,886 (10.95%) for our country. A total of 229 liver transplants were performed in 214 pediatric patients. Retransplantation was performed in 15 patients (6.55%). Cadaveric liver transplantation was performed in 9 patients. Graft survival rates were 87%, 83%, 78%, 78%, 78%, and 78% at <30 days, 30 to 90 days, 91 to 364 days, 1 to 3 years, and >3 years, respectively. Patient survival rates for <30 days, 30 to 90 days, 91 to 364 days, 1 to 3 years, and >3 years were 91.5%, 85.7%, 82%, 81.5%, and 81.5%, respectively. Our 5-year survival rates in metabolic diseases and the acute fulminant failure group are 93.8% and 100%, respectively. CONCLUSIONS: The fact that the 1- and 5-year survival rates are the same shows that when patients overcome biliary vascular and infectious problems, their survival is prolonged.
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Falência Hepática , Transplante de Fígado , Adulto , Humanos , Criança , Transplante de Fígado/métodos , Falência Hepática/cirurgia , Portoenterostomia Hepática , Reoperação/efeitos adversos , Taxa de Sobrevida , Sobrevivência de Enxerto , Resultado do Tratamento , Estudos Retrospectivos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgiaRESUMO
BACKGROUND: Patients with liver graft failures have an extremely low chance of finding a cadaveric graft in countries with a scarcity of deceased donors. We compared the outcomes of liver re-transplantation with living-donor liver grafts (re-LDLT) and deceased-donor liver grafts (re-DDLT) in adult patients (>18 years). METHODS: The medical records of 1513 (1417 [93.6%] LDLT and 96 [6.3%] DDLT) patients who underwent liver transplantation at Memorial Hospital between January 2011 and October 2022 were reviewed. Forty patients (24 adults and 16 pediatric) were re-transplanted (2.84%); 24 adult patients (2.72%: 25 re-LDLT, 1 patient with second re-LDLT) were divided into 2 groups: re-DDLT (n = 6) and re-LDLT (n = 18). The groups were compared in demographics, pre-, peri-, postoperative characteristics, and outcomes. RESULTS: The overall survival rates were 91.7%, 79.2%, 75.0%, and 75% for <30 days, 31 to 90 days, 1, and 3 years, respectively. The LDLT group was significantly younger (P = .022), had smaller graft weight (P = .03), shorter mechanical ventilation (P = .036) but longer operation time (P = .019), and hospitalization period (P = .003). The groups were otherwise comparable. There was no statistically significant difference in survival rates between the groups (P = .058), although the re-LDLT group had an evidently higher survival rate (88.9% and 83.3 % vs 50.0%). CONCLUSION: Re-LDLT has shown comparable outcomes to re-DDLT, if not better (even not far from significance P = .058). These results may encourage performing re-LDLTs in patients with indications for re-LT without worrying about low chances of survival, especially in countries with limited sources of deceased donors.
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Transplante de Fígado , Doadores Vivos , Adulto , Humanos , Criança , Transplante de Fígado/métodos , Resultado do Tratamento , Estudos Retrospectivos , Sobrevivência de Enxerto , FígadoRESUMO
BACKGROUND: Exercise interventions improve muscle performance and functionality when applied more than 6 months after liver trans- plantation, but no studies have reported on earlier exercise interventions. Hence, we assessed the effects of early resistance training on functional outcomes in adult liver recipients. METHODS: The study included 30 liver transplantation patients (53.2 ± 12.4 years) randomly assigned to a training group (n = 15) or a control group (n = 15). Data collected preoperatively and 4 and 8 weeks post-surgery were analyzed, including peripheral and respiratory muscle strength, exercise capacity, physical performance, and fatigue. An 8-week physiotherapy program was applied (training group: standard physiotherapy + resistance training; control group: standard physiotherapy) for 2 sessions/day, 5 days/week. RESULTS: Baseline data showed a homogeneous distribution in the between-group comparisons. In the within-group analysis; EG showed higher improvements in physical performance (TG: P = .001, CG: P = .05) and fatigue perception (TG: P = .001; CG: P = .006), than the CG. The TG showed eight-week improvements in exercise capacity, peripheral muscle strength, and maximal inspiratory pressure (P = .001), and maximal expiratory pressure (P = .047), while CG remained unchanged (P > .05). In the between-group analysis; the improvements indicated significant differences in deltoid strength and fatigue perception, in favor of the TG (P < .05). A change of 0.9 kg in peripheral muscle strength and >37.8 m in 6-min walk distance (6MWD) was determined, representing clinically significant improvement in liver recipients. CONCLUSION: Early resistance training may improve muscle strength, exercise capacity, physical performance, and fatigue perception in liver recipients, when added to standard physiotherapy. The estimated minimal clinically important differences are meaningful to clini- cians in setting liver transplanted patient-specific goals.
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Transplante de Fígado , Treinamento Resistido , Adulto , Fadiga , Humanos , Projetos Piloto , Qualidade de Vida , Músculos Respiratórios/fisiologiaRESUMO
BACKGROUND: Liver transplantation is the ultimate treatment for end-stage liver failure. As organ donation systems improve, more reproductive-age women are expected to undergo liver transplantation. Current studies indicate increased risk of some perinatal and maternal complications; however, the available data is still scarce. Therefore, we aimed to evaluate the maternal and fetal outcomes of pregnancies in liver transplant recipients. METHODS: We retrospectively evaluated liver transplantations performed between 2011 and 2020 in a tertiary center. Perinatal, maternal, fetal outcomes and transplant status were assessed among pregnancies conceived after liver transplantation. RESULTS: Among 1137 patients, 82 (7.2%) were reproductive-age females. Ten pregnancies in nine patients were identified after liver transplantation. The mean age of patients was 29.3±6.1 at transplantation, and 32.5±5.4 at conception. The mean interval between conception and transplantation was 30.3±11.7 months. There were eight live births (80%), one miscarriage (10%) and one termination (10%). Three patients delivered<37th gestational week (37.5%). The median gestational age at birth was 38.5 (IQR: 5.21) weeks. The mean birth weight of infants was 2669.3±831 g. Two patients were diagnosed with preeclampsia (25%) and acute graft rejection episode was observed in one patient (10%) during pregnancy. CONCLUSION: Although the incidence of some perinatal complications, such as hypertensive disorders and preterm delivery, is increased in liver transplant recipients, pregnancy after liver transplantation appears to have favorable outcomes for the mother, fetus and transplant with close monitoring by a multidisciplinary team.
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Doença Hepática Terminal , Transplante de Fígado , Complicações na Gravidez , Gravidez , Recém-Nascido , Feminino , Humanos , Lactente , Resultado da Gravidez , Complicações na Gravidez/epidemiologia , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Turquia/epidemiologia , TransplantadosRESUMO
In this study, possible risk factors of gastrointestinal perforations (GIP) that increase mortality after liver transplantation in children were investigated. One hundred and thirty-one pediatric patients who underwent 139 liver transplants between January 2016 and February 2020 were evaluated retrospectively based on preoperative and surgical data. Furthermore, cases with biliary atresia, which constitute 26.7% (35) of the patients, were compared within themselves and with other groups. It was found that the cases that developed perforations were younger, lower in weight, and had higher number of surgeries than those who did not, while the mortality and morbidity rates were higher in these patients. When cases with biliary atresia were analyzed within themselves, no significant difference was found between perforated biliary atresia and non-perforated cases in terms of age, weight, and previous surgery. When biliary atresia and other etiologies were compared, biliary atresia cases were found to be transplanted at a younger age, at a lower weight, and this group had a higher risk for perforation. Early laparotomy should be performed in order to reduce mortality in GIPs. Patients that are younger, underweight, previously operated, and using mesh must be closely monitored.
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Perfuração Intestinal/epidemiologia , Transplante de Fígado , Complicações Pós-Operatórias/epidemiologia , Ruptura Gástrica/epidemiologia , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco , Ruptura EspontâneaRESUMO
BACKGROUND: Liver transplantation (LT) is the best treatment in selected patients with hepatocellular carcinoma (HCC). Morphologic criteria alone are not sufficient to predict survival. In this study, we investigated the clinical, biochemical, and pathologic factors affecting survival in patients who underwent LT due to HCC. METHODS: Between October 2011 and January 2018, 165 of 749 LT for HCC cases performed at the Memorial Atasehir Hospital were evaluated retrospectively. Survival, demographic characteristics and etiology, preoperative alpha-fetoprotein (AFP) level, Model for End-Stage Liver Disease (MELD) score, prognostic staging, and morphologic and histologic properties were evaluated. RESULTS: One hundred and thirty-nine cases of 165 were living donor liver transplantation (LDLT). The mean age was 57.7 ± 7.3 years, the mean follow-up period was 27.8 ± 20 months, and 41 patients (24%) died before follow-up. Recurrence of HCC was detected in 23 (14%) cases. Overall survival was 85%, 71%, and 64% for 1, 3, and 5 years, respectively. In terms of 1-, 3-, and 5-year survival within vs beyond Milan criteria was 90%, 80%, and 76% vs 75%, 66%, and 44%, respectively. In the University of California San Francisco criteria, it was 86%, 76%, and 70% vs 76%, 60%, and 30% compared with 1-, 3-, and 5-year survival. While histopathological poor differentiation and AFP elevation affected the course negatively. Good differentiation did not have a significant effect on survival. It was determined that poor differentiation, lymphovascular invasion, and an increased number of nodules significantly affected survival in both within and beyond cases. CONCLUSION: A transplant decision is controversial in patients with HCC with other than previously defined morphologic criteria. In these cases, AFP level and histologic differentiation determine survival. The results were not satisfactory in both high and/or poorly differentiated cases.
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Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado/mortalidade , Transplante de Fígado/métodos , Adulto , Idoso , Carcinoma Hepatocelular/mortalidade , Feminino , Humanos , Neoplasias Hepáticas/mortalidade , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , São FranciscoRESUMO
Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic syndrome. The etiology of PRES is still unclear. Some factors were described. We present a case of a pediatric patient with liver transplant who developed PRES following blood transfusion while receiving tacrolimus therapy. A 5½-year-old boy who underwent living donor liver transplantation, and PRES developed on the sixth day post transplant under tacrolimus treatment after 6 hours of red blood transfusion. PRES is a rare condition; it should be kept in mind about patients who have received organ transplants and develop sudden neurologic symptoms.
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Transplante de Fígado/efeitos adversos , Síndrome da Leucoencefalopatia Posterior/etiologia , Transfusão de Sangue , Pré-Escolar , Humanos , Imunossupressores/uso terapêutico , Doadores Vivos , Masculino , Tacrolimo/uso terapêuticoRESUMO
INTRODUCTION: Several markers of systemic inflammation, including blood C-reactive protein, platelet lymphocyte ratio (PLR) and neutrophil lymphocyte ratio (NLR) have been identified as independent prognosticators for hepatocellular carcinoma (HCC). METHODS: To attempt to understand the significance of these markers, they were examined in relation to 4 tumour parameters, namely maximum tumour diameter (MTD), tumour multifocality, portal vein thrombosis (PVT) and blood alpha-fetoprotein (AFP) levels. RESULTS: Using linear and logistic regression models, we found that C-reactive protein and PLR on single variables, were statistically significantly related to the tumour parameters. In a logistic regression final model, CRP was significantly related to MTD, AFP and PVT, and the Glasgow Index significantly related to MTD and AFP. Results of the area under the receiver operating characteristic curves (ROC), showed that the areas for PLR and CRP were statistically significant for high versus low MTD and for presence versus absence of PVT. CRP alone was significant for high versus low AFP. CONCLUSIONS: These analyses suggest that the prognostic usefulness of the inflammatory markers PLR and CRP (but not NLR) may be due to their reflection of parameter values for tumour growth and invasiveness.
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The hepatocellular carcinoma (HCC) tumor marker alpha-fetoprotein (AFP) is only elevated in about half of the HCC patients, limiting its usefulness in following the effects of therapy or screening. New markers are needed. It has been previously noted that the inflammation markers C-reactive protein (CRP) and platelet-lymphocyte ratio (PLR) are prognostically important and may reflect HCC aggressiveness. We therefore examined these 2 markers in a low-AFP HCC cohort and found that for HCCs > 2 cm, both markers significantly rise with an increasing maximum tumor diameter (MTD). We calculated the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and Youden index value for each marker, and their area-under-the-curve values for each MTD group. Patients were dichotomized into 2 groups based on the CRP and PLR from the receiver-operating characteristic curve analysis. In the logistic regression models of the 4 different MTD patient groups, CRP and PLR levels were statistically significant to estimate MTD in univariate logistic regression models of MTD groups > 2 cm. CRP and PLR were then combined, and the combination was statistically significant to estimate MTD groups of 3-, 4-, and 5-cm cutoffs. CRP and PLR thus have potential as tumor markers for low-AFP HCC patients, and possibly for screening.
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Biomarcadores Tumorais , Proteína C-Reativa , Carcinoma Hepatocelular/sangue , Neoplasias Hepáticas/sangue , Contagem de Linfócitos , Contagem de Plaquetas , alfa-Fetoproteínas , Área Sob a Curva , Proteína C-Reativa/metabolismo , Carcinoma Hepatocelular/diagnóstico , Humanos , Neoplasias Hepáticas/diagnóstico , Prognóstico , Curva ROC , Análise de Regressão , Carga Tumoral , alfa-Fetoproteínas/metabolismoRESUMO
Macroscopic portal vein invasion (PVT) by hepatocellular carcinoma (HCC) in the liver is one of the most important negative prognostic factors for HCC patients. The characteristics of a large cohort of such patients were examined. We found that the percent of patients with PVT significantly increased with increasing maximum tumor diameter (MTD), from 13.7% with tumors of MTD <5cm to 56.4% with tumors of MTD >10cm. There were similar numbers of HCC patients with very large tumors with and without PVT. Thus, MTD alone was insufficient to explain the presence of PVT, as were high AFP levels, since less than 50% of high AFP patients had PVT. However, the percent of patients with PVT was also found to significantly increase with increasing blood alpha-fetoprotein (AFP) levels and tumor multifocality. A logistic regression model that included these 3 factors together showed an odds ratio of 17.9 for the combination of MTD>5.0cm plus tumor multifocality plus elevated AFP, compared to low levels of these 3 parameters. The presence or absence of macroscopic PVT may therefore represent different HCC aggressiveness phenotypes, as judged by a significant increase in tumor multifocality and AFP levels in the PVT positive patients. Factors in addition to MTD and AFP must also contribute to PVT development.
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Carcinoma Hepatocelular/patologia , Neoplasias Hepáticas/patologia , Neoplasias Primárias Múltiplas/patologia , Células Neoplásicas Circulantes , Veia Porta/patologia , Trombose Venosa/etiologia , Idoso , Carcinoma Hepatocelular/sangue , Carcinoma Hepatocelular/complicações , Feminino , Humanos , Neoplasias Hepáticas/sangue , Neoplasias Hepáticas/complicações , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Neoplasias Primárias Múltiplas/sangue , Neoplasias Primárias Múltiplas/complicações , Carga Tumoral , alfa-Fetoproteínas/metabolismoRESUMO
C-reactive protein (CRP) is a blood marker for inflammation and is an independent prognostic factor for many human cancers. Combined with albumin levels, it forms the basis of the Glasgow Index for cancer prognosis. We reviewed the literature on CRP and HCC and also evaluated blood CRP levels and combination CRP plus albumin levels in a large HCC cohort. In order to understand the prognostic significance of CRP, we retrospectively examined a large HCC cohort and examined the relationship of CRP levels to tumor parameters. We report, that CRP alone and CRP plus albumin combined as well, significantly correlated with parameters of HCC aggressiveness, such as maximum tumor dimension (MTD), portal vein thrombosis (PVT) and blood alpha-fetoprotein (AFP) levels, both as individual parameters and all parameters together (Aggressiveness Index). This extends current thinking, to suggest a possible explanation for the usefulness of blood CRP levels in HCC prognostication.
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A large database of 1773 HCC patients in Turkey was examined. 41.9% had alpha-fetoprotein (AFP) levels <20 IU/ml and an additional 16.123% had values between 20-100 IU/ml. This 58% of the cohort (<100 IU/ml AFP levels) was examined in detail. 66% of patients with small (<5 cm) HCCs had low AFP, compared to 49% of patients with larger (>5 cm) HCCs. The mean diameter (MTD) of larger MTD, low AFP tumors was 8.4cm. Therefore, factors other than AFP must contribute to HCC tumor growth. Larger tumors in low AFP patients had both higher platelet levels and increased PVT percent. Linear regression analysis for both MTD and multifocality showed that platelet numbers and presence of PVT were significant variables; whereas for PVT, significant variables were albumin, alkaline phosphatase and MTD. Comparisons between patients with AFP levels <20, 20-<100, 100-<1000 and >1000 IU/ml showed the most significant tumor finding was an increase in PVT percent between each group, and to a lesser extent, MTD. Thus, low- or normal-AFP HCCs constitute the majority of patients and have slightly lower MTD and much lower PVT percent than HCCs associated with elevated blood AFP levels. New, non-AFP markers are thus needed, especially for small HCCs.
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Liver transplantation (LT) has become a favorable therapeutic option for patients with end-stage liver diseases. Gilbert's syndrome (GS) is a benign condition characterized by intermittent mild jaundice due to unconjugated hyperbilirubinemia. It is not obvious whether living-donor liver transplantation (LDLT) from a donor with GS could result in a normal outcome for both the recipient and the donor. We aimed to determine whether right lobe hepatectomy is a safe procedure for living donors with GS and LT recipients. Between September 2011 and March 2015, 305 LDLT procedures using right lobe grafts were performed at Atasehir Memorial Hospital, Istanbul, Turkey. Nineteen of 305 LT candidates who had been diagnosed with GS were included in the current study. After a 12-h overnight fast, total and indirect bilirubin levels of donors and recipients were measured. The median follow-up after transplant was 16 months (range 3-36 months). The median age of donors was 25 (range 20-55 yr). Four donors (21%) were female, and 15 donors (89%) were male. The median age of donors was 51 (range 23-68 yr). Eleven recipients (57%) were female, and 8 (43%) were male. The median preoperative total bilirubin level of donors was 1.69 mg/dL (range 1.26-2.43 mg/dL) (normal range <1.2 mg/dL). The median total bilirubin level of donors on postoperative day 7 was 1.04 mg/dL (range 0.71-3.23 mg/dL). As our study has included a large number of donors with GS, it produced reliable evidence that right lobe hepatectomy is a safe procedure for living donors with GS and LT recipients.
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Seleção do Doador , Doença Hepática Terminal/cirurgia , Doença de Gilbert/cirurgia , Transplante de Fígado , Doadores Vivos , Adulto , Idoso , Feminino , Seguimentos , Hepatectomia , Humanos , Testes de Função Hepática , Masculino , Pessoa de Meia-Idade , Prognóstico , Adulto JovemRESUMO
Acute appendicitis is uncommon after renal transplant. Infection with Candida albicans can produce serious complications by compromising the vascular anastomosis. In such cases, the origin of Candida albicans is often in the gastrointestinal system. Here, we report 2 uncommon complications that occurred in the same patient. A 27-year-old female renal transplant patient with appendicitis presented to our institution with acute graft failure. The patient was treated with an appendectomy and a transplant nephrectomy. Subsequently, the patient had a mycotic pseudoaneurysm rupture of the external iliac artery secondary to Candida albicans infection that originated possibly in the gastrointestinal system. This complication was further treated with a cross-femoral bypass. The occurrence of these 2 complications together is rare.
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Aneurisma Infectado/microbiologia , Aneurisma Roto/microbiologia , Apendicite/complicações , Candida albicans/isolamento & purificação , Aneurisma Ilíaco/microbiologia , Transplante de Rim/efeitos adversos , Adulto , Aneurisma Infectado/diagnóstico , Aneurisma Infectado/cirurgia , Aneurisma Roto/diagnóstico , Aneurisma Roto/cirurgia , Antifúngicos/uso terapêutico , Apendicectomia , Apendicite/diagnóstico , Apendicite/cirurgia , Implante de Prótese Vascular , Feminino , Humanos , Aneurisma Ilíaco/diagnóstico , Aneurisma Ilíaco/cirurgia , Nefrectomia , Diálise Renal , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND: Intra-abdominal hypertension (IAH) has negative effects on the functions of intra- and extra-abdominal organs and systems. Intra- and extraperitoneal hemorrhage, diffuse intestinal and retroperitoneal edema, intestinal ileus or obstruction, necrotizing pancreatitis, intra-abdominal packing, intra-abdominal sepsis, and pneumoperitoneum can all cause IAH. No studies were found in the literature relating to the effects of IAH on the endocrine functions of the pancreas. Therefore, the objective of this study was to investigate the effects of IAH on the endocrine functions of the pancreas. METHODS: Forty male rats were divided into four groups: control, sham control, and two study groups, each containing 10 rats. In one of the study groups, animals were subjected to IAH of up to 20 mm Hg and in the other study group to 25 mm Hg, for 3 hours. At the end of the study, blood samples were collected for biochemical analysis and pancreatic tissue samples for histopathologic examination. RESULTS: The results showed that glucagon levels were increased in the study groups (p<0.001) and insulin levels were decreased (p<0.001). There were no differences between the control and sham control groups. Histopathologic examination showed inflammatory cell infiltration in exocrine pancreatic tissue and vascular congestion in the islets of Langerhans. CONCLUSION: IAH causes an increase in glucagon levels and a decrease in insulin levels. These changes may be due to both the direct effects of IAH and the indirect effects of other organs that are affected by IAH.
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Hipertensão Intra-Abdominal/fisiopatologia , Ilhotas Pancreáticas/fisiopatologia , Animais , Glucagon/sangue , Insulina/sangue , Hipertensão Intra-Abdominal/sangue , Hipertensão Intra-Abdominal/patologia , Ilhotas Pancreáticas/patologia , Masculino , Ratos , Ratos Sprague-DawleyRESUMO
OBJECTIVES: Posttransplant leukopenia is frequently observed in renal transplant. Granulocyte colony-stimulating factor controls the production of functional neutrophils and their release into peripheral blood. Granulocyte colony-stimulating factor has been widely and frequently used for many conditions and disorders in the field of hematology and oncology. MATERIALS AND METHODS: We present the cases of valacyclovir-related and valganciclovir-related neutropenia in 2 renal transplant recipients. RESULTS: Both cases had renal transplants from live donors. The first one was an 18-year-old man. Laboratory investigations revealed his leukocyte count as 1.7 x 10(9)/L. The patient was using mycophenolate mofetil, cyclosporine, and valganciclovir. Mycophenolate mofetil was stopped because he had neutropenia, and later, valganciclovir was also stopped because the neutropenia persisted. Because the neutropenia did not recover after we discontinued valganciclovir, the patient was administered granulocyte colony-stimulating factor. The neutrophil count increased to 2.2 x 10(9)/L (leucocyte count to 6.5 x 10(9)/L) after 24 hours. The second case was a 37-year-old man and was using mycophenolic acid, tacrolimus, and valacyclovir. Laboratory investigations revealed his leukocyte count to be 1.3 x 10(9)/L. Mycophenolic acid and valganciclovir were stopped owing to neutropenia. The patient was administered granulocyte colony-stimulating factor, and the neutrophil count increased to 3.8 x 10(9)/L (leucocyte count to 5.8 x 10(9)/L). The kidney functions did not deteriorate in either patient, and the patients' kidney functions were similar to baseline levels 12 months after surgery. CONCLUSIONS: We conclude that granulocyte colony-stimulating factor can be used safely and effectively in renal transplant patients.
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Aciclovir/análogos & derivados , Antivirais/efeitos adversos , Infecções por Citomegalovirus/prevenção & controle , Ganciclovir/análogos & derivados , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Transplante de Rim , Neutropenia/tratamento farmacológico , Valina/análogos & derivados , Aciclovir/efeitos adversos , Adolescente , Adulto , Infecções por Citomegalovirus/etiologia , Filgrastim , Ganciclovir/efeitos adversos , Humanos , Transplante de Rim/efeitos adversos , Masculino , Neutropenia/induzido quimicamente , Proteínas Recombinantes , Resultado do Tratamento , Valaciclovir , Valganciclovir , Valina/efeitos adversosRESUMO
OBJECTIVE: Our goal is to investigate the distribution of MEFV mutations in patients with renal amyloidosis who are in renal transplant waiting list which is prepared for transplantation. MATERIALS AND METHODS: FMF was diagnosed in 25 of the 297 patients between the years 2004 and 2008, who were involved in the study (15 male, 10 female; age 34±7.8). 5 out of 25 patients were transplanted, remaining were waiting for Tx. Biopsy results were amyloidosis and taken from renal (n:16), rectal (n:8) and duodenal (1).All of them were carrier of mutations in both pyrin alleles.The primer cause of chronic renal failure in our group was secondary AA amyloidosis. DNA was isolated from 25 whole blood samples. The NanoChip Molecular Biology Workstation (Nanogen) uses electronic microarrays for mutation detection. Exon 2,3,5 and 10 of pyrin gene genotypes were identified in the NanoChip. RESULTS: Genetic analysis of the patients demonstrated that each subject carries either homozygote or compound heterozygote mutations of the gene. The most common mutations were M694V, V726A, E148Q and M680I. CONCLUSIONS: The clinic manifestation and complain of our patients were febrile and painful attacks such as in the abdomen, chest and joints due to inflammation of the peritoneum, pleura and synovial membrane. The major problem in FMF is the occurrence of amyloidosis that primarily affects the kidneys causing proteinuria and renal failure. Dialysis and renal transplantation can be treatment, but it is important to diagnose FMF at earliest stages. The percentage of FMF patients in our waiting list was 8.4%. Moreover, in our region FMF incidence is highly frequent, so FMF should be chased by genetically so as to prevent chronic renal failure due to amyloidosis.