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1.
Rev. gastroenterol. Perú ; 43(2)abr. 2023.
Article in English | LILACS-Express | LILACS | ID: biblio-1450021

ABSTRACT

Retroperitoneal cystic lymphangiomas (RCL) are rare benign tumors of the lymphatic system. They account for less than 1% of all lymphangiomas. Surgical resection is the recommended treatment option; however, obtaining a pre-operative diagnosis is often difficult and, in most cases, the final diagnosis is only possible following histological assessment of the surgical specimen. This report describes a case of RCL in a 58-year-old female cirrhotic patient who presented to our center with dull aching abdominal pain and distension. To our knowledge, this is the first case of a RCL in a cirrhotic patient reported in the literature.


Los linfangiomas quísticos retroperitoneales (LQR) son tumores benignos raros del sistema linfático y representan menos del 1% de todos los linfangiomas. La resección quirúrgica es la opción de tratamiento recomendada; sin embargo, obtener un diagnóstico preoperatorio suele ser difícil y, en la mayoría de los casos, el diagnóstico final solo es posible tras la evaluación histológica de la pieza operatoria. En este estudio, describimos un caso de un LQR en una paciente cirrótica de 58 años que se presentó en nuestro centro con dolor abdominal sordo y distensión. Hasta donde sabemos, este es el primer caso de LQR en un paciente cirrótico reportado en la literatura.

2.
Journal of Clinical Hepatology ; (12): 205-210, 2023.
Article in Chinese | WPRIM | ID: wpr-960693

ABSTRACT

Acute decompensated cirrhosis is a stage of end-stage liver disease during which patients often experience decompensated complications and rapid progression. Systemic inflammatory response is characterized by excessive secretion of inflammatory factors caused by bacterial infection of local tissue and rapid spread to the whole body, thereby affecting the physiological activities of the body and causing organ damage or disorder, and it is a relatively serious inflammatory state. This article elaborates on the occurrence of systemic inflammation, the factors affecting the severity of systemic inflammation, the manifestation of systemic inflammation in different stages of decompensated cirrhosis, and the role of systemic inflammation in complications, in order to gain a deeper understanding of systemic inflammation and apply it in the research and development of new therapies and drugs.

3.
Journal of Clinical Hepatology ; (12): 1010-1018, 2023.
Article in Chinese | WPRIM | ID: wpr-973185

ABSTRACT

End-stage liver disease (ESLD) includes decompensated liver cirrhosis and liver failure, which usually have dangerous conditions and a poor prognosis. Liver transplantation is the only effective therapy for ESLD, but its clinical application is limited due to shortage of liver donors, immunological rejection, and expensive costs. Mesenchymal stem cells (MSCs) can differentiate into hepatocyte-like cells and alleviate liver fibrosis by regulating immune function through paracrine, and therefore, MSCs have a wide application prospect in the field of ESLD treatment. A number of clinical studies have shown that MSC infusion is safe and effective in the treatment of ESLD during a short period of time, and there is also certain clinical evidence for its long-term safety and efficacy. MSC-derived exosomes (MSC-Exo) do not have a complete cellular structure and can promote hepatocyte regeneration through a variety of mechanisms, and their clinical value has attracted more and more attention, but there are few studies on this issue. Currently, the core mechanism of MSC therapy for ESLD and the standardized process of MSC preparation are the problems needing to be solved urgently.

4.
Organ Transplantation ; (6): 905-912, 2023.
Article in Chinese | WPRIM | ID: wpr-997826

ABSTRACT

Patients with end-stage liver disease after liver transplantation constantly suffer from malnutrition due to primary diseases and transplantation-related factors. Malnutrition will worsen clinical condition of the patients, increase the incidence of complication, length of hospital stay and medical expense after transplantation, and lower the survival rate. Sufficient nutritional support at all stages of liver transplantation is of significance. Accurate assessment of nutritional status and timely intervention are prerequisites for perioperative nutritional treatment in liver transplantation. In this article, the latest nutritional risk screening indexes and evaluation tools, nutritional support methods and other perioperative nutritional intervention measures for liver transplantation were reviewed, aiming to deepen the understanding and cognition of perioperative nutritional therapy for liver transplantation and provide reference for improving nutritional status and clinical prognosis of liver transplant recipients.

5.
Chinese Journal of Laboratory Medicine ; (12): 52-61, 2023.
Article in Chinese | WPRIM | ID: wpr-995697

ABSTRACT

Objective:To investigate the diagnostic accuracy of serological indicators and evaluate the diagnostic value of a new established combined serological model on identifying the minimal hepatic encephalopathy (MHE) in patients with compensated cirrhosis.Methods:This prospective multicenter study enrolled 263 compensated cirrhotic patients from 23 hospitals in 15 provinces, autonomous regions and municipalities of China between October 2021 and August 2022. Clinical data and laboratory test results were collected, and the model for end-stage liver disease (MELD) score was calculated. Ammonia level was corrected to the upper limit of normal (AMM-ULN) by the baseline blood ammonia measurements/upper limit of the normal reference value. MHE was diagnosed by combined abnormal number connection test-A and abnormal digit symbol test as suggested by Guidelines on the management of hepatic encephalopathy in cirrhosis. The patients were randomly divided (7∶3) into training set ( n=185) and validation set ( n=78) based on caret package of R language. Logistic regression was used to establish a combined model of MHE diagnosis. The diagnostic performance was evaluated by the area under the curve (AUC) of receiver operating characteristic curve, Hosmer-Lemeshow test and calibration curve. The internal verification was carried out by the Bootstrap method ( n=200). AUC comparisons were achieved using the Delong test. Results:In the training set, prevalence of MHE was 37.8% (70/185). There were statistically significant differences in AMM-ULN, albumin, platelet, alkaline phosphatase, international normalized ratio, MELD score and education between non-MHE group and MHE group (all P<0.05). Multivariate Logistic regression analysis showed that AMM-ULN [odds ratio ( OR)=1.78, 95% confidence interval ( CI) 1.05-3.14, P=0.038] and MELD score ( OR=1.11, 95% CI 1.04-1.20, P=0.002) were independent risk factors for MHE, and the AUC for predicting MHE were 0.663, 0.625, respectively. Compared with the use of blood AMM-ULN and MELD score alone, the AUC of the combined model of AMM-ULN, MELD score and education exhibited better predictive performance in determining the presence of MHE was 0.755, the specificity and sensitivity was 85.2% and 55.7%, respectively. Hosmer-Lemeshow test and calibration curve showed that the model had good calibration ( P=0.733). The AUC for internal validation of the combined model for diagnosing MHE was 0.752. In the validation set, the AUC of the combined model for diagnosing MHE was 0.794, and Hosmer-Lemeshow test showed good calibration ( P=0.841). Conclusion:Use of the combined model including AMM-ULN, MELD score and education could improve the predictive efficiency of MHE among patients with compensated cirrhosis.

6.
Chinese Journal of General Practitioners ; (6): 62-68, 2023.
Article in Chinese | WPRIM | ID: wpr-994694

ABSTRACT

Objective:To evaluate neutrophil/lymphocyte ratio(NLR) and the model for end-stage liver disease-sodium(MELD-Na)score in predicting short-term prognosis of patients with HBV-related acute-on-chronic liver failure(HBV-ACLF).Methods:A total of 234 consecutive HBV-ACLF patients(194 males and 40 females, aged 23-85 years)admitted to Hangzhou Xixi Hospital from January 2019 to December 2021 were enrolled. According to the 12-week clinical outcomes, patients were divided into good prognosis group( n=141)and poor prognosis group( n=93). Univariate and multivariate Logistic regression were performed to identify independent risk factors for poor prognosis of HBV-ACLF patients. Receiver operating characteristics(ROC)curve was applied to evaluate the accuracy of risk factors in predicting short-term prognosis of HBV-ACLF patients. Results:The age [(48.7±11.9) vs. (52.5±9.9) years old, t=-2.59, P=0.011], proportion of males [78.0%(110/141) vs. 90.3%(84/93), χ2=5.99, P=0.014], total bilirubin[202.9(141.2, 287.6) vs. 320.0(224.4, 400.0) μmol/L, Z=-5.14, P<0.001], creatinine [71.0(59.0, 78.0) vs. 81.0(64.0, 111.0)μmol/L, Z=-3.98, P<0.001], international normalized ratio[1.66(1.52, 1.86) vs. 1.91(1.66, 2.27), Z=-5.46, P<0.001], leukocyte count[5.16(3.99, 6.95)×10 9/L vs. 6.57(4.83, 8.30)×10 9/L, Z=-4.14, P=0.001], NLR[2.77(2.02, 3.55) vs. 5.48(3.44, 8.53), Z=-8.48, P<0.001], MELD score[22.0(20.0, 24.0) vs. 26.0(24.0, 29.0), Z=-9.22, P<0.001], MELD-Na score[22.8(20.0, 25.6) vs. 29.0(25.0, 36.0), Z=-9.16, P<0.001], liver cirrhosis[77.3%(109/141) vs. 88.2%(82/93), χ2=4.41, P=0.036], hepatorenal syndrome[4/141(2.8%) vs. 12/93(12.9%), χ2=8.91, P=0.003] and the proportion of artificial liver treatment[21/141(14.9%) vs. 24/93(25.8%), χ2=4.30, P=0.038] were significantly elevated in poor prognosis group compared with survival group. Logistic regression analysis showed that NLR( OR=3.76, 95 %CI: 2.10-6.74, P<0.001)and MELD-Na score( OR=2.24, 95 %CI: 1.17-4.29, P=0.015) were independent risk factors for poor short-term prognosis of HBV-ACLF patients. The area under the ROC curve(AUC)of NLR, and MELD-Na for the short-term prognosis of HBV-ACLF patients was 0.792 and 0.822, respectively. The AUC of the combination of NLR with MELD-Na was 0.858, which was significantly higher than that of NLR( Z=-3.04, P=0.001) or MELD-Na score( Z=-2.16, P=0.031)alone. Based on the cut-off value of the combined model, patients were classified into high combined model score (≥0.04) group and low combined model score (<0.04) group, the survival rate of the high group was significantly higher than that of the low group( χ2=67.47, P<0.001). Conclusions:NLR and MELD-Na score are independent risk factors of the short-term prognosis of HBV-ACLF patients. The combination of NLR and MELD-Na score will be beneficial to predict the short-term prognosis of HBV-ACLF patients.

7.
Chinese Journal of Clinical Infectious Diseases ; (6): 234-240, 2023.
Article in Chinese | WPRIM | ID: wpr-993736

ABSTRACT

Myeloid-derived suppressor cells (MDSCs) are a group of immature and heterogeneous cells that can inhibit T cell function. In pathological conditions such as tumors, infections, and chronic inflammation, the large expansion of MDSCs is involved in processes of immune escape, immune tolerance and inflammatory reactions. MDSCs are also crucial in the pathophysiology of hepatitis B virus (HBV) infection, however, their activation, differentiation, and function during HBV infection are still unclear. This article reviews the general characteristics and roles of MDSCs in HBV infection, as well as related drug therapies, in order to provide information for further research on the related mechanism and potential targeted treatment.

8.
Organ Transplantation ; (6): 154-2023.
Article in Chinese | WPRIM | ID: wpr-959034

ABSTRACT

Hepatic venous pressure gradient (HVPG) is the "gold standard" for the diagnosis of portal hypertension, which could be applied in the evaluation of liver cirrhosis. Combined use of HVPG with model for end-stage liver disease (MELD) scoring system may more accurately match the donors and recipients undergoing liver transplantation for liver cirrhosis, select the appropriate timing of surgery, and provide guidance for bridging treatment for patients on the waiting list for liver transplantation. Besides, HVPG may also predict clinical prognosis of liver transplant recipients, and provide evidence for early detection and intervention of potential complications. Therefore, the value of HVPG in preoperative evaluation and prognosis prediction of liver transplant recipients was reviewed, aiming to provide guidance for clinical diagnosis and treatment of liver transplant recipients before and after surgery.

9.
Chinese Journal of Hepatobiliary Surgery ; (12): 808-811, 2022.
Article in Chinese | WPRIM | ID: wpr-957048

ABSTRACT

Objective:To evaluate the value of preoperative aspartate aminotransferaseto platelet ratio index (APRI) and fibrosis index 4 (Fib4) in predicting posthepatectomy liver failure (PHLF) of primary hepatocellular carcinoma.Methods:The data of 587 patients with hepatocellular carcinoma admitted to the First Affiliated Hospital of Xinjiang Medical University from January 2014 to January 2020 were retrospectively collected and analyzed, including 412 males and 175 females, aged (56.8±11.2) years. Univariate and multivariate logistic regression were used to analyze the influencing factors of PHLF. The ability of Child-Pugh score, model for end-stage liver diseas (MELD) score, APRI and Fib4 to predict PHLF was evaluated through the receiver operating characteristic (ROC) curve of subjects.Results:Among 587 patients, 186 (31.7%) had liver failure after hepatectomy. In multivariate logistic regression analysis, APRI ( OR=2.660, 95% CI: 1.314-5.384, P=0.007) and Fib4 ( OR=1.322, 95% CI: 1.157-1.511, P<0.001) were risk factors for PHLF in patients with hepatocellular carcinoma. The higher the number, the greater the risk of PHLF. The predicted area under the ROC curve of PHLF in patients with hepatocellular carcinoma was Fib4(0.719)>APRI(0.686)>MELD score(0.618)>Child-Pugh score(0.565). Conclusion:APRI and Fib4 were risk factors of PHLF in patients with hepatocellular carcinoma. They predict the occurrence of PHLF better than Child-Pugh score and MELD score.

10.
Chinese Journal of Hepatobiliary Surgery ; (12): 624-628, 2022.
Article in Chinese | WPRIM | ID: wpr-957014

ABSTRACT

End-stage liver disease refers to the advanced stage of liver disease caused by various acute and chronic liver damage, of which decompensated cirrhosis and liver failure are most common. Currently, liver transplantation is the most effective treatment option for patients with end-stage liver disease, but several limitations regarding transplantation currently restrict its application. Mesenchymal stem cells are the potential treatment option for end-stage liver disease because of the biological properties such as multidirectional differentiation, paracrine and immunomodulatory properties, as well as anti-inflammatory, anti-apoptotic and anti-fibrosis, but the therapeutic effect is affected by low homing rate, poor colonization and low conversion rate. The researchers aimed to improve them through genetic modification, chemical or hypoxic pretreatment, and three dimensional culture. This article reviewed and discussed the pretreatment methods, their application status of treatment for end-stage liver disease and the existing problems.

11.
Chinese Journal of Infectious Diseases ; (12): 613-619, 2022.
Article in Chinese | WPRIM | ID: wpr-956458

ABSTRACT

Objective:To analyze the predictive ability of model for end-stage liver disease (MELD)-sarcopenia score in short-term prognosis of patients with acute-on-chronic liver failure (ACLF).Methods:Two hundred and seventy-one patients with ACLF hospitalized in Tianjin Third Central Hospital from January 2013 to December 2019 were selected, among whom 157 cases with sarcopenia and 114 cases without sarcopenia.According to ACLF classification, the patients were divided into group A (no cirrhosis basis) of 61 cases, group B (compensated cirrhosis basis) of 99 cases, and group C (previous history of uncompensated cirrhosis) of 111 cases.The basic data, laboratory examination results, computed tomography (CT) examination results and prognosis of the patients were retrospectively collected, and the MELD score, MELD-Na score and MELD-sarcopenia score were calculated. Multivariate logistic regression, multivariate Cox proportional hazards regression, Kaplan-Meier method, log-rank method and area under receiver operating characteristic curve were used for statistical analysis.Results:Low body mass index (odds ratio ( OR)=0.93, P<0.001), complicated cirrhosis ( OR=1.14, P=0.004), complicated hepatic encephalopathy ( OR=1.31, P<0.001), high white blood cell level ( OR=1.18, P=0.009) and high platelet level ( OR=1.08, P<0.001) were independent risk factors for sarcopenia in patients with ACLF. High MELD score (hazard ratio ( HR)=1.02, P=0.001), high MELD-Na score ( HR=1.07, P=0.038), high MELD-sarcopenia score ( HR=1.14, P<0.001), high total bilirubin ( HR=1.00, P<0.001) and high international normalized ratio (INR) ( HR=1.71, P<0.001) were independent risk factors for death in patients with ACLF. In subgroup analysis, the cumulative survival rate of sarcopenia patients in group A and B was lower than that of non-sarcopenia patients ( χ2=5.97 and 8.34, respectively, P=0.015 and 0.004, respectively), while there was no significant difference in the cumulative survival rate between sarcopenia patients and non-sarcopenia patients in group C ( χ2=4.90, P=0.053). In groups A and B, the area under the curve (AUC) of MELD-sarcopenia score in predicting short-term prognosis was 0.87, which was higher than MELD score (0.78) and MELD-Na score (0.78), and the differences were both statistically significant ( Z=2.86 and 2.56, respectively, P=0.004 and 0.011, respectively). The AUC of MELD-Na score in predicting short-term prognosis in group C (0.83) was higher than that of MELD score (0.71) and MELD-sarcopenia score (0.69), and the differences were both statistically significant ( Z=2.52 and 2.64, respectively, P=0.012 and 0.008, respectively). Conclusions:Patients with ACLF with no cirrhosis basis or compensated cirrhosis basis complicated with sarcopenia have shorter survival time and worse prognosis than those without sarcopenia. For patients with ACLF with no cirrhosis basis or compensated cirrhosis basis, MELD-sarcopenia score has better predictive value for the short-term prognosis.

12.
Chinese Journal of Clinical Nutrition ; (6): 310-317, 2022.
Article in Chinese | WPRIM | ID: wpr-955966

ABSTRACT

Sarcopenia is one of the most common complications of end-stage liver disease (ESLD) and is an independent risk factor for mortality in ESLD patients. Increasing evidence has indicated that nutritional intervention plays an important role in improving the prognosis of ESLD complicated with sarcopenia. Timely identification and early treatment of sarcopenia in ESLD are indispensable for improving patient outcome and quality of life. Accumulating in-depth researches on the pathogenesis and metabolic characteristics of sarcopenia in ESLD patients have provided increasing evidence for the nutritional treatment of sarcopenia in ESLD. Here we reviewed and summarized the research progress regarding the early identification, nutritional risk screening, assessment, and intervention of sarcopenia in ESLD.

13.
International Journal of Biomedical Engineering ; (6): 93-98,111, 2022.
Article in Chinese | WPRIM | ID: wpr-954198

ABSTRACT

Objective:To analyze the risk factors of death within two years of the patients with liver cirrhosis after transjugular intrahepatic portosystemic shunt(TIPS), and to explore the predictive value of 6 common clinical evaluation systems on the risk of death after TIPS.Methods:TIPS clinical data from 132 patients with liver cirrhosis were analyzed retrospectively. According to the 2-year clinical outcome after TIPS, the patients were divided into the death group and the survival group. Logistic regression was used to analyze the risk factors of death within 2 years after TIPS. According to the scores of CTP, MELD, MELD Na, BioCliM, FIB-4, and ALBI evaluation systems, the prediction efficiency of death risk of the six evaluation systems was evaluated by using the receiver operating characteristic (ROC) curves and the area under the curve (AUC).Results:During the 2-year follow-up period after TIPS, the age, urea nitrogen level, platelet count, and proportion of hepatic encephalopathy in the death group were higher than those in the survival group one month after TIPS, and the serum sodium level was lower than those in the survival group (all P<0.05). Multivariate analysis showed that the elderly and hepatic encephalopathy one month after operation were independent risk factors for death (all P<0.05). At 1 week after the surgery, there were significant differences in CTP, MELD, and MELD-Na scores between the survival group and the death group (all P<0.05). One week after operation, the AUC of ROC of CTP, MELD, MELD-Na, and ALBI scores were 0.685, 0.721, 0.805, and 0.658 respectively, and the optimal critical values were 8.5, 12.99, 14.51 and -1.52 respectively. Conclusions:The elderly and the occurrence of hepatic encephalopathy one month after TIPS are independent risk factors for the death of liver cirrhosis patients after TIPS. The evaluation of CTP, MELD, MELD-Na, and ALBI one week after TIPS can predict the death risk of decompensated liver cirrhosis patients within 2 years after TIPS, and MELD-Na has the best predictive effect.

14.
Fisioter. Mov. (Online) ; 35: e35138, 2022. tab
Article in English | LILACS-Express | LILACS | ID: biblio-1404789

ABSTRACT

Abstract Introduction Children with chronic liver diseases are exposed to biological and/or environmental risk factors that can compromise their neuromotor acquisition and development of functional skills. Objective To describe the neuropsychomotor development (NPMD) and functional skills of children with chronic liver diseases. Methods Cross-sectional, descriptive and exploratory study carried out with children up to 6 years old who were selected at a reference hospital in the state of Bahia, Brazil, from November 2019 to March 2020. Children in outpatient care with clinical, laboratory and histological diagnosis compatible with chronic liver disease were considered eligible. The instrument for assessing neuropsychomotor development was Denver II. Functional skills were obtained by applying the Pediatric Evaluation of Disability Inventory computer-adaptive test (PEDI-CAT) to parents or primary caregivers, Speedy version (Speedy-CAT). Results Of the 34 children with chronic liver disease, 52.9% were female, aged between 4 and 6 years (64%). The results of the Denver II test showed that 68.7% (22/32) of the sample were at risk for NPMD. In the PEDI-CAT, the scores of children with liver disease at risk for NPMD were 60.7 ± 9.1 in the daily activity domains, 57.6 ± 11.8 in mobility and 48.3 ± 6.2 in the social/cognitive domains. Conclusion Children with chronic liver disease are at risk for NPMD, although not presenting impaired functional skills when evaluated by the PEDI-CAT.


Resumo Introdução Crianças com doenças hepáticas crônicas são expostas a fatores de risco biológicos e/ou ambientais que podem comprometer suas aquisições neuromotoras e o desenvolvimento de suas habilidades funcionais. Objetivo Descrever o desenvolvimento neuropsicomotor (DNPM) e habilidades funcionais de crianças com doenças hepáticas crônicas. Métodos Estudo seccional, descritivo e exploratório realizado com crianças de até 6 anos, que foram selecionadas em um hospital de referência do estado da Bahia, Brasil, no período de novembro de 2019 a março de 2020. Foram consideradas elegíveis para o estudo crianças em atendimento ambulatorial, com diagnóstico clínico, laboratorial e histológico compatíveis com doença hepática crônica. O instrumento de avaliação do desenvolvimento neuropsicomotor foi o Denver II. As habilidades funcionais foram obtidas pela aplicação do Inventário de Avaliação Pediátrica de Incapacidade Testagem Computadorizada Adaptativa (PEDI-CAT) aos pais ou cuidadores principais, versão rápida (Speedy-CAT). Resultados Das 34 crianças com hepatopatias crônicas, 52,9% eram do sexo feminino, com idade entre 4 e 6 anos (64%). Os resultados do teste de Denver II demonstraram que 68,7% (22/32) da amostra apresentaram risco para DNPM. No PEDI-CAT, os escores das crianças hepatopatas com risco para DNPM foram de 60,7 ± 9,1 nos domínios atividade diária, 57,6 ± 11,8 em mobilidade e 48,3 ± 6,2 em social/cognitivo. Conclusão Crianças com hepatopatias crônicas apresentam risco para DNPM, apesar de não possuírem comprometimento de suas habilidades funcionais quando avaliadas pelo PEDI-CAT.

15.
Journal of Clinical Hepatology ; (12): 215-219, 2022.
Article in Chinese | WPRIM | ID: wpr-913146

ABSTRACT

There is a high prevalence rate of malnutrition in patients with end-stage liver disease, which often promotes disease progression and has a negative impact on the prognosis of patients. This article briefly describes the etiology of malnutrition in end-stage liver disease and introduces the research advances in nutrition screening, evaluation, and treatment in end-stage liver disease in China and globally, hoping to provide inspiration for nutritional support in patients with end-stage liver disease in China.

16.
Organ Transplantation ; (6): 61-2022.
Article in Chinese | WPRIM | ID: wpr-907034

ABSTRACT

Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.

17.
Organ Transplantation ; (6): 489-2022.
Article in Chinese | WPRIM | ID: wpr-934770

ABSTRACT

Objective To explore the predictive values of the initial model for end-stage liver disease (MELD) score, MELD combined with serum sodium (MELD-Na) score and MELD combined with serum lactic acid (MELD-Lac) score for early survival rate after liver transplantation in patients with liver failure. Methods Clinical data of 135 recipients undergoing liver transplantation for liver failure were retrospectively analyzed. All patients were divided into the early survival group (n=110) and early death group (n=25) according to the survival at postoperative 28 d. Clinical data were compared between two groups. The optimal cut-off values of MELD, MELD-Na and MELD-Lac scores for predicting early survival rate after liver transplantation in patients with liver failure were determined by the receiver operating characteristic (ROC) curve. The predictive values of different scores for early survival rate after liver transplantation in patients with liver failure were evaluated. Results Significant differences were observed in the initial MELD, MELD-Na and MELD-Lac scores after liver transplantation between two groups (all P < 0.05). For the initial MELD, MELD-Na and MELD-Lac scores in predicting early survival rate after liver transplantation in patients with liver failure, the AUC were 0.653 [95% confidence interval (CI) 0.515-0.792], 0.648 (95%CI 0.514-0.781) and 0.809 (95%CI 0.718-0.900), the optimal cut-off values were 18.09, 18.09 and 19.97, Youden's indexes were 0.398, 0.380 and 0.525, the sensitivity was 0.680, 0.680 and 0.840, and the specificity was 0.720, 0.700 and 0.690, respectively. The AUC of MELD-Lac score was higher than those of MELD and MELD-Na scores, and the differences were statistically significant (both P < 0.05). Conclusions Compared with the initial MELD and MELD-Na scores after liver transplantation, the initial MELD-Lac score is a more reliable index for predicting early survival rate after liver transplantation in patients with liver failure.

18.
Organ Transplantation ; (6): 611-2022.
Article in Chinese | WPRIM | ID: wpr-941482

ABSTRACT

Objective To evaluate the predictive values of albumin-bilirubin (ALBI) and easy albumin-bilirubin (EZ-ALBI) scores for early survival (postoperative 3 months) of recipients with liver failure after liver transplantation. Methods Clinical data of 137 recipients diagnosed with liver failure and underwent liver transplantation were retrospectively analyzed. The optimal cut-off values of preoperative ALBI, EZ-ALBI and MELD scores to predict early survival of recipients with liver failure after liver transplantation were determined by the area under the receiver operating characteristic (ROC) curve. The risk factors of early death of recipients with liver failure after liver transplantation were identified by univariate and multivariate Cox regression analyses. The effects of different ALBI and EZ-ALBI levels upon early prognosis of recipients with liver failure after liver transplantation were analyzed. Results The optimal cut-off values of ALBI, EZ-ALBI and MELD scores were 0.21, -19.83 and 24.36, and the AUC was 0.706, 0.697 and 0.686, respectively. Univariate Cox regression analysis showed that preoperative alanine aminotransferase(ALT)≥50 U/L, aspartate aminotransferase(AST)≥60 U/L, ALBI score≥0.21 and EZ-ALBI score≥-19.83 were the risk factors for early postoperative death of recipients with liver failure after liver transplantation (all P < 0.05). Multivariate Cox regression analysis demonstrated that preoperative ALBI score≥0.21 was an independent risk factor for early postoperative death of recipients with liver failure after liver transplantation (P < 0.05). According to the optimal cut-off value of ALBI score, the early survival rates in the ALBI < 0.21 (n=46) and ALBI≥0.21(n=91) groups were 93.5% and 64.8%, and the difference was statistically significant (P < 0.05). According to the optimal cut-off value of EZ-ALBI score, the early survival rates in the EZ-ALBI < -19.83(n=60) and EZ-ALBI≥-19.83(n=77) groups were 88.3% and 63.6%, and the difference was statistically significant (P < 0.05). Conclusions Preoperative ALBI score is of high predictive value for early survival of recipients with liver failure after liver transplantation, which could be utilized as a reference parameter for selecting liver transplant recipients.

19.
Organ Transplantation ; (6): 338-2022.
Article in Chinese | WPRIM | ID: wpr-923579

ABSTRACT

Drug-induced liver injury (DILI) is a type of necrotizing and inflammatory liver disease caused by certain commonly-used drugs, Chinese herbal medicines or dietary supplements. In severe cases, it may lead to acute liver failure. Without liver transplantation, the fatality could reach up to 80%. It is of significance to master the indications of liver transplantation. Several prognostic scoring systems have been developed to help clinicians to decide which patients need urgent liver transplantation, such as King's College criteria (KCC) and model for end-stage liver disease (MELD) scoring systems. However, these scoring methods have been developed for a long period of time and lack of modifications. Therefore, scholars have proposed several new scoring systems, such as acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) and sequential organ failure assessment (SOFA) scoring systems, which provide novel ideas for the evaluation of liver transplantation. As an important treatment measure for drug-induced acute liver failure, urgent liver transplantation has greatly improved the survival rate of patients. In this article, the classification, clinical diagnosis, liver transplantation evaluation and prognosis of DILI were summarized, aiming to provide reference for the treatment of DILI by liver transplantation.

20.
Organ Transplantation ; (6): 333-2022.
Article in Chinese | WPRIM | ID: wpr-923578

ABSTRACT

Acute-on-chronic liver failure (ACLF) is a specific category of liver failure, which is mainly characterized by rapid progression and multiple organ failure. At present, patients with ACLF are mainly given with systematic and comprehensive medical therapy to promote liver regeneration. However, liver transplantation is the only potentially curative treatment for patients who failed to respond to medical treatment and rapidly progress into multiple organ failure. Considering the differences of disease progression and clinical prognosis, and the shortage of donor liver in China, it is necessary to actively prevent the triggering factors of ACLF in patients with chronic liver diseases, screen out the recipients who are most likely to benefit from liver transplantation and deliver precision management during perioperative period of liver transplantation. In this article, the application of liver transplantation in ACLF was illustrated from the perspectives of accurate evaluation of ACLF, proper control of liver transplantation indications and meticulous perioperative management, aiming to optimize the therapeutic strategy of liver transplantation in patients with ACLF.

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