Gynura root has been used extensively in Chinese folk medicine and plays a role in promoting microcirculation and relieving pain.However,its hepatic toxicity should not be neglected.Recently,we admitted a 62-year old ...Gynura root has been used extensively in Chinese folk medicine and plays a role in promoting microcirculation and relieving pain.However,its hepatic toxicity should not be neglected.Recently,we admitted a 62-year old female who developed hepatic veno-occlusive disease(HVOD)after ingestion of Gynura root.Only a few articles on HVOD induced by Gynura root have been reported in the literature.It is suspected that pyrrolizidine alkaloids in Gynura root might be responsible for HVOD.In this paper,we report a case of HVOD and review the literature.展开更多
Hepatic veno-occlusive disease (HVOD) is rarely encountered and easily misjudged as Budd-Chiari syndrome. It is often related to stem cell transplantation in recent years. We report a case of HVOD that is related to i...Hepatic veno-occlusive disease (HVOD) is rarely encountered and easily misjudged as Budd-Chiari syndrome. It is often related to stem cell transplantation in recent years. We report a case of HVOD that is related to ingestion of some palatable local dishes. The diagnosis was confirmed by liver biopsy pathology with specific observation of inflammatory changes and fibrosis of venules intima, dilated sinusoids and central veins. Chronic diarrhea is unique for this case as a result of ingesting harmful stuffs. This case demonstrated that supervision and instruction of food recipe and traditional medicine are crucial, and prompt diagnosis, supportive care and specific treatment are essential to decreasing the morbidity and mortality of HVOD.展开更多
BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury...BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury and bleeding in rural areas in China. This study was undertaken to better understand VOD and its relations to the effect of Gymura segetum. METHODS: Between 2000 and 2002,two patients were admitted to our department because of VOD. Before admission, both of them had been injured and taken oral decoction of patent drug Gymura segetum. We analyzed the clinical manifestations, diagnosis and therapy of the two patients. RESULTS: Pyrrolizidine in Panax notginseng was proved to induce VOD. The diagnosis of VOD depended on hepatic biopsy. CONCLUSION: Gymura segetum can induce VOD. More attention should be paid to its unsuscepted side effects.展开更多
Nowadays hepatic veno-occlusive disease is mainly caused by Sedum aizoon in China,and its prognosis dependents on the dosage and courses of the Sedum aizoon treatment but lacks other objective indicators.There are a l...Nowadays hepatic veno-occlusive disease is mainly caused by Sedum aizoon in China,and its prognosis dependents on the dosage and courses of the Sedum aizoon treatment but lacks other objective indicators.There are a lot relationships between CA125 level and liver cirrhosis,this case had a obvious increased CA125 level in the serum,hydrothorax and ascites,following by the liver cirrhosis in a short time,and then died of upper gastrointestinal bleeding.By now we guess that CA125 level could forecast the liver cirrhosis following by hepatic veno-occlusive,which will become the prognosis of the hepatic veno-occlusive.展开更多
BACKGROUND:Hepatic veno-occlusive disease(HVOD)is a severe complication of chemotherapy before hematopoietic stem cell transplantation and dietary ingestion of pyrrolizidine alkaloids.Many experimental models were est...BACKGROUND:Hepatic veno-occlusive disease(HVOD)is a severe complication of chemotherapy before hematopoietic stem cell transplantation and dietary ingestion of pyrrolizidine alkaloids.Many experimental models were established to study its mechanisms or therapy,but few are ideal.This work aimed at evaluating a rat model of HVOD induced by monocrotaline to help advance research into this disease. METHODS:Thirty-two male rats were randomly classified into 5 groups,and PBS or monocrotaline was administered (100 mg/kg or 160 mg/kg).They were sacrificed on day 7(groups A,B and D)or day 10(groups C and E).Blood samples were collected to determine liver enzyme concentrations.The weight of the liver and body and the amount of ascites were measured.Histopathological changes of liver tissue on light microscopy were assessed by a modified Deleve scoring system.The positivity of proliferating cell nuclear antigen(PCNA)was estimated. RESULTS:The rats that were treated with 160 mg/kg monocrotaline presented with severe clinical symptoms (including two deaths)and the histopathological picture of HVOD.On the other hand,the rats that were fed with 100 mg/kg monocrotaline had milder and reversible manifestations.Comparison of the rats sacrificed on day 10 with those sacrificed on day 7 showed that the positivity of PCNA increased,especially that of hepatocytes.CONCLUSIONS:Monocrotaline induces acute,dose- dependent HVOD in rats.The model is potentially reversible with a low dose,but reliable and irreversible with a higher dose.The modified scoring system seems to be more accurate than the traditional one in reflecting the histopathology of HVOD.The enhancement of PCNA positivity may be associated with hepatic tissue undergoing recovery.展开更多
Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is co...Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is commonly given to transplant recipients from the start of conditioning through the early weeks of transplant. However, high quality evidence from randomized controlled trials is scarce with small sample sizes and the trials yielded conflicting results. Although various treatment options for hepatic VOD are available, most have not undergone stringent evaluation with randomized controlled trial and therefore it remains uncertain which treatment offers real benefit. It remains controversial whether VOD prophylaxis should be given, which prophylactic therapy should be given, who should receive prophylaxis, and what treatment should be offered once VOD is established.展开更多
Objective: Hepatic veno-occlusive disease(HVOD) has attracted increasing attention in recent years due to its relationship with ingestion of Gynura segetum. The mortality of severe HVOD remains high due to the lack of...Objective: Hepatic veno-occlusive disease(HVOD) has attracted increasing attention in recent years due to its relationship with ingestion of Gynura segetum. The mortality of severe HVOD remains high due to the lack of specific therapies. The aim of the study was to delineate the clinical characteristics and outcomes and explore the potential prognostic factors of HVOD.Methods: This was a single-center retrospective study. Eighty-nine HVOD patients were screened from the First Affiliated Hospital of Zhejiang University with an ingestion history of G. segetum before developing symptoms from January 2009 to May 2018. The enrolled patients were divided into the survivor and death groups according to the clinical follow-up that ended on September 1, 2019. The demographic variables and clinical data of the patients were recorded. A binary logistic regression analysis and receiver operating characteristic curve were conducted to identify the prognostic factors and assess the prognostic value for predicting death, and a survival analysis was performed to evaluate the clinical outcomes.Results: Sixty-four patients were eligible for further analysis. Most patients showed abdominal distension and were positive for migrating dullness in the abdomen(P = 0.740 and P = 0.732, respectively). The patients who died had higher levels of model for end-stage liver disease score, and higher prothrombin time than those who survived(both P < 0.001). All HVOD patients in both the survival and death groups showed ascites with abnormal imaging presentations of the liver parenchyma and hepatic blood vessels.Unexpectedly, we found that hydrothorax was detected in 21(65.63%) patients in the death group and 19(59.38%) patients in the survivor group during hospitalization, which was rarely mentioned in previous studies. Furthermore, international normalized ratio(INR) and creatinine are found to be potential independent prognostic factors for predicting death. Six severe patients achieved clinical improvements and survived after liver transplantation.Conclusion: HVOD can be induced by the ingestion of G. segetum, and INR combined with creatinine has prognostic value for predicting death. Liver transplantation may be an effective treatment option for severe HVOD patients.展开更多
Hepatic stellate cells(HSCs),a distinct category of non-parenchymal cells in the liver,are critical for liver homeostasis.In healthy livers,HSCs remain non-proliferative and quiescent.However,under conditions of acute...Hepatic stellate cells(HSCs),a distinct category of non-parenchymal cells in the liver,are critical for liver homeostasis.In healthy livers,HSCs remain non-proliferative and quiescent.However,under conditions of acute or chronic liver damage,HSCs are activated and participate in the progression and regulation of liver diseases such as liver fibrosis,cirrhosis,and liver cancer.Fatty liver diseases(FLD),including nonalcoholic(NAFLD)and alcoholrelated(ALD),are common chronic inflammatory conditions of the liver.These diseases,often resulting from multiple metabolic disorders,can progress through a sequence of inflammation,fibrosis,and ultimately,cancer.In this review,we focused on the activation and regulatory mechanism of HSCs in the context of FLD.We summarized the molecular pathways of activated HSCs(aHSCs)in mediating FLD and their role in promoting liver tumor development from the perspectives of cell proliferation,invasion,metastasis,angiogenesis,immunosuppression,and chemo-resistance.We aimed to offer an in-depth discussion on the reciprocal regulatory interactions between FLD and HSC activation,providing new insights for researchers in this field.展开更多
BACKGROUND Colorectal polyps are commonly observed in patients with chronic liver disease(CLD)and pose a significant clinical concern because of their potential for malignancy.AIM To explore the clinical characteristi...BACKGROUND Colorectal polyps are commonly observed in patients with chronic liver disease(CLD)and pose a significant clinical concern because of their potential for malignancy.AIM To explore the clinical characteristics of colorectal polyps in patients with CLD,a nomogram was established to predict the presence of adenomatous polyps(AP).METHODS Patients with CLD who underwent colonoscopy at Tianjin Second People’s Hospital from January 2020 to May 2023 were evaluated.Clinical data including laboratory results,colonoscopy findings,and pathology reports were collected.Key variables for the nomogram were identified through least absolute shrinkage and selection operator regression,followed by multivariate logistic regression.The performance of the model was evaluated using the area under the receiver area under curve,as well as calibration curves and decision curve analysis.RESULTS The study enrolled 870 participants who underwent colonoscopy,and the detection rate of AP in patients with CLD was 28.6%.Compared to individuals without polyps,six risk factors were identified as predictors for AP occurrence:Age,male sex,body mass index,alcohol consumption,overlapping metabolic dysfunction-associated steatotic liver disease,and serum ferritin levels.The novel nomogram(AP model)demonstrated an area under curve of 0.801(95%confidence interval:0.756-0.845)and 0.785(95%confidence interval:0.712-0.858)in the training and validation groups.Calibration curves indicated good agreement among predicted and actual probabilities(training:χ^(2)=11.860,P=0.157;validation:χ^(2)=7.055,P=0.530).The decision curve analysis underscored the clinical utility of the nomogram for predicting the risk of AP.CONCLUSION The AP model showed reasonable accuracy and provided a clinical foundation for predicting the occurrence of AP in patients with CLD,which has a certain predictive value.展开更多
Hepatitis B virus(HBV)reactivation is a clinically significant challenge in disease management.This review explores the immunological mechanisms underlying HBV reactivation,emphasizing disease progression and manageme...Hepatitis B virus(HBV)reactivation is a clinically significant challenge in disease management.This review explores the immunological mechanisms underlying HBV reactivation,emphasizing disease progression and management.It delves into host immune responses and reactivation’s delicate balance,spanning innate and adaptive immunity.Viral factors’disruption of this balance,as are interac-tions between viral antigens,immune cells,cytokine networks,and immune checkpoint pathways,are examined.Notably,the roles of T cells,natural killer cells,and antigen-presenting cells are discussed,highlighting their influence on disease progression.HBV reactivation’s impact on disease severity,hepatic flares,liver fibrosis progression,and hepatocellular carcinoma is detailed.Management strategies,including anti-viral and immunomodulatory approaches,are critically analyzed.The role of prophylactic anti-viral therapy during immunosuppressive treatments is explored alongside novel immunotherapeutic interventions to restore immune control and prevent reactivation.In conclusion,this compre-hensive review furnishes a holistic view of the immunological mechanisms that propel HBV reactivation.With a dedicated focus on understanding its implic-ations for disease progression and the prospects of efficient management stra-tegies,this article contributes significantly to the knowledge base.The more profound insights into the intricate interactions between viral elements and the immune system will inform evidence-based approaches,ultimately enhancing disease management and elevating patient outcomes.The dynamic landscape of management strategies is critically scrutinized,spanning anti-viral and immunomodulatory approaches.The role of prophylactic anti-viral therapy in preventing reactivation during immunosuppressive treatments and the potential of innovative immunotherapeutic interventions to restore immune control and proactively deter reactivation.展开更多
BACKGROUND Although hepatitis B virus infection is the leading cause of chronic liver injury globally,nonalcoholic fatty liver disease(NAFLD)is gradually gaining attention as another major chronic liver disease.The nu...BACKGROUND Although hepatitis B virus infection is the leading cause of chronic liver injury globally,nonalcoholic fatty liver disease(NAFLD)is gradually gaining attention as another major chronic liver disease.The number of patients having chronic hepatitis B(CHB)with concomitant hepatic steatosis has increased.AIM To analyze the effect of NAFLD on the response to antiviral treatment in patients with CHB.METHODS Relevant English studies were systematically searched across PubMed,EMBASE,Web of Science,and Cochrane Library until October 2023.Studies in which the treatment outcomes were compared between patients with CHB only and those with CHB and hepatic steatosis were included.RESULTS Of the 2502 retrieved studies,11 articles were finally included.Biochemical response until 48 wk(OR=0.87,95%CI:0.50–1.53,P=0.000)and 96 wk(OR=0.35,95%CI:0.24–0.53,P=0.24)and virological response until 96 wk(OR=0.80,95%CI:0.43–1.49,P=0.097)were lower in patients with hepatic steatosis than in patients with CHB alone.CONCLUSION Hepatic steatosis lowers the biochemical response to antiviral treatment in patients with CHB.展开更多
Objective:To investigate the impact of metabolic dysfunction-associated steatotic liver disease(MASLD)on the efficacy of immune checkpoint inhibitor(ICI)-based therapy in patients with chronic hepatitis B(CHB)-related...Objective:To investigate the impact of metabolic dysfunction-associated steatotic liver disease(MASLD)on the efficacy of immune checkpoint inhibitor(ICI)-based therapy in patients with chronic hepatitis B(CHB)-related hepatocellular carcinoma(HCC).Methods:A total of 155 patients with CHB-related HCC who received ICI–based therapy(in the Department of Hepatology,Tianjin Second People’s Hospital and Department of Hepatobiliary Oncology,Tianjin Medical University Cancer Institute&Hospital)between April 2021 and December 2023 were evaluated.Patients were divided into two groups:MASLD concurrent with CHB[MASLD-CHB](n=38),and CHB(n=117).Results:The median progression-free survival(PFS,6.9 months vs.9.3 months;P=0.001),progressive disease(57.89%vs.37.61%;P=0.028),and disease control rate(42.11%vs.62.39%;P=0.028)in the MASLD-CHB group were significantly worse than the CHB group.The median overall survival was not attained.The percentage of CD4+PD1+(17.56%vs.8.89%;P<0.001)and CD8+PD1+T cells(10.50%vs.7.42%;P=0.005)in patient samples from the MASLD-CHB group were significantly higher than the CHB group.Concurrent MASLD[hazard ratio(HR)=1.921;95%CI,1.138–3.245;P=0.015]and alpha-fetoprotein levels after 3 months of treatment(HR=2.412;95%CI,1.360–4.279;P=0.003)were independent risk factors for PFS in all patients.Conclusions:ICI-based therapy in patients with CHB-related HCC and concurrent MASLD resulted in poorer efficacy and shorter PFS compared to patients with CHB-related HCC alone.展开更多
BACKGROUND The prevalence of nonalcoholic fatty liver disease(NAFLD)in patients with chronic hepatitis B(CHB)has increased in recent clinical practice;however,the relationship between CHB and hepatic steatosis(HS)rema...BACKGROUND The prevalence of nonalcoholic fatty liver disease(NAFLD)in patients with chronic hepatitis B(CHB)has increased in recent clinical practice;however,the relationship between CHB and hepatic steatosis(HS)remains controversial.AIM To shed light on the potential association between NAFLD and hepatitis B virus(HBV)infection.METHODS We conducted a systematic literature search using multiple databases,including PubMed,the Cochrane Library,Web of Science,and EMBASE,to identify relevant studies.Predefined inclusion criteria were used to determine the eligibility of the studies for further analysis.RESULTS Comprehensive meta-analysis software was used for statistical analysis,which covered 20 studies.The results indicated a lower NAFLD susceptibility in HBVinfected individuals(pooled OR=0.87;95%CI=0.69-1.08;I2=91.1%),with diabetes(P=0.015),body mass index(BMI;P=0.010),and possibly age(P=0.061)as heterogeneity sources.Of note,in four studies(6197 HBV patients),HBV-infected individuals had a reduced NAFLD risk(OR=0.68,95%CI=0.51-0.89,P=0.006).A positive link between hyperlipidemia and metabolic syndrome emerged in hepatitis B patients,along with specific biochemical indicators,including BMI,creatinine,uric acid,fasting blood glucose,and homeostasis model assessment of insulin resistance.CONCLUSION HBV infection may provide protection against HS;however,the occurrence of HS in patients with HBV infection is associated with metabolic syndrome and specific biochemical parameters.展开更多
BACKGROUND A new nomenclature of metabolic associated steatotic liver disease(MASLD)was proposed in 2023,thus expanding the diagnostic name of“MASLD combined with other etiologies”.AIM To investigate the clinical pr...BACKGROUND A new nomenclature of metabolic associated steatotic liver disease(MASLD)was proposed in 2023,thus expanding the diagnostic name of“MASLD combined with other etiologies”.AIM To investigate the clinical profiles of patients with concurrent MASLD and ch-ronic hepatitis B virus(HBV)infection.METHODS This study included participants from the Taiwan Bio-bank.The diagnostic cri-teria of MASLD encompassed hepatic steatosis and any cardio-metabolic risk factors.Positive hepatitis B surface antigen was considered indicative of chronic HBV infection.Dual etiology was defined as MASLD combined with chronic HBV infection(MASLD-HBV).Fibrosis 4(FIB-4)score determined the severity of liver fibrosis,and athero-sclerosis was diagnosed by the presence of carotid plaques on duplex ultrasound.RESULTS In a total of 18980 participants(mean age,55.18±10.35 years;males,30.42%),there were 7654(40.3%)MASLD patients and 2128(11.2%)HBV carriers.After propensity score matching for age and gender,HBV carriers had a lower percentage of MASLD than healthy controls.Those with dual etiology had higher aspartate aminotrans-ferase,alanine aminotransferase(ALT),and FIB-4 levels,but lower gamma glutamyl transferase(GGT)levels than MASLD patients.In contrast,those with dual etiology had higher ALT and GGT levels,but lower FIB-4 than“HBV alone”patients.The risk of atherosclerosis was similar among these three groups.CONCLUSION MASLD-HBV patients have worse liver fibrosis severity than MASLD patients,but better liver fibrosis stage than“HBV alone”patients,suggesting a complex interaction between MASLD and chronic HBV infection.展开更多
BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune...BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.展开更多
BACKGROUND The effect of nonalcoholic fatty liver disease(NAFLD)on the efficacy of nucleoside analogues(NAs)in antiviral therapy for patients with chronic hepatitis B(CHB)remains controversial.AIM To investigate the i...BACKGROUND The effect of nonalcoholic fatty liver disease(NAFLD)on the efficacy of nucleoside analogues(NAs)in antiviral therapy for patients with chronic hepatitis B(CHB)remains controversial.AIM To investigate the influence of NAFLD on virological response in CHB patients undergoing NAs treatment.METHODS Logistic regression analysis was conducted on a cohort of 465 CHB patients from two hospitals to determine whether NAFLD was a risk factor for adverse reactions to NAs.CHB patients were followed up for more than 28 months after initial antiviral treatment,and further validation was performed using different viral load populations.RESULTS NAFLD was identified as an independent risk factor for partial virological response following antiviral therapy with NAs(odds ratio=1.777,P=0.017).In our subsequent analysis focusing on CHB patients with high viral load,the NAFLD group exhibited significantly longer virus shedding time and lower proportion of the complete virological response compared with the non-NAFLD group(16.8±6.1 vs 13.0±6.8,P<0.05).During the 24-month period of antiviral treatment with NAs,hepatitis B virus(HBV)DNA levels decreased slowly in the NAFLD group,and the negative conversion rate of HBV was notably lower than that observed in non-NAFLD group(P=0.001).Similar results were obtained when analyzing patients with low baseline HBV viral load within the NAFLD group.CONCLUSION Coexistence of NAFLD may diminish virological response among CHB patients receiving antiviral treatment with NAs.展开更多
BACKGROUND Cirrhosis is a chronic late stage liver disease associated with hepatitis viruses,alcoholism, and metabolic disorders, such as Wilson disease(WD). There are no clear markers or clinical features that define...BACKGROUND Cirrhosis is a chronic late stage liver disease associated with hepatitis viruses,alcoholism, and metabolic disorders, such as Wilson disease(WD). There are no clear markers or clinical features that define cirrhosis originating from these disparate origins. We hypothesized that cirrhosis is not one disease and cirrhosis of different etiology may have differential clinical hepatic features.AIM To delineate the liver features between WD-associated cirrhosis and hepatitis Bassociated cirrhosis in the Chinese population.METHODS In this observational study, we reviewed the medical data of consecutive inpatients who had WD-associated cirrhosis or hepatitis B-associated cirrhosis from January 2010 to August 2018, and excluded patients who had carcinoma,severe heart or pulmonary diseases, or other liver diseases. According to the etiology of cirrhosis, patients were divided into two groups: WD-associated cirrhosis group(60 patients) and hepatitis B-associated cirrhosis group(56 patients). The liver fibrosis degree, liver function indices, and portal hypertension features of these patients were compared between the two groups.RESULTS No inter-group differences were observed in the diagnostic liver fibrosis markers,however, clinical features clearly defined the origin of cirrhosis. WD-associated cirrhosis patients(16-29 years) had lower levels of alanine transaminase,aspartate transaminase, and bilirubin, lower prothrombin time, lower incidence of hepatic encephalopathy, and lower portal vein diameter(P < 0.05), compared to cirrhosis resulting from hepatitis B in older patients(45-62 years). Importantly,they had decreased risks of progression from Child-Pugh grade A to B(odds ratio = 0.046, 95% confidence interval: 0.006-0.387, P = 0.005) and of ascites(odds ratio = 0.08, 95% confidence interval: 0.01-0.48, P = 0.005). Conversely, WDassociated cirrhosis patients had a higher risk of splenomegaly(odds ratio = 4.15,95% confidence interval: 1.38-12.45, P = 0.011).CONCLUSION WD-associated cirrhosis presents a higher risk of splenomegaly associated with leukopenia and thrombocytopenia, although revealing milder liver dysfunction and portal hypertension symptoms, which recommends WD patients to be monitored for associated complications.展开更多
Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far ...Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far is an 8-mo-old Japanese boy with low ceruloplasmin levels, and the youngest child with elevated aminotransferase ever reported so far is a 9-mo-old Korean boy with confirmed by genetic testing. Here we report an 8-mo-old Chinese boy presented with elevated liver enzymes, and low serum ceruloplasmin level. Genetic analysis of ATP7 B gene detected two heterozygous disease causing mutations(c.2621C>T/p.A874 V and c.3809A>G/p.N1270S), and parental origins were determined. Persistent elevation of serum aminotransferase in this infant was normalized after zinc therapy. To our best knowledge, this is the youngest patient with elevated liver enzymes ever reported worldwide. We hope that this will raise awareness among pediatricians, leading to earlier diagnosis, timely treatment, and better clinical outcome.展开更多
Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiolo...Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiological, epidemiological and therapeutic aspects. Studies examining the most relevant information concerning the prevalence, etiological factors, diagnostic and therapeutic aspects involved in HO were identified by a systematic literature search of the PubMed database. HO generically defines overall alterations in bone mineral density (BMD) (osteoporosis or osteopenia) which appear as a possible complication of chronic liver disease. The origin of HO is multifactorial and its etiology and severity vary in accordance with the underlying liver disease. Its exact prevalence is unknown, but different studies estimate that it could affect from 20% to 50% of patients. The reported mean prevalence of osteoporosis ranges from 13%-60% in chronic cholestasis to 20% in chronic viral hepatitis and 55% in viral cirrhosis. Alcoholic liver disease is not always related to osteo-penia. HO has been commonly studied in chronic cholestatic disease (primary biliary cirrhosis and primary sclerosing cholangitis). Several risk factors and pathogenic mechanisms have been associated with the loss of BMD in patients with chronic liver disease. However, little information has been discovered in relationship to most of these mechanisms. Screening for osteopenia and osteoporosis is recommended in advanced chronic liver disease. There is a lack of randomized studies assessing specific management for HO.展开更多
In this article, we have reviewed the hepatic perfusion disorder (HPD), etiopathogenesis of HPD and corresponding diseases. Review of the literature was based on computer searches (PubMed, Index Medicus) and perso...In this article, we have reviewed the hepatic perfusion disorder (HPD), etiopathogenesis of HPD and corresponding diseases. Review of the literature was based on computer searches (PubMed, Index Medicus) and personal experiences. We considered HPD reflects perfusion differences due to redistribution of arterial blood flow among segments, subsegments and lobes of the liver. The plain CT scan findings of HPD manifests as triangular or wedge-shaped areas of low attenuation. On contrast-enhanced CT scan, HPD manifests multiple (or single) transient wedge-shaped, rotundloid or irregular appearance, homogeneous hyperattenuation (in less cases, hypoattenuation) during the hepatic arterial phase (HAP) and isoattenuated or slightly hyperattenuated areas during the portal arterial phase. Dynamic enhanced magnetic resonance (MR) features are similar to enhanced CT scan. Angiographic findings include non-opacification of portal vein on portograms or wedge-shaped segmental staining in arterial and parenchymal phases on hepatic angiograms. The causes of HPD are arterioportal shunts (APS), intrahepatic vascular compressions and portal vein occlusion, steal phenomenon by hypervascular tumors, vascular variations and any other unknown reasons. It is very important for radiologists to be familiar with the various appearances of HPD to avoid false-positive diagnosis of pseudolesions and not to overestimate the extent of the disease.展开更多
文摘Gynura root has been used extensively in Chinese folk medicine and plays a role in promoting microcirculation and relieving pain.However,its hepatic toxicity should not be neglected.Recently,we admitted a 62-year old female who developed hepatic veno-occlusive disease(HVOD)after ingestion of Gynura root.Only a few articles on HVOD induced by Gynura root have been reported in the literature.It is suspected that pyrrolizidine alkaloids in Gynura root might be responsible for HVOD.In this paper,we report a case of HVOD and review the literature.
文摘Hepatic veno-occlusive disease (HVOD) is rarely encountered and easily misjudged as Budd-Chiari syndrome. It is often related to stem cell transplantation in recent years. We report a case of HVOD that is related to ingestion of some palatable local dishes. The diagnosis was confirmed by liver biopsy pathology with specific observation of inflammatory changes and fibrosis of venules intima, dilated sinusoids and central veins. Chronic diarrhea is unique for this case as a result of ingesting harmful stuffs. This case demonstrated that supervision and instruction of food recipe and traditional medicine are crucial, and prompt diagnosis, supportive care and specific treatment are essential to decreasing the morbidity and mortality of HVOD.
文摘BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury and bleeding in rural areas in China. This study was undertaken to better understand VOD and its relations to the effect of Gymura segetum. METHODS: Between 2000 and 2002,two patients were admitted to our department because of VOD. Before admission, both of them had been injured and taken oral decoction of patent drug Gymura segetum. We analyzed the clinical manifestations, diagnosis and therapy of the two patients. RESULTS: Pyrrolizidine in Panax notginseng was proved to induce VOD. The diagnosis of VOD depended on hepatic biopsy. CONCLUSION: Gymura segetum can induce VOD. More attention should be paid to its unsuscepted side effects.
文摘Nowadays hepatic veno-occlusive disease is mainly caused by Sedum aizoon in China,and its prognosis dependents on the dosage and courses of the Sedum aizoon treatment but lacks other objective indicators.There are a lot relationships between CA125 level and liver cirrhosis,this case had a obvious increased CA125 level in the serum,hydrothorax and ascites,following by the liver cirrhosis in a short time,and then died of upper gastrointestinal bleeding.By now we guess that CA125 level could forecast the liver cirrhosis following by hepatic veno-occlusive,which will become the prognosis of the hepatic veno-occlusive.
文摘BACKGROUND:Hepatic veno-occlusive disease(HVOD)is a severe complication of chemotherapy before hematopoietic stem cell transplantation and dietary ingestion of pyrrolizidine alkaloids.Many experimental models were established to study its mechanisms or therapy,but few are ideal.This work aimed at evaluating a rat model of HVOD induced by monocrotaline to help advance research into this disease. METHODS:Thirty-two male rats were randomly classified into 5 groups,and PBS or monocrotaline was administered (100 mg/kg or 160 mg/kg).They were sacrificed on day 7(groups A,B and D)or day 10(groups C and E).Blood samples were collected to determine liver enzyme concentrations.The weight of the liver and body and the amount of ascites were measured.Histopathological changes of liver tissue on light microscopy were assessed by a modified Deleve scoring system.The positivity of proliferating cell nuclear antigen(PCNA)was estimated. RESULTS:The rats that were treated with 160 mg/kg monocrotaline presented with severe clinical symptoms (including two deaths)and the histopathological picture of HVOD.On the other hand,the rats that were fed with 100 mg/kg monocrotaline had milder and reversible manifestations.Comparison of the rats sacrificed on day 10 with those sacrificed on day 7 showed that the positivity of PCNA increased,especially that of hepatocytes.CONCLUSIONS:Monocrotaline induces acute,dose- dependent HVOD in rats.The model is potentially reversible with a low dose,but reliable and irreversible with a higher dose.The modified scoring system seems to be more accurate than the traditional one in reflecting the histopathology of HVOD.The enhancement of PCNA positivity may be associated with hepatic tissue undergoing recovery.
文摘Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is commonly given to transplant recipients from the start of conditioning through the early weeks of transplant. However, high quality evidence from randomized controlled trials is scarce with small sample sizes and the trials yielded conflicting results. Although various treatment options for hepatic VOD are available, most have not undergone stringent evaluation with randomized controlled trial and therefore it remains uncertain which treatment offers real benefit. It remains controversial whether VOD prophylaxis should be given, which prophylactic therapy should be given, who should receive prophylaxis, and what treatment should be offered once VOD is established.
基金supported by the Foundation of Key Discipline Construction of Zhejiang Province for Traditional Chinese Medicine(Grant No. 2017-XK-A36)the Foundation of Key Research Project of Zhejiang Province for Traditional Chinese Medicine (Grant No. 2019ZZ014)。
文摘Objective: Hepatic veno-occlusive disease(HVOD) has attracted increasing attention in recent years due to its relationship with ingestion of Gynura segetum. The mortality of severe HVOD remains high due to the lack of specific therapies. The aim of the study was to delineate the clinical characteristics and outcomes and explore the potential prognostic factors of HVOD.Methods: This was a single-center retrospective study. Eighty-nine HVOD patients were screened from the First Affiliated Hospital of Zhejiang University with an ingestion history of G. segetum before developing symptoms from January 2009 to May 2018. The enrolled patients were divided into the survivor and death groups according to the clinical follow-up that ended on September 1, 2019. The demographic variables and clinical data of the patients were recorded. A binary logistic regression analysis and receiver operating characteristic curve were conducted to identify the prognostic factors and assess the prognostic value for predicting death, and a survival analysis was performed to evaluate the clinical outcomes.Results: Sixty-four patients were eligible for further analysis. Most patients showed abdominal distension and were positive for migrating dullness in the abdomen(P = 0.740 and P = 0.732, respectively). The patients who died had higher levels of model for end-stage liver disease score, and higher prothrombin time than those who survived(both P < 0.001). All HVOD patients in both the survival and death groups showed ascites with abnormal imaging presentations of the liver parenchyma and hepatic blood vessels.Unexpectedly, we found that hydrothorax was detected in 21(65.63%) patients in the death group and 19(59.38%) patients in the survivor group during hospitalization, which was rarely mentioned in previous studies. Furthermore, international normalized ratio(INR) and creatinine are found to be potential independent prognostic factors for predicting death. Six severe patients achieved clinical improvements and survived after liver transplantation.Conclusion: HVOD can be induced by the ingestion of G. segetum, and INR combined with creatinine has prognostic value for predicting death. Liver transplantation may be an effective treatment option for severe HVOD patients.
基金supported by grants from the Ministry of Science and Technology of the People’s Republic of China(grant number:2020YFA0803300)the National Natural Science Foundation of China(grant numbers:32270783,32100949,32300642)Figures were prepared using Figdraw。
文摘Hepatic stellate cells(HSCs),a distinct category of non-parenchymal cells in the liver,are critical for liver homeostasis.In healthy livers,HSCs remain non-proliferative and quiescent.However,under conditions of acute or chronic liver damage,HSCs are activated and participate in the progression and regulation of liver diseases such as liver fibrosis,cirrhosis,and liver cancer.Fatty liver diseases(FLD),including nonalcoholic(NAFLD)and alcoholrelated(ALD),are common chronic inflammatory conditions of the liver.These diseases,often resulting from multiple metabolic disorders,can progress through a sequence of inflammation,fibrosis,and ultimately,cancer.In this review,we focused on the activation and regulatory mechanism of HSCs in the context of FLD.We summarized the molecular pathways of activated HSCs(aHSCs)in mediating FLD and their role in promoting liver tumor development from the perspectives of cell proliferation,invasion,metastasis,angiogenesis,immunosuppression,and chemo-resistance.We aimed to offer an in-depth discussion on the reciprocal regulatory interactions between FLD and HSC activation,providing new insights for researchers in this field.
基金Supported by the National Natural Science Foundation of China,No.62375202Natural Science Foundation of Tianjin,No.23JCYBJC00950+1 种基金Tianjin Health Science and Technology Project Key Discipline Special,No.TJWJ2022XK034Research Project in Key Areas of Traditional Chinese Medicine in 2024,No.2024022.
文摘BACKGROUND Colorectal polyps are commonly observed in patients with chronic liver disease(CLD)and pose a significant clinical concern because of their potential for malignancy.AIM To explore the clinical characteristics of colorectal polyps in patients with CLD,a nomogram was established to predict the presence of adenomatous polyps(AP).METHODS Patients with CLD who underwent colonoscopy at Tianjin Second People’s Hospital from January 2020 to May 2023 were evaluated.Clinical data including laboratory results,colonoscopy findings,and pathology reports were collected.Key variables for the nomogram were identified through least absolute shrinkage and selection operator regression,followed by multivariate logistic regression.The performance of the model was evaluated using the area under the receiver area under curve,as well as calibration curves and decision curve analysis.RESULTS The study enrolled 870 participants who underwent colonoscopy,and the detection rate of AP in patients with CLD was 28.6%.Compared to individuals without polyps,six risk factors were identified as predictors for AP occurrence:Age,male sex,body mass index,alcohol consumption,overlapping metabolic dysfunction-associated steatotic liver disease,and serum ferritin levels.The novel nomogram(AP model)demonstrated an area under curve of 0.801(95%confidence interval:0.756-0.845)and 0.785(95%confidence interval:0.712-0.858)in the training and validation groups.Calibration curves indicated good agreement among predicted and actual probabilities(training:χ^(2)=11.860,P=0.157;validation:χ^(2)=7.055,P=0.530).The decision curve analysis underscored the clinical utility of the nomogram for predicting the risk of AP.CONCLUSION The AP model showed reasonable accuracy and provided a clinical foundation for predicting the occurrence of AP in patients with CLD,which has a certain predictive value.
文摘Hepatitis B virus(HBV)reactivation is a clinically significant challenge in disease management.This review explores the immunological mechanisms underlying HBV reactivation,emphasizing disease progression and management.It delves into host immune responses and reactivation’s delicate balance,spanning innate and adaptive immunity.Viral factors’disruption of this balance,as are interac-tions between viral antigens,immune cells,cytokine networks,and immune checkpoint pathways,are examined.Notably,the roles of T cells,natural killer cells,and antigen-presenting cells are discussed,highlighting their influence on disease progression.HBV reactivation’s impact on disease severity,hepatic flares,liver fibrosis progression,and hepatocellular carcinoma is detailed.Management strategies,including anti-viral and immunomodulatory approaches,are critically analyzed.The role of prophylactic anti-viral therapy during immunosuppressive treatments is explored alongside novel immunotherapeutic interventions to restore immune control and prevent reactivation.In conclusion,this compre-hensive review furnishes a holistic view of the immunological mechanisms that propel HBV reactivation.With a dedicated focus on understanding its implic-ations for disease progression and the prospects of efficient management stra-tegies,this article contributes significantly to the knowledge base.The more profound insights into the intricate interactions between viral elements and the immune system will inform evidence-based approaches,ultimately enhancing disease management and elevating patient outcomes.The dynamic landscape of management strategies is critically scrutinized,spanning anti-viral and immunomodulatory approaches.The role of prophylactic anti-viral therapy in preventing reactivation during immunosuppressive treatments and the potential of innovative immunotherapeutic interventions to restore immune control and proactively deter reactivation.
文摘BACKGROUND Although hepatitis B virus infection is the leading cause of chronic liver injury globally,nonalcoholic fatty liver disease(NAFLD)is gradually gaining attention as another major chronic liver disease.The number of patients having chronic hepatitis B(CHB)with concomitant hepatic steatosis has increased.AIM To analyze the effect of NAFLD on the response to antiviral treatment in patients with CHB.METHODS Relevant English studies were systematically searched across PubMed,EMBASE,Web of Science,and Cochrane Library until October 2023.Studies in which the treatment outcomes were compared between patients with CHB only and those with CHB and hepatic steatosis were included.RESULTS Of the 2502 retrieved studies,11 articles were finally included.Biochemical response until 48 wk(OR=0.87,95%CI:0.50–1.53,P=0.000)and 96 wk(OR=0.35,95%CI:0.24–0.53,P=0.24)and virological response until 96 wk(OR=0.80,95%CI:0.43–1.49,P=0.097)were lower in patients with hepatic steatosis than in patients with CHB alone.CONCLUSION Hepatic steatosis lowers the biochemical response to antiviral treatment in patients with CHB.
基金supported by the National Natural Science Foundation of China (Grant No. 62375202)Natural Science Foundation of Tianjin (Grant No. 23JCYBJC00950)+2 种基金Tianjin Health Science and Technology Project Key Discipline Special (Grant No. TJWJ2022XK034)Tianjin Key Medical Discipline (Specialty) Construction Project (Grant No.TJYXZDXK-059B)Research Project in Key Areas of TCM in 2024 (Grant No. 2024022)
文摘Objective:To investigate the impact of metabolic dysfunction-associated steatotic liver disease(MASLD)on the efficacy of immune checkpoint inhibitor(ICI)-based therapy in patients with chronic hepatitis B(CHB)-related hepatocellular carcinoma(HCC).Methods:A total of 155 patients with CHB-related HCC who received ICI–based therapy(in the Department of Hepatology,Tianjin Second People’s Hospital and Department of Hepatobiliary Oncology,Tianjin Medical University Cancer Institute&Hospital)between April 2021 and December 2023 were evaluated.Patients were divided into two groups:MASLD concurrent with CHB[MASLD-CHB](n=38),and CHB(n=117).Results:The median progression-free survival(PFS,6.9 months vs.9.3 months;P=0.001),progressive disease(57.89%vs.37.61%;P=0.028),and disease control rate(42.11%vs.62.39%;P=0.028)in the MASLD-CHB group were significantly worse than the CHB group.The median overall survival was not attained.The percentage of CD4+PD1+(17.56%vs.8.89%;P<0.001)and CD8+PD1+T cells(10.50%vs.7.42%;P=0.005)in patient samples from the MASLD-CHB group were significantly higher than the CHB group.Concurrent MASLD[hazard ratio(HR)=1.921;95%CI,1.138–3.245;P=0.015]and alpha-fetoprotein levels after 3 months of treatment(HR=2.412;95%CI,1.360–4.279;P=0.003)were independent risk factors for PFS in all patients.Conclusions:ICI-based therapy in patients with CHB-related HCC and concurrent MASLD resulted in poorer efficacy and shorter PFS compared to patients with CHB-related HCC alone.
基金Supported by Program of Zhejiang Provincial TCM Sci-tech Plan,No.2024ZL039.
文摘BACKGROUND The prevalence of nonalcoholic fatty liver disease(NAFLD)in patients with chronic hepatitis B(CHB)has increased in recent clinical practice;however,the relationship between CHB and hepatic steatosis(HS)remains controversial.AIM To shed light on the potential association between NAFLD and hepatitis B virus(HBV)infection.METHODS We conducted a systematic literature search using multiple databases,including PubMed,the Cochrane Library,Web of Science,and EMBASE,to identify relevant studies.Predefined inclusion criteria were used to determine the eligibility of the studies for further analysis.RESULTS Comprehensive meta-analysis software was used for statistical analysis,which covered 20 studies.The results indicated a lower NAFLD susceptibility in HBVinfected individuals(pooled OR=0.87;95%CI=0.69-1.08;I2=91.1%),with diabetes(P=0.015),body mass index(BMI;P=0.010),and possibly age(P=0.061)as heterogeneity sources.Of note,in four studies(6197 HBV patients),HBV-infected individuals had a reduced NAFLD risk(OR=0.68,95%CI=0.51-0.89,P=0.006).A positive link between hyperlipidemia and metabolic syndrome emerged in hepatitis B patients,along with specific biochemical indicators,including BMI,creatinine,uric acid,fasting blood glucose,and homeostasis model assessment of insulin resistance.CONCLUSION HBV infection may provide protection against HS;however,the occurrence of HS in patients with HBV infection is associated with metabolic syndrome and specific biochemical parameters.
基金Supported by Taipei Tzu Chi Hospital,Buddhist Tzu Chi Medical Foundation,No.TCRD-TPE-112-11.
文摘BACKGROUND A new nomenclature of metabolic associated steatotic liver disease(MASLD)was proposed in 2023,thus expanding the diagnostic name of“MASLD combined with other etiologies”.AIM To investigate the clinical profiles of patients with concurrent MASLD and ch-ronic hepatitis B virus(HBV)infection.METHODS This study included participants from the Taiwan Bio-bank.The diagnostic cri-teria of MASLD encompassed hepatic steatosis and any cardio-metabolic risk factors.Positive hepatitis B surface antigen was considered indicative of chronic HBV infection.Dual etiology was defined as MASLD combined with chronic HBV infection(MASLD-HBV).Fibrosis 4(FIB-4)score determined the severity of liver fibrosis,and athero-sclerosis was diagnosed by the presence of carotid plaques on duplex ultrasound.RESULTS In a total of 18980 participants(mean age,55.18±10.35 years;males,30.42%),there were 7654(40.3%)MASLD patients and 2128(11.2%)HBV carriers.After propensity score matching for age and gender,HBV carriers had a lower percentage of MASLD than healthy controls.Those with dual etiology had higher aspartate aminotrans-ferase,alanine aminotransferase(ALT),and FIB-4 levels,but lower gamma glutamyl transferase(GGT)levels than MASLD patients.In contrast,those with dual etiology had higher ALT and GGT levels,but lower FIB-4 than“HBV alone”patients.The risk of atherosclerosis was similar among these three groups.CONCLUSION MASLD-HBV patients have worse liver fibrosis severity than MASLD patients,but better liver fibrosis stage than“HBV alone”patients,suggesting a complex interaction between MASLD and chronic HBV infection.
基金Supported by National Natural Science Foundation of China,No.82060123National Health Commission of Guizhou Province,No.gzwjk2019-1-082.
文摘BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.
基金Supported by National Science and Technology Major Project of China,No.92169121National Key R and D Projects,No.2022YFC2305100Wuhan Science and Technology Bureau Knowledge Innovation Special Foundation of Hubei Province,No.2022020801010588.
文摘BACKGROUND The effect of nonalcoholic fatty liver disease(NAFLD)on the efficacy of nucleoside analogues(NAs)in antiviral therapy for patients with chronic hepatitis B(CHB)remains controversial.AIM To investigate the influence of NAFLD on virological response in CHB patients undergoing NAs treatment.METHODS Logistic regression analysis was conducted on a cohort of 465 CHB patients from two hospitals to determine whether NAFLD was a risk factor for adverse reactions to NAs.CHB patients were followed up for more than 28 months after initial antiviral treatment,and further validation was performed using different viral load populations.RESULTS NAFLD was identified as an independent risk factor for partial virological response following antiviral therapy with NAs(odds ratio=1.777,P=0.017).In our subsequent analysis focusing on CHB patients with high viral load,the NAFLD group exhibited significantly longer virus shedding time and lower proportion of the complete virological response compared with the non-NAFLD group(16.8±6.1 vs 13.0±6.8,P<0.05).During the 24-month period of antiviral treatment with NAs,hepatitis B virus(HBV)DNA levels decreased slowly in the NAFLD group,and the negative conversion rate of HBV was notably lower than that observed in non-NAFLD group(P=0.001).Similar results were obtained when analyzing patients with low baseline HBV viral load within the NAFLD group.CONCLUSION Coexistence of NAFLD may diminish virological response among CHB patients receiving antiviral treatment with NAs.
基金Supported by the Science and Technology Planning Project of Guangdong Province,No.2015A030302085 and No.2016A020212022
文摘BACKGROUND Cirrhosis is a chronic late stage liver disease associated with hepatitis viruses,alcoholism, and metabolic disorders, such as Wilson disease(WD). There are no clear markers or clinical features that define cirrhosis originating from these disparate origins. We hypothesized that cirrhosis is not one disease and cirrhosis of different etiology may have differential clinical hepatic features.AIM To delineate the liver features between WD-associated cirrhosis and hepatitis Bassociated cirrhosis in the Chinese population.METHODS In this observational study, we reviewed the medical data of consecutive inpatients who had WD-associated cirrhosis or hepatitis B-associated cirrhosis from January 2010 to August 2018, and excluded patients who had carcinoma,severe heart or pulmonary diseases, or other liver diseases. According to the etiology of cirrhosis, patients were divided into two groups: WD-associated cirrhosis group(60 patients) and hepatitis B-associated cirrhosis group(56 patients). The liver fibrosis degree, liver function indices, and portal hypertension features of these patients were compared between the two groups.RESULTS No inter-group differences were observed in the diagnostic liver fibrosis markers,however, clinical features clearly defined the origin of cirrhosis. WD-associated cirrhosis patients(16-29 years) had lower levels of alanine transaminase,aspartate transaminase, and bilirubin, lower prothrombin time, lower incidence of hepatic encephalopathy, and lower portal vein diameter(P < 0.05), compared to cirrhosis resulting from hepatitis B in older patients(45-62 years). Importantly,they had decreased risks of progression from Child-Pugh grade A to B(odds ratio = 0.046, 95% confidence interval: 0.006-0.387, P = 0.005) and of ascites(odds ratio = 0.08, 95% confidence interval: 0.01-0.48, P = 0.005). Conversely, WDassociated cirrhosis patients had a higher risk of splenomegaly(odds ratio = 4.15,95% confidence interval: 1.38-12.45, P = 0.011).CONCLUSION WD-associated cirrhosis presents a higher risk of splenomegaly associated with leukopenia and thrombocytopenia, although revealing milder liver dysfunction and portal hypertension symptoms, which recommends WD patients to be monitored for associated complications.
基金Supported by National Natural Science Foundation of China,No.81070281
文摘Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far is an 8-mo-old Japanese boy with low ceruloplasmin levels, and the youngest child with elevated aminotransferase ever reported so far is a 9-mo-old Korean boy with confirmed by genetic testing. Here we report an 8-mo-old Chinese boy presented with elevated liver enzymes, and low serum ceruloplasmin level. Genetic analysis of ATP7 B gene detected two heterozygous disease causing mutations(c.2621C>T/p.A874 V and c.3809A>G/p.N1270S), and parental origins were determined. Persistent elevation of serum aminotransferase in this infant was normalized after zinc therapy. To our best knowledge, this is the youngest patient with elevated liver enzymes ever reported worldwide. We hope that this will raise awareness among pediatricians, leading to earlier diagnosis, timely treatment, and better clinical outcome.
文摘Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiological, epidemiological and therapeutic aspects. Studies examining the most relevant information concerning the prevalence, etiological factors, diagnostic and therapeutic aspects involved in HO were identified by a systematic literature search of the PubMed database. HO generically defines overall alterations in bone mineral density (BMD) (osteoporosis or osteopenia) which appear as a possible complication of chronic liver disease. The origin of HO is multifactorial and its etiology and severity vary in accordance with the underlying liver disease. Its exact prevalence is unknown, but different studies estimate that it could affect from 20% to 50% of patients. The reported mean prevalence of osteoporosis ranges from 13%-60% in chronic cholestasis to 20% in chronic viral hepatitis and 55% in viral cirrhosis. Alcoholic liver disease is not always related to osteo-penia. HO has been commonly studied in chronic cholestatic disease (primary biliary cirrhosis and primary sclerosing cholangitis). Several risk factors and pathogenic mechanisms have been associated with the loss of BMD in patients with chronic liver disease. However, little information has been discovered in relationship to most of these mechanisms. Screening for osteopenia and osteoporosis is recommended in advanced chronic liver disease. There is a lack of randomized studies assessing specific management for HO.
文摘In this article, we have reviewed the hepatic perfusion disorder (HPD), etiopathogenesis of HPD and corresponding diseases. Review of the literature was based on computer searches (PubMed, Index Medicus) and personal experiences. We considered HPD reflects perfusion differences due to redistribution of arterial blood flow among segments, subsegments and lobes of the liver. The plain CT scan findings of HPD manifests as triangular or wedge-shaped areas of low attenuation. On contrast-enhanced CT scan, HPD manifests multiple (or single) transient wedge-shaped, rotundloid or irregular appearance, homogeneous hyperattenuation (in less cases, hypoattenuation) during the hepatic arterial phase (HAP) and isoattenuated or slightly hyperattenuated areas during the portal arterial phase. Dynamic enhanced magnetic resonance (MR) features are similar to enhanced CT scan. Angiographic findings include non-opacification of portal vein on portograms or wedge-shaped segmental staining in arterial and parenchymal phases on hepatic angiograms. The causes of HPD are arterioportal shunts (APS), intrahepatic vascular compressions and portal vein occlusion, steal phenomenon by hypervascular tumors, vascular variations and any other unknown reasons. It is very important for radiologists to be familiar with the various appearances of HPD to avoid false-positive diagnosis of pseudolesions and not to overestimate the extent of the disease.