Current pathogenetic aspects of hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy

Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor,intellectual and cognitive abnormalities with emotional/affective and behavioral disturbances.This article focuses on the underlying mechanisms of the condition and the differences between hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy.Hepatic encephalopathy is a serious condition that can cause neurological death with brain edema and intracranial hypertension.It is assumed that approximately 60%-80% of patients with liver cirrhosis develop hepatic encephalopathy This review explores the complex mechanisms that lead to hepatic encephalopathy.However,noncirrhotic hyperammonemic encephalopathy is not associated with hepatic diseases and has a completely different etiology.Noncirrhotic hyperammonemic encephalopathy is a severe occurrence that is connected with multiple pathogeneses.

Systemic lupus erythematosus complicated by noncirrhotic portal hypertension: A case report and review of literature

A 48 year-old Chinese woman suffering from polyarthritis,irregular fever and trichomadesis was admitted to the hospital.A diagnosis of systemic lupus erythematosus(SLE)was made based on polyarthritis,pancytopenia,reduced complement 3,multiple positive autoantibodies,a positive Coomb’s test and protein in her urine.In addition,splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging,indicating that the patient had SLE and portal hypertension.Further negative investigations ruled out the possibility of cirrhosis.The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension(NCPH)without liver histopathology,due to the patient’s refusal for liver biopsy.Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone,hydroxychloroquine and metoprolol tartrate.To date,SLE complicated by NCPH has rarely been reported,as it is under-recognized clinically as well as pathologically.Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics,which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.

Noncirrhotic portal hypertension due to peripheral T-cell lymphoma,not otherwise specified:A case report

BACKGROUND Peripheral T-cell lymphoma(PTCL),an aggressive and rare disease that belongs to a heterogeneous group of mature T-cell lymphomas,develops rapidly and has a poor prognosis.Early detection and treatment are essential to improve patient cure and survival rates.Here,we report a rare case of PTCL with clinical presentation of noncirrhotic portal hypertension,which provides a basis for early vigilance of lymphomas in the future.CASE SUMMARY A 65-year-old Chinese woman was admitted to our hospital because of abdominal distension for 3 months and pitting oedema of both lower limbs for 2 months.Physical examinations and associated auxiliary examinations showed the presence of hepatosplenomegaly,and her hepatic venous pressure gradient was 10 mmHg.Immunohistochemical analysis of the liver biopsy confirmed the diagnosis of PTCL.The patient underwent combination therapy with dexamethasone,VP-16,and chidamide.Unfortunately,after 41 days of chemotherapy,the patient died of multiple organ failure.CONCLUSION PCTL accompanied by noncirrhotic portal hypertension is rarely reported.This case report discusses the diagnosis of a patient according to the literature.

Efficacy and Safety of All-oral,12-week Ravidasvir Plus Ritonavir-boosted Danoprevir and Ribavirin in Treatment-naive Noncirrhotic HCV Genotype 1 Patients:Results from a Phase 2/3 Clinical Trial in China

Background and Aims:Ravidasvir(RDV)is a new generation pangenotypic hepatitis C virus(HCV)NS5A inhibitor,with high barrier to baseline resistance-associated species.This is the first phase 2/3 study conducted in China's Mainland confirming the efficacy and safety of RDV+ritonavir-boosted danoprevir+ribavirin for 12 weeks in treatment-naive noncirrhotic patients with genotype 1 infection in a large population.Methods:In this multicenter,randomized,doubleblinded,placebo-controlled phase 2/3 trial(NCT03362814),we enrolled 424 treatment-nafve,noncirrhotic adult HCV genotype 1 patients.All patients were randomized at 3∶1 ratio to receive a combination of RDV 200mg once daily plus ritonavir-boosted danoprevir 100mg/100mg twice daily and oral ribavirin 1000/1200mg/day(body weight<75/≥75 kg)(n=318)or placebo(n=106)for 12 weeks.The primary end-point was the rate of sustained virologic response 12 weeks after the end of treatment,and the safety was evaluated and compared between treatment and placebo groups.Results:The overall rate of sustained virological response at 12 weeks after treatment is 99%(306/309,95%,CI:97%-100%)under per protocol set analysis.All patients harboring baseline NS5A resistance-associated species in the treatment group(76/76,per protocol set)achieved sustained virological response at 12 weeks after treatment.No treatment-related serious adverse events were reported.Laboratory abnormalities showed mild or moderate severity(grade 1 and grade 2)in liver function tests.Conclusions:In treatment-na(i)ve,noncirrhotic HCV Chinese patients infected with HCV genotype 1,all-oral regimen of RDV+ritonavir-boosted danoprevir+ribavirin for 12 weeks was highly efficacious,safe,and well tolerated.

Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.