INTRODUCTIONThis study aimed to quantify and investigate factors affecting the health-related quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers.

MATERIALS AND METHODSA cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.

RESULTSChildren with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).

CONCLUSIONHRQoL in children with BA living with their native livers is comparable to healthy children.

Adolescent ; Age Factors ; Biliary Atresia ; complications ; physiopathology ; psychology ; surgery ; Case-Control Studies ; Child ; Child, Preschool ; Chronic Disease ; Cross-Sectional Studies ; Female ; Health Status ; Humans ; Hypertension, Portal ; etiology ; physiopathology ; psychology ; Liver Diseases ; physiopathology ; psychology ; Liver Transplantation ; Malaysia ; Male ; Quality of Life ; Serum Albumin

BACKGROUND/AIMS: Hepatic damage during transarterial chemoembolization (TACE) is a critical complication in patients with hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC). Apart from its role in preventing HBV reactivation, there is some evidence for the benefits of preemptive antiviral therapy in TACE. This study evaluated the effect of preemptive antiviral therapy on acute hepatic deterioration following TACE. METHODS: This retrospective observational study included a prospectively collected cohort of 108 patients with HBV-related HCC who underwent TACE between January 2007 and January 2013. Acute hepatic deterioration following TACE was evaluated. Treatment-related hepatic decompensation was defined as newly developed encephalopathy, ascites, variceal bleeding, elevation of the bilirubin level, prolongation of prothrombin time, or elevation of the Child-Pugh score by ≥2 within 2 weeks following TACE. Univariate and multivariate analyses were conducted to identify factors influencing treatment-related decompensation. Preemptive antiviral therapy involves directing prophylaxis only toward high-risk chronic hepatitis B patients in an attempt to prevent the progression of liver disease. We regarded at least 6 months as a significant duration of preemptive antiviral treatment before diagnosis of HCC. RESULTS: Of the 108 patients, 30 (27.8%) patients received preemptive antiviral therapy. Treatment-related decompensation was observed in 25 (23.1%) patients during the follow-up period. Treatment-related decompensation following TACE was observed more frequently in the nonpreemptive group than in the preemptive group (29.5% vs. 6.7%, P=0.008). In the multivariate analysis, higher serum total bilirubin (Hazard ratio [HR] =3.425, P=0.013), hypoalbuminemia (HR=3.990, P=0.015), and absence of antiviral therapy (HR=7.597, P=0.006) were significantly associated with treatment-related hepatic decompensation. CONCLUSIONS: Our findings suggest that preemptive antiviral therapy significantly reduces the risk of acute hepatic deterioration. Preventing hepatic deterioration during TACE by applying such a preemptive approach may facilitate the continuation of anticancer therapy and thus improve long-term outcomes.
Aged ; Antiviral Agents/*therapeutic use ; Bilirubin/blood ; Carcinoma, Hepatocellular/*therapy ; Chemoembolization, Therapeutic/*adverse effects ; Female ; Gastrointestinal Hemorrhage/etiology ; Guanine/*analogs & derivatives/therapeutic use ; Hepatitis B/complications/*drug therapy ; Humans ; Hypoalbuminemia/etiology ; Incidence ; Liver/physiopathology ; Liver Diseases/epidemiology/*etiology ; Liver Neoplasms/*therapy ; Male ; Middle Aged ; Proportional Hazards Models ; Retrospective Studies ; Risk Factors ; Treatment Outcome

Portal hypertension is a severe consequence of chronic liver diseases and is responsible for the main clinical complications of liver cirrhosis. Hepatic venous pressure gradient (HVPG) measurement is the best available method to evaluate the presence and severity of portal hypertension. Clinically significant portal hypertension is defined as an increase in HVPG to >10 mmHg. In this condition, the complications of portal hypertension might begin to appear. HVPG measurement is increasingly used in the clinical fields, and the HVPG is a robust surrogate marker in many clinical applications such as diagnosis, risk stratification, identification of patients with hepatocellular carcinoma who are candidates for liver resection, monitoring of the efficacy of medical treatment, and assessment of progression of portal hypertension. Patients who had a reduction in HVPG of > or =20% or to < or =12 mmHg in response to drug therapy are defined as responders. Responders have a markedly decreased risk of bleeding/rebleeding, ascites, and spontaneous bacterial peritonitis, which results in improved survival. This review provides clinical use of HVPG measurement in the field of liver disease.
Chronic Disease ; Hemodynamics ; Hemorrhage/etiology ; Hepatic Veins/physiology ; Humans ; Hypertension, Portal/complications ; Liver Cirrhosis/diagnosis ; Liver Diseases/complications/*physiopathology ; Portal Pressure

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and potentially fatal syndrome that results from inappropriate activation of lymphocytes and macrophages. It is characterized by fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and pathologic findings of hemo- phagocytosis in the bone marrow or other tissues. We report an adult HLH case admitted to hepatology department.
Humans ; Liver Diseases ; complications ; physiopathology ; Liver Function Tests ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; etiology ; Male ; Middle Aged ; Prednisone ; therapeutic use

Malaria is one of the most widespread infectious diseases of tropical countries with an estimated 207 million cases globally. In India, there are endemic pockets of this disease, including Aligarh. Hundreds of Plasmodium falciparum and P. vivax cases with severe pathological conditions are recorded every year in this district. The aim of this study is to find out changes in liver enzymes and kidney markers. Specific diagnosis for P. falciparum and P. vivax was made by microscopic examination of Giemsa stained slides. Clinical symptoms were observed in both of these infections. Liver enzymes, such as AST, ALT, and ALP, and kidney function markers, such as creatinine and urea, were estimated by standard biochemical techniques. In Aligarh district, P. vivax, P. falciparum, and mixed infections were 64%, 34%, and 2%, respectively. In case of P. falciparum infection, the incidences of anemia, splenomegaly, renal failure, jaundice, and neurological sequelae were higher compared to those in P. vivax infection. Recrudescence and relapse rates were 18% and 20% in P. falciparum and P. vivax infections, respectively. Liver dysfunctions and renal failures were more common in P. falciparum patients, particularly in elderly patients. Artesunate derivatives must, therefore, be introduced for the treatment of P. falciparum as they resist to chloroquine as well as sulfadoxine-pyrimethamine combinations.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Clinical Laboratory Techniques ; Female ; Humans ; India/epidemiology ; Infant ; Infant, Newborn ; Kidney/physiopathology ; Kidney Diseases/epidemiology/etiology ; Kidney Function Tests ; Liver/physiopathology ; Liver Diseases/epidemiology/etiology ; Liver Function Tests ; Malaria, Falciparum/complications/*epidemiology/*pathology ; Malaria, Vivax/complications/*epidemiology/*pathology ; Male ; Middle Aged ; Prevalence ; Recurrence ; Young Adult

OBJECTIVETo determine the levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) and evaluate their relationships with cardiac structure and function and liver function in patients with cirrhosis.

METHODSFifty patients with cirrhosis underwent two-dimensional Doppler echocardiography. The cirrhotic patients were divided into groups according to Child-Pugh score:Child-Pugh class A, n=15; Child-Pugh class B, n=20; Child-Pugh class C, n=15. Cardiac dimensions and left and right ventricular functions were evaluated. In addition, the plasma NT-proBNP was detected in the 50 cirrhotic patients and 11 healthy controls.

RESULTSThe levels of plasma NT-proBNP was significantly higher in cirrhotic patients than in healthy controls (240.15+/-80.87 pg/mL vs.55.86+/-20.13 pg/mL, P=0.000).The Child-Pugh class A, B and C groups showed no differences for left ventricular diameter, right ventricular diameter, septal thickness, left ventricular wall thickness, E wave, A wave, aortic annulus diameter, and the value of E/A.However, the left atrial diameter was significantly lower in the A group than in the C group (29.83+/-3.76 mm vs.35.08+/-3.68 mm, P=0.015) and in the B group than in the C group (31.78+/-4.05 mm vs.35.08+/-3.68 mm, P=0.000); there was no significant difference between the A and B groups. The plasma NT-proBNP was significantly lower in the A group than the C group (189.20+/-20.25 pg/mL vs.300.13+/-34.96 pg/mL, P=0.000) and in the B group than in the C group (202.34+/-31.20 pg/mL vs.300.13+/-34.96 pg/mL, P=0.000); there was no significant difference between the A and B groups (P=0.302).The NT-proBNP level was positively correlated with the left atrial diameter and the left ventricular wall thickness (r=0.540, P=0.000 andr=0.309, P=0.029 respectively).In addition, the NT-proBNP showed correlation with Child-Turcotte-Pugh score (r=0.454, P=0.001), albumin level (r=-0.376, P=0.007) and total bilirubin level (r=0.283, P=0.047).

CONCLUSIONs Increased levels of plasma NT-proBNP are related to disease severity in patients with cirrhosis.Furthermore, cardiac dysfunction in patients with cirrhosis may be related to increased plasma levels of NT-proBNP.

Heart Diseases ; complications ; Humans ; Liver Cirrhosis ; complications ; physiopathology ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood

OBJECTIVEEndogenous hydrophobic bile acids may be a pathogenetic factor of biliary complications after orthotopic liver transplantation (OLT).This study was designed to investigate the effects of hydrophilic ursodeoxycholic acid (UDCA), when administered early after OLT, on serum liver tests and on the incidence of biliary complications.

METHODSA total of 112 adult patients undergoing OLT were randomly assigned to one of two groups for receipt of UDCA (13 to 15 mg/kg/d for 4 weeks, n=56) or a placebo (n=56). All patients underwent serum liver testing and measurement of serum bile acids during the 4 weeks following OLT.Patients with T-tube underwent measurement of biliary bile acids during the 4 weeks following OLT.Biliary complications, as well as patient and graft survival rates, were analyzed during the follow-up period (mean of 65.6 months).

RESULTSAt post-OLT days 7, 21 and 28, the UDCA-treated patients showed significantly lower levels of alanine aminotransferase, aspartate aminotransferase and gamma glutamyl transpeptidase (all P less than 0.05).In addition, the UDCA-treated patients showed significantly lower incidence of biliary sludge and casts within the first year post-OLT (3.6% vs.14.3%; x2=3.953, P=0.047). However, there were no significant differences for the incidence of other biliary complications at post-OLT years 1, 3 and 5.The graft and patient survival rates were also similar between the two groups.

CONCLUSIONUDCA, when administered early after OLT, improves results from serum liver tests and decreases the incidence of biliary sludge and casts within the first postoperative year.

Alanine Transaminase ; Aspartate Aminotransferases ; Bile ; Bile Acids and Salts ; Biliary Tract Diseases ; drug therapy ; physiopathology ; Humans ; Liver ; physiopathology ; Liver Cirrhosis, Biliary ; Liver Function Tests ; Liver Transplantation ; Postoperative Complications ; physiopathology ; Ursodeoxycholic Acid ; therapeutic use ; gamma-Glutamyltransferase

The lymphatic system is part of the circulatory system and plays a key role in normal vascular function. Its failure plays a crucial role in the development and maintenance of various diseases including liver diseases. Lymphangiogenesis (the growth of lymphatic vessels) and changes in the properties of lymphatic vessels are associated with pathogenesis of tumor metastases, ascites formation, liver fibrosis/cirrhosis and portal hypertension. Despite its significant role in liver diseases and its importance as a potential therapeutic target for those diseases, the lymphatic vascular system of the liver is poorly understood. Therefore, how the lymphatic vascular system in general and lymphangiogenesis in particular are mechanistically related to the pathogenesis and maintenance of liver diseases are largely unknown. This article summarizes: 1) the lymphatic vascular system; 2) its role in liver tumors, liver fibrosis/cirrhosis and portal hypertension; and 3) its role in ascites formation.
Ascites/*etiology ; Humans ; Hypertension, Portal/complications/pathology ; Liver Cirrhosis/complications/pathology ; Liver Diseases/complications/*pathology ; Liver Neoplasms/complications/pathology ; Lymphangiogenesis ; Lymphatic Vessels/metabolism/physiopathology

OBJECTIVETo investigate the effect of enteral nutrition(EN) on liver function and inflammatory response after abdominal operation in patients with liver dysfunction.

METHODSA prospective multicenter study was conducted. Patients requiring EN for at least 5 days after abdominal surgery with at least 1 abnormal liver function index were included. After operations, EN suspensions(TPF-FOS) were administered for 5 days after the return of bowel function with targeted content of 125.52 kJ(30 kcal)·kg(-1)·d(-1) maintained for a minimum of 3 days. Levels of serum pre-albumin, C-reaction protein(CRP), and liver function index were measured and the incidence of systemic inflammatory response syndrome(SIRS) was recorded before operation and 6 days after EN. Occurrence of gastrointestinal discomfort was monitored during the treatment.

RESULTSNo statistically significant difference was found in pre-albumin between preoperative level and post-EN level[(175.94±71.79) mg/L vs.(192.22±91.26) mg/L, P=0.162]. Patients with abnormal level of γ-glutamyl transpeptidase were less after EN compared to the preoperative period(30 vs. 40, P=0.041), as was total bilirubin (3 vs. 9, P=0.034). No significant differences in other indices of liver function were found. Total bilirubin and direct bilirubin decreased after EN support(P=0.000 and P=0.015, respectively). CRP was notably reduced after EN support [(48.74±65.16) mg/L vs.(25.79±23.63) mg/L, P=0.009] and the incidence of SIRS largely declined after EN support(19.0% vs. 10.3%, P=0.059). The incidence of gastrointestinal discomfort was 22.4% on postoperative day 1 and declined to 19.0% on postoperative day 5.

CONCLUSIONFor patients with liver dysfunction, enteral nutrition support with TPF-FOS after abdominal operation can reduce inflammatory response, improve liver function, and maintain serum protein level.

Abdomen ; surgery ; Adult ; Digestive System Surgical Procedures ; Enteral Nutrition ; Female ; Humans ; Inflammation ; therapy ; Liver ; physiopathology ; Liver Diseases ; complications ; physiopathology ; Male ; Middle Aged ; Postoperative Complications ; therapy ; Postoperative Period ; Prospective Studies

BACKGROUND/AIMS: The blunted ventricular systolic and diastolic contractile responses to physical and pharmacological stress in cirrhosis are termed cirrhotic cardiomyopathy (CCM). CCM has been known to involve multiple defects in the beta-adrenergic signaling pathway. The aim of this study was to determine whether cirrhotic patients have blunted cardiac responses to catecholamine stimulation through dobutamine stress echocardiography (DSE). METHODS: Seventy-one cirrhotic patients with normal left ventricular (LV) chamber size and ejection fraction were enrolled. The LV systolic and diastolic functions were evaluated by two-dimensional and Doppler echocardiography at rest and during peak dobutamine infusion (40 microg/kg/min). An abnormal response was defined as a decrease of less than 10% in LV end-diastolic volume, a decrease of less than 20% in end-systolic volume, and an increase of less than 10% in LV ejection fraction (EF) at peak dobutamine infusion, based on previously used criteria. The early/late diastolic flow (E/A) ratio and diastolic parameters were also measured. RESULTS: A blunted LV response to dobutamine was observed in 18 of 71 cirrhotic patients (25.4%). The baseline EF was significantly higher in 18 patients with a blunted DSE response than that of those with a normal DSE response (P<0.05). The baseline and peak E/A ratios, which are common diastolic dysfunction markers, were higher in the cirrhosis group than in the control group (P<0.001). No adverse events associated with DSE were observed. CONCLUSIONS: Blunted cardiac responses to dobutamine stimulation, which are implicated in defects in the beta-adrenergic signaling pathway, might contribute to the pathogenesis of CCM in patients with cirrhosis.
Adrenergic beta-1 Receptor Agonists/*diagnostic use ; Adult ; Aged ; Dobutamine/*diagnostic use ; Echocardiography, Stress ; Female ; Heart Diseases/complications/physiopathology/*ultrasonography ; Humans ; Liver Cirrhosis/*complications/physiopathology ; Male ; Middle Aged ; Receptors, Adrenergic, beta-1/chemistry/metabolism ; Severity of Illness Index ; Ventricular Function, Left/physiology