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

INTRODUCTIONHepatic venous pressure gradient (HVPG) measurement is recommended for prognostic and therapeutic indications in centres with adequate resources and expertise. Our study aimed to evaluate the quality of HVPG measurements at our centre before and after introduction of a standardised protocol, and the clinical relevance of the HVPG to variceal bleeding in cirrhotics.

METHODSHVPG measurements performed at Singapore General Hospital from 2005-2013 were retrospectively reviewed. Criteria for quality HVPG readings were triplicate readings, absence of negative pressure values and variability of ≤ 2 mmHg. The rate of variceal bleeding was compared in cirrhotics who achieved a HVPG response to pharmacotherapy (reduction of the HVPG to < 12 mmHg or by ≥ 20% of baseline) and those who did not.

RESULTS126 HVPG measurements were performed in 105 patients (mean age 54.7 ± 11.4 years; 55.2% men). 80% had liver cirrhosis and 20% had non-cirrhotic portal hypertension (NCPH). The mean overall HVPG was 13.5 ± 7.2 mmHg, with a significant difference between the cirrhosis and NCPH groups (p < 0.001). The proportion of quality readings significantly improved after the protocol was introduced. HVPG response was achieved in 28 (33.3%, n = 84) cirrhotics. Nine had variceal bleeding over a median follow-up of 29 months. The rate of variceal bleeding was significantly lower in HVPG responders compared to nonresponders (p = 0.025).

CONCLUSIONThe quality of HVPG measurements in our centre improved after the introduction of a standardised protocol. A HVPG response can prognosticate the risk of variceal bleeding in cirrhotics.

Esophageal and Gastric Varices ; complications ; physiopathology ; Female ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; etiology ; physiopathology ; prevention & control ; Humans ; Hypertension, Portal ; complications ; physiopathology ; Liver Cirrhosis ; complications ; physiopathology ; Male ; Middle Aged ; Portal Pressure ; physiology ; Prognosis ; Retrospective Studies

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

Cirrhosis with portal hypertension is a common disease which has a significant impact on the quality of patients' life. Esophagogastric devascularization (EGDV) has been demonstrated to be an effective method to treat portal hypertension, however certain complications are associated with it. The purpose of this study was to evaluate the effectiveness and clinical outcome of the selective EGDV (sEGDV) for the treatment of portal hypertension. The study was conducted prospectively from Jan. 1 2011 to Dec. 31, 2012, and 180 patients were randomized to the sEGDV group (n=90) or the non-sEGDV (n-sEGDV) group (n=90). Patients' demographics, preoperative lab test results and operative details were comparable between the two groups. Postoperative and short-term complications were analyzed in two groups. There was statistically significant difference (P<0.01) in the PVF reduction between the two groups. Post-operative complications showed no statistically significant difference between the two groups in the incidence of bleeding, ascites, acute portal vein thrombosis, fever and hepatic encephalopathy. Mortality between two groups was comparable. The incidence of splenic fossa effusion after the surgery was lower in sEGDV group than in n-sEGDV group. There were no significant differences in the short-term follow-up data such as esophageal varices and portal hypertensive gastropathy (P>0.05). It is suggested that sEGDV is a safe, simple and effective surgical procedure. It has both the advantages of the shunt and devascularization because it preserves body's voluntary diversion. With the advantage of low incidence of postoperative complications, it is an ideal surgical approach for the treatment of portal hypertension.
Adult ; Esophagus ; blood supply ; surgery ; Female ; Gastrointestinal Hemorrhage ; etiology ; pathology ; physiopathology ; Hepatic Encephalopathy ; pathology ; physiopathology ; Humans ; Hypertension, Portal ; pathology ; physiopathology ; surgery ; In Vitro Techniques ; Male ; Middle Aged ; Postoperative Complications ; pathology ; physiopathology ; Prospective Studies ; Stomach ; blood supply ; surgery ; Thrombosis ; etiology ; pathology ; physiopathology

BACKGROUND/AIMS: The relationship between portal hemodynamics and fundal varices has not been well documented. The purpose of this study was to understand the pathophysiology of fundal varices and to investigate bleeding risk factors related to the presence of spontaneous portosystemic shunts, and to examine the hepatic venous pressure gradient (HVPG) between fundal varices and other varices. METHODS: In total, 85 patients with cirrhosis who underwent HVPG and gastroscopic examination between July 2009 and March 2011 were included in this study. The interrelationship between HVPG and the types of varices or the presence of spontaneous portosystemic shunts was studied. RESULTS: There was no significant difference in the HVPG between fundal varices (n=12) and esophageal varices and gastroesophageal varices type 1 (GOV1) groups (n=73) (17.1+/-7.7 mm Hg vs 19.7+/-5.3 mm Hg). Additionally, there was no significant difference in the HVPG between varices with spontaneous portosystemic shunts (n=28) and varices without these shunts (n=57) (18.3+/-5.8 mm Hg vs 17.0+/-8.1 mm Hg). Spontaneous portosystemic shunts increased in fundal varices compared with esophageal varices and GOV1 (8/12 patients [66.7%] vs 20/73 patients [27.4%]; p=0.016). CONCLUSIONS: Fundal varices had a high prevalence of spontaneous portosystemic shunts compared with other varices. However, the portal pressure in fundal varices was not different from the pressure in esophageal varices and GOV1.
Adult ; Aged ; Endoscopy, Gastrointestinal ; Esophageal and Gastric Varices/etiology/*physiopathology ; Esophagus ; Female ; Gastric Fundus ; Gastrointestinal Hemorrhage/etiology/*physiopathology ; Humans ; Hypertension, Portal/complications/*physiopathology ; Liver Cirrhosis/complications/*physiopathology ; Male ; Middle Aged ; *Portal Pressure ; *Renal Veins ; Risk Factors ; *Splenic Vein ; Stomach/*blood supply ; Vascular Fistula/complications/*physiopathology

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

Animals ; Colon ; blood supply ; pathology ; Colonic Diseases ; drug therapy ; etiology ; physiopathology ; Hemodynamics ; Hepatic Veins ; pathology ; physiopathology ; Hypertension, Portal ; complications ; physiopathology ; Intestinal Mucosa ; blood supply ; drug effects ; pathology ; Liver Cirrhosis, Experimental ; complications ; Losartan ; administration & dosage ; therapeutic use ; Male ; Microscopy ; Portal Pressure ; drug effects ; Random Allocation ; Rats ; Rats, Wistar

Hyperdynamic circulation in patients with liver cirrhosis is characterized by increased cardiac output and heart rate, and decreased systemic vascular resistance with low arterial blood pressure and currently focused on understanding the pathogenesis because of possibility of developing novel treatment modality. Basically, these hemodynamic alternations arise from portal hypertension. Portosystemic collaterals develop to counterbalance the increased intrahepatic vascular resistance to portal blood flow and induce an increase in venous return to heart. Increased shear stress in vascular endothelial cell related high blood flow by portosystemic shunting contributes to this up-regulation of eNOS resulting in NO overproduction. Additionally, bypassing through portosystemic collaterals and escaping degradation of over-produced circulating vasodilators in the diseased liver can promote the peripheral arterial vasodilation. Vasodilation of the systemic and splanchnic circulations lead to a reduced systemic vascular resistance, and increased cardiac output and splanchnic blood flow. Furthermore, neurohumoral vasoconstrictive systems including systemic nervous system, rennin angiotensin aldosterone system, and vasopressin are intensively activated secondary to vasodilation. However, hyperdynamic circulation would be more aggravated by the activated vasoconstrictive systems. With the progression of the cirrhotic process, hyperdynamic alternations can be more profound due to hyporesponsiveness to vasoconstrictors and increased shunt formation in conjunction with autonomic neuropathy. Eventually, splanchnic arterial vasodilation results in an increase portal venous inflow, maintaining the elevated portal venous pressure. Hyperdynamic circulation is intimately involved in portal hypertension with liver cirrhosis, therefore it is reasonable to have an interest in complete understanding of the pathogenensis of hyperdynamic circulation to develop novel treatment modality.
Blood Circulation/*physiology ; Blood Flow Velocity/physiology ; Humans ; Hypertension, Portal/etiology/*physiopathology ; Liver/blood supply/physiopathology ; Liver Cirrhosis/drug therapy/etiology/*physiopathology ; Nitric Oxide/metabolism ; Nitric Oxide Synthase Type III/metabolism ; Vasodilation

Adult ; Blood Circulation ; Blood Flow Velocity ; Blood Pressure ; Female ; Heart Rate ; Hemodynamics ; physiology ; Humans ; Hypertension, Portal ; etiology ; physiopathology ; Liver ; blood supply ; physiopathology ; Liver Cirrhosis ; etiology ; physiopathology ; Male ; Middle Aged ; Ventricular Dysfunction ; physiopathology

Adult ; Aged ; Blood Flow Velocity ; Esophageal and Gastric Varices ; complications ; physiopathology ; Female ; Gastrointestinal Hemorrhage ; diagnostic imaging ; etiology ; physiopathology ; Hemodynamics ; Humans ; Hypertension, Portal ; diagnostic imaging ; etiology ; physiopathology ; Liver Cirrhosis ; complications ; Liver Diseases ; complications ; Male ; Middle Aged ; Portal System ; diagnostic imaging ; physiopathology ; Spleen ; diagnostic imaging ; physiopathology ; Ultrasonography, Doppler, Color