Progression of disease in extra hepatic biliary atresia is associated with reduced total hepatic blood flow: descriptive results of a prospective pilot study

Hepatic fibrosis and cirrhosis develop progressively in extrahepatic biliary atresia despite timely surgical intervention. We aimed to study total hepatic blood flow [hepatic artery and portal vein flow] as a possible predictive factor of outcome of infants having biliary atresia who had underwent Kasai portoenterostomy. Twenty Infants having biliary atresia underwent colored and pulsed Doppler ultrasound studies. They were done before and 2-3 months after portoenterostomy. Hepatic artery, portal vein and single hepatic vein mean flow, mean diameter, mean velocity, hepatic arterial to portal venous flow ratio and total hepatic flow/kg were calculated and correlated to final outcome. The detected mean total hepatic flow and total hepatic flow/kg preoperatively was 685.5 +/- 296 ml/min and 147.1 +/- 51.4 ml/min/kg and post-operatively in those who became anicteric was 854.4 +/- 107 ml/min and 149.5 +/- 37.2 ml/min/kg, 539.2 +/- 337.7 ml/min and 112.1 +/- 78.6 ml/min/kg in those who developed chronic disease and in those who died was 157.6 +/- 79.6 and 30.9 +/- 16.1 ml/min/kg respectively. Unresolving cholestasis in infants having biliary atresia with poor outcome following portoenterostomy is associated with decreased post-operative total hepatic flow. Preoperative total hepatic flow did not correlate with postoperative total hepatic flow

Evaluation of TC sign by 3 dimensional sonography in extrahepatic biliary atresia: a prospective study

The tubular or triangular cord sign [TC sign] is very sensitive and specific in diagnosis of extrahepatic biliary atresia. It is a b and -like periportal echogenicity, which represents a cone shaped fibrotic mass cranial to the bifurcation of portal vein. TC sign anatomic characterization using 3 dimensional sonography in infants with biliary atresia. 20 infants having biliary atresia underwent both 2 dimensional and 3 dimensional ultrasonographic examinations using both 5 and 7 MHz convex linear transducers. Ultrasonographic findings were correlated to intraoperative details. 11 infants with neonatal hepatitis with absent TC sign were evaluated as a control group. The TC sign identified on 2 dimensional sonography was identified by 3 dimensional sonography. 3D ultra-sonography characterized the TC sign as the left hepatic bile duct, which was completely obliterated in 18 infants, [confirmed by intra-operative findings] and partially occluded in 2. It converged to meet the right hepatic duct which was also seen occluded [in all cases] as a fibrotic b and seen through a right modified oblique lateral scan [while using the liver as an acoustic window]. The common hepatic duct was seen occluded in all infants. The common bile duct was seen occluded in all except one which showed non-communicating cystic dilatation [confirmed by intra-operative cholangiogra-phy]. All infants with idiopathic neonatal hepatitis had patent intact biliary systems and recovered in the following 6-10 months. TC sign represents the obliterated left hepatic bile duct. 3Dimensional ultrasonography is superior to 2D sonography in providing better diagnostic imaging of the biliary tract in infants with cholestasis

Elevated levels of interleukin 18 in children with acute and chronic liver disease: further evidence of T cell-mediated liver injury

IL-18, derived from macrophages and kupffer cells, is the central pro-inflammatory cytokine leading to experimental liver failure. The objective of this study was to evaluate IL-18 in sera of infants and children suffering from acute hepatitis, chronic hepatitis and cirrhosis and to define its role as a predictive factor for chronicity of liver disease. Eighteen children suffering from acute viral hepatitis, 26 from chronic hepatitis and 15 suffering from cirrhosis were included in this study. They were attendants of the Hepatology Clinic of the New Children's Hospital, Cairo University. Twenty-three age and sex matched, clinically free infants and children, were also included as a control group. All studied infants and children, and the control group underwent quantitative determination of IL-18 in serum by ELISA. The mean IL-18 was found to be statistically significantly lower among the control group compared to the others. The mean IL-18 values were 38.65 +/- 15.46, 510.27 +/- 757, 305.03 +/- 647 and 257.86 +/- 395 pg/ml for the healthy control, acute hepatitis, chronic hepatitis and cirrhosis groups respectively. IL-18 level was not found to be predictive of pathology or etiology [P=0.067]. No correlation was found between IL-18 level and total bilirubin [P=0.70], direct bilirubin [P=0.79], ALT [P=0.29], AST [P=0.48] or alkaline phosphatase [P=0.222]. Higher levels of IL-18 were encountered in those children having more severe AIH. High IL-18 was present in children having various liver diseases. This supports the view that hepatocytes destruction is in part immune mediated. Immune modulation remains a potential future perspective for liver disease intervention

Pulmonary function testing in children with chronic liver disease

Hepatopulmonary syndrome [HPS] is a rare but severe complication in children with chronic liver disease [CLD] that may necessitate "urgent" liver transplantation. This study aimed at studying the feasibility and usefulness of pulmonary function testing [PFT] in the diagnosis of lung affections in a group of Egyptian children with chronic liver disease [CLD] in order to diagnose HPS in them. Twenty five Egyptian children [aged 10-16 years with a mean of 12.4 +/- 1.8 years] with different causes of CLD and 8 age-matched, healthy controls were subjected to arterial blood gas analysis, PFT and measurement of mean pulmonary artery pressure [MPAP]. Arterial oxygen pressure [Pa0[2]] less than 80 mmHg was present in 10 patients, forced expiratory volume in I second [FEV[1]] less than 80% predicted in 15, forced vital capacity [FVC] less than 80% predicted in 15 and the ratio FEV[1]:FVC less than 65% predicted in 20. Carbon monoxide diffusion capacity [DL[CO]] was less than 80% predicted in 15 patients and 4 controls. Hypoxemia occurred in 60% of patients with decreased DL[co] but none of controls. MPAP was significantly lower in patients than controls, it increased with exercise but not significantly. Neither portal hypertension nor sclerotherapy showed a significant relation to hypoxemia, or low MPAP. Also the duration of CLD, its histological severity and grading of fibrosis were not related to any of the pulmonary function tests. Arterial hypoxemia is present in CLD. PFT was rather "tedious" but non-invasive investigation in children with CLD. It did demonstrate obstructive airway as well as diffusion defects in these children. Therefore we may recommend PFT in the work-up of children with CLD, particularly if liver transplantation is contemplated