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

EEG changes in liver glycogenoses: a cross-sectional study

Children with glycogen storage disease [GSD] suffer from hypoglycemia that is life long and needs frequent feedings and nocturnal intragastric infusions of solutions containing glucose or cornstarch, a regimen that is not possible for most of our followed up patients. The objective of this study was the evaluation of permanent EEG changes in children suffering from liver glycogenoses with attacks of hypoglycemia, hypoglycemic convulsions and those with euglycemia. In a cross-sectional study, 25 children suffering from liver GSD [with no current neurological symptoms] and 20 age and sex matched clinically free children [control group] underwent EEG studies. The results showed that 11 [44%] of the studied children were found to have nocturnal convulsions. Abnormal EEG studies were found in 79 children [76%]. One had a left temporal epileptic focus, 1 had increased slow activity, 7 had abnormal background symmetry and 16 [64%] had absent alpha rhythm in alert state EEG recording. The focal findings did not prove to correlate to hypoglycemia but rather to the level of direct bilirubin above 0.3mg% and total bilirubin above 1mg% [P=0.018%]. Colchicine intake and its prolonged use [>36 months] were associated with abnormal discharge [P=0.004 and respectively P=0.008].The absence of alpha rhythm was related to positive consanguinity [P=0.003] and was detected in children with other affected sib [P=0.000]

Conclusion: liver glycogenosis are associated with EEG changes. These changes were found to be attributable to familial, genetic factors and factors affecting the liver capacity to maintain direct bilirubin within a tight normal range of normal population

Abnormal iron metabolism in non alcoholic steatohepatitis in Egyptian children and adolescents

Non-alcoholic steatohepatitis [NASH] is a form of liver disease resembling alcoholic liver disease in a patient who did not consume significant amount of alcohol. Our study is concerned with the relation between NASH and iron level in Serum and liver tissue. Thirty four patients with NASH proved by liver biopsy were the subjects of our study. They were 12 males [35.3%] and 22 females [64.7%] with mean age of 11.6 +/- 6.4 years [range from 8 to 18 years]. The investigations done to all of them included: Fasting and post prandial blood glucose, hepatitis markers, lipid profile, liver function tests, serum iron, total iron binding capacity [TIBC], serum ferritin, prothrombin time and concentration, abdominal ultrasonography, ultrasonography guided liver biopsy and histopathological examination of the specimen. Our results showed elevation of the serum levels of iron total iron binding capacity, serum ferritin and total iron score in 82.4% of our NASH patients. There was no significant difference in the studied iron parameters in different grades of steatosis and in different grades of activity while there was signiticant difference in different stages of fibrosis

Conclusion: In addition to diabetes mellitus lipid abnormalities and obesity, increased iron stores may play an important role in the deveIOpment of NASH and this may be through increased oxidative stress that ultimately leads to fibrosis and iron reduction might be of benefit in the treatment of this disease

Protein C, protein S, antithrombin lll and factor V leiden [Q506] mutation in veno-occlusive disease in Egyptian children

Thickening of the subintimai zone of the central vein and sublobular venules leads to decreased venous outflow, increased resistance and severe hemodynamic changes characteristic of veno-occlusive disease [VOD]. The role of the coagulation pathways in the pathophysiology of VOD is an area of controversy. This work aimed at evaluation of the presence of protein C, protein S, antithrombin III deficiency and the presence of factor V Leiden in children having VOD. This cross-sectional study included 20 children suffering from veno-occlusive disease and 20 age and sex matched clinically free infants as a control group. They underwent protein C, protein S, antithrombin III assays. Molecular study of factor V mutation [mutation Q506] detection was carried out. The results proved that protein C deficiency was present in 14 children [70%], and antithrombin III in 2 children [10%]. Protein 8 deficiency was not encountered in our studied group. Two children were heterozygous for the mutation Q506. The 2 children with heterozygous factor V Leiden mutation had protein C deficiency. The percutaneous liver biopsies revealed central vein obstruction in 11 children [55%], extensive fibrosis in one and cirrhosis in one. The biopsy findings did not correlate with the clinical stage [P=0.47] or duration of disease [P=0.49]. None of these changes was encountered in the control group

Conclusion: We report the presence of protein C and antithrombin III deficiency and heterozygous Factor V Leiden [Q506] mutation in children with VOD. We support that thrombophilia is a host susceptibility factor and not the etiology of VOD

Plasma retinol in cholestatic infants and children: evaluation of therapy

Plasma retinol level was estimated in 20 patients with infantile cholestasis, 10 of them were supplemented with oral vitamin a 50, 000 iu/week. Ten children of matched age and sex were included as control. 70% of untreated cases had plasma retional level < 20 micro g dl[-1], in contrast to only 40% of treated ones. None of the control group had plasma retional level < 20 micro g dl[-1]. The least plasma retional level was observed at 7-9 months and it increased progressively with continuing oral therapy. Cases with neonatal hepatitis showed a significantly lower plasma retinol than those with extrahepatic biliary atresia [EHBA], paucity of intrahepatic bile ducts [PIHBD] and controls. It is realized that vitamin A deficiency is an established problem in infantile cholestasis. Supplementation with vit A is mandatory. Oral dose of 50,000 IU/week, attempated in this study is nearly satisfactory to prevent hypovitaminosis A in this group of children, however, parenteeral administration may be required in severe cholestasis and with non compliance with oral therapy