Variceal bleeding results in significant morbidity and mortality in both children and adults. The guidelines for the management of variceal bleeding are well established in adults but not in children as there have been insufficient pediatric studies of this disorder. In addition, the adult guidelines for treatment of variceal bleeding cannot be applied directly to children as the etiology and n...

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conc...

Newborns have limited reserve supplies of vitamin A. Infants with chronic cholestasis are in a precarious nutritional state because of their limited ability to build these stores even though the vitamin is present in their diet. In this study, we investigated liver concentrations of vitamin A in 30 children with extrahepatic biliary atresia. We demonstrate that correction of the deficiency occu...

The objective of this study was to find any association of extrahepatic biliary atresia (EHBA) with a possible infectious etiology. Infants diagnosed to have EHBA were tested by blood PCR for cytomegalovirus (CMV), Ebstein-Barr virus, HBsAg, anti-HCV and IgM antibodies for CMV, toxoplasma, rubella, and herpes infections. Thirty-three infants of EHBA were included in the study, male 22, median a...

AMA Nagi SA, Zakaria H, Elkhadry SW, Hamed W, Gaballa N, Elkholy S. APRI and FIB-4 indices as diagnostic noninvasive scores for prediction of severe fibrosis in patients with biliary atresia. Clinical Experimental Hepatology - Manuscripts Accepted. 2023. doi:10.5114/ceh.2023.130699. APA Nagi, A., Zakaria, H., Elkhadry, W., Hamed, Gaballa, N., & Elkholy, (2023). https://doi.org/10.5114/ceh.2023....

Neonatal cholestatic disorders are a group of hepatobiliary diseases occurring within the first 3 months of life. Bile flow is impaired, and patients have conjugated hyperbilirubinemia, acholic stools, and hepatomegaly. Overall, 1 in 2,500 live births is affected with a neonatal cholestatic disorder (1). The two most common causes of neonatal cholestasis are biliary atresia and idiopathic neona...

One hundred and three infants with prolonged cholestasis beginning before 3 months were classified as having alpha-1-antitrypsin deficiency (17 patients), scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70 patients). Twenty-two gradually developed chronic liver disease and the remaining 81 recovered within a few months. Prognosis was found to be poor for infants with alph...

Biliary Atresia (BA) is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus (SI) has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation ha...

Galactosemia is an autosomal recessive disorder caused by deficient or absent activities of one of the three enzymes involved in the galactose metabolic pathway. The predominant form is classic type galactosemia caused by severe reduction or absence of the galactose1-phosphate uridyl transferase (GALT) enzyme. Coexistence of extrahepatic biliary atresia (EHBA) with Duarte 1 and 2 variants of ga...

nt matter & 2005 2005.12.004 thor. Tel.: +44 174 . [email protected] Summary Prolonged jaundice in premature infants (born at o37 weeks gestation) is a frequent clinical problem. The delay in physiological adaptation following premature birth, together with the promotion of breast milk feeds among neonatal units, increases the age at which nonpathological jaundice subsides compared with infants ...