1/17. Cholestatic syndromes of infancy and childhood.cholestasis results from structural and functional impairment of the hepatobiliary system, which is often the target of several environmental factors and disease processes. This review focuses on the clinical consequences of this impairment. When evaluating an infant or child with cholestasis, a broad differential diagnosis must be considered; viral infections, metabolic disorders, and toxic insults may often lead to cholestasis. In the infant, cholestasis associated with severe hepatic synthetic dysfunction points to life-threatening metabolic disorders. In this setting, early diagnosis and prompt treatment offer the only chance for survival. Fortunately, cholestasis in infants presents more frequently with initially normal liver synthetic function. In those infants without evidence of infection, evaluation for patency of the extra-hepatic biliary system is a high priority. biliary atresia comprises a significant portion of these patients and requires surgical intervention with portoenterostomy in an attempt to improve biliary flow. In a substantial group of infants and older children in whom the cause for cholestasis is not apparent, typical clinical and biochemical markers may allow the identification of specific genetic defects of syndromes that result from abnormal canalicular transporters.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/17. biliary atresia and the Dandy-Walker anomaly in a neonate with 45,X Turner's syndrome.A case is described of a neonate with Turner's syndrome (45,X) whose clinical course was complicated by jaundice. autopsy findings included intraphepatic biliary atresia, coarctation of the aorta of the infantile type, and the dandy-walker syndrome.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
3/17. Neonatal cholestatic hepatitis from carbamazepine exposure during pregnancy and breast feeding.OBJECTIVE: To report a case of transient cholestatic hepatitis occurring in an infant between the third and seventh weeks of life, most likely due to carbamazepine exposure during pregnancy and breast feeding. CASE SUMMARY: A boy, born to an epileptic mother who had been treated with carbamazepine monotherapy throughout pregnancy and breast feeding, experienced asphyxia at birth with transient hepatic dysfunction in the first week of life. After full recovery from asphyxia, he experienced a second period of liver dysfunction, presenting as cholestatic hepatitis that lasted approximately 5 weeks. Infectious and metabolic etiologies as well as extrahepatic biliary atresia were excluded. DISCUSSION: carbamazepine is known to induce hepatic damage in children and adults. As the drug crosses the placenta and is excreted into breast milk, infants of mothers taking carbamazepine might also develop liver dysfunction. In addition to the present case, there are 2 well-documented case reports of cholestasis in association with transplacental and transmammary carbamazepine exposure. CONCLUSIONS: carbamazepine-induced hepatitis may occur in association with prenatal exposure and breast feeding. This may expose infants to unnecessary diagnostic procedures, and should therefore be mentioned in the company's product information.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
4/17. Congenital atresia of the portal vein and extrahepatic portocaval shunt associated with benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and atrial septal defect.A rare case of congenital atresia of the portal vein and ductus venosus, extrahepatic portocaval shunt, benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and an atrial septal defect is reported. Twenty-two cases of congenital extrahepatic end-to-side shunts have been described before. Although additional anomalies are common in this type of shunt, hemangiomatosis has been described only once. Adrenal hyperplasia has never been reported in this anomaly.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
5/17. Anomalous biliary drainage associated with pancreaticobiliary maljunction and nondilatation of the common bile duct.A case report is presented of an infant with jejunal atresia and hyperbilirubinemia with diagnosed pancreaticobiliary maljunction without choledochal dilatation. Common channel insertion was into the third portion of the duodenum.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
6/17. biliary atresia associated with hypoplastic left heart syndrome: a case report and review of the literature.biliary atresia is a cholestatic disorder of infancy that is associated with other anatomic anomalies in approximately 20% of cases. These frequently are defects in situs determination and laterality, causing syndromes of heterotaxy and complex heart defects. The authors describe an infant with both biliary atresia and hypoplastic left heart syndrome (HLHS) in the absence of a laterality defect or evidence of any other morphologic defect. The presence of a hypoplastic left ventricle in a patient with biliary atresia has previously been reported only in the context of a heterotaxy syndrome. The coexistence of these 2 disorders raises etiologic considerations and represents a potential challenge for the surgical treatment of both conditions.- - - - - - - - - - ranking = 7keywords = atresia (Clic here for more details about this article) |
7/17. Ocular pathologic features of arteriohepatic dysplasia (Alagille's syndrome).Arteriohepatic dysplasia (Alagille's syndrome), an autosomal dominant condition involving jaundice caused by a developmental scarcity of intrahepatic bile ducts, has characteristic cardiovascular, skeletal, facial, and ocular features that distinguish it from extrahepatic biliary atresia and an array of other neonatal intrahepatic cholestatic disorders. Two children who died of this syndrome had prominent Schwalbe's rings with attached iris strands characteristic of Axenfeld's syndrome. Additional histologic findings of iris atrophy and stromal nodules, however, made the designation Axenfeld-Reiger's syndrome more appropriate. Pigmentary retinopathy, degeneration of Bruch's membrane, and prominent lipofuscin deposition in the ciliary muscle noted in one of the patients were not regarded as primary changes of Alagille's syndrome, but were believed to be secondary to acquired deficiency of the fat-soluble vitamins A and E. Early recognition of the ocular changes in arteriohepatic dysplasia is helpful in establishing the proper diagnosis to avoid unnecessary abdominal surgery and institute vitamin therapy.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
8/17. Serial ultrasonic examination to differentiate biliary atresia from neonatal hepatitis--special reference to changes in size of the gallbladder.We performed serial ultrasonic examinations to differentiate biliary atresia from neonatal hepatitis. The subjects studied were 144 children (100 normal neonates and infants, 31 patients with neonatal hepatitis and 13 patients with biliary atresia). They were examined by ultrasound before, during and after feeding. In 97 out of 100 normal children and all patients with neonatal hepatitis, the gallbladder was identified, and the change in size following oral feeding was observed. In four children with severe neonatal hepatitis which could not be differentiated from biliary atresia by clinical and laboratory data, we readily identified the gallbladder and observed the change in the size following oral feeding. In 8 of 13 patients with biliary atresia, we identified a small gallbladder whose size was not affected by oral feeding. In the other patients the gallbladder was not identified before, during or after oral feeding. On the basis of these results, we consider that serial ultrasonic examination with oral feeding aids in a differential diagnosis of biliary atresia and neonatal hepatitis.- - - - - - - - - - ranking = 9keywords = atresia (Clic here for more details about this article) |
9/17. Neonatal gallbladder enlargement and alpha 1-antitrypsin deficiency.patients with clinical signs of alpha 1-antitrypsin deficiency in the neonatal period usually present with prolonged obstructive jaundice. We report a patient with alpha 1-antitrypsin deficiency who presented with gallbladder enlargement in the neonatal period. This gallbladder enlargement may be due to cystic duct hypoplasia or atresia, which has been reported in association with alpha 1-antitrypsin deficiency. The diagnosis of alpha 1-antitrypsin deficiency should be considered in neonates with gallbladder enlargement and prolonged obstructive jaundice.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
10/17. cystic fibrosis mistaken for idiopathic biliary atresia.Previous reports of prolonged jaundice in cystic fibrosis have not described operative and histopathological findings in the liver and biliary tree. In the two cases reported here, obstructive jaundice in the neonatal period was associated with anatomical evidence of intra- or extrahepatic biliary obstruction. Hepatoportoenterostomy, a surgical procedure that is not without complications, was performed on one of the patients for biliary atresia before the diagnosis of cystic fibrosis was suspected. Prolonged obstructive jaundice may be an early manifestation of cystic fibrosis and may resolve without operative management. A sweat test should be performed on all patients with prolonged obstructive neonatal jaundice to rule out cystic fibrosis.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
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