Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Biliary Atresia”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed1979

Hepatic portocholecystostomy for biliary atresia.

Four infants with biliary atresia had gross obliteration of the common hepatic duct but residual patency of the gallbladder, cystic duct and common bile duct. The patients were treated by hepatic portocholecystostomy utilizing the extant bile ducts for biliary reconstruction. Bile drainage was achieved in all four infants. There was a conspicuous absence of postoperative cholangitis. Subsequent obstruction of the distal ducts in two patients necessitated reoperation and construction of a standard biliointestinal conduit. The other two children are surviving, jaundice-free, 5 1/2 and 5 years after operation with minimal sequelae of biliary atresia. Hepatic portocholecystostomy is a feasible surgical alternative to intestinal reconstruction in patients with biliary atresia in whom the disease is limited to the proximal extrahepatic bile ducts.

Bile Ducts↗
PubMed1977

Studies of the aetiology of neonatal hepatitis and biliary atresia.

Aetiological factors were sought prospectively in 55 babies with extrahepatic biliary atresia, in 105 with neonatal hepatitis, and in 11 with intrahepatic biliary atresia, seen as a result of nearly complete ascertainment of these conditions in the State of Victoria between 1963 and 1974. In neonatal hepatitis infective causes were shown in 22 babies, galactosaemia in 6 and alpha1-antitrypsin deficiency in 8; familial occurrence was noted in 10 further babies and unrelated second diseases were present in 24 of the remaining 59 babies. The only clues to aetiology in extrahepatic biliary atresia were a suspicion of time-space clusters, a deficiency of affected babies born to young primiparous women, and an unexpected number of spontaneous abortions in the histories given by the mothers. Genetic factors appeared to be important in intrahepatic biliary atresia, but are not reported in detail. Hypotheses for the aetiology of neonatal hepatitis and of extrahepatic biliary atresia are presented. Both are considered syndromes with multiple causes. Recurrence risks in sibs are discussed, and are 1 in 7 for neonatal hepatitis of unknown cause, negligible in extrahepatic biliary atresia, and usually 1 in 2 or 1 in 4 in intrahepatic biliary atresia, depending upon the family history.

Abortion, Spontaneous↗
PubMed1977

Biliary atresia in a twin.

The occurrence of biliary atresia in one of a pair of male twins is reported. The current concepts regarding the aetiology of biliary atresia are examined in relation to this case; and neither the concept of a transplacental agent nor that of a genetic trigger mechanism seems probable. It is concluded that, as with many congenital defects, biliary atresia is probably the end-result of different aetiological factors, among which viruses and other agents may cross the placenta to cause it, whether or not there is a genetic predisposition in the individual fetus, or the biliary system may simply fail to develop.

Bile Ducts↗
PubMed1979

Rose bengal sodium I 131 studies in infants with suspected biliary atresia.

Twenty-eight infants suspected of having biliary atresia were studied with radionuclide imaging and quantitation of liver activity clearance using rose bengal sodium I 131. Scanning the abdomen for the presence or absence of gut radioactivity was a sensitive indicator of complete biliary obstruction. Absence of gut radioactivity correctly predicted biliary atresia in all 11 infants proved to have that diagnosis. Biliary atresia was excluded by the presence of gut radioactivity in 13 of 17 infants with other forms of neonatal juandice. When present, gut activity was identifiable within 24 hours after injection. Serial tests improved specificity. Plotting the rate of clearance of liver activity did not aid diagnosis.

Bile Ducts↗
PubMed1975

An attempt to determine surgical indication for biliary atresia by laboratory examination.

To determine the surgical indication of biliary atresia, we analyzed the history, physical findings and laboratory examinations of 90 cases of biliary atresia operated upon at the Second Department of Surgery, Tohoku University Hospital during the past 10 years, from 1963 through 1972 inclusive. A score table from the data of these examinations was made for the determination of surgical indication. According to this score table, all of the patients who showed good excretion of bile took plus score, whereas a half of the patients who showed poor or no excretion of bile took minus score. Although the remaining patients who showed poor excretion of bile took plus score, these cases had some elements for good excretion of bile. From these results, the patients who takes plus score is considered to be a good indication for operation. The score test is of great value for determination of the surgical indication of biliary atresia.

Age Factors↗
PubMed1978

New perspectives on biliary atresia.

An investigation into the aetiology, diagnosis, and treatment of biliary atresia was carried out because the prognosis remains so poor.In an electron microscopical study no viral particles or viral inclusion bodies were seen, nor were any specific ultrastructural features observed. An animal experiment suggested that obstruction within the biliary tract of newborn rabbits could be produced by maternal intravenous injection of the bile acid lithocholic acid.A simple and atraumatic method of diagnosis was developed using(99) (m)Tc-labelled compounds which are excreted into bile. Two compounds, (99m)Tc-pyridoxylidene glutamate ((99m)Tc-PG) and (99m)Tc-dihydrothioctic acid ((99m)Tc-DHT) were first assessed in normal piglets and piglets with complete biliary obstruction. Intestinal imaging correlated with biliary tract patency, and the same correlation was found in jaundiced human adults, in whom the (99m)Tc-PG scan correctly determined biliary patency in 21 out of 24 cases. The (99m)Tc-PG scan compared well with liver biopsy and (131)I-Rose Bengal in the diagnosis of 11 infants with prolonged jaundice.A model of extrahepatic biliary atresia was developed in the newborn piglet so that different methods of bile drainage could be assessed. Priorities in biliary atresia lie in a better understanding of the aetiology and early diagnosis rather than in devising new bile drainage procedures.

Adult↗
PubMed1979

[Lipoprotein X (LP-X) in the differential diagnosis of cholestasis in children, with special reference to biliary atresia].

Lipoprotein-X (LP-X) in the serum of infants with persistent jaundice is indicative of cholestasis. In early infancy biliary atresia and biliary agenesis are the most common cause of cholestasis, whereas neonatal hepatitis is a less frequent cause of cholestasis. The authors introduced and described the qualitative and quantitative methods of LP-X determination for diagnostic purposes. LP-X estimations were carried out in 9 children with persistent jaundice. LP-X was found to be present in 4 infants-in 2 with complete absence of extrahepatic biliary tracts, in 1 with extrahepatic biliary atresia and in 1 with hypoplastic extrahepatic biliary tract. LP-X was also found in a 5 year old boy with mechanical occlusion of bile ducts caused by a malignant tumor ( rhabdomyoblastoma ), and in 3 year old girl with inborn enzymatic liver dysfunction. In this case LP-X concentration was estimated before and after 3 week course of cholestyramine, after which there was a 35% decrease in the LP-X concentration. In a 4 month old child LP-X was not found in spite of the absence of extra and intrahepatic biliary tracts. This finding may be explained by the far advanced hepatic cirrhosis. The authors stress the importance of introducing of LP-X estimation in the differential diagnosis of jaundice in early infancy.

Bile Ducts↗
PubMed1978

The portoenterostomy procedure for biliary atresia: a five year experience.

The portoenterostomy (Kasai) procedure in infants with biliary atresia has dramatically altered the outlook for this heretofore fatal disease. When performed on infants under three months of age, bile drainage can be achieved in a majority of the patients. Since 1972, 37 infants have been treated with this operation at our institution. Diagnostic operative cholangiography and liver biopsy are recommended if the cause of conjugated hyperbilirubinemia is presumed to be obstructive. When biliary atresia is encountered, identification of the atretic ducts with transection high in the porta hepatis is carried out. Thirty-two infants have had the portojejunostomy, while five, in whom the proximal hepatic ducts were atretic but the gall bladder and distal ducts were patent, underwent portocholecystostomy. Examination of the resected fibrous duct tissue revealed a statistically significant correlation between ductal histology and postoperative outcome. Extended bile drainage has been achieved in 26 of 37 patients. Seventeen exhibit near normal growth and development four months to five years postoperatively. Seven have died with progressive liver disease despite bile drainage. Two additional patients died, jaundice free, from unrelated causes. Despite bile drainage, progressive hepatic fibrosis has been confirmed by serial biopsies in 14 patients. This finding indicates that biliary obstruction is not the sole component in the development of biliary cirrhosis. These data suggest that extrahepatic biliary atresia is a dynamic obliterative process, which can be favorably modified in approximately 50% of the infants by early surgical treatment.

Bile Ducts↗
PubMed1976

25-Hydroxycholecalciferol inthe management of rickets associated with extrahepatic biliary atresia.

In children with extrahepatic biliary atresia, impaired hydroxylation and defective intestinal absorption of cholecalciferol may lead to a deficiency of vitamin D and rickets. The data presented herein demonstrate that in such patients serum levels of vitamin D measured as 25-hydroxycalciferol are reduced. A moderate therapeutic oral dose of 25-hydroxycholecalciferol, by circumventing the hepatic conversion of cholecalciferol to 25-hydroxycholecalciferol, will replete vitamin D stores and maintain the serum concentration of 25-hydroxycalciferol required to prevent or heal rickets in these patients.

Administration, Oral↗
PubMed1977

Long-term results in treatment of biliary atresia.

The 10-year follow-up results of operation for biliary atresia from 11 pediatric surgical clinics are evaluated; 638 cases were analyzed. The results for non-correctable biliary atresia before Kasaï's operation were uniformly bad, and even now only a very small percentage of the cases is cured.

Child↗
PubMed1977

Congenital extrahepatic biliary atresia.

Ten cases of congenital extrahepatic biliary atresia were studied ultrastructurally. Samples of liver were obtained from each and in six of the cases, fibrous tracts, which we hoped would contain extrahepatic bile ducts, were also secured. The observed extrahepatic biliary structures were real, but hypoplastic, bile ducts. In places, necrosed epithelial cells, without obvious inflammatory processes, could be observed. The ductular cell cytoplasmic changes and the inflammatory reaction are different according to whether extrahepatic or intrahepatic sites are considered. These differences, as well as the cytoplasmic modifications of liver parenchymal cells, seem to be the result of impaired bile flow. On the contrary, extrahepatic bile duct hypoplasia and necrosis seem to be directly related to the unknown origin of this disease. Whether the nuclear changes of hepatocytes are the expression of direct injury of the liver is another important question. If there is direct injury, it is possible that the disease might evolve as an independent liver disease despite a correctly performed and uncomplicated surgical intervention.

Bile Ducts↗
PubMed1977

Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia.

An 11-year-old girl developed cholangiocellular carcinoma in association with biliary cirrhosis due to congenital biliary atresia. An exploratory laparotomy and an operative cholangiogram at 3 months of age had confirmed the diagnosis of extrahepatic biliary atresia. A liver biopsy specimen taken at 6 months of age showed biliary cirrhosis. The subsequent clinical course was characterized by persistent moderate jaundice, anemia, malnutrition, rickets, pathologic fractures, and recurrent gastrointestinal bleeding. The presence of cholangiocellular carcinoma of the liver with advanced biliary cirrhosis was established at an exploratory laparotomy a week before her death. We discuss here the pathogenesis of biliary cirrhosis and carcinoma of the liver; there may be a relation between the two in the childhood population.

Adenoma, Bile Duct↗
PubMed1979

The surgical management of the unusual forms of biliary atresia.

Of 50 infants undergoing laparotomy for biliary atresia during the last 9 yr at the Kobe Children's Hospital, 9 had variant forms, including (A) a gallbladder with a demonstrable connection with the intrahepatic biliary system, (B) a gallbladder with a connection with the extrahepatic biliary system and duodenum, and (C) a grossly visible subhepatic cyst. Three patients with form A underwent cholecystoduodenostomy. All these patients died of cholangitis and hepatic failure within 2 mo after operation. Two patients with form B were successfully treated by hepatic portocholecystostomy without any episodes of cholangitis. Two of the patients with form C, occurring in 1970, underwent an anastomosis between the subhepatic cyst and the duodenum. There was no bile excretion in these patients. The other two patients were treated subsequently by hepatic portoenterostomy with complete excision of the subhepatic cyst and excellent bile excretion was obtained. Infants with forms A and C should be treated by hepatic portoenterostomy instead of a direct anastomosis between the dilated portion of the extrahepatic biliary system and the alimentary tract. For patients with form B, hepatic portocholecystostomy seems to be the most suitable procedure relative to the prevention of postoperative cholangitis, which is the most serious complications in infants obtaining successful bile excretion.

Bile Ducts↗
PubMed1977

Histopathological studies on the prognosis of biliary atresia.

Liver specimens from 68 cases of biliary atresia were histologically studied in reference to postoperative prognosis. The histological findings were classified into 4 to 5 groups by the degree of fibrosis, bile duct proliferation, bile plugs, giant cell transformation, and intralobular cholestasis. Furthermore, morphometrical studies of interstitial area, bile duct (ductules), intrahepatic portal vein, and intrahepatic artery were done and correlated with the age of patients and with operative results. From this study, it was shown that 1) marked fibrosis (cirrhosis) and interstitial areas occupying more than 35% of the sectional area of the liver specimen, 2) severe ductular proliferation, shown by proliferated bile ducts occupying more than 20% of interstitial areas, 3) no or slight intralobular cholestasis and 4) marked interlobular cholestasis are considered to predict poor postoperative bile excretion.

Age Factors↗
PubMed1975

Differentiation of biliary atresia from neonatal hepatitis by routine clinical examinations.

After analysis of 33 cases of neonatal hepatitis and 90 cases of biliary atresia, a score test from history, physical findings and daily laboratory examinations was studied for the differential diagnosis of these two diseases. The biliary atresia takes plus score, whereas the neonatal hepatitis takes minus score. The score test is of great value for the differential diagnosis between these two diseases.

Alkaline Phosphatase↗
PubMed1977

Serum bile acid patterns in neonatal hepatitis and extrahepatic biliary atresia.

Serum bile acid patterns were determined in 31 infants ranging in age from 4 days to 24 weeks. Fifteen infants with extrahepatic biliary atresia had a mean concentration of serum bile acids of 90 microng/ml +/- 47 SD;; 16 infants with neonatal hepatitis had a mean concentration of 60 +/- 35.5 SD. The chenodeoxycholate/cholate ratio was greater than one in 13 infants (87%) with atresia and in 10 infants (66%) with neonatal hepatitis. Except for the tendency of a higher total concentration of serum bile acids in infants with atresia, a single serum bile acid value does not differentiate neonatal hepatitis from extrahepatic biliary atresia. The high proportion of chenodeoxycholate in extrahepatic atresia is different from the pattern in other types of cholestatic disease and may reflect an underlying hepatitis.

Bile Acids and Salts↗
PubMed1977

A histopathological study of the remnant of extrahepatic bile duct in so-called uncorrectable biliary atresia.

Histopathological study of the remnant of extrahepatic bile ducts in 40 cases of so-called uncorrectable biliary atresia, upon which we operated the last three years, has been performed. The histological findings of the remnant were classified into three types. Only two cases were found to have type 1a ducts in the porta hepatis area, from which we can expect better prognosis postoperatively. We also found that as the patients become older, the size of the duct in the remnant becomes smaller and the hepatic fibrosis becomes more remarkable. Therefore the operation should be performed in the infant with this lesion as young as possible. As for the evaluation of operative results of hepatic portoenterostomy for this lesion, a proper evaluation can be made only in those cases in which a microscopic examination of the remnant of extrahepatic bile duct at the porta hepatis area has been adequately performed. Concerning the pathogenesis of biliary atresia, we presume that congenital abnormalities of bile ducts are a basic factor, and additional nonspecific inflammation and bile stasis complete its pathological condition.

Age Factors↗