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PubMedDate not supplied

Duodenal atresia, biliary atresia, and intestinal infarct in truncal aplasia cutis congenita.

A newborn boy with aplasia cutis congenita had biliary atresia, distal duodenal atresia, and a severe infarct of the intestine resulting in complete absence of the entire midgut. The boy died due to biliary atresia and severe short gut syndrome approximately 3 weeks after birth. While the association of duodenal atresia with aplasia cutis congenita has been described, the findings of biliary atresia and midgut atresia in association with aplasia cutis congenita have not been described previously.

Adult↗
PubMed2000

Combined pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia: prenatal diagnostic features and review of the literature.

The authors recently managed a case of combined pure esophageal, duodenal, biliary, and pancreatic ductal atresia in a fetus and newborn with Trisomy 21. The authors present a case report and review of the literature, emphasizing the prenatal radiographic features of the combined lesions and the high incidence of associated anomalies including Down's syndrome. Prenatal suspicion of these anomalies warrants karyotype analysis as well as careful pre- and postnatal screening for other anomalies.

Abnormalities, Multiple↗
PubMed2001

Etiopathogenesis of biliary atresia.

Biliary atresia, a progressive sclerosis of the extrahepatic biliary tree that occurs only within the first 3 months of life, is one of the most common causes of neonatal cholestasis and accounts for over half of children who undergo liver transplantation. In biliary atresia, a number of prenatal or perinatal insults to the biliary tree appear to culminate in complete obliteration of the lumen of the extrahepatic biliary tree and continued injury and sclerosis of intrahepatic bile ducts, even after portoenterostomy is successful. A minority of cases of biliary atresia may be caused by defects in morphogenesis of the bile ducts. Potential etiologies for the more common perinatal form of biliary atresia include viral infections, immune-mediated bile duct injury, and autoimmune disease involving the bile ducts. Two viruses, reovirus and rotavirus, have received increasing attention as possible inciters of an immune-mediated injury to the biliary tree. Fas ligand upregulation and apoptosis of bile duct epithelia have been demonstrated in human specimens, as well as T-lymphocyte and macrophage activation in portal tracts. An experimental model using rotavirus infection in newborn mice has been useful in characterizing the mechanisms underlying bile duct injury. It is proposed that virally induced neoantigens displayed on biliary epithelium may play a role in initiating the immune processes involved in destruction of the extrahepatic bile duct and ongoing intrahepatic ductal injury in the perinatal form of biliary atresia. The short window of time after birth during which this disease presents suggests that immaturity of the neonatal immune system and genetic susceptibility also may be key factors. Delineation of the mechanisms underlying bile duct injury will be essential to the development of new potential therapies for this important pediatric disorder.

Animals↗
PubMed2006

Cellular and humoral autoimmunity directed at bile duct epithelia in murine biliary atresia.

Biliary atresia is an inflammatory fibrosclerosing lesion of the bile ducts that leads to biliary cirrhosis and is the most frequent indication for liver transplantation in children. The pathogenesis of biliary atresia is not known; one theory is that of a virus-induced, subsequent autoimmune-mediated injury of bile ducts. The aim of this study was to determine whether autoreactive T cells and autoantibodies specific to bile duct epithelia are present in the rotavirus (RRV)- induced murine model of biliary atresia and whether the T cells are sufficient to result in bile duct inflammation. In vitro analyses showed significant increases in IFN-gamma-producing T cells from RRV-diseased mice in response to bile duct epithelial autoantigen. Adoptive transfer of the T cells from RRV-diseased mice into naïve syngeneic SCID recipients resulted in bile duct-specific inflammation. This induction of bile duct pathology occurred in the absence of detectable virus, indicating a definite response to bile duct autoantigens. Furthermore, periductal immunoglobulin deposits and serum antibodies reactive to bile duct epithelial protein were detected in RRV-diseased mice. In conclusion, both cellular and humoral components of autoimmunity exist in murine biliary atresia, and the progressive bile duct injury is due in part to a bile duct epithelia-specific T cell-mediated immune response. The role of cellular and humoral autoimmunity in human biliary atresia and possible interventional strategies therefore should be the focus of future research.

Adoptive Transfer↗
PubMed2003

The pattern of differentially expressed genes in biliary atresia.

Biliary atresia is a progressive obliterative cholangiopathy, but the etiology of this disorder remains uncertain. Identifying genes specifically expressed in biliary atresia and analyzing the pattern of expression may lead to a better understanding of the pathogenesis. Liver tissues were taken from a recipient with biliary atresia and a normal donor during liver transplantation. Total RNA was extracted from each sample and reversely transcribed to cDNA. Then radiolabeled cDNA probe pools were made by random primed DNA labeling method and used for screening of differentially expressed genes by hybridizing with expressed sequence tags (EST) dot blot panel. Northern blot hybridization was done to confirm that these genes are also differentially expressed in other liver tissues. Among 1730 EST clones, 26 cDNA clones were significantly overexpressed in biliary cirrhosis, while 2 clones were significantly decreased in biliary atresia. By Northern blot hybridization, the results of tissue inhibitor of metalloproteinase (TIMP)-1 and IGFBP-2 were well correlated with differential EST screening (DES). This study identified the pattern of differentially expressed genes in the biliary cirrhosis due to biliary atresia using DES technique.

Biliary Atresia↗
PubMed2006

Effect of rotavirus strain on the murine model of biliary atresia.

Biliary atresia is a devastating disorder of the newborn in which afflicted infants develop inflammation and fibrosis of the extrahepatic biliary tract, resulting in cirrhosis and end-stage liver disease. Infection with a virus is thought to be a contributing factor in the etiology of biliary atresia. In the murine model of biliary atresia, perinatal exposure to rhesus rotavirus (RRV) results in biliary epithelial cell infection causing bile duct obstruction. The purpose of this study was to determine if tropism for the biliary epithelial cell was unique to RRV. Newborn mice underwent intraperitoneal injection with five strains of rotavirus: RRV (simian), SA11-FM (simian/bovine), SA11-SM (simian), EDIM (murine), and Wa (human). RRV and SA11-FM caused clinical manifestations of bile duct obstruction and high mortality. SA11-SM caused clinical signs of hepatobiliary injury but the mortality was markedly reduced. EDIM and Wa caused no sign of hepatobiliary disease. The systemic and temporal distribution of viral protein and live virus varied according to the injected strain. Immunohistochemistry revealed that RRV and SA11-FM targeted the biliary epithelial cells. In contrast, SA11-SM was found in the liver but in not in the biliary epithelium. These results indicate that strain-specific characteristics dictate tropism for cells of hepatobiliary origin which in turn impact the ability to induce the murine model of biliary atresia.

Animals↗
PubMed1997

Biliary atresia.

Biliary atresia, a progressive obliterative process involving the bile ducts, has its onset in the newborn period. It is characterized by worsening cholestasis, hepatic fibrosis, and cirrhosis, which lead to portal hypertension and a decline in hepatic synthetic function. Untreated, the outcome is uniformly fatal. The two major milestones toward improved treatment of this disease have been the Kasai portoenterostomy and orthotopic liver transplantation. There has been discussion regarding transplantation as primary therapy, but portoenterostomy remains the standard of care as first-line intervention. Hepatic transplantation, done more frequently for biliary atresia than for any other cause of liver failure in the pediatric population, offers improved survival and quality of life to those for whom the Kasai operation fails. The etiology of biliary atresia remains poorly understood. Working toward a better understanding of this disease, recent investigations target more precise characterization of the hepatic pathology and seek to identify possible causative agents and predictors of favorable outcome. Recent advances in the understanding of biliary atresia published between December 1995 and November 1996 are the focus of this review.

Age Factors↗
PubMedDate not supplied

Screening for biliary atresia.

Biliary atresia is a severe progressive cholangiopathy which leads to early liver cirrhosis and is uniformly fatal. Early surgical intervention (the Kasai procedure) is needed for an improved outcome. However, early recognition and diagnosis is not easy during the neonatal period because of the high incidence of neonatal jaundice, ill-informed and less than urgent appraisal of the clinical manifestations among jaundiced neonates. A mass screening program for biliary atresia using a stool color card was conducted in Taiwan from March 2002 (in 49 hospitals and clinics) to December 2003 (in 95 hospitals and clinics). The stool color card for infants has proved to be a sensitive and specific screening method for biliary atresia in infants younger than two months of age.

Biliary Atresia↗
PubMed2005

Computerized three-dimensional study of a rotavirus model of biliary atresia: comparison with human biliary atresia.

Biliary atresia is a panbiliary disease causing obstructive jaundice in neonates and infants. The clinical spectrum can be broadly categorized into the fetal and perinatal types. A consistent animal model that accurately mimics the whole clinical spectrum of biliary atresia is not yet available. However, rotavirus infection of neonatal mice has been shown to produce atresia in the biliary system. This study investigates the three-dimensional computerized morphology of the murine neonatal model comparing with age-matched control mice. Newborn Balb/c mice were injected intraperitoneally with rhesus rotavirus within 24-48 h after birth. Control mice received 0.9% NaCl. Pups with symptoms of cholestasis were sacrificed from the 5th to the 15th postinjection day, as were age-matched controls. Their hepatobiliary tissues were prepared for three-dimensional computerized image reconstruction. Rotavirus infection caused obliteration of the intrahepatic bile ducts and single to multiple atresias in the extrahepatic bile duct. At 15 days postinjection, intrahepatic ductal proliferation appeared, and the three-dimensional appearances of the intrahepatic biliary structures were similar to the human disease. Cystic duct and gallbladder dilatation was frequently seen in this model, and this feature distinguishes it from the human disease in which the gallbladder is almost always atretic. This rotavirus murine model demonstrates many of the features of human perinatal biliary atresia, and can be used as an investigative tool to further study the pathogenesis of biliary atresia.

Animals↗
PubMed2001

Portal vein phlebolithiasis found post-liver transplantation in the native liver of a child with biliary atresia.

Biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspection of the hilar region of the native liver post-Tx revealed the formation of a pouch in the hepatic duct and a stone in the lumen of the portal vein. X-ray diffraction analysis showed that the stone was composed of cholesteryl cinnamate, gluconic acid phenylhydrazide, Na beta broma-allyl mercaptomethyl penicillinate, and Al2O3 crystals. While the cholesterol component is a known element of gallstones, we attributed the Na beta broma-allyl mercaptomethyl penicillinate to the patient's drug therapy. Our literature search revealed no previous record or crystallographic analysis of portal vein phlebolithiasis. In this report we describe this rare finding.

Biliary Atresia↗
PubMed2004

Isolation and culture of biliary epithelial cells from bile duct remnants of patients with biliary atresia.

Biliary atresia(BA), the most common cause of obstructive jaundice in infancy, has been considered to be a result of progressive destruction of the bile ducts through a necroinflammatory process. Many immunohistochemical studies of BA remnant have been done, but it has not been shown that biliary epithelial cells (BECs) can be cultured from BA remnant. For this study, we obtained bile duct remnants from three patients with biliary atresia (one male, two females) who received Kasai's operation from 2002 to 2003. The successive cultivation rate of BECs from explants was 100% (3/3 patients). Culture of BECs on collagen gel was possible up to at least four passages. Under a phase-contrast microscope, primary and passed cultured cells on collagen gel showed a cobblestone-like spread in 2 weeks. The BECs had immunoreactivity to anti-human cytokeratin 7 antibody. In this study, we proved that BECs in the remnants of BA could be cultured, and defined the maturation of biliary epithelial cells of BA by immunocytochemistry with anti-human cytokeratin 7 antibody. In conclusion, BECs in BA remnant are "alive", and their proliferation activity can be maintained.

Bile Ducts↗
PubMed1996

'Triangular cord': a sonographic finding applicable in the diagnosis of biliary atresia.

Biliary atresia (BA) is characterized by luminal obstruction of the extrahepatic bile duct with fibrous remnants. The authors reviewed ultrasonographic examinations of the fibrous tissue in the bifurcation of the portal vein at the porta hepatis and identified the triangular- or tubular-shaped echogenic density, the so-called "triangular cord" (TC), in the vicinity of the portal vein on a transverse or longitudinal scan. In this prospective study, the authors investigated whether TC was useful in the noninvasive diagnosis of biliary atresia in 18 infants who had persistent neonatal jaundice. This was done by comparing the ultrasonographic examination with the histopathologic examination (HPE) of liver specimens obtained from a needle biopsy. The TC was identified in nine patients, all of whom were confirmed to have BA by HPE. The TC was not observed in the other nine patients, who had neonatal hepatitis (NH). The mean size of the TC was 13 mm (wide) x 6 mm (thick) (width range, 5 to 21 mm; thickness range, 4 to 12 mm). The diagnosis of BA was confirmed at the time of Kasai hepatoportojejunostomy in eight of the nine patients whose TC was noted by ultrasonography (US). The other patient was discharged because his parents refused the operation; he died of liver failure at 15 months of age. The nine patients with absent TC were treated medically for NH. Eight of them improved clinically. The other, diagnosed to have NH by needle and wedge liver biopsies, was reexamined 40 days after the initial examination because of worsening jaundice. A 18 x 12-mm TC was visualized ultrasonographically. Additionally, a percutaneous liver biopsy specimen showed BA with severe portal fibrosis and ductal proliferation. The patient underwent a Kasai hepatoportoenterostomy. On the basis of these results, the authors conclude that TC is a very specific ultrasonographic finding, representing the fibrous cone at the porta hepatis, and is a useful tool in the noninvasive diagnosis of BA. However, early exploration or close US follow-up is recommended for any patient suspected of having BA clinically, even if a liver biopsy confirms the NH.

Biliary Atresia↗
PubMed2006

Biliary atresia.

Biliary atresia (BA) is a rare disease characterised by a biliary obstruction of unknown origin that presents in the neonatal period. It is the most frequent surgical cause of cholestatic jaundice in this age group. BA occurs in approximately 1/18,000 live births in Western Europe. In the world, the reported incidence varies from 5/100,000 to 32/100,000 live births, and is highest in Asia and the Pacific region. Females are affected slightly more often than males. The common histopathological picture is one of inflammatory damage to the intra- and extrahepatic bile ducts with sclerosis and narrowing or even obliteration of the biliary tree. Untreated, this condition leads to cirrhosis and death within the first years of life. BA is not known to be a hereditary condition. No primary medical treatment is relevant for the management of BA. Once BA suspected, surgical intervention (Kasai portoenterostomy) should be performed as soon as possible as operations performed early in life is more likely to be successful. Liver transplantation may be needed later if the Kasai operation fails to restore the biliary flow or if cirrhotic complications occur. At present, approximately 90% of BA patients survive and the majority have normal quality of life.

Biliary Atresia↗
PubMed1996

[Histologic factors as elements of prognostic value in biliary atresia].

Biliary atresia (BA) is one of the biliary tree anomaly more frequent. Occurs in about 0.8 to 1/10.000 live births. BA is defined as a progressive biliary tree. The prognosis depends on the age of the diagnosis and precocity surgery. We present the results of a retrospective analysis of 71 RA carried out at the Garrahan Hospital from 1987 to 1993. 47 were female and 24 were male. Age ranged from 45 to 120 days of life. This study involved a consecutive series of 58 patients with histopathologic study of Porta-hepatis (PH) and liver biopsy obtained during the Kasai. The purpose of this study was to determine the value of histological factors as type of PH and hepatocytic giant cell transformation (GCT). 82.8% had favorable type of PH and the CCT was mild in 84.5%. 72.4% had bad outcome and was independent of the type of PH. Neither of them were statistically significant with survive and evolution. In our service neither PH non CGT were predictors of a bad outcome. There were good outcome in 27.5%, died 37.9% and 10.3% undergo liver transplantation. The precocity in a diagnosis and surgical procedure before two months of age are the most important factors in correlation with survival. Others immunomorphologic factors must be studied in BA that explained the etiopathogenic process. Orthotopic liver transplantation is the successful therapy in children with BA.

Biliary Atresia↗
PubMed1995

An association of multiple intestinal atresia and biliary atresia: a case report.

This is the first report of a 2-day-old girl having an association of biliary atresia and multiple intestinal atresia. She developed intractable vomiting in the first hours of life. An upper gastrointestinal series and barium enema showed jejunal atresia and rectal atresia. A laparotomy was performed on the fourth day of life. The operative findings were multiple sites of atresia of the intestine, 10 atretic sites in the small bowel, and 3 in the colon. The gall bladder was normal in size and position. After the operation, the patient's stool remained acholic and her jaundice did not resolve. On the 58th day of life, another laparotomy was performed with the presumptive diagnosis of biliary atresia. The operative finding was biliary atresia, type 3; the Kasai operation with external jejunostomy was performed. The patient suffered from postoperative cholangitis and enterocolitis because of her short bowel and subsequently died at 9 months of age. This is the first case of biliary atresia associated with multiple intestinal atresia reported in the literature.

Barium Sulfate↗
PubMed1998

Clinical significance of plasma endothelin levels in patients with biliary atresia.

Biliary atresia (BA) is the end-result of a destructive inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tracts with the development of biliary cirrhosis and portal hypertension (PH). Endothelins (ET) are 21-amino-acid peptides of endothelial origin with potent vasoconstrictor activity that bind to various cells of the liver. Nothing is presently known about plasma ET levels in BA. The aim of this study was to determine the clinical significance of plasma ET levels in patients with BA after hepatic portoenterostomy (Kasai's procedure) and to correlate these with liver function tests (LFT) and PH. We measured plasma concentrations of ET in 19 patients with BA (5 boys and 14 girls; mean age 11.6 +/- 5.5 years) after portoenterostomy and 10 age-matched controls. Patients were grouped according to outcome based on LFT: group A consisted of 9 patients with an "unfavorable outcome" and Group B 10 patients with a "favorable outcome". The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA). No patient had ascites or hepatorenal syndrome. Plasma ET levels were significantly higher in patients with BA than in controls (3.42 +/- 0.42 vs 1.75 +/- 0.39 pg/ml, respectively; P < 0.01) and in patients in group A than in group B. (3.75 +/- 0.25 vs 3.06 +/- 0.23 pg/ml, respectively; P < 0.01). In group A, plasma ET levels were higher in patients with PH (n = 4) than in those without PH (n = 5) (3.99 +/- 0.06 vs 3.64 +/- 0.22 pg/ml, respectively; P < 0.05). We conclude that plasma ET levels are high in patients with BA, especially those with severe biliary cirrhosis, and that ET may partially contribute to development of PH in BA. The results of the present study also suggest that plasma ET concentrations may be a useful marker in the follow-up of patients with BA.

Biliary Atresia↗
PubMed2006

Pathogenesis and treatment opportunities for biliary atresia.

Biliary atresia is the single most common cause of chronic cholestasis in children and is the leading indication for liver transplantation worldwide. It results from an inflammatory and fibrosing obstruction of the extrahepatic bile ducts in the first few months of life. Early diagnosis and timely surgical portoenterostomy are necessary for improved biliary drainage, but the liver disease progresses to end-stage biliary cirrhosis in most patients. Although the pathogenesis of disease is largely unknown, recent patient- and animal-based experiments indicate interactions between infectious agents and inflammatory circuits may be important pathogenic mechanisms of disease.

Animals↗