Ad by PromoteBurner.com'; }
?>
The biliary atresia (AVB) is characterized by obstruction of the bile ducts of unknown origin, occurring in the perinatal period (1). The AVB is the leading cause of neonatal cholestasis. The histological picture is usually inflammation of the bile ducts within and outside the liver, leading to sclerosis with narrowing or obliteration (2). Untreated AVB leads to biliary cirrhosis and death of the child in the first years of life. Surgical treatment is sequential in the neonatal period, the intervention of Kasai (3), a bilio-digestive shunt between the affirmative hilum of the liver and jejunum; later if necessary, liver transplantation in case of failure Recovery of bile flow into the intestine and / or complications of biliary cirrhosis (4). The AVB is the main indication for liver transplantation in children.
Epidemiology:
The reported incidence of AVB varied from 5 / 100000 live births in the Netherlands (5), 5.1 / 100000 in France (6), 6 / 100000 in Great Britain (7), 6,5 / 100000 Texas (8), 7 / 100000 in Victoria (Australia) (9), 7.4 / 100000 in Atlanta (USA) (10) and Japan (11), 10.6 / 100000 in Hawaii (12) until 32 / 100000 in French Polynesia (13). Studies on temporal distribution and spatial-temporal cases have not provided convincing evidence for seasonal variations in incidence or epidemics, which were suspected only in studies involving limited numbers of cases (8 -10), but were not confirmed in studies including larger numbers of patients (5, 6, 14, 15).
Anatomy:
Two forms are distinguished AVB (16):
The AVB syndromic (~ 10%), in which the hepatobiliary lesions are associated with various congenital malformations such as polysplenia, congenital heart disease, abnormal intra-abdominal (situs inversus, median liver, portal vein pre-duodenal absence of inferior vena cava reverse liver, intestinal malrotation).
The AVB nonsyndromic (~ 90%), in which the bladder is isolated anomaly.
Several surgical classifications have been proposed. The French classification based on anatomical characteristics of the remaining extra-hepatic bile (17, 18):
Tableau 1 : Types anatomiques d'AVB
| Type 1 | (~3%) | Cholédoque | Type 1 | |
| Type 2 | (~6%) | Canal hépatique | Type 2 | |
| Type 3 | (19%) | Porta hepatis | Type 2 | |
| Type 4 | (72%) | Porta hepatis | Type 3 |
- Ne préjuge pas de l’état des voies biliaires intra-hépatiques
Classification française
Incidence
Description
Niveau supérieur de l’obstruction des voies biliaires extra-hépatiques
Correspondance dans la classification japonaise/USA/UK
Type 1 (~3%)
Atrésie limitée au cholédoque
Cholédoque Type 1
Type 2 (~6%)
Kyste du hile hépatique communiquant avec des voies biliaires intrahépatiques dystrophiques
Canal hépatique Type 2
Type 3 (19%)
Vésicule, canal cystique et cholédoque perméables
Porta hepatis Type 2
Type 4 (72%)
Atrésie extra-hépatique complète
Porta hepatis Type 3
* Ne préjuge pas de l’état des voies biliaires intra-hépatiques
Table 1: Types of anatomical AVB
French classification Impact Description Up to the obstruction of extrahepatic bile Correspondence in the Japanese classification / USA / UK
Type 1 (~ 3%) limited to the bile duct atresia bile duct Type 1
Type 2 (~ 6%) cyst liver hilum communicating with dystrophic intrahepatic bile duct hepatic duct type 2
Type 3 (19%) gallbladder, cystic duct and bile duct permeable Porta hepatis Type 2
Type 4 (72%) extrahepatic atresia complete Porta hepatis Type 3
• Do not prejudge the status of biliary intrahepatic
Etiology:
The cause of AVB is unknown. Low levels of g GT in amniotic fluid (enzyme normally excreted in bile and from amniotic fluid by fetal defecation) were identified from 18 weeks of gestation in fetuses born with AVB (19). The cystic forms (syndromic or not) AVB can be detected by prenatal ultrasound at 20 weeks gestation (20, 21). AVB some cases could be secondary to abnormal morphogenesis of bile ducts, and other cases to a secondary alteration of bile ducts developed normally.
Studies on human embryos have shown similarities between the developing bile canaliculi in the first trimester, and residual bile ducts at the hilum of the liver in patients with AVB, suggesting a possible defect in the process of remodeling bile ducts from the ductal plate (22). The persistence of bile ducts of fetal type could lead to bile leakage and inflammation severe secondary. Recent studies have investigated the morphogenesis of normal and pathologic biliary tract (23, 24) and the mechanisms of hepatic fibrosis (25).
The possible role of viral infection has been studied extensively. The association of AVB with cytomegalovirus (26, 27), respiratory syncytial virus (28), Epstein-Barr (29), and human papilloma virus (30) was reported, while no association was found with the virus of hepatitis A, B and C (31, 32). The Reovirus type 3 can cause cholangiopathy resembling the AVB in mice (33), and may be associated with spontaneous AVB rhesus monkeys (34). In human neonates, the association of AVB with reovirus type 3 has been suggested in several studies (35-38), but has not been confirmed by others (39-41). Rotavirus type A can cause biliary obstruction resembling AVB (42), and the deleterious effects of the virus can be prevented by interferon alpha (43). In humans, the possible role of rotavirus type C is controversial (44, 45).
Several observations suggest a genetic component in the pathogenesis of the AVB. Familial cases of AVB were reported (46-50), although discordant pairs of twins have been observed (51-53). Variations inter-racial incidence of AVB were reported in Hawaii (12) and Atlanta (10). The incidence of HLA B12 and haplotypes A9-B5 and A28-B35 was found higher in the patients with AVB compared to a control group in the United Kingdom (54).
Diagnosis
From the early diagnosis depends on the chances of success of the initial response (17, 18, 55-57). While neonatal jaundice that lasts beyond two weeks of life should be explored, and AVB must be eliminated rapidly (58, 59).
Prenatal diagnosis: Prenatal diagnosis of AVB remains exceptional. Types 1 and 2, which are rare, may be suspected on antenatal ultrasound if a cystic structure is detected in the hilum of the liver (20, 60): The post-natal must distinguish a cystic form of AVB, which requires urgent surgery, a choledochal cyst, whose treatment can generally be deferred.
Clinic: After birth, the characteristic clinical triad associated (1.60): 1) jaundice that persists after two weeks of life. 2) stool discolored (gray-white) and dark urine. 3) an enlarged liver. The general condition of the baby is usually excellent, and the growth height and weight is normal at this point. The late signs are: an enlarged spleen (portal hypertension), ascites, bleeding which can be intra-cranial (lack of absorption of vitamin K).
Figure 1: Selles décolorées
Ultrasound: Ultrasound of the liver was performed with a strict fast of 12 hours (the child being infused): it does not show dilated bile ducts. The AVB may be suspected if the gallbladder was atrophic despite the prolonged fasting, if the hilum of the liver is hyperechoic (sign of the "fibrous cone"), if there is a cyst in the hilum of the liver, if the elements of the syndrome of polysplenia are identified: Multiple rates, Preduodenal portal vein, absence of vena cava iférieure retro-hepatic.
Cholangiography: In cases where the gallbladder seems normal on ultrasonography, cholangiography is needed to check the patency of bile ducts. This cholangiography can be performed percutaneously (under ultrasound), endoscopic retrograde (ERCP) or surgery.
Liver biopsy: The main features suggestive of AVB are bile plugs, ductulaire proliferation, edema and / or portal fibrosis. As in all other causes of neonatal cholestasis, giant hepatocytes can be observed.
Other: The blood biochemistry showed cholestasis (with elevated cholesterol and g GT). The HIDA scan showed the absence of marker excretion from the liver to the intestine, but this can also be observed in other severe neonatal cholestasis. In addition, scintigraphy can be falsely reassuring in the early stages of the AVB.
Exclusion of other causes of neonatal cholestasis: The medical causes of neonatal cholestasis must be excluded. Differential diagnoses the most common are: Alagille syndrome, cholestasis fibrogenic family, the deficit has 1 antitrypsin, cystic fibrosis.
The diagnosis of AVB can in most cases be strongly suspected with the clinical, ultrasound, and after a quick review to eliminate the other major causes of neonatal cholestasis. Cholangiography and / or biopsy hépaique are indicated only when the diagnosis remains uncertain, particularly in cases of non-atrophic gallbladder on ultrasound (61, 62).
Treatment
Current treatment of AVB is sequential: 1) In the neonatal period, the intervention of Kasai, with the objective to restore biliary flow into the intestine. 2) The secondary liver transplantation in cases of failure to restore holérèse and / or complications of biliary cirrhosis.
The Kasai operation: hépatoporto-enterostomy:
Following a transverse incision above the umbilicus, the diagnosis of AVB was confirmed by inspection of the liver and bile ducts extrahepatic: in most cases (type 4: Extra-hepatic atresia complete), the diagnosis is obvious with a liver cholestatic (Green) + / - fibrous or cirrhosis, and gallbladder reduced to the status of a fibrous cord, if the vesicle is permeable, the color of its contents (bile colored or colorless epithelial secretions) is relieved, and cholangiography is performed. The elements of polysplenia syndrome are sought as well as other intra-abdominal abnormalities (including Meckel diverticulum). The portal pressure was measured by the umbilical vein.
After section of the falciform ligament and left triangular ligament, the liver is exteriorized through abdominal incision. The tree extrahepatic bile is excised, and the fibrous cone located above the portal bifurcation. A Y jejunal loop of 45 cm in length is prepared and then passed through the mesocolon to the liver hilum. The anastomosis is performed by ventousage the end of the ilostomy open its antimesenteric border, on the edge of the bottom section of remaining bile cut at the hilar plate. A liver biopsy is performed.
Figure 2: Vue opératoire d’une forme complète (type 4) d’AVB
Figure 3 : Hépatoporto-enterostomie (Opération de Kasai) (63)
Several technical variations are possible, depending on the anatomy of the remaining extra-hepatic bile:
• AVB Type 1: cholecysto-enterostomy, or hepatic-enterostomy.
• AVB Type 2: kysto-enterostomy. This operation can be performed after having verified by cholangiography that hilar cyst communicates with the dystrophic intrahepatic bile ducts.
• AVB Type 3: hépatoporto-cholecystostomy. The gallbladder, the cystic and common bile duct, all permeable (cholangiography) are preserved. The gallbladder is freed from his bed, preserving its vascular pedicle. The anastomosis is performed between the edge of the hilar plate and the bottom vesicular mobilized to the hilum of the liver. As this intervention does not in direct contact with intestinal bile ducts, it is supposed to reduce the risk of postoperative cholangitis (57). Its specific complications are anastomotic bile leak with biliopéritoine postoperative torsion and obstruction of the cystic and common bile duct (64-66).
Figure 4: Hépatoporto-cholecystostomie (63)
In post-operative treatments have been proposed to either increase the cholereses or to reduce inflammation at the hilar plate, which could lead to granulation tissue and fibrosis obstructing the bile ducts still permeable. Although advocated by some surgeons (67-69), corticosteroids remain controversial because their interest on the long-term has not been demonstrated, and may increase the risk of severe cholangitis postoperatively.
Become a success after the operation when the Kasai Kasai intervention can restore bile flow to the intestines, the stool is recolorent (yellow or green), and the jaundice subsides gradually. This may take several weeks to several months. The development of biliary cirrhosis is stopped or slowed significantly, and survival with native liver were reported to adulthood (70, 71).
Figure 5: Survie avec le foie natif de 271 enfants operés d’une intervention de Kasa pour AVB entre 1968 et 1983 à l’hôpital de Bicêtre (Paris) (70)
However, several complications can occur:
• Cholangitis: Direct contact between the intestine and dystrophic intrahepatic bile ducts, seats bile stasis may be the cause of ascending cholangitis, especially in the first weeks or months following the intervention of Kasai, in 30 to 60% of cases (72, 73). This infection can be severe and fulminant. It is revealed by signs of sepsis (fever, hypothermia, poor hemodynamics), an outbreak of jaundice (cholestasis and biological), pale stools, pain on palpation of the liver. The diagnosis can be confirmed by blood cultures and / or culture bios liver (73). Treatment involves IV antibiotics, and resuscitation measures nonspecific. In cases of recurrent cholangitis and / or late reflux in ilostomy too short occlusion of the loop in Y, and cavities infected bile ducts must be sought. Of recurrent cholangitis without cause "surgery" may require continuous antibiotic prophylaxis.
• Portal hypertension: Portal hypertension occurs in at least two thirds of children after portoentérostomie (74, 75), even after complete regression of cholestasis. The commonest sites of varicose veins are: esophagus, stomach, ilostomy, rectum. In case of failure of restoration of bile flow, portal hypertension is treated by liver transplantation, but may require sclerotherapy or ligation of varicose veins in anticipation of transplantation. In case of complete regression of jaundice after surgery of Kasai, conservative treatments are shown: the more often sclerotherapy or variceal ligation (76, 77). Diversion Puerto sushépatique percutaneous trans-hépatrique (TIPS) is rarely used in this indication, given the young age of these patients, the usual hypoplasia of the portal vein in children with AVB, the frequent existense cavities of intrahepatic bile, and the risk of intimal proliferation and obstruction secondary to the bypass (78). The porto-systemic shunt surgery remain rare indications, when portal hypertension is isolated, with summary functions of normal liver, and a non-progressive liver disease, and varicose veins are not accessible to endoscopic treatment (79) . Exceptionally, hypersplenism can be treated by arterial embolization of the spleen (80).
• The hepato-pulmonary syndrome and pulmonary hypertension: As in patients with porto-systemic shunts spontannés (cirrhosis or portal hypertension pre-hepatic) or acquired (porto-systemic shunt surgery), the arterio-venous shunts or pulmonary pulmonary hypertension may occur in patients with biliary cirrhosis secondary to AVB, even in cases of complete regression of jaundice. These pulmonary complications may be due to the lack of detoxification by liver vasoactive substances of intestinal origin, coming directly into the pulmonary vasculature due to porto-systemic derivations. Pulmonary arteriovenous shunts are responsible for hypoxia, cyanosis, dyspnea, clubbing, while the pulmonary hypertension causes discomfort or even unexpected death. The diagnosis is confirmed by pulmonary perfusion scintigraphy, and echocardiography, respectively. Liver transplantation allows the regression of pulmonary shunts (81), and pulmonary hypertension in its early stage (not set) (82).
• Cavities intrahepatic bile: Cavities large bile ducts may develop months or years after surgery Kasai, even in cases of complete regression of jaundice. These cavities may become infected and / or compress the portal vein, requiring external drainage. A kysto-enterostomy (83) or transplantation may then be necessary.
• Neoplasia: The hepatocellular, hepatoblastoma (84) and cholangiocarcinoma (85) were observed in liver cirrhotic patients with AVB in childhood or adulthood. Screening of a liver tumor should be systematic monitoring of children after intervention Kasai.
Become failed after the intervention of Kasai
In case of failure of restoration of bile flow into the intestine, biliary cirrhosis continues to evolve and liver transplantation is necessary. The AVB represents more than half of the indications for liver transplantation in children. Failing immediate intervention Kasai, transplantation is usually performed in the second year of life, but may sometimes be necessary early (at 6 months of life) in cases of rapidly progressive cirrhosis. If successful initial response to Kasai, the transplant may be needed later in childhood or adulthood, either because of recurrence of jaundice (secondary failure of the intervention of Kasai) or because of complications of cirrhosis despite regression of jaundice.
The graft may come from a deceased donor organs: rarely a whole liver, from a donor child size near the recipient, usually a left lobe (segments 2 +3) or a left liver (segments 2 +3 4) obtained after reduction or sharing an adult donor liver. The graft may also be collected from one parent to the child.
Survival of patients 5 and 10 years after liver transplantation for AVB currently exceeds 80% (86, 87). In most cases, quality of life for these children is close to normal, both for growth to thrive, for their physical, intellectual, and subsequent fertility (88-90).
Become a global children with AVB
The overall prognosis of children with AVB has improved since the early days of liver transplantation, and currently over 90% of children with AVB can survive.
Tableau 2: Pronostic de l’AVB en France et au Royaume Uni :
France 1986-96 | France 1997-2002 | UK 1999-2002 | |
| Survie globale des patients à 4 ans | |||
| Survie avec le foie natif à 4 ans | |||
| Survie 4 ans après transplantation hépatique |
Several prognostic factors have been identified. Some are related to characteristics of the disease (and can not be modified): the presence of polysplenia syndrome (17, 18, 92), anatomical type of the remaining extra-hepatic bile (17, 55-57), histological lesions of remaining extrahepatic bile (93, 94), extent of liver fibrosis at the time of the intervention of Kasai (95-100). Other prognostic factors are related to the care and could be improved: age at Kasai intervention (17, 55, 57, 59), accessibility to liver transplantation (availability in pediatric liver graft) (101 ), experience of dealing center in the care of such children (7, 17, 102). This last point has led to centralize patients with AVB in England and Wales in three medical-surgical units of Pediatric Hepatology (91).
Acknowledgments:
Pediatricians and surgeons from 45 French centers participating in the work of the French Observatory of biliary atresia.
References
Références
1. Alagille D. Extrahepatic biliary atresia. Hepatology 1984;4:7S-10S.
2. Gautier M, Eliot N. Extrahepatic biliary atresia: morphological study of 98 biliary remnants. Arch Path Lab Med 1981;105:397-402.
3. Kasai M, Kimura S, Asakura Y, Suzuki Y, Taira Y, Obashi E. Surgical treatment of biliary atresia. J Pediatr Surg 1968;3:665-675.
4. Otte JB, de Ville de Goyet J, Reding R, Hausleithner V, Sokal E, Chardot C, et al. Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review. Hepatology 1994;20(1 Pt 2):41S-48S.
5. Houwen RH, Kerremans I, van Steensel-Moll HA, van Romunde LK, Bijleveld CM, Schweizer P. Time-space distribution of extrahepatic biliary atresia in The Netherlands and West Germany. Z Kinderchir 1988;43(2):68-71.
6. Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B. Epidemiology of biliary atresia in France: a national study 1986-96. J Hepatol 1999;31(6):1006-13.
7. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000;355(9197):25-9.
8. Strickland AD, Shannon K. Studies in the aetiology of extrahepatic biliary atresia: time-space clustering. Pediatrics 1982;100:749-53.
9. Danks DM, Campbell PE, Jack I, Rogers J, Smith AL. Studies of the aetiology of neonatal hepatitis and biliary atresia. Arch Dis Child 1977;52:360-7.
10. Yoon PW, Bresee JS, Olney RS, James LM, Khoury MJ. Epidemiology of biliary atresia: a population-based study. Pediatrics 1997;99(3):376-82.
11. Chiba T, Ohi R, Kamiyama T, Nio M, Ibrahim M. Japanese biliary atresia registry. In: Ohi R, editor. Biliary atresia. Tokyo (Japan): ICOM Associates; 1991. p. 79-86.
12. Shim WK, Kasai M, Spence MA. Racial influence on the incidence of biliary atresia. Prog Pediatr Surg 1974;6:53-62.
13. Vic P, Gestas P, Mallet EC, Arnaud JP. Atrésie des voies biliaires en Polynésie française. Etude rétrospective de 10 ans. Arch Pediatr 1994;1(7):646-51.
14. Ayas MF, Hillemeier AC, Olson AD. Lack of evidence for seasonal variation in extrahepatic biliary atresia during infancy. J Clin Gastroenterol 1996;22(4):292-4.
15. Davenport M, Dhawan A. Epidemiologic study of infants with biliary atresia. Pediatrics 1998;101(4 Pt 1):729-30.
16. Howard ER. Biliary atresia. In: Stringer MD, Oldham KT, Mouriquand PDE, Howard ER, editors. Pediatric surgery and urology: long term outcomes. London: WB Saunders; 1998. p. 402-16.
17. Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology 1999;30(3):606-11.
18. Gauthier F, Luciani JL, Chardot C, Branchereau S, de Dreuzy O, Lababidi A, et al. Determinants of life span after Kasai operation at the era of liver transplantation. Tohoku J Exp Med 1997;181(1):97-107.
19. Brunero M, de Dreuzy O, Herrera JM, Gauthier F, Valayer J. Diagnosi ecografica prenatale di una immagine cistica a carico dell'ilo epatico. Interpretazione per un adeguato trattamento. Minerva pediatr 1996;48:485-94.
20. Hinds R, Davenport M, Mieli-Vergani G, Hadzic N. Antenatal presentation of biliary atresia. J Pediatr 2004;144(1):43-6.
21. Muller F, Oury JF, Dumez Y. Microvillar enzymes assays in amniotic fluid and fetal tisssues at different stages of development. Prenatal Diagnosis 1988;8:189-98.
22. Tan CE, Driver M, Howard ER, Moscoso GJ. Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry. J Pediatr Surg 1994;29(6):808-14.
23. Desmet VJ. Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 1998;73(1):80-9.
24. Mazziotti MV, Willis LK, Heuckeroth RO, LaRegina MC, Swanson PE, Overbeek PA, et al. Anomalous development of the hepatobiliary system in the Inv mouse. Hepatology 1999;30(2):372-8.
25. Ramm GA, Nair VG, Bridle KR, Shepherd RW, Crawford DH. Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia. Am J Pathol 1998;153(2):527-35.
26. Fischler B, Ehrnst A, Forsgren M, Orvell C, Nemeth A. The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1998;27(1):57-64.
27. Hart MH, Kaufman SS, Vanderhoof JA, Erdman S, Linder J, Markin RS, et al. Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus infection in twins. Am J Dis Child 1991;145(3):302-5.
28. Nadal D, Wunderli W, Meurmann O, Briner J, Hirsig J. Isolation of respiratory syncytial virus from liver tissue and extrahepatic biliary atresia material. Scand J Infect Dis 1990;22(1):91-3.
29. Weaver LT, Nelson R, Bell TM. The association of extrahepatic bile duct atresia and neonatal Epstein Barr virus infection. Acta Paediatr Scand 1984;73:155-7.
30. Drut R, Drut RM, Gomez MA, Cueto Rua E, Lojo MM. Presence of human papillomavirus in extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1998;27(5):530-5.
31. A-Kader HH, Nowicki MJ, Kuramoto KL, Baroudy B, Zeldis JB, Balistreri WF. Evaluation of the role of hepatitis C virus in biliary atresia. Pediatr Infect Dis J 1994;13:657-9.
32. Balistreri WF, Tabor E, Gerety RJ. Negative serology for hepatitis A and B viruses in 18 cases of neonatal cholestasis. Pediatrics 1980;66:269-71.
33. Bangaru B, Morecki R, Glaser JH, Gartner LM, Horwitz MS. Comparative studies of biliary atresia in the human newborn and reovirus-induced cholangitis in weanling mice. Lab Invest 1980;43(5):456-62.
34. Rosenberg DP, Morecki R, Lollini LO, Glaser J, Cornelius CE. Extrahepatic biliary atresia in a rhesus monkey (Macaca mulatta). Hepatology 1983;3(4):577-80.
35. Glaser JH, Balistreri WF, Morechi R. Role of reovirus type 3 in persistent infantile cholestasis. J Pediatr 1984;105:912-5.
36. Morecki R, Glaser JH, Cho S, Balistreri WF, Horwitz MS. Biliary atresia and reovirus type 3 infection. New Engl J Med 1982;307:481-4.
37. Morecki R, Glaser JH, Johnson AB, Kress Y. Detection of reovirus type 3 in the porta hepatis of an infant with extrahepatic biliary atresia: ultrastructural and immunocytochemical study. Hepatology 1984;4:1137-42.
38. Tyler KL, Sokol RJ, Oberhaus SM, Le M, Karrer FM, Narkewicz MR, et al. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts. Hepatology 1998;27(6):1475-82.
39. Brown WR, Sokol RJ, Levin MJ, Silverman A, Tamaru T, Lilly JR, et al. Lack of correlation between infection with reovirus 3 and extrahepatic biliary atresia or neonatal hepatitis. J Pediatr 1988;113(4):670-6.
40. Dussaix E, Hadchouel M, Tardieu M, Alagille D. Biliary atresia and reovirus type 3 infection. New Engl J Med 1984;310:658.
41. Steele MI, Marshall CM, Lloyd RE, Randolph VE. Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease. Hepatology 1995;21(3):697-702.
42. Petersen C, Biermanns D, Kuske M, Schakel K, Meyer-Junghanel L, Mildenberger H. New aspects in a murine model for extrahepatic biliary atresia. J Pediatr Surg 1997;32(8):1190-5.
43. Petersen C, Bruns E, Kuske M, von Wussow P. Treatment of extrahepatic biliary atresia with interferon-alpha in a murine infectious model. Pediatr Res 1997;42(5):623-8.
44. Bobo L, Ojeh C, Chiu D, Machado A, Colombani P, Schwarz K. Lack of evidence for rotavirus by polymerase chain reaction/enzyme immunoassay of hepatobiliary samples from children with biliary atresia. Pediatr Res 1997;41(2):229-34.
45. Riepenhoff-Talty M, Gouvea V, Evans MJ, Svensson L, Hoffenberg E, Sokol RJ, et al. Detection of group C rotavirus in infants with extrahepatic biliary atresia. J Infect Dis 1996;174(1):8-15.
46. Cunningham ML, Sybert VP. Idiopathic extrahepatic biliary atresia: recurrence in sibs in two families. Am J Med Genet 1988;31(2):421-6.
47. Gunasekaran TS, Hassall EG, Steinbrecher UP, Yong SL. Recurrence of extrahepatic biliary atresia in two half sibs. Am J Med Genet 1992;43(3):592-4.
48. Lachaux A, Descos B, Plauchu H, Wright C, Louis D, Raveau J, et al. Familial extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1988;7(2):280-3.
49. Silveira TR, Salzano FM, Howard ER, Mowat AP. Extrahepatic biliary atresia and twinning. Braz J Med Biol Res 1991;24(1):67-71.
50. Smith BM, Laberge JM, Schreiber R, Weber AM, Blanchard H. Familial biliary atresia in three siblings including twins. J Pediatr Surg 1991;26(11):1331-3.
51. Hyams JS, Glaser JH, Leichtner AM, Morecki R. Discordance for biliary atresia in two sets of monozygotic twins. J Pediatr 1985;107:420-2.
52. Poovorawan Y, Chongsrisawat V, Tanunytthawongse C, Norapaksunthorn T, Mutirangura A, Chandrakamol B. Extrahepatic biliary atresia in twins: zygosity determination by short tandem repeat loci. J Med Assoc Thai 1996;79 Suppl 1:S119-24.
53. Strickland AD, Shannon K, Coln CD. Biliary atresia in two sets of twins. J Pediatr 1985;107(418-20).
54. Silveira TR, Salzano FM, Donaldson PT, Mieli-Vergani G, Howard ER, Mowat AP. Association between HLA and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1993;16(2):114-7.
55. Altman RP, Lilly JR, Greenfeld J, Weinberg A, van Leeuwen K, Flanigan L. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg 1997;226(3):348-55.
56. Ibrahim M, Miyano T, Ohi R, Saeki M, Shiraki K, Tanaka K, et al. Japanese Biliary Atresia Registry, 1989 to 1994. Tohoku J Exp Med 1997;181(1):85-95.
57. Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J Pediatr Surg 1990;25(10):1076-81.
58. Bernard O. Plaidoyer pour un diagnostic précoce de l'atrésie des voies biliaires. Douze erreurs à éviter. Arch Pediatr 1995;2(10):937-9.
59. Mieli-Vergani G, Howard ER, Portman B, Mowat AP. Late referral for biliary atresia--missed opportunities for effective surgery. Lancet 1989;1(8635):421-3.
60. Redkar R, Davenport M, Howard ER. Antenatal diagnosis of congenital anomalies of the biliary tract. J Pediatr Surg 1998;33(5):700-4.
61. Bernard O. Diagnostic précoce des cholestases néonatales. Arch Pediatr 1998;5(9):1031-5.
62. Bernard O, Gauthier F. Progrès récents en hépatologie pédiatrique. Arch Fr Pediatr 1991;48(1):53-6.
63. Valayer J, Chardot C. Atrésie des voies biliaires. Encyclopédie Médico Chirurgicale 2002;techniques chirurgicales - appareil digestif(40-890):1-11.
64. Freitas L, Gauthier F, Valayer J. Second operation for repair of biliary atresia. J Pediatr Surg 1987;22(9):857-60.
65. Lilly JR. Hepatic portocholecystostomy for biliary atresia. J Pediatr Surg 1979;14(3):301-4.
66. Lilly JR, Stellin G. Catheter decompression of hepatic portocholecystostomy. J Pediatr Surg 1982;17(6):904-5.
67. Altman RP, Anderson KD. Surgical management of intractable cholangitis following successful Kasai procedure. J Pediatr Surg 1982;17(6):894-900.
68. Karrer FM, Lilly JR. Corticosteroid therapy in biliary atresia. J Pediatr Surg 1985;20(6):693-5.
69. Kasai M, Suzuki H, Ohashi E, Ohi R, Chiba T, Okamoto A. Technique and results of operative management of biliary atresia. World J Surg 1978;2(5):571-9.
70. Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 2005;41(2):366-71.
71. Ohi R, Nio M, Chiba T, Endo N, Goto M, Ibrahim M. Long-term follow-up after surgery for patients with biliary atresia. J Pediatr Surg 1990;25(4):442-5.
72. Burnweit CA, Coln D. Influence of diversion on the development of cholangitis after hepatoportoenterostomy for biliary atresia. J Pediatr Surg 1986;21(12):1143-6.
73. Ecoffey C, Rothman E, Bernard O, Hadchouel M, Valayer J, Alagille D. Bacterial cholangitis after surgery for biliary atresia. J Pediatr 1987;111(6 Pt 1):824-9.
74. Kasai M, Okamoto A, Ohi R, Yabe K, Matsumura Y. Changes of portal vein pressure and intrahepatic blood vessels after surgery for biliary atresia. J Pediatr Surg 1981;16(2):152-9.
75. Ohi R, Mochizuki I, Komatsu K, Kasai M. Portal hypertension after successful hepatic portoenterostomy in biliary atresia. J Pediatr Surg 1986;21(3):271-4.
76. Sasaki T, Hasegawa T, Nakajima K, Tanano H, Wasa M, Fukui Y, et al. Endoscopic variceal ligation in the management of gastroesophageal varices in postoperative biliary atresia. J Pediatr Surg 1998;33(11):1628-32.
77. Stringer MD, Howard ER, Mowat AP. Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg 1989;24(5):438-42.
78. Rossle M, Siegerstetter V, Huber M, Ochs A. The first decade of the transjugular intrahepatic portosystemic shunt (TIPS): state of the art. Liver 1998;18(2):73-89.
79. Valayer J, Branchereau S. Portal hypertension: porto systemic shunts. In: Stringer MD, Oldham KT, Mouriquand PDE, Howard ER, editors. Pediatric surgery and urology: long term outcomes. London: W.B. Saunders; 1998. p. 439-46.
80. Chiba T, Ohi R, Yaoita M. Partial splenic embolization for hypersplenism in pediatric patients with special reference to its long-term efficacy. In: Ohi R, editor. Biliary atresia. Tokyo: ICOM Associates Inc; 1991. p. 154-8.
81. Yonemura T, Yoshibayashi M, Uemoto S, Inomata Y, Tanaka K, Furusho K. Intrapulmonary shunting in biliary atresia before and after living-related liver transplantation. Br J Surg 1999;86(9):1139-43.
82. Losay J, Piot D, Bougaran J, Ozier Y, Devictor D, Houssin D, et al. Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension. J Hepatol 1998;28(2):337-42.
83. Tsuchida Y, Honna T, Kawarasaki H. Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy. J Pediatr Surg 1994;29(5):630-4.
84. Tatekawa Y, Asonuma K, Uemoto S, Inomata Y, Tanaka K. Liver transplantation for biliary atresia associated with malignant hepatic tumors. J Pediatr Surg 2001;36(3):436-9.
85. Kulkarni PB, Beatty E, Jr. Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. Am J Dis Child 1977;131(4):442-4.
86. Diem HV, Evrard V, Vinh HT, Sokal EM, Janssen M, Otte JB, et al. Pediatric liver transplantation for biliary atresia: results of primary grafts in 328 recipients. Transplantation 2003;75(10):1692-7.
87. Fouquet V, Alves A, Branchereau S, Grabar S, Debray D, Jacquemin E, et al. Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl 2005;11(2):152-60.
88. Asonuma K, Inomata Y, Uemoto S, Egawa H, Kiuchi T, Okajima H, et al. Growth and quality of life after living-related liver transplantation in children. Pediatr Transplant 1998;2(1):64-9.
89. Codoner-Franch P, Bernard O, Alvarez F. Long-term follow-up of growth in height after successful liver transplantation. J Pediatr 1994;124(3):368-73.
90. Midgley DE, Bradlee TA, Donohoe C, Kent KP, Alonso EM. Health-related quality of life in long-term survivors of pediatric liver transplantation. Liver Transpl 2000;6(3):333-9.
91. Davenport M, De Ville de Goyet J, Stringer MD, Mieli-Vergani G, Kelly DA, McClean P, et al. Seamless management of biliary atresia in England and Wales (1999-2002). Lancet 2004;363(9418):1354-7.
92. Vazquez J, Lopez Gutierrez JC, Gamez M, Lopez-Santamaria M, Murcia J, Larrauri J, et al. Biliary atresia and the polysplenia syndrome: its impact on final outcome. J Pediatr Surg 1995;30(3):485-7.
93. Schweizer P, Kirschner HJ, Schittenhelm C. Anatomy of the porta hepatis (PH) as rational basis for the hepatoporto-enterostomy (HPE). Eur J Pediatr Surg 1999;9(1):13-8.
94. Tan CE, Davenport M, Driver M, Howard ER. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg 1994;29(11):1459-64.
95. Ohya T, Miyano T, Kimura K. Indication for portoenterostomy based on 103 patients with Suruga II modification. J Pediatr Surg 1990;25(7):801-4.
96. Schweizer P, Schweizer M, Schellinger K, Kirschner HJ, Schittenhelm C. Prognosis of extrahepatic bile-duct atresia after hepatoportoenterostomy. Pediatr Surg Int 2000;16(5-6):351-5.
97. Tanano H, Hasegawa T, Kimura T, Sasaki T, Kawahara H, Kubota A, et al. Proposal of fibrosis index using image analyzer as a quantitative histological evaluation of liver fibrosis in biliary atresia. Pediatr Surg Int 2003;19(1-2):52-6.
98. Vazquez-Estevez J, Stewart B, Shikes RH, Hall RJ, Lilly JR. Biliary atresia: early determination of prognosis. J Pediatr Surg 1989;24(1):48-50; discussion 50-1.
99. Weerasooriya VS, White FV, Shepherd RW. Hepatic fibrosis and survival in biliary atresia. J Pediatr 2004;144(1):123-5.
100. Wildhaber BE, Coran AG, Drongowski RA, Hirschl RB, Geiger JD, Lelli JL, et al. The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients. J Pediatr Surg 2003;38(10):1480-5.
101. Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 2003;38(7):997-1000.
102. McClement JW, Howard ER, Mowat AP. Results of surgical treatment for extrahepatic biliary atresia in United Kingdom 1980-2. Survey conducted on behalf of the British Paediatric Association Gastroenterology Group and the British Association of Paediatric Surgeons. Br Med J (Clin Res Ed) 1985;290(6465):345-7.
Copyright : (c) Christophe CHARDOT
Auteur : M Christophe CHARDOT
Date de création : 22/04/2002
Date de mise à jour : 29/01/2010
Search Engine Submission - AddMe
20 Tools for Tracking Social Media Marketing
![Reblog this post [with Zemanta]](http://img.zemanta.com/reblog_a.png?x-id=d5f4fcc2-8943-463f-9ddf-a377695e7c14)
