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Etiology & basic research > Epidemiology - Genetics

Gene mutation and biliary atresia with polysplenia syndrome.

Jacquemin E, Cresteil D, Raynaud N, Hadchouel M.

J Pediatr Gastroenterol Nutr 2002 Mar;34(3):326-7

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Abstract


Epidemiology - Genetics

Gene mutation and biliary atresia with polysplenia syndrome.

Jacquemin E, Cresteil D, Raynaud N, Hadchouel M.

J Pediatr Gastroenterol Nutr 2002 Mar;34(3):326-7

Gene mutation and biliary atresia with polysplenia syndrome

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Diagnosis and therapy > Diagnosis

MR cholongiography in the evaluation of neonatal cholestasis: initial results

Norton KI, Glass RB, Kogan D, Lee JS, Emre S, Shneider BL.

Radiology 2002 Mar;222(3):687-91

Department of Radiology, Mount Sinai School of Medicine, 1 Gustave L Levy Pl, Box 1234, New York, NY 10029, USA. karen.norton@msnyuhealth.org

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Abstract


Diagnosis

MR cholongiography in the evaluation of neonatal cholestasis: initial results

Norton KI, Glass RB, Kogan D, Lee JS, Emre S, Shneider BL.

Radiology 2002 Mar;222(3):687-91

Department of Radiology, Mount Sinai School of Medicine, 1 Gustave L Levy Pl, Box 1234, New York, NY 10029, USA. karen.norton@msnyuhealth.org

PURPOSE: To retrospectively analyze prospective magnetic resonance (MR) cholangiographic interpretations of findings and compare them with clinical outcome and to determine the accuracy of MR cholangiography in depicting extrahepatic biliary atresia and helping to distinguish it from other causes of neonatal jaundice. MATERIALS AND METHODS: Twenty-six infants (15 male, 11 female; median age, 2 months) underwent MR cholangiography with a 1.5-T MR imaging unit. Original interpretations were compared with clinical outcome. Statistical analysis was performed to determine the accuracy of MR cholangiography in depicting extrahepatic biliary atresia. Equivocal cases and any cases lost to follow-up were excluded. RESULTS: Findings in six of 26 infants were interpreted as normal, and none of five patients (one lost to follow-up) had biliary atresia or other surgical lesions; two were abnormal but not suggestive of biliary atresia (one false-negative finding); 12 were consistent with biliary atresia (three false-positive findings); fourdemonstrated a choledochal cyst; and two were equivocal. MR cholangiography accuracy was 82% (19 of 23); sensitivity, 90% (nine of 10); and specificity, 77% (10 of 13) for the detection of extrahepatic biliary atresia, with a positive predictive value of 75% (nine of 12) and a negative predictive value of 91% (10 of 11). CONCLUSION: Results of this study found that MR cholangiography is 82% accurate, 90% sensitive, and 77% specific for depicting extrahepatic biliary atresia. Contrary to previous reports, false-positive and false-negative findings occur at MR cholangiography.

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Diagnosis and therapy > Diagnosis

Atypical morphologic presentation of biliary atresia and value of serial liverbiopsies.

Azar G, Beneck D, Lane B, Markowitz J, Daum F, Kahn E.

J Pediatr Gastroenterol Nutr 2002 Feb;34(2):212-5

Department of Pathology, North Shore University Hospital, New York University School of Medicine, Manhasset, New York 11030, USA.

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Abstract


Diagnosis

Atypical morphologic presentation of biliary atresia and value of serial liverbiopsies.

Azar G, Beneck D, Lane B, Markowitz J, Daum F, Kahn E.

J Pediatr Gastroenterol Nutr 2002 Feb;34(2):212-5

Department of Pathology, North Shore University Hospital, New York University School of Medicine, Manhasset, New York 11030, USA.

BACKGROUND: Liver biopsy findings are important in diagnosing extrahepatic biliary atresia. Diffuse ductular proliferation is a characteristic finding. We describe four patients with conjugated hyperbilirubinemia in whom the initial liver biopsy findings showed a lack of ductular proliferation, despite subsequent development of biliary atresia. RESULTS: On initial biopsy, paucity of intrahepatic bile ducts was present in three of four patients, with a bile duct to portal space ratio of 0.3 to 0.4 (normal, 0.9-1.8). A normal bile duct to portal space ratio of 1.0 was observed in the fourth patient. Ductular proliferation became apparent in three subjects between 9 and 12 weeks of age, and biliary atresia was noted at the time of a Kasai portoenterostomy. The fourth child had well-developed biliary cirrhosis at liver transplantation. CONCLUSIONS: Changes characteristic of biliary atresia may appear even after 9 weeks of age. Bile duct paucity and normal bile duct to portal space ratio do not preclude the subsequent development of biliary atresia. Infants with unexplained conjugated hyperbilirubinemia and acholic stools should undergo sequential liver biopsies until clinical improvement occurs or until biliary atresia can be excluded from the differential diagnosis.

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Liver transplantation > Split and living related liver transplantation

Living-related liver transplantation for biliary atresia associated with polysplenia syndrome.

Hasegawa T, Kimura T, Sasaki T, Okada A.

Pediatr Transplant 2002 Feb;6(1):78-81

Department of Pediatric Surgery, Osaka University Medical School, Osaka, Japan.
toshi@pedsurg.med.osaka-u.ac.jp

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Abstract


Split and living related liver transplantation

Living-related liver transplantation for biliary atresia associated with polysplenia syndrome.

Hasegawa T, Kimura T, Sasaki T, Okada A.

Pediatr Transplant 2002 Feb;6(1):78-81

Department of Pediatric Surgery, Osaka University Medical School, Osaka, Japan.
toshi@pedsurg.med.osaka-u.ac.jp

This report describes a 1-yr-old boy with biliary atresia (BA) and polysplenia
syndrome (PS) who underwent successful living-related liver transplantation
(LTx). At the time of initial hepatic portoenterostomy, he was noticed to have a
preduodenal portal vein (PV), non-rotation of the intestine, and polysplenia.
Because he did not achieve good bile excretion, he underwent a living-related
LTx (using a left lateral segment from his mother) at the age of 14 months.
Evaluation of the vascular anatomy was made by angiography, magnetic resonance
imaging (MRI), computerized tomography (CT), and Doppler ultrasound. The PV was
stenotic from the confluence of the superior mesenteric vein (SMV) and splenic
vein (SpV) to the hepatic hilum. The retrohepatic inferior vena cava (IVC) was
deficient cranially to the renal vein and was connected to the azygous vein. The
supra-hepatic IVC was detected below the diaphragm and was connected to three
hepatic veins. The common hepatic artery (HA) originated from the superior
mesenteric artery. At LTx, the PV was dissected to the level of confluence of
the SMV and the SpV, from which the venous graft was interposed using the
donor's ovarian vein. Three hepatic veins were plastied into one orifice, which
was anastomosed to the graft's hepatic vein under the diaphragm. The graft
vascularity and function has been good for 1 yr after LTx. In the present case,
sufficient pre-LTx evaluation of vascular anomalies seemed to help performance
of the successful LTx.

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Liver transplantation > Technical aspects

Laparoscopic living donor hepatectomy for liver transplantation in children.

Cherqui D, Soubrane O, Husson E, Barshasz E, Vignaux O, Ghimouz M, Branchereau S, Chardot C, Gauthier F, Fagniez PL, Houssin D.

Lancet 2002 Feb 2;359(9304):392-6

Comment in:
Lancet. 2002 Feb 2;359(9304):368-70.

Department of General and Digestive Surgery, Hopital Henri Mondor 94010,
Creteil, France. daniel.cherqui@hmn.ap-hop-paris.fr

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Technical aspects

Laparoscopic living donor hepatectomy for liver transplantation in children.

Cherqui D, Soubrane O, Husson E, Barshasz E, Vignaux O, Ghimouz M, Branchereau S, Chardot C, Gauthier F, Fagniez PL, Houssin D.

Lancet 2002 Feb 2;359(9304):392-6

Comment in:
Lancet. 2002 Feb 2;359(9304):368-70.

Department of General and Digestive Surgery, Hopital Henri Mondor 94010,
Creteil, France. daniel.cherqui@hmn.ap-hop-paris.fr

BACKGROUND: Because cadaveric organ donors are in short supply, living donors
are increasingly being used in transplantations. We have developed a safe and
reproducible method for laparoscopic liver resection. METHODS: Left hepatic
lobectomy (resection of segments 2 and 3) was done by laparoscopy in one woman
aged 27 years and one man aged 31 years. The grafts were prepared under
laparoscopy, without any vascular clamping, and were externalised through a
suprapubic Pfannenstiel incision. Both grafts were transplanted conventionally
to the patients' respective sons, who were both aged 1 year and had biliary
atresia. FINDINGS: Donor operations lasted 7 h for the woman and 6 h for the
man, and warm ischaemia times were 4 and 10 min, respectively. Blood loss was
150 and 450 mL, respectively, and no transfusions were required. Neither patient
had complications during or after surgery; and hospital stay was 7 and 5 days,
respectively. Both recipients are alive and have excellent graft function.
INTERPRETATION: We have shown the feasibility of laparoscopic living donor
hepatectomy from parent to child. If the safety and feasibility of this
procedure can be shown in larger series, laparoscopic donor left lobectomy could
become a new option for paediatric living donor liver transplantation.

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Portoenterostomy > Technical aspects

Laparoscopic Kasai portoenterostomy for biliary atresia.

Esteves E, Clemente Neto E, Ottaiano Neto M, Devanir J Jr, Esteves Pereira R.

Pediatric Surgery Division, University of Goias, Goiania, Brazil, edward@doctor.com Pediatr Surg Int. 2002 Dec;18(8):737-40

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Abstract


Technical aspects

Laparoscopic Kasai portoenterostomy for biliary atresia.

Esteves E, Clemente Neto E, Ottaiano Neto M, Devanir J Jr, Esteves Pereira R.

Pediatric Surgery Division, University of Goias, Goiania, Brazil, edward@doctor.com Pediatr Surg Int. 2002 Dec;18(8):737-40

Conventional surgery for extrahepatic bile-duct atresia (EHBDA) usually requires a large, painful, muscle-cutting laparotomy, dislodgment of the liver, and wide manipulations, followed by adhesions and possible complications that may disturb the postoperative course and hamper liver transplantation (LT). The main role of laparoscopy in EHBDA has been for diagnostic purposes. Besides all the advantages of minimally-invasive access, it allows excellent visibility and dissection of tiny hilar structures. The authors present the first two cases of successful Roux-en-Y laparoscopic portoenterostomy (LARP) for EHBDA, showing the importance of advanced technical skills and a new approach for extracorporeal enteroanastomosis. Laparoscopic hilar dissection and portoenterostomy was accomplished using four trocars. The umbilical site was used for extracorporeal Roux-en-Y enteroenterostomy, in the first case using a laparoscopic stapler and in the second a hand-sewn suture. Mean operative time was 190 min, and no operative complications were observed. Both girls became anicteric. The first is doing well 15 months after the operation with good hepatic function. The other was anicteric for 6 months, had one episode of cholangitis, developed an umbilical hernia, has shown slow and progressive hepatic failure, and is now being evaluated for possible LT. It is concluded that LARP for EHBDA can be done safely in infants using an extracorporeal transumbilical enteric anastomosis, with several advantages compared with open surgery. The role of LARP in facilitating LT is yet to be defined.

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Portoenterostomy > Outcome

Surgical management for intractable cholangitis in biliary atresia.

Muraji T, Tsugawa C, Nishijima E, Satoh S, Takamizawa S, Ise K, Maekawa T.

Department of Surgery, Kobe Children's Hospital, Kobe, Hyogoken, Japan. J Pediatr Surg. 2002 Dec;37(12):1713-5

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Outcome

Surgical management for intractable cholangitis in biliary atresia.

Muraji T, Tsugawa C, Nishijima E, Satoh S, Takamizawa S, Ise K, Maekawa T.

Department of Surgery, Kobe Children's Hospital, Kobe, Hyogoken, Japan. J Pediatr Surg. 2002 Dec;37(12):1713-5

BACKGROUND/PURPOSE: The efficacy of antireflux surgical procedures involving the Roux-en-Y jejunal limb for cholangitis was evaluated retrospectively in patients with biliary atresia (BA). METHODS: From July 1993 to December 2001, 41 patients with BA underwent hepatic portojejunostomy with Roux-en-Y reconstruction. Of these patients, 11 had intractable cholangitis that was treated by creation of a value with or without lengthening of the Roux-en-Y limb. RESULTS: Among the 11 patients, the first episode of cholangitis occurred within 6 months after portojejunostomy in 10 patients and at the age of 4 years in one patient. Cholangitis developed at various intervals from once every week to once every 2 months requiring hospitalization each time. All patients underwent valve creation at 2 months to 5 years postoperatively, whereas 2 had an additional lengthening of the limb. Cholangitis resolved completely after surgery in all cases. Two patients underwent liver transplantation, and the third patient died of an unrelated cause. The 8 survivors with native livers are doing well after 1 to 8 years of follow-up. CONCLUSION: Early surgical intervention could control intractable cholangitis in all patients, both delaying the time of liver transplantation and improving the quality of life.

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Miscellaneous

Biliary atresia, the next generation: a review of liver function, social activity, and sexual development in the late postoperative period.

Kuroda T, Saeki M, Nakano M, Morikawa N.

Department of Surgery, National Center for Child Health and Development, Tokyo, Japan. J Pediatr Surg. 2002 Dec;37(12):1709-12

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Miscellaneous

Biliary atresia, the next generation: a review of liver function, social activity, and sexual development in the late postoperative period.

Kuroda T, Saeki M, Nakano M, Morikawa N.

Department of Surgery, National Center for Child Health and Development, Tokyo, Japan. J Pediatr Surg. 2002 Dec;37(12):1709-12

PURPOSE: The current study aimed to establish the management for biliary atresia (BA) patients in the late postoperative period. METHODS: Of 165 BA patients operated on in the authors' department, 44 patients (16 boys, 28 girls) with a follow-up period of more than 15 years were reviewed retrospectively. RESULTS: Forty-one of 44 patients (93.2%) currently are employed or highly educated, 7 are married, whereas 2 (4.5%) died, and 10 (22.7%) required liver transplantation after puberty. Four babies have been born from BA parents without congenital anomalies. Four girls conceived 5 times and delivered 3 newborns weighing 2,330 to 2,474 g including one delivered after transplantation. Maternal portal hypertension uniformly deteriorated during pregnancy, and one pregnancy was terminated. Menstrual disorder correlated significantly with the biochemical data related to liver function at puberty such as serum choline esterase (266 +/- 70.4 in 19 normal patients v 159 +/- 34.3 IU/L in 9 abnormal patients, P =.00057), asparate aminotransferase (42 +/- 30.8 v 96.0 +/- 63.6 IU/L; P =.0031), and serum albumin (4.6 +/- 0.4 v 3.9 +/- 0.6 g/dL; P =.013). CONCLUSIONS: The long-term survivors of Kasai's operation, with or without liver transplantation, have reached the next generation. Transgenerational follow-up and management including conception and perinatal care should be required for BA patients.

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Etiology & basic research > Epidemiology - Genetics

Genetic induction of proinflammatory immunity in children with biliary atresia.

Bezerra JA, Tiao G, Ryckman FC, Alonso M, Sabla GE, Shneider B, Sokol RJ, Aronow BJ.

Division of Pediatric Gastroenterology, Children's Hospital Medical Center and University of Cincinnati, Cincinnati, OH 45229, USA. jorge.bezerra@chmcc.org Lancet. 2002 Nov 23;360(9346):1653-9

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Epidemiology - Genetics

Genetic induction of proinflammatory immunity in children with biliary atresia.

Bezerra JA, Tiao G, Ryckman FC, Alonso M, Sabla GE, Shneider B, Sokol RJ, Aronow BJ.

Division of Pediatric Gastroenterology, Children's Hospital Medical Center and University of Cincinnati, Cincinnati, OH 45229, USA. jorge.bezerra@chmcc.org Lancet. 2002 Nov 23;360(9346):1653-9

BACKGROUND: Biliary atresia is the commonest cause of pathological jaundice in infants and the leading indication for liver transplantation in children worldwide. The cause and pathogenesis remain largely unknown. Because of clinical heterogeneity and experimental difficulties in addressing molecular mechanisms underlying multifactorial disorders in human beings, we searched for genomic signatures of biliary atresia in affected infants. METHODS: We generated pools of biotinylated cRNA from livers of 14 infants with biliary atresia and six with neonatal intrahepatic cholestasis (diseased controls) and hybridised the cRNA against oligonucleotide-based gene chips. Immunohistochemistry and reverse transcriptase (RT)-PCR were used to assess the specificity of the findings and functional commitment of lymphocytes in affected livers. FINDINGS: Data filtering, to identify genes that are differentially expressed, and cluster analysis revealed a predominant and coordinated activation of immunity/inflammation genes within the livers of infants with biliary atresia. Most of the genes showed differential lymphocyte function, with activation of osteopontin, a regulator of cell-mediated (T-helper 1 [Th-1]) immunity in T-helper lymphocytes, and suppression of immunoglobulin genes in early stages of disease. These findings were associated with production of interferon gamma in 65% of infants with biliary atresia and no diseased control. However, histologically similar inflammatory infiltrates were present in livers of both groups, implying differential activation states of similar cell types. INTERPRETATION: Livers of infants with biliary atresia have a coordinated activation of genes involved in lymphocyte differentiation. Among these genes, the overexpression of osteopontin and interferon gamma points to a potential role of Th-1-like cytokines in disease pathogenesis.
Comment in: Lancet. 2003 Mar 15;361(9361):971-2; author reply 972.

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Miscellaneous

Biliary atresia associated with a fatty acid oxidation defect.

Matthews RP, Russo P, Berry GT, Piccoli DA, Rand EB.

J Pediatr Gastroenterol Nutr. 2002 Nov;35(5):624-8

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Miscellaneous

Biliary atresia associated with a fatty acid oxidation defect.

Matthews RP, Russo P, Berry GT, Piccoli DA, Rand EB.

J Pediatr Gastroenterol Nutr. 2002 Nov;35(5):624-8

Biliary atresia associated with a fatty acid oxidation defect.

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Miscellaneous

Transjugular intrahepatic portosystemic shunts in children with biliary atresia.

Huppert PE, Goffette P, Astfalk W, Sokal EM, Brambs HJ, Schott U, Duda SH, Schweizer P, Claussen CD.

Department of Diagnostic Radiology, Eberhard Karls University of Tubingen, Hoppe-Seyler-Strasse 3, D-72076 Tubingen, Germany. PeterHuppert@t-online.de Cardiovasc Intervent Radiol. 2002 Nov-Dec;25(6):484-93

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Miscellaneous

Transjugular intrahepatic portosystemic shunts in children with biliary atresia.

Huppert PE, Goffette P, Astfalk W, Sokal EM, Brambs HJ, Schott U, Duda SH, Schweizer P, Claussen CD.

Department of Diagnostic Radiology, Eberhard Karls University of Tubingen, Hoppe-Seyler-Strasse 3, D-72076 Tubingen, Germany. PeterHuppert@t-online.de Cardiovasc Intervent Radiol. 2002 Nov-Dec;25(6):484-93

PURPOSE: We retrospectively evaluated the technical and long-term clinical results of transjugular intrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). METHODS: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. RESULTS: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture. Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n = 11) and sonographically suspected restenosis (n = 5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. CONCLUSION: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults.

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Etiology & basic research > Epidemiology - Genetics

The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia.

Kohsaka T, Yuan ZR, Guo SX, Tagawa M, Nakamura A, Nakano M, Kawasasaki H, Inomata Y, Tanaka K, Miyauchi J.

Departments of Hepato-Entero-Nephrology, Immunology, Pediatric Surgery, and Pathology, National Center for Child Health and Development, Children's Medical Research Center, Tokyo, Japan. kohsaka-t@ncchd.go.jp Hepatology. 2002 Oct;36:904-12

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Epidemiology - Genetics

The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia.

Kohsaka T, Yuan ZR, Guo SX, Tagawa M, Nakamura A, Nakano M, Kawasasaki H, Inomata Y, Tanaka K, Miyauchi J.

Departments of Hepato-Entero-Nephrology, Immunology, Pediatric Surgery, and Pathology, National Center for Child Health and Development, Children's Medical Research Center, Tokyo, Japan. kohsaka-t@ncchd.go.jp Hepatology. 2002 Oct;36:904-12

Mutations of human jagged 1 (JAG1) gene are responsible for Alagille Syndrome (AGS), whose 2 main symptoms are intrahepatic bile duct hypoplasia and pulmonary stenosis. We examined the JAG1 mutation in extrahepatic biliary atresia (EHBA), which is similar in phenotype to AGS, although a different pathogenesis is suggested. In 102 cases of EHBA, 9 missense mutations were detected, including 2 intrafamilial expressions in the propositus and an aunt of one family. These mutations were all missense and sporadic except for those of this particular family. The JAG1 gene mutations were generally found in severely ill patients subjected to liver transplantation at less than 5 years of age. None of the 9 cases of EHBA revealed any of the 5 major symptoms of AGS nor any identical pathological findings after 3 years of follow-up. Our cases were clearly separated from AGS by pathological findings and clinical features, and could be diagnosed as EHBA and not as atypical AGS. The increase of interleukin 8 (IL-8) production induced by tumor necrosis factor alpha (TNF-alpha) in Huh 7 cells was suppressed by the coexistence of JAG1 protein. We examined the different influences between wild-type cells and the 3 kinds of mutants detected in EHBA on Huh 7 cells and found that 2 of 3 mutants showed about half of the repressed activity compared with that of wild type. In conclusion, these results suggest that the JAG1 gene abnormality may be an aggravating factor in EHBA.

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Review and overview papers

History of pediatric liver transplantation. Where are we coming from? Where do we stand?

Otte JB.

Department of Pediatric Surgery and Liver Transplantation, Universite Catholique de Louvain, Cliniques Saint-Luc, Brussels, Belgium. otte@chex.ucl.ac.be Pediatr Transplant. 2002 Oct;6(5):378-87

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Review and overview papers

History of pediatric liver transplantation. Where are we coming from? Where do we stand?

Otte JB.

Department of Pediatric Surgery and Liver Transplantation, Universite Catholique de Louvain, Cliniques Saint-Luc, Brussels, Belgium. otte@chex.ucl.ac.be Pediatr Transplant. 2002 Oct;6(5):378-87

The history of pediatric liver transplantation cannot be dissociated from one man, Thomas E. Starzl, whose pioneer efforts contributed more than anyone else to what has become a routinely successful clinical procedure. During the pre-cyclosporine era, the pediatric experience was confined nearly exclusively in Denver: first attempt in 1963, first success with survival beyond one year in 1967, cumulative experience with 84 pediatric cases in the pre-cyclosporine era (1967-1979) with a 2-year patient survival rate of 30%. The stampede for the development of other liver transplant centers came with the introduction of cyclosporine in the early eighties. Besides Pittsburgh, seven centers (Brussels, Cambridge and Hanover in Europe; Boston, Dallas, UCLA, Minneapolis in USA) had performed up to 1986 at least 20 pediatric liver transplants each with a long-term (>1 year) patient survival rate ranging between 57% and 83%. At the moment, a long-term patient survival rate in excess of 90% in elective patients -including infants - is commonly obtained in experienced centers. The shortage of size matched liver donors which was responsible for a high death rate on the cadaveric waiting list stimulated the development or technical innovations based on the segmental anatomy of the liver: reduced ('cutdown') liver graft, split graft and living liver transplantation. Challenging technical aspects in the recipient have been solved in order to reduce the incidence of surgical complications like outflow obstruction, arterial and portal thrombosis, and biliary problems. The indications of liver transplantation have been refined; regarding biliary atresia, which is the most frequent indication, a consensus has developed to propose a sequential strategy with a single attempt at hepatoportoenterostomy followed, when it fails, by liver transplantation. Some contra-indications accepted in the past are not currently valid with better understanding of the pathophysiology and/or increased clinical experience; such is the case of the hepatopulmonary syndrome. A major progress in preoperative management has been achieved through a multidisciplinary approach, particularly regarding nutrition and control of portal hypertension-related bleeding and ascites. Perioperatively, liver transplantation has derived benefit from the expertise of anesthetists managing babies with serious conditions and increased experience of the transplant surgeons regarding the knowledge of all the technical modalities, good strategy, technical skills and meticulous control of bleeding. It is well-recognized that children require more immunosuppression than adults. As in adults, the first breakthrough came with the introduction of cyclosporine which more than doubled the one-year patient survival rate. The next advance during the last decade was afforded by FK 506 - Tacrolimus which allows steroid withdrawal with the first year post-transplant in most patients. Besides its efficacy in reducing the incidence of rejection and absence of cosmetic side-effects, the steroid-sparing effect of Tacrolimus is of utmost importance to preserve the growth potential of children. The use of OKT-3 both for induction and treatment of rejection has been abandoned nearly universally because its use, cumulated with other immunosuppressants, resulted in a high incidence of lymphoproliferative disorder. In contrast, anti-IL2-receptor monoclonal antibodies, will most likely gain an increasing place in induction, with the availability of chimeric or humanized preparations. The side-effects of immunosuppression can endanger both the quality of life and the life expectancy; they are a special source of concern in pediatric recipients whose survival can be expected to be more than a few decades. Children would benefit most from the development of a marker able to identify the patients who have developed graft acceptance, allowing complete wearing of immunosuppression. Also they would benefit most from research protocols of tolerance induction. Since the vast majority of liver-transplanted children will have a reasonably normal life expectancy, the focus should be switched to their long-term rehabilitation and the assessment of their quality of life when they reach adulthood.

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Etiology & basic research > Immunology

HLA in Egyptian children with biliary atresia.

A-Kader HH, El-Ayyouti M, Hawas S, Abdalla A, Al-Tonbary Y, Bassiouny M, Boneberg A, El-Sallab S.

Department of Pediatrics, Upstate Medical University, Syracuse, New York 13210, USA. J Pediatr. 2002 Sep;141(3):432-3

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Immunology

HLA in Egyptian children with biliary atresia.

A-Kader HH, El-Ayyouti M, Hawas S, Abdalla A, Al-Tonbary Y, Bassiouny M, Boneberg A, El-Sallab S.

Department of Pediatrics, Upstate Medical University, Syracuse, New York 13210, USA. J Pediatr. 2002 Sep;141(3):432-3

We studied the human leukocytes antigens in 18 Egyptian children with biliary atresia (BA) without extrahepatic congenital malformations. There was a significant increased frequency of both B8 and DR3 (83.3% and 94.4% in patients with BA compared with 6.5% and 14.9% in the general population, respectively). Ten patients had the B8/DR3 haplotype. Our results support the hypothesis that genetic factors may play a role in susceptibility to BA.

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Diagnosis and therapy > Diagnosis

Prenatal ultrasonographic appearance of type IIId (uncorrectable type with cystic dilatation) biliary atresia.

Hasegawa T, Sasaki T, Kimura T, Sawai T, Nose K, Kamata S, Okada A, Wada K, Kanzaki T.

Department of Pediatric Surgery, Osaka University Medical School, 2-2 Yamadaoka, Suita City, Osaka 565-0871, Japan. toshi@pedsurg.med.osaka-u.ac.jp Pediatr Surg Int. 2002 Sep;18(5-6):425-8

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Abstract


Diagnosis

Prenatal ultrasonographic appearance of type IIId (uncorrectable type with cystic dilatation) biliary atresia.

Hasegawa T, Sasaki T, Kimura T, Sawai T, Nose K, Kamata S, Okada A, Wada K, Kanzaki T.

Department of Pediatric Surgery, Osaka University Medical School, 2-2 Yamadaoka, Suita City, Osaka 565-0871, Japan. toshi@pedsurg.med.osaka-u.ac.jp Pediatr Surg Int. 2002 Sep;18(5-6):425-8

Although prenatal ultrasonographic (US) diagnosis has been reported in biliary atresia (BA), most cases are type I (correctable with cystic dilatation). We report three prenatal cases of type IIId BA (uncorrectable with cystic dilatation). Routine fetal US at 22 to 24 weeks of gestation showed two communicating cystic lesions 12 to 16 mm in diameter. On color Doppler US, the lesions were separate from the portal vein or hepatic artery. The size did not change during the prenatal period in any case. Choledochal cyst (CC) was considered the most likely diagnosis, although BA with cystic lesions was also considered. After birth, the patients developed acholic stools and prolonged neonatal jaundice. Hepatobiliary scintigraphy showed negative passage. Duodenal fluid showed a negative or slightly positive Gmelin test. The neonates underwent laparotomy at the age of 36, 46, and 32 days, respectively. Intraoperative cholangiography showed the gallbladder and slightly-dilated common-bile duct without entering the proximal or distal bile ducts in all cases. They were classified as type IIId BA and underwent excision of the cystic lesions and dissection of the portal bile-duct remnants, followed by hepatic portoenterostomy. Case 1 showed persistent jaundice and finally underwent liver transplantation (LTx), case 2 became anicteric. Case 3 remained jaundiced and is to undergo LTx. In conclusion, type IIId BA may be one of the differential diagnoses when a cystic lesion is detected under the hepatic hilum by fetal US. However, prenatal diagnosis of BA is still difficult with respect to differentiation from a CC or type I BA. Early postnatal diagnosis followed by immediate treatment is important, especially in type IIId BA.

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Liver transplantation > Outcome

The Italian experience on paediatric liver transplantation: 1988-1999 report.

Pompili M, Mirante VG, Fagiuoli S, Beccaria S, Leandro G, Rapaccini GL, Gasbarrini A, Naccarato R, Pagliaro L, Rizzetto M, Gasbarrini G;

Monotematica AISF 2000-OLT Study Group.Dept. of Internal Medicine and Geriatrics, Catholic University of Sacro Cuore, Rome, Italy. mpompili@rm.unicatt.it Dig Liver Dis. 2002 Sep;34(9):649-55

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Outcome

The Italian experience on paediatric liver transplantation: 1988-1999 report.

Pompili M, Mirante VG, Fagiuoli S, Beccaria S, Leandro G, Rapaccini GL, Gasbarrini A, Naccarato R, Pagliaro L, Rizzetto M, Gasbarrini G;

Monotematica AISF 2000-OLT Study Group.Dept. of Internal Medicine and Geriatrics, Catholic University of Sacro Cuore, Rome, Italy. mpompili@rm.unicatt.it Dig Liver Dis. 2002 Sep;34(9):649-55

BACKGROUND: Liver transplantation is the treatment of choice for end-stage liver disease in both adult and paediatric patients. The Italian experience in paediatric liver transplantation during the period 1988-1999 is reported herein. PATIENTS AND METHODS: This report concerns 228 liver transplantations performed in 207 patients (100 male, 107 female, mean age 5.1+/-4.4 years) in 11 Italian centres. The mean waiting time on the transplantation list was 6.1+/-8.9 months and the main indications for the procedure were biliary atresia, inborn metabolic disorders, liver cirrhosis, liver neoplasms, Alagille syndrome, and fulminant hepatic failure. RESULTS: The cumulative survival rate was 77%, 76%, 73%, and 71% at 1, 3, 5, and 7 years. The overall prevalence of acute rejection was 54%. Survival was significantly affected by re-transplantation (p=0.0002), by United Network for Organ Sharing 4 status at transplantation (p=0.016), and, among the indications for the procedure, by fulminant hepatic failure (p=0.004). Fifty patients (24%) died during the observation period. The main causes of death were primary non-function of the graft and sepsis CONCLUSIONS: This study shows that liver transplantation in paediatric age, in Italy, is an effective procedure providing a 5-year survival rate comparable to that attained in the largest published series.
Comment in: Dig Liver Dis. 2002 Sep;34(9):619-20.

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Etiology & basic research > Animal model

The role of reovirus type 3 infection in an established murine model for biliary atresia.

Szavay PO, Leonhardt J, Czech-Schmidt G, Petersen C.

Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany. Eur J Pediatr Surg. 2002 Aug;12(4):248-50

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Abstract


Animal model

The role of reovirus type 3 infection in an established murine model for biliary atresia.

Szavay PO, Leonhardt J, Czech-Schmidt G, Petersen C.

Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany. Eur J Pediatr Surg. 2002 Aug;12(4):248-50

AIM OF THE STUDY: Infection of newborn Balb/c-mice with Rhesus rotavirus (RRV) leads to cholestasis and biliary atresia. In this current model, Reovirus Type 3 was investigated to ascertain whether Reovirus Type 3 causes the same or similar hepatobiliary lesions as RRV. METHODS: Newborn Balb/c-mice were infected with Reovirus Type 3 Dearing and Reovirus Type 3 Abney on the first day of life. Clinical observation followed for a period of at least 10 days. Cholestatic and/or dystrophic mice were prepared and specimens were taken for histological examination. RESULTS: Infection with RRV showed a 85 % morbidity for biliary atresia as described before. Clinical disease, following an infection with Reovirus T3 Dearing, showed neurological symptoms such as ataxia, and all mice died within 3 weeks. No obstructive or atretic changes of the hepatobiliary ducts could be seen either macroscopically or histomorphologically. 60 % of the mice having been infected with Reovirus T3 Abney showed signs of cholestasis and oily fur syndrome, but almost 15 % recovered from the disease. Although the histological findings did not reveal biliary atresia, inflammation and destruction of bile ducts could be observed. CONCLUSION: In comparison to the RRV infection in a Balb/c-mice model, where biliary atresia could be induced, infection with Reovirus T3 in this model did not lead to biliary atresia. But Reovirus T3 Abney infection revealed inflammatory signs as described in the literature before. The question as to why different hepatotrophic viruses lead to different changes in the murine hepatobiliary tract has to be investigated in further studies.

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Etiology & basic research > Epidemiology - Genetics

A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia.

Fischler B, Haglund B, Hjern A.

Department of Pediatrics, Huddinge University Hospital, Karolinska Institutet, Stockholm, Sweden. J Pediatr. 2002 Aug;141(2):217-

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Abstract


Epidemiology - Genetics

A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia.

Fischler B, Haglund B, Hjern A.

Department of Pediatrics, Huddinge University Hospital, Karolinska Institutet, Stockholm, Sweden. J Pediatr. 2002 Aug;141(2):217-

OBJECTIVE: To study the incidence, etiologic risk factors, and outcome of biliary atresia. STUDY DESIGN: Register study using Swedish national health databases. The study population consisted of 1,204,791 children, corresponding to 99% of the entire cohort of Swedish children born between 1987 and 1997, with an end point of follow-up at 2 years of age. Cases with biliary atresia with and without major heart malformations were identified with indicators from various national health databases. RESULTS: Eighty-five cases with biliary atresia were identified, the incidence being 1 in 14,000. A major heart malformation was found in 13 (15%) cases. In a multivariate analysis, 4 independent risk factors were identified: high maternal age (odds ratio [OR] = 3.0), parity of at least 4 (OR = 2.2), prematurity (OR = 2.9), and low birth weight for gestational age (OR = 4.7). No significant differences were found in the distribution of birth months. The outcome did not differ between the two groups with and without major heart malformations, nor with respect to any of the risk factors. CONCLUSIONS: The Swedish incidence of biliary atresia is similar to that found in other European countries. The identified risk factors may suggest the existence of a maternal vulnerability and the importance of viral infections transmitted from mother to fetus/neonate.

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Diagnosis and therapy > Diagnosis

The evaluation by magnetic resonance imaging of hepatic periportal fibrosis in infants with neonatal cholestasis: preliminary report.

Avni FE, Segers V, De Maertelaer V, Cadranel S, Dassonville M, Delaet MH, Nicaise N, Metens T.

Department of Pediatric Imaging, University Children Hospital, Brussels, Belgium. J Pediatr Surg. 2002 Aug;37(8):1128-33

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Abstract


Diagnosis

The evaluation by magnetic resonance imaging of hepatic periportal fibrosis in infants with neonatal cholestasis: preliminary report.

Avni FE, Segers V, De Maertelaer V, Cadranel S, Dassonville M, Delaet MH, Nicaise N, Metens T.

Department of Pediatric Imaging, University Children Hospital, Brussels, Belgium. J Pediatr Surg. 2002 Aug;37(8):1128-33

PURPOSE: The aim of this study was to evaluate the potential role of magnetic resonance (MR) imaging cholangiography for the assessment of periportal fibrosis associated with neonatal cholestasis. METHODS: The authors have compared the findings on MR imaging cholangiography and on pathology in 10 infants evaluated because of neonatal cholestasis. The series included 3 patients with biliary atresia (BA), 3 patients with choledocal cyst, 2 with a neonatal hepatitis, and 2 with an inspissated bile syndrome. MR examinations were performed on a 0.5 Tesla magnet unit using Turbo Field Echo T1 and Turbo spin echo T2 sequences. A periportal hyposignal paralleling the portal vein branches and disappearing after Gadolinium injection was considered consistent with periportal fibrosis. The final type of hepatobiliary anomaly was established based on surgery (n = 6) or on laparoscopic cholangiogram (n = 10). The degree of periportal fibrosis was evaluated on pathology using a grading system from grade 0 (no fibrosis) to 4 (fibrosis with cirrhosis). The relationship between periportal hyposignal and fibrosis was tested using the exact chi2 test. RESULTS: MR imaging assessed correctly and more completely than ultrasound scan the morphology of the biliary tract in all 10 patients. A periportal hyposignal was present in the 3 patients with BA (2 patients displayed a grade 3 and one a grade 4 fibrosis on pathology) and in one with choledocal cyst (grade 3 fibrosis on pathology). No hyposignal was visualized in the 2 other patients with a choledocal cyst (grades 1 and 2), in the 2 patients with neonatal hepatitis (grades 1 and 2), or in the 2 patients with inspissated bile syndrome (both grade 0). A relationship between the hyposignal seen on MR and the degree of fibrosis seen on pathology was confirmed by the exact chi2 test (P =.019). CONCLUSIONS: This preliminary series confirms the potential role of MR imaging for the assessment of the morphology of the abnormal biliary tract and of the degree of periportal fibrosis. The presence of an hyposignal on an echo gradient TFE T1 sequence suggests an advanced fibrosis (grade 3 and higher).

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Portoenterostomy > Outcome

Role of plasma thrombopoietin level in thrombocytopenia of postoperative biliary atresia patients.

Hasegawa T, Sasaki T, Kimura T, Okada A.

Department of Pediatric Surgery, Osaka University Medical School, Suita City, Osaka, Japan. J Pediatr Surg. 2002 Aug;37(8):1195-9

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Abstract


Outcome

Role of plasma thrombopoietin level in thrombocytopenia of postoperative biliary atresia patients.

Hasegawa T, Sasaki T, Kimura T, Okada A.

Department of Pediatric Surgery, Osaka University Medical School, Suita City, Osaka, Japan. J Pediatr Surg. 2002 Aug;37(8):1195-9

BACKGROUND/PURPOSE: To evaluate if thrombocytopenia may be related to plasma thrombopoietin level (P-TPO) in postoperative biliary atresia (BA). METHODS: Forty-three postoperative BA patients aged 1 to 20 years were included. P-TPO was measured by enzyme immunoassay. P-TPO was compared with platelet counts (Plt), Child's classification, presence of splenomegaly, and liver function tests. RESULTS: P-TPO significantly correlated with Plt, child's classification, serum albumin, and cholinesterase level, respectively. In 4 patients undergoing portal decompression procedure, preoperative and postoperative Plt and P-TPO were 87.5 +/- 69.1 x 10(3) and 50.3 +/- 28.0, 118.8 +/- 62.3 x 10(3)/mm3, and 53.0 +/- 55.0 pg/mL, respectively, without significant difference. In 6 patients undergoing liver transplantation (LTx), Plt and P-TPO after LTx was 157.5 +/- 83.5 x 10(3) and 143.5 +/- 75.2, respectively, which were significantly higher than those before LTx (55.0 +/- 15.6 x 10(3)/mm3 and 53.2 +/- 32.9 pg/mL). CONCLUSION: Thrombocytopenia in postoperative BA may be caused by decreased plasma TPO level in accordance with the severity of liver dysfunction rather than hypersplenism. Copyright 2002, Elsevier Science (USA). All rights reserved.

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Claus Petersen | Department of Pediatric Surgery | Hannover Medical School | Carl-Neuberg-Strasse 1 | 30625 Hannover, Germany
Tel.: +49-(0)511-532-9040 | Fax: +49-(0)511-532-8052 | www.bard-online.com | biliary-atresia@mh-hannover.de