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Chapter 11 - Biliary atresia and other disorders of the extrahepatic bile ducts
- from Section II - Cholestatic liver disease
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- By William F. Balistreri, University of Cincinnati College of Medicine , Jorge A. Bezerra, Pediatric Liver Care Center; The William and Rebecca Balistreri Chair of Pediatric Hepatology, Division of Gastroenterology, Hepatology and Nutrition, University of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA, Frederick C. Ryckman, Pediatric Surgery Training Program, Pediatric Liver Care Center, Cincinnati Children’s Hospital, Cincinnati, OH, USA
- Edited by Frederick J. Suchy, University of Colorado Medical Center, Ronald J. Sokol, University of Colorado Medical Center, William F. Balistreri
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- Book:
- Liver Disease in Children
- Published online:
- 05 March 2014
- Print publication:
- 20 February 2014, pp 155-176
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Summary
Introduction
Biliary atresia and related disorders of the biliary tract, such as choledochal cysts, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in the newborn (neonatal cholestasis).
Neonatal hepatobiliary diseases, including biliary atresia, choledochal cysts, and “idiopathic” neonatal hepatitis, have historically been viewed as a continuum – a gradation of manifestations of a basic underlying disease process in which giant cell transformation of hepatocytes is strongly associated with inflammation at any level of the hepatobiliary tract. These disease entities may be polar end-points of a common initial insult, as originally stated in the unifying hypothesis of Landing [1]. The end result represents the sequelae of the inflammatory process at the primary site of injury. Landing suggested that this inflammatory process may injure bile duct epithelial cells, leading to either duct obliteration (biliary atresia) or weakening of the bile duct wall with subsequent dilatation (choledochal cyst). The lesions may be dependent on the stage of fetal or early postnatal development when the injury occurs and the site within the developing hepatobiliary tree at which the injury occurs [1,2]. The recent observation that extrahepatic bile ducts develop cystic dilatations following rotavirus infection in newborn mice genetically primed to have a prominent T helper lymphocyte type 2 response suggests that the lesions may also be dependent on the type of immune response to the viral insult [3]. A relationship between the pathogenesis of these obstructive cholangiopathies of infancy and the process of development (embryogenesis) is suggested by the association with disorders of situs determination such as the polysplenia syndrome and the observation of the so-called ductal plate malformation within the liver of a few patients with biliary atresia. The ductal plate malformation is postulated to represent either a primary developmental anomaly or disruption of a developmental sequence early in fetal life, resulting in incomplete regression of the immature bile ducts [2]. In contrast, most patients with biliary atresia have the late-onset type, which probably occurs after the anatomic formation of intra- and extrahepatic bile ducts; this represents injury (destruction) of fully formed structures [1]. The dynamic nature of the underlying process has been further suggested by an apparent postnatal evolution of patent to atretic ducts: patients initially shown to have “neonatal hepatitis” with a patent biliary system were subsequently found to have acquired biliary atresia.
Chapter 43 - Liver transplantation in children: indications and surgical aspects
- from Section V - Other considerations and issues in pediatric hepatology
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- By M. Kyle Jensen, University of Utah, Primary Children’s Medical Center, Salt Lake City, UT, USA, Maria H. Alonso, Department of Pediatrics, Northwestern University, Chicago, and Director of Hepatology, Department of Gastroenterology, Hepatology and Nutrition, Children’s Memorial Hospital, Chicago, IL, USA, Jaimie D. Nathan, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA, Frederick C. Ryckman, Pediatric Surgery Training Program, Pediatric Liver Care Center, Cincinnati Children’s Hospital, Cincinnati, OH, USA, Gregory M. Tiao, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA, William F. Balistreri, University of Cincinnati College of Medicine
- Edited by Frederick J. Suchy, University of Colorado Medical Center, Ronald J. Sokol, University of Colorado Medical Center, William F. Balistreri
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- Book:
- Liver Disease in Children
- Published online:
- 05 March 2014
- Print publication:
- 20 February 2014, pp 760-772
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Summary
Introduction
Liver transplantation has become the standard of care for end-stage liver disease in children and successful outcomes are now achieved in the vast majority of transplant recipients. Progressive improvement has occurred through better preoperative care of patients with liver disease, improved operative techniques that has allowed the donor pool to expand, and improved immunosuppression strategies to prevent rejection while avoiding complications of over-immunosuppression. The success of the past, however, has also bred unique challenges for the future. With the increasing number of liver transplant candidates, improved donor awareness and organ availability must occur. A delicate balance between the risks assumed by living donors and the needs of their children must be struck. The increasing numbers of surviving patients present unique challenges and complications related to lifelong immunosuppression. The future success of pediatric liver transplantation will require appreciation of the increasingly complex care needs of this population and a national focus on donor organ shortages.
The evaluation process
Collective experience suggests that the progression of chronic liver disease is not linear, but rather exponential, suggesting that early warning signs of hepatic compromise, such as deteriorating synthetic function or refractory nutritional failure should lead to prompt evaluation. In children with acute liver failure (ALF) or rapidly progressive decompensation of chronic disease, aggressive critical care intervention is essential to maintain all other physiologic systems until a suitable donor organ becomes available.
41 - Liver Transplantation in Children
- from SECTION V - OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
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- By Greg Tiao, M.D., Assistant Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Attending Surgeon, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Maria H. Alonso, M.D., Assistant Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Associate Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Frederick C. Ryckman, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
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- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 975-994
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Summary
Pediatric hepatologists and transplant surgeons have transformed the outcome of severe end-stage liver disease in children from hopelessness to success. Liver and combined multivisceral transplantation procedures have become the state-of-the-art treatment for these complex clinical problems, with anticipated success. The progressive improvement appreciated has been advanced through the use of innovative operative procedures using unique technical solutions in response to donor shortages. Although preoperative care advancements have significantly improved pretransplant morbidity, the full potential of improved transplant success has been limited by longer waiting lists and limited donor availability. Expanding indications for transplantation to children and adults with previously fatal diseases have increased this discrepancy. Parallel advances in critical care, immunosuppression, and postoperative management have also played a pivotal role in improved survival. However, the success of the past has bred unique problems that must be met in the future. If we are to succeed in meeting the needs of the increasing number of candidates, improved donor awareness and availability must occur. A delicate balance between the risks assumed by living donors and the needs of their children must be struck. The increasing numbers of surviving patients present unique challenges and complications related to lifelong immunosuppression. The future success of pediatric liver transplantation will require appreciation of the increasingly complex care needs of this population and a national focus on donor organ shortages.
THE SELECTION PROCESS
The primary aim of the evaluation process is to identify appropriate candidates for liver transplantation (LTx) and establish an effective pretransplant management plan.
12 - Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
- from SECTION II - CHOLESTATIC LIVER DISEASES
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- By William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Jorge A. Bezerra, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Ryckman C. Frederick, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
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- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 247-269
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Summary
Biliary atresia and related disorders of the biliary tract, such as choledochal cysts, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in the newborn (neonatal cholestasis). In this chapter, we review the current status of diagnosis and management of these disorders, as well as advances in the intriguing quest for an understanding of their pathogenesis.
OVERVIEW
Neonatal hepatobiliary diseases, including biliary atresia, choledochal cysts, and “idiopathic” neonatal hepatitis, have historically been viewed as a continuum – a gradation of manifestations of a basic underlying disease process in which giant cell transformation of hepatocytes is strongly associated with inflammation at any level of the hepatobiliary tract. These disease entities may be polar end points of a common initial insult, as originally stated in the unifying hypothesis of Landing [1]. The end result represents the sequela of the inflammatory process at the primary site of injury. Landing suggested that this inflammatory process may injure bile duct epithelial cells, leading to either duct obliteration (biliary atresia) or weakening of the bile duct wall with subsequent dilatation (choledochal cyst). The lesions may be dependent on the stage of fetal development when the injury occurs and the site within the developing hepatobiliary tree at which the injury occurs [1, 2]. A relationship of the pathogenesis of these obstructive cholangiopathies of infancy to the process of development is suggested by the association with disorders of situs determination such as the polysplenia syndrome and the observation of the so-called ductal plate malformation within the liver of a few patients with biliary atresia.
64 - Renal failure and transplantation
- from Part VII - Transplantation
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- By Maria H. Alonso, Department of Pediatric General and Thoracic Surgery, Transplantation Division, Cincinnati Children's Hospital Medical Center, OH, USA, Greg Tiao, Department of Pediatric General and Thoracic Surgery, Transplantation Division, Cincinnati Children's Hospital Medical Center, OH, USA, Frederick C. Ryckman, Department of Pediatric General and Thoracic Surgery, Transplantation Division, Cincinnati Children's Hospital Medical Center, OH, USA
- Edited by Mark D. Stringer, Keith T. Oldham, Pierre D. E. Mouriquand
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- Book:
- Pediatric Surgery and Urology
- Published online:
- 08 January 2010
- Print publication:
- 09 November 2006, pp 845-857
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Summary
Introduction
The management of end-stage renal disease, encompassing the principles of medical management, dialysis, and renal transplantation, formed the early experience with organ failure and replacement. The lessons learned were the basis for the exploding growth of solid organ replacement in children and adults. The care continuum for these patients can be divided into several phases: pretransplant medical management, transplantation, short- and long-term outcome. In this chapter, we will attempt to review the factors affecting success in medical pretransplant management, the transplant procedure, and throughout the short- and long-term follow-up periods. At each stage of care, factors which can determine the likelihood of success may be assigned (age, sex, ethnicity, primary disease, etc.). Some determinants of outcome are variable, that is, they are influenced by choices in care delivery (type of transplant, immunosuppression protocol, experience of transplant center, etc.). As the phases of transplantation are reviewed, we will attempt to identify avenues to improve outcome based on the application of current data.
Information reviewing the management of children with end-stage renal disease has been regularly evaluated and compiled in several large registries, allowing analysis of large cohorts. The United Network for Organ Sharing (UNOS)(www.unos.org) collects information on renal replacement in the United States, the United States Renal Data Systems (www.usrds.org) collects information regarding transplantation in Medicare patients.