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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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- Chapter
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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
-
- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 247-269
-
- Chapter
- Export citation
-
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.