SECTION I: PAT H O P H YS I O LO G Y O F PE D IAT R I C LIVER DISEASE
1. Liver Development: From Endoderm to Hepatocyte 3
Torsten Wuestefeld and Kenneth S. Zaret
2. Functional Development of the Liver 14
Frederick J. Suchy
3. Mechanisms of Bile Formation and Cholestasis 28
Saul J. Karpen
4. The Cholangiopathies 35
Valeer J. Desmet and Tania A. D. Roskams
5. Acute Liver Failure in Children 71
Estella M. Alonso, Robert H. Squires, and Peter F. Whitington
6. Cirrhosis and Chronic Liver Failure 97
Stephen Hardy and Ronald E. Kleinman
7. Portal Hypertension 138
Benjamin L. Shneider
8. Laboratory Assessment of Liver Function and Injury in Children 163
Vicky Lee Ng
SECTION II: CH O LE S TAT I C LIVER DISEASES
9. Approach to the Infant with Cholestasis 179
Frederick J. Suchy
10. Medical and Nutritional Management of Cholestasis in Infants and Children 190
Andrew P. Feranchak and Ronald J. Sokol
11. Neonatal Hepatitis and Congenital Infections 232
Philip Rosenthal
12. Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts 247
William F. Balistreri, Jorge A. Bezerra, and Frederick C. Ryckman
13. Neonatal Jaundice and Disorders of Bilirubin Metabolism 270
Glenn R. Gourley
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14. Familial Hepatocellular Cholestasis 310
Frederick J. Suchy and Benjamin L. Shneider
15. Alagille Syndrome 326
Binita M. Kamath, Nancy B. Spinner, and David A. Piccoli
16. Diseases of the Gallbladder in Infancy, Childhood, and Adolescence 346
James E. Heubi
SECTION III: HE PAT I T I S A ND IMMUNE DISORDERS
17. Acute and Chronic Viral Hepatitis 369
Jay A. Hochman and William F. Balistreri
18. Autoimmune Hepatitis 447
Giorgina Mieli-Vergani and Diego Vergani
19. Sclerosing Cholangitis 459
Nissa I. Erickson and William F. Balistreri
20. Drug-Induced Liver Disease 478
Eve A. Roberts
21. Liver Disease in Immunodeficiencies 513
Nedim Hadzi ́ ˇ c
SECTION IV: ME TA B O LI C LIVER DISEASE
22. Laboratory Diagnosis of Inborn Errors of Metabolism 531
Devin Oglesbee and Piero Rinaldo
23. α1-Antitrypsin Deficiency 545
David H. Perlmutter
24. Cystic Fibrosis Liver Disease 572
Andrew P. Feranchak
25. Inborn Errors of Carbohydrate Metabolism 595
Fayez K. Ghishan and Mona Zawaideh
26. Copper Metabolism and Copper Storage Disorders 626
Judith A. O’Connor and Ronald J. Sokol
27. Iron Storage Disorders 661
Alex S. Knisely and Michael R. Narkewicz
28. Heme Biosynthesis and the Porphyrias 677
Robert J. Desnick, Kenneth H. Astrin, and Karl E. Anderson
29. Tyrosinemia 694
Grant Mitchell, Pierre A. Russo, Jos ́ee Dubois, and Fernando Alvarez
30. The Liver in Lysosomal Storage Diseases 714
T. Andrew Burrow, Kevin E. Bove, and Gregory A. Grabowski
31. Disorders of Bile Acid Synthesis and Metabolism: A Metabolic Basis
for Liver Disease 736
Kenneth D. R. Setchell and Nancy C. O’Connell
32. Inborn Errors of Mitochondrial Fatty Acid Oxidation 767
Jerry Angdisen, Majed Dasouki, and Jamal A. Ibdah
33. Mitochondrial Hepatopathies 803
Ronald J. Sokol
34. Nonalcoholic Fatty Liver Disease 830
Jeffrey B. Schwimmer
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35. Peroxisomal Diseases 840
Paul A.Watkins and Kathleen B. Schwarz
36. Urea Cycle Disorders 858
Marshall L. Summar
SECTION V: OTHER CONDITIONS AND ISSUES IN PE D IAT R I C HE PATO LO G Y
37. Bacterial, Parasitic, and Fungal Infections of the Liver 871
Donald A. Novak, Gregory Y. Lauwers, and Richard L. Kradin
38. Systemic Disease and the Liver 897
Michael K. Farrell and John C. Bucuvalas
39. Fibrocystic Liver Disease 928
Maureen M. Jonas and Antonio R. Perez-Atayde
40. Tumors of the Liver 943
Dolores L ́opez-Terrada and Milton J. Finegold
41. Liver Transplantation in Children 975
Greg Tiao, Maria H. Alonso, and Frederick C. Ryckman
Index 995
Color plates appear after page 366
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