About this book
In recent years there have been huge advances in the understanding of the genetic and molecular basis of the fibrocystic diseases. This volume provides a thorough review of fibrocyctic diseases that affect the liver. It contains in-depth discussions of the genetics, molecular biology, pathogenesis, histology, clinical presentations, complications of, treatment, and prognosis of the conditions affecting children and adults, and hence will be the gold-standard reference for these conditions. In addition, the histological features that distinguish these conditions from other potentially fibrosing hepatopathies are illustrated. Conditions with syndromic features involving the kidney or other organ systems are also reviewed. Thorough review of the clinical phenotypes, their presentations, treatment, potential complications of, and prognosis is discussed. Fibrocystic Diseases of the Liver will be an invaluable resource for hepatologists, gastroenterologists, nephrologists, and hepatic surgeons who care for children and adults with liver disease, as well as basic scientists in molecular genetics, hepatobiliary pathophysiology, hepatology and nephrology.
Gastroenterologists, hepatic surgeons, nephrologists and the pediatric component of these specialities
Table of contents
- Embryology and development of the ductal plate and renal tubule.
- Cholangiocyte biology as relevant to cystic disease.
- Biliary cilia and basal bodies.
- Genetics of cystic diseases of the liver and kidney.
- Ragiologic findings in the fibrocystic diseases.
- Pathology of fibrocystic diseases of the liver.
- ARPKD in infancy.
- CHF/Caroli syndrome/ARPKD
- Caroli disease.
- Choledochal cysts.
- Cystadenoma and Cystadenocarcinoma
- Simple hepatic cysts
- Echinococcal cyst
- Jag 1/Notch sequence and Alagille’s syndrome.
- Biliary atresia and the ductal plate.
- Nephronopthisis and Renal-hepatic-pancreatic dysplasia
- Joubert syndrome
- Meckel-Gruber, Bardet-Biedel, and Jeune syndromes
- Congenital disorder of glycosylation (PMM2)
- Complications: Vascular and Portal hypertension.
- Complications: Cholangitis and Cholangiocarcinoma.
- Treatment of the liver disease: shunts and transplantation
- Treatment of the renal disease