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Journal of Gastroenterology and Digestive Diseases | Volume 3
May 25-26, 2018 | New York, USA
World Liver Conference 2018
C
ystic fibrosis (CF) is a multisystem genetic disorder
caused by a defect in the cystic fibrosis transmembrane
conductance regulator (CFTR) gene, encoding the
transporter protein responsible for chloride flux across
the apical membrane of the epithelial cell. Significant liver
disease is an unusual presentation in CF population with the
incidence of 4-7%; however an emerging cause of mortality
as a result of advanced care in pulmonary, nutritional and
lung transplantation care. The cause of CFLD is unknown.
The presentation of CFLD varies widely in the CF population
with most liver disease occurring at or before puberty.
Although dual-pass liver biopsy increases diagnostic yield
with the presence of fibrosis, the distribution of fibrotic
liver lesions in CFLD is focal and yet the procedure is rather
invasive. Currently there are no known risk factors predicting
the development of CFLD. The development of non-invasive
fibrosis markers has progressed rapidly. Such markers
include hyaluronic acid, metalloproteinases, tissue Inhibitors
of matrix metalloproteinases (TIMPS), transforming growth
factor (TGF-β1), etc. These are not routine laboratory
investigations and tend to be expensive. In addition, they
may be elevated secondary to fibrosis in extrahepatic organs
such as the lungs. Many simple and inexpensive methods
such as APR, FIB-4, liver ultrasound have been used as a non-
invasive biochemical marker. Furthermore, in CF the role of
these tests to reflect CFLD is unclear but may help for early
detection of a child with CFLD. Novel therapies exist in CF
children. It is still unknown if any therapy can prevent the
development and progression of liver disease in CF children.
Speaker Biography
Wikrom Karnsakul is an Assistant Professor of Pediatrics at the Johns Hopkins University
School of Medicine. His clinical interests are in the care of pediatric liver diseases, and
general gastrointestinal diseases. He received his Medical degree in 1992 fromMahidol
University School of Medicine in Bangkok, Thailand. He completed his Residency in
Pediatrics at Advocate Hope Children’s Hospital, University of Illinois at Chicago in
1998 and did a fellowship in Pediatric Gastroenterology, Hepatology and Nutrition at
Texas Children’s Hospital, Baylor College of Medicine in Houston, Texas. He completed
his Post-doctoral research training at USDA/ARS Children’s Nutrition Research Center,
Baylor College of Medicine. He joined the faculty at Johns Hopkins University School of
Medicine in 2008. His research interests center on the understanding and treatment of
chronic liver disease, ascites, cholestasis, viral hepatitis, metabolic liver diseases and
living related liver transplantation. He has a particular focus on hepatitis E infection. He
is also involved in NIH-funded multicenter research studies including the Cholestatic
Liver Disease Consortium and Cystic Fibrosis Related Liver Disease Project.
e:
wkarnsa1@jhmi.eduNew perspectives in cystic fibrosis associated liver diseases in children
Wikrom Karnsakul
Johns Hopkins School of Medicine, USA