Overview of Congenital Hepatic Fibrosis in Pediatrics: A Review
Ahmed Abdelsamie Fadl *
Doctor Samir Abbas Hospital, Saudi Arabia and Department of pediatrics Alazhar Unversity Hospitals, Cairo, Egypt.
Sarah jamal almujil
KAUH, Saudi Arabia.
Ahmed Saeed S. Banheem
King Abdullah Medical Complex Jeddah, Saudi Arabia.
Nasser Naif Alsuhaymi
Al-Rayan Colleges, Medina, Saudi Arabia.
Mansour Hemaid Alhelali
Medical Rehabilitation Hospital, Saudi Arabia.
Dhiyaa Hassan A. Aldukhi
Medical Graduate, Saudi Arabia.
Khalil Rafed B. Alsaedi
Medical Rehabilitation Hospital, Saudi Arabia.
Naif Mohammed Al-Wagdani
King Abdulaziz Hospital, Jeddah, Saudi Arabia.
Ebtesam Ali S. Almadi
Qassim University, Saudi Arabia.
Khalid Mohammed A. Aladhadh
King Saud bin Abdulaziz University for Health Sciences, Saudi Arabia.
Fahad Amrh AlAjmi
Farwaniyah Hospital, Kuwait.
*Author to whom correspondence should be addressed.
Abstract
Congenital hepatic fibrosis is a rare developmental illness caused by a ductal plate malformation, often known as ciliopathy or fibrocystic liver disease. Hepatosplenomegaly and portal hypertension are two symptoms. The disease affects 1/10000–20000 people. frequently associated with a variety of illnesses caused by genetic abnormalities, such as autosomal recessive polycystic kidney disease (ARPKD) and Caroli syndrome. There hasn't been a way to stop or reverse the progression of congenital hepatic fibrosis until now. Clinical trials of anti-fibrotic medicines such as colchicine, interferon gamma, angiotensin II receptor blockers, pirfenidone, and ursodeoxycholic acid found no significant benefit. The only known cure for CHF is liver transplantation, which is recommended when the condition has progressed to the point when symptoms of liver failure have appeared. In this article we will be making overview of the disease. It’s symptoms and diagnosis, different treatment method, and we will compare some of the articles published about the disease.
Keywords: Congenital hepatic fibrosis, genetic abnormalities, autosomal recessive polycystic kidney disease