What is congenital hepatic fibrosis?
Congenital hepatic fibrosis (CHF) is an inherited disease that both the liver and kidneys. The condition can cause the liver, kidneys and bile ducts to form abnormally before birth and may also cause fibrosis and scarring on the liver.
What is Fibropolycystic liver disease?
Fibropolycystic liver diseases are a unique group of entities that are thought to stem from a derangement of embryonic ductal plate development at various stages (,1–,4). These entities include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, and choledochal cysts.
Is Alagille syndrome detectable prior to birth?
How is Alagille syndrome diagnosed? Although Alagille syndrome is present from birth, it is often not diagnosed until later in childhood, particularly in less severe cases with few symptoms.
Is liver fibrosis hereditary?
The various syndromes that include congenital hepatic fibrosis can have different inheritance patterns. Most of these disorders are inherited in an autosomal recessive pattern , which means both copies of the associated gene in each cell have mutations.
Is liver fibrosis genetic?
Genetic Disorders Several genetic diseases predispose the liver to fibrosis (Scorza et al., 2014). In all of these diseases, the initiation of fibrosis begins with tissue injury due to a consequence of the genetic defect followed by a fibrogenic wound healing response, as discussed above.
What are the symptoms of polycystic liver disease?
GE The main symptom of polycystic liver disease is abdominal distension due to the large mass of cysts in the liver. Other symptoms that are also caused by the large bulk of cysts within the abdomen include shortness of breath, early postprandial fullness, abdominal discomfort, and back discomfort.
How do you treat polycystic liver disease?
The only definitive treatment of PLD, used in only the most severe cases, is liver transplant [2, 8, 33]. Medication to slow down cyst growth and fluid secretion in the liver (somatostatin analogs, namely octreotide and lanreotide) is also useful in reducing liver volume [7, 10].
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