Hanot Syndrome

Hanot Syndrome

Article
Digestive Health
Diseases & Conditions
+3
Contributed byKrish Tangella MD, MBAOct 07, 2020

The topic Hanot Syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition Primary Biliary Cirrhosis.

Quick Summary:

  • Primary Biliary Cirrhosis (PBC) is an autoimmune disease that is characterized by inflammation of the biliary duct (duct that carries bile from the liver to the small intestine). This condition usually affects the small and medium sized intrahepatic bile ducts
  • When inflammation and scarring of the bile duct occurs, the bile cannot be properly carried from the liver to the intestine. As a result, bile builds up in the liver (cholestasis) and causes the characteristic yellowing of the skin (jaundice)
  • The name Primary Biliary Cirrhosis is a misnomer (misleading term) for this condition. Although the term “cirrhosis” usually refers to a terminal condition, it may not be so in this case, as individuals with PBC are usually diagnosed early, even before liver cirrhosis (scarring of the liver) occurs
  • A majority of PBC cases are found in middle-aged women; and for some reason, the condition is more common in some European countries
  • A family history of PBC, smoking, and other factors increase one’s risk of developing the disorder Though the exact cause of Primary Biliary Cirrhosis is mostly unknown, researchers believe that it may be due to multiple factors, such as autoimmune mechanisms, genetic predisposition, and other unknown factors
  • Most patients are usually asymptomatic (without symptoms) and the diagnosis is suspected during blood tests for other conditions. Yellowing of the eyes, dark urine, itching, and diarrhea are commonly observed with Primary Biliary Cirrhosis
  • The treatment options available include nutritional supplements, symptomatic treatment (treating only the symptoms), ursodeoxycholic acid, and liver transplantation
  • Unfortunately, Primary Biliary Cirrhosis is a progressive condition that in most cases requires liver transplantation. This is not always curative. The average survival rate is 7-10 years after the onset of symptoms

Please find comprehensive information on Primary Biliary Cirrhosis regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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