Thanks to Scott for presenting a super interesting case of a young man with no known medical history who presented with altered mental status and was found to have cirrhosis and acute liver dysfunction likely due to a flare of previously undiagnosed perinatally acquired chronic hepatitis B! Pearls below:
- In a recent U.S. study, the most common cause of cirrhosis in adults <40 years old was alcohol!
- The risk of perinatal transmission of HBV has been drastically reduced by passive/active immunization within 12 hours of birth.
- Patients who acquire HBV via perinatal transmission and progress to chronic infection frequently do not manifest signs of liver disease for up to 30 years.
For those who want more info:
Cirrhosis in young patients:
Cirrhosis is rare in young patients, more common in young men compared to young women, and diagnosed more commonly in Caucasian and Hispanic patients than in African-American patients.
In a 1988 Indian study of 63 young cirrhotic patients of mean age 22 years (Sarin SK, Gut 1988;29:101-107), the most common causes were HBV (50%), followed by alcohol (10%), cryptogenic (19%), and rarely, Wilson disease or alpha-1-antitrypsin deficiency (1% each). Notably, HCV was discovered in 1989 so would have been part of the cryptogenic group in this study.
A 2014 U.S. study of 219 cirrhotic patients under the age of 40 (Sajja KC, J Investig Med 2014;62:920-926) showed that the most common causes of cirrhosis in patients <40 years old were alcohol (45%), HCV (33%), autoimmune (5%), cryptogenic/NASH (4%), HBV (4%), and PBC/PSC (2%). Notably, this study was single-hospital in Texas. The patients were 69% male, 41% Hispanic, 40% Caucasian, 14% African-American, and only 1% Asian/Pacific Islander, so the prevalence of vertically transmitted HBV is likely different than in the San Francisco population.
Chronic hepatitis B:
- Worldwide, 2 billion people have evidence of past or present infection, and 250 million are chronic carriers. In the U.S., prevalence of chronic HBV is <2%.
- Perinatal transmission: Rate of progression from acute to chronic HBV is 90% for perinatally acquired infection but <5% for adult acquired infection. Most transmission occurs at or before birth, but passive and active immunization within 12 hours of delivery has reduced transmission by >95%, and maternal antiviral therapy further reduces transmission risk. Breastfeeding has not been shown to increase transmission risk, and the decision to breastfeed should be based on patient preference.
- In chronic hepatitis B infection (including perinatal transmission, see diagram below), the immune tolerance phase lasts approximately 10-30 years before evidence of liver disease arises. Exacerbations begin to occur in the clearance phase and can mimic acute HBV infection if the infection was not previously diagnosed. Rarely, these exacerbations can result in liver decompensation and death.
- Prognosis is variable, but 5 year rate of progression from chronic hepatitis to cirrhosis is as high as 20% in some studies.
- The decision to treat chronic hepatitis B is complex but is overall based on cirrhosis status, liver dysfunction (ALT), and HBV DNA level. Goals of treatment are HBV DNA suppression, loss of HBV sAg, and loss of HBV eAg in patients who were initially eAg+. Tenofovir or entecavir are first-line treatments. Resistant virus is more likely to arise in patients failing therapy with lamivudine than with tenofovir or entecavir.
Have a great day everyone!