Primary Biliary Cirrhosis (cont.)

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What is the role of mathematical models in predicting the outcome (prognosis) in PBC?

Investigators at the Mayo Clinic performed statistical analyses of many variables (different types of data) among a large group of patients with PBC followed for many years. They used the results to derive a mathematical equation to calculate a so-called Mayo Risk Score (MRS). It turns out that the calculation is based on the results of three of the patient's blood tests (total bilirubin, albumin, and prothrombin time), the age of the patient, and the presence of enough fluid retention to swell the legs (edema) or abdomen (ascites). The Mayo Risk Score provides accurate information about the outcome (prognosis) of individual patients over time. It has been validated and is currently used to determine which patients with PBC need to be put on a liver transplant waiting list.

Physicians can rather easily calculate a Mayo Risk Score for their patients by going to the Internet site of the Mayo Clinic. There is no charge. The results provide an estimated survival for the patient over the next several years. Patients with an estimated life expectancy of 95% or less over one year meet the minimal listing criteria set by the United Network of Organ Sharing (UNOS) for liver transplant candidates.

What about pregnancy in PBC?

As discussed earlier, some women experience itching during their last trimester of pregnancy when the hormone levels of estrogens are high. A minority of these women may have a predisposition to develop PBC or may actually have early PBC that has not yet been diagnosed.

In the medical literature, pregnancy in women with an established diagnosis of PBC has not been reported frequently. While early reports suggested that the outcome was suboptimal for both fetus and mother, later reports indicated that women with PBC can deliver healthy babies. However, these women may develop itching or jaundice during the last trimester. Otherwise, the clinical course of PBC does not tend to worsen or improve during most pregnancies. Although some babies may be born several weeks prematurely, only one miscarriage has been reported. Furthermore, the risk of fetal abnormalities does not appear to increase in the pregnancies of PBC women.

Since advanced cirrhosis interferes with the processing (metabolism) of sex hormones, the likelihood of a woman with advanced liver disease becoming pregnant is small. Nevertheless, it is important to know that PBC patients who might become pregnant should not receive injections of vitamin A because it can cause birth defects (See the section on treatment of fat malabsorption). The chance that ursodeoxycholic acid therapy causes fetal harm is classified as remote but possible since adequate studies have not been done in pregnant women. The safety of ursodeoxycholic acid therapy taken by PBC mothers for their breast-feeding infants is unknown and considered controversial.

Medically reviewed by Stephanie Hawthorne, MD; American Board of Internal Medicine with subspecialty of Gastroenterology

REFERENCES:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2864616/
http://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-natural-history-of-primary-biliary-cirrhosis
http://www.ojrd.com/content/3/1/1
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2266722/
http://www.uptodate.com/contents/metabolic-bone-disease-in-primary-biliary-cirrhosis?source=see_link
http://www.uptodate.com/contents/overview-of-the-treatment-of-primary-biliary-cirrhosis
http://onlinelibrary.wiley.com/doi/10.1002/hep.510260508/pdf
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2758170/
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3488395/


Medically Reviewed by a Doctor on 1/9/2014

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