John M. Vierling M.D. is Professor of Medicine and Surgery at the Baylor College of Medicine in Houston, Texas, where he also serves as Director of Baylor Liver Health and Chief of Hepatology. In addition, he is the Director of Advanced Liver Therapies, a center devoted to clinical research in hepatobiliary diseases at St. Luke's Episcopal Hospital. Dr. Vierling is board certified in internal medicine and gastroenterology and a Fellow of the American College of Physicians.
Dr. Schoenfield served as associate professor of medicine and consultant in gastroenterology on the faculty of the Mayo Clinic for seven years. He became a professor of medicine in residence at UCLA from 1972 to 1999 (now emeritus). He was the director of gastroenterology at Cedars-Sinai Medical Center in Los Angeles for 25 years, where he received the chief resident's teaching award, the president's award, and the pioneer of medicine award.
This phase is defined by a positive AMA, persistently abnormal liver blood
tests, and the presence of symptoms of PBC. The duration of this phase among
patients is also quite variable, lasting from 3 to 11 years.
In this phase, symptomatic patients develop the complications of cirrhosis
and progressive liver failure. The duration of this phase ranges from months to
2 years. These patients are at risk of dying unless they undergo successful
liver transplantation.
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.
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.
Gallstones are stones that form when substances in the bile harden. Gallstones (formed in the gallbladder) can be as small as a grain of sand or as large as a golf ball. There can be just one large stone, hundreds of tiny stones, or any combination. The majority of gallstones do not cause symptoms.
Hypothyroidism is any state in which thyroid hormone production is below normal. Normally, the
rate of thyroid hormone production is controlled by the brain at the pituitary.
Hypothyroidism is a very common condition and the symptoms of hypothyroidism are
often subtle.
Liver cancer is the fifth most common cancer in the world and the majority of patients with liver cancer will die within one year as a result. Patients with associated cirrhosis caused by chronic hepatitis B or C infections, alcohol, and hemochromatosis are at the greatest risk of developing liver cancer. Many patients with liver cancer do not develop symptoms until the advanced stages of the tumor which usually makes prognosis poor. The combination of an imaging study (ultrasound, CT, or MRI scans) and an elevated blood level of alpha-fetoprotein will most effectively diagnose liver cancer, while a liver biopsy can make a definitive diagnosis. Medical treatments, including chemotherapy, chemoembolization, ablation, and proton beam therapy, are not very effective. Surgical removal of the tumor or a liver transplant may be most effective in certain cases.
Cirrhosis of the liver refers to a disease in which normal liver cells are replaced by scar tissue caused by alcohol and viral hepatitis B and C. This disease leads to abnormalities in the liver's ability to handle toxins and blood flow, causing internal bleeding, kidney failure, mental confusion, coma, body fluid accumulation, and frequent infections. Symptoms include yellowing of the skin, itching, and fatigue.
Sjögren's syndrome is an autoimmune disease involving the abnormal production of extra antibodies that attack the glands and connective tissue. Sjögren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Though there is no cure for Sjögren's syndrome, the symptoms may be treated by using lubricating eye ointments, drinking plenty of water, humidifying the air, and using glycerin swabs. Medications are also available to treat dry eye and dry mouth.
Edema is the swelling of tissues as a result of excess water accumulations. Peripheral edema occurs in the feet and legs. There are two types of edema, non-pitting edema and pitting edema. Causes of pitting edema is caused by systemic diseases, most commonly involving the heart, liver, and kidneys. Local conditions that cause edema are thrombophlebitis and varicose veins. Edema is generally treated with medication.
Itching can be a common problem. Itches can be localized or generalized. There are many causes of itching to include: infection (jock itch, vaginal itch), disease (hyperthyroidism, liver or kidney), reactions to drugs, and skin infestations (pubic or body lice). Treatment for itching varies depending on the cause of the itch.
Jaundice is a yellowish staining of the skin and whites of the eyes (sclerae) with bilirubin, the pigment found in bile. Jaundice can be an indicator of liver or gallbladder disease, or it may result from the rupture of red blood cells (hemolysis).
Ulcerative colitis is a chronic inflammation of the colon. Symptoms include abdominal pain, diarrhea, and rectal bleeding. Ulcerative colitis is closely related to Crohn's disease, and together they are referred to as inflammatory bowel disease. Treatment depends upon the type of ulcerative colitis diagnosed.
Learn about osteoporosis, a condition characterized by the loss of bone density, which leads to an increased risk of bone fracture. Unless one experiences a fracture, a person may have osteoporosis for decades without knowing it. Treatment for osteoporosis may involve medications that stop bone loss and increase bone strength and bone formation, as well as quitting smoking, regular exercise, cutting back on alcohol intake, and eating a calcium- and vitamin D-rich balanced diet.
Scleroderma is an autoimmune disease of the connective tissue. It is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body, leading to thickness and firmness of involved areas. Scleroderma is also referred to as systemic sclerosis, and the cause is unknown. Treatment of scleroderma is directed toward the individual features that are most troubling to the patient.
The liver is the largest solid organ in the body, and is actually an gland. The liver has a wide variety of critical functions such as manufacturing proteins and metabolizing fats and carbohydrates. The liver also eliminates harmful biochemical waste products from the body (alcohol, drugs, toxins). The liver secretes bile that aids in digestion. Examples of diseases of the liver include cirrhosis, hepatitis, cancer, and fatty liver. Symptoms of liver disease include bleeding, easy bruising, edema, fatigue, and jaundice.
Digestion is the complex process of turning food you eat into the energy you need to survive. The digestive process also involves creating waste to be eliminated, and is made of a series of muscles that coordinate the movement of food.
Thyroiditis is the inflammation of the thyroid gland. The inflamed thyroid gland can release an excess of thyroid hormones into the blood stream, resulting in a temporary hyperthyroid state. Some forms of thyroiditis can be diagnosed based on tenderness and enlargement of the thyroid gland. A thyroid scan sometimes is used in making the diagnosis. Thyroiditis can also be diagnosed with a biopsy of the thyroid gland.
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