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.
Cholesterol may deposit in the skin around the eyes or in skin creases of the
palms, soles, elbows, knees, or buttocks. Collectively, these waxy, raised
deposits are called xanthomas. Such deposits around the eyes are also referred
to as xanthalasma. Xanthomas are more common in PBC than in any other liver
diseases associated with cholestasis. Most xanthomas do not cause symptoms, but
those on the palms sometimes can be painful. Rarely, xanthomas deposit in nerves
and cause a neuropathy (disease of the nerve). This neuropathy is characterized
by abnormal sensation in the parts of the body, most often the limbs, supplied
by the affected nerves.
Although elevated levels of cholesterol in the blood are common in PBC and
other liver diseases with cholestasis, xanthomas are found in only 25% of
patients at the time of diagnosis. Xanthomas tend not to occur until the serum
cholesterol rises to very high levels, for example, above 600 mg/dL. Xanthomas
tend to spontaneously disappear in patients with advanced liver disease due to
impaired production of cholesterol by the damaged liver. Importantly, the high
levels of serum cholesterol in PBC do not seem to increase the risk of heart
disease because the composition of the cholesterol is different from the usual
cholesterol (atypical) and does not easily deposit in blood vessels.
As the amount of bile acids entering the gut decrease with increasing
cholestasis, patients can loose the ability to absorb all of the fat present in
their diet. This reduction in fat absorption, called malabsorption, occurs
because the bile acids are needed for the normal intestinal absorption of fat.
So, when advanced cholestasis prevents adequate amounts of bile acids from
reaching the small bowel, the absorption of dietary fat and of the vitamins A,
D, E and K is reduced. As a result, undigested fat passing into the large
intestine causes diarrhea, while continuing malabsorption of fat can lead to
weight loss and vitamin deficiencies. A laboratory measurement of the amount of
fat in the bowel movements can reveal whether the dietary fat is being absorbed
normally or not.
Vitamins A, D, E, and K, referred to collectively as the fat-soluble
vitamins, are absorbed from the gut in the same way that dietary fat is
absorbed. Therefore, deficiencies of these vitamins can occur in advanced
cholestasis. Also, bear in mind that some of the other conditions associated
with PBC, such as pancreatic insufficiency, celiac sprue, and scleroderma with
bacterial overgrowth, can also lead to malabsorption of fat and of the
fat-soluble vitamins. Prior to the development of jaundice, however,
deficiencies of vitamins A and E actually occur in only a minority of PBC
patients. Vitamin A deficiency causes decreased vision in the dark. Vitamin E
deficiency can cause abnormal skin sensations or muscular weakness due to its
effects on the nerves that extend from the spinal cord.
As already noted, deficiency of vitamin D results in osteomalacia (bones with
inadequate amounts of calcium deposited in them.) Deficiency of vitamin K
reduces the liver's production of blood clotting proteins and consequently,
causes a tendency to bleed easily. Also, the resulting deficiency of clotting
factors makes a blood test called the prothrombin time (blood clotting test) to
become abnormal. Prothrombin is a clotting factor that is produced in the liver
and needed for the normal clotting of blood. It is important to recognize that
the liver damage itself also can impair production of blood clotting factors and
cause easy bleeding and an abnormal prothrombin time.
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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