Bay Biosciences provides high quality, clinical grade bio-samples, cryogenically preserved biopsy tissue, FFPE tissue blocks, sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Primary Biliary Cholangitis (PBC) disease.
The PBMC, sera (serum), plasma biofluid samples are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The human Primary Biliary Cholangitis (PBC) bio-specimens are collected from unique patients diagnosed with Primary Biliary Cholangitis (PBC) disease and are provided to a valued pharmaceutical customer for research, development and drug discovery.
Primary Biliary Cholangitis (PBC) Overview
Primary Biliary Cholangitis (PBC), also known as Primary Biliary Cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Over time, this pressure build-up destroys the bile ducts leading to liver cell damage. As the disease progresses and enough liver cells die, cirrhosis and liver failure occur. Bile is produced in the liver and then transported through the bile ducts to the gallbladder and intestine where it helps digest fats and fat soluble vitamins (A, D, E, K). When the bile cannot be drained from the liver, it causes inflammation that leads to cell death. Scared tissue gradually replaces the areas of damaged liver and then the body cannot perform necessary functions.
Primary Biliary Cholangitis is considered an autoimmune disease, which means the body’s immune system is mistakenly attacks healthy cells and tissue. Researchers think a combination of genetic and environmental factors triggers the disease. It usually develops slowly.
Primary Biliary Cholangitis (PBC) Symptoms
More than half the people with Primary Biliary Cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons. Symptoms eventually develop over the next five to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes.
Common early symptoms of PBC include:
- Fatigue
- Itchy skin
- Dry eyes and mouth
Later signs and symptoms of PBC may include:
- Pain in the upper right abdomen
- Swelling of the spleen
- Bone, muscle or joint (musculoskeletal) pain
- Swollen feet and ankles (edema)
- Buildup of fluid in the abdomen due to liver failure (ascites)
- Fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees
- Yellowing of the skin and eyes (jaundice
- Darkening of the skin that’s not related to sun exposure (hyperpigmentation)
- Weak and brittle bones (osteoporosis), which can lead to fractures
- High cholesterol
- Diarrhea, which may include greasy stools (steatorrhea)
- Under-active thyroid (hypothyroidism)
- Weight loss
Causes of Primary Biliary Cholangitis (PBC)
It’s not clear what causes Primary Biliary Cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. The liver inflammation seen in Primary Biliary Cholangitis starts when certain types of white blood cells called T cells (T lymphocytes) start to collect in the liver. Normally, these immune cells detect and help defend against germs, such as bacteria. But in Primary Biliary Cholangitis, they mistakenly destroy the healthy cells lining the small bile ducts in the liver. Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue (fibrosis) that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for the liver to work properly.
Risk factors of Primary Biliary Cholangitis (PBC)
The following factors may increase the risk of Primary Biliary Cholangitis:
- Sex- Most patients with Primary Biliary Cholangitis are women.
- Age- It’s most likely to occur in people between 30 to 60 years old.
- Genetic factors- Persons more likely to get the condition if they have a family member who has or had it.
- Geography- PBC disease most common in northern Europe and North America.
Researchers think that genetic factors combined with certain environmental factors trigger Primary Biliary Cholangitis (PBC) disease. These environmental factors may include:
- Infections caused by bacteria, fungi or parasites
- Smoking
- Toxic chemicals
Complications of Primary Biliary Cholangitis (PBC)
As liver damage worsens, Primary Biliary Cholangitis (PBC) disease can cause serious health problems, including:
- Liver scarring (cirrhosis): Cirrhosis makes it difficult for the liver to work and may lead to liver failure. It indicates the later stage of Primary Biliary Cholangitis. People with Primary Biliary Cholangitis disease and cirrhosis have a poor prognosis (likely outcome) and higher risk of other complications.
- Increased pressure in the portal vein (portal hypertension): Blood from the intestine, spleen and pancreas enters the liver through a large blood vessel called the portal vein. When scar tissue from cirrhosis blocks normal blood flow through the liver, blood backs up. This causes increased pressure inside the vein. Also, because blood doesn’t flow normally through the liver, drugs and other toxins aren’t filtered properly from the bloodstream.
- Enlarged spleen (Splenomegaly): The spleen can become swollen with white blood cells and platelets because the body no longer filters toxins out of the bloodstream as it should.
- Gallstones and bile duct stones: If bile cannot flow through the bile ducts, it may harden into stones, causing pain and infection
- Enlarged veins (Varices): When blood flow through the portal vein is slowed or blocked, blood may back up into other organs, usually in the stomach and esophagus. Increased pressure may cause delicate veins to break open and bleed. Bleeding in the upper stomach or esophagus is a life-threatening emergency that requires immediate medical care.
- Liver cancer: Liver scarring (Cirrhosis) increases the risk of liver cancer.
- Weak bones (Osteoporosis): Patients with Primary Biliary Cholangitis (PBC) disease have an increased risk of weak, brittle bones that may break more easily.
- Vitamin deficiencies: A lack of bile affects the digestive system’s ability to absorb fats and the fat-soluble vitamins, A, D, E and K. Because of this, some people with advanced Primary Biliary Cholangitis (PBC) may have low levels of these vitamins.
- Decreased mental function (hepatic encephalopathy): Some patients with Primary Biliary Cholangitis with liver failure have personality changes and problems with memory and concentration.
- Increased risk of other disease: Primary Biliary Cholangitis (PBC) disease is associated with metabolic or immune system disorders, including thyroid problems, limited scleroderma (CREST syndrome) and rheumatoid arthritis.
Detailed clinical data, elevated levels of antibodies, serology, biomarkers, M2, genetic information, biopsy tissue, pathology annotations associated with the Primary Biliary Cholangitis (PBC) disease patient’s specimens is provided to a valued customer for diagnostics, development and drug discovery. The Primary Biliary Cholangitis (PBC) disease sera (serum), plasma and PBMC biofluid samples are processed from patients peripheral whole-blood using customized processing protocols provided by the researcher.
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Bay Biosciences provides human tissue samples (human specimens) from diseased and normal healthy donors; including peripheral whole-blood, amniotic fluid, bronchoalveolar lavage fluid (BAL), sputum, pleural effusion, cerebrospinal fluid (CSF), serum (sera), plasma, peripheral blood mononuclear cells (PBMC’s), saliva, Buffy coat, urine, stool samples, aqueous humor, vitreous humor, kidney stones, renal calculi, nephrolithiasis, urolithiasis and other bodily fluids from most diseases including cancer. We can also procure most human bio-specimens and can do special collections and requests of human samples that are difficult to find. All our human tissue samples are procured through IRB approved clinical protocols and procedures.
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