Primary Sclerosing Cholangitis (PSC)

1. Primary Sclerosing Cholangitis (PSC) By: Adriana Lopez

2. Primary Sclerosing Cholangitis (PSC) • Is a disease in the digestive system where the Bile Ducts are blocked & damaged outside & inside the liver. • The Bile Ducts are the tubes that carry out the Bile from the liver into the Gallbladder and Small Intestine. • The Bile is the liquid which is made inside the liver & helps break down fat in foods.

3. Progression of PSC • Inflammation of the Bile Ducts lead to scarring that narrows the bile ducts and if worsens; the scarring could in time lead to blocked Bile Ducts& that leads to Cirrhosis & Liver Failure since the bile cannot continue its routine.

4. Symptoms • The main symptoms of PSC are: • Itching of the Skin(may occur when toxins in bile get into the bloodstream) • Fatigue • Jaundice (Yellowing of the skin or whites of the eyes.) • An infection in the bile ducts can cause chills and fever. • In the United States 6 out of 100,000 people are diagnosed with PSC; average age of diagnosis is at 40(can also occur in children). PSC is more common in men; approximately 70% of PSC patients are men. • A person can have the disease for years before symptoms develop. **Having family members with PSC may increase a person's risk of developing PSC.**

5. AdvancedEffects • As the disease progresses: • chronic fatigue • loss of appetite • weight loss • In the advanced stages, swelling can occur in the abdomen and feet. Liver failure may take many years to develop.

6. Complications of PSC • Main complications that occur due to PSC. • These include: • Deficiencies in Vitamins A, D, E, & K • Infections of the Bile Ducts • Cirrhosis-Extensive scarring of the liver • Liver Failure • Bile Duct Cancer

7. Causes of PSC • The causes for PSC are not yet known. PSC could be Genetic, caused by an Immune System disorder, Bacteria, and or a Virus; all these may play roles in the development of the disease. • PSC is interconnected to Inflammatory Bowel Disease (IBD); 3 out of 4 people with PSC have a type of IBD called Ulcerative Colitis. The link between PSC and IBD is not yet understood.

8. Treatment • Although researchers have studied many treatments, none has been made known to cure or slow the progress of PSC. • Treatment of PSC targets the relieving of symptoms and is meant to manage complications. • Medical treatment may include • Various medications to relieve itching • Antibiotics to treat infections • Vitamin supplements • Instruments passed through an endoscope during ERCP can help open blocked bile ducts. • Liver transplantation may be an option if the liver begins to fail.

9. Effects of Primary Sclerosing Cholangitis (PSC) • Since this disease causes the deficiency of certain vitamins; it could cause other parts of the body to malfunction which can lead to deformities that at most times are permanent and cause life-long problems.

10. Diagnosis Duodenum • Blood tests to check levels of liver enzymes are the first step in diagnosing PSC. Cholangiography can be performed in the following ways: • Endoscopic Retrograde Cholangiopancreatography (ERCP). ERCP uses an endoscope—a long, flexible, lighted tube—that goes down the mouth, into the duodenum where dye is injected into the bile ducts. X rays are taken. • Percutaneous Trans Hepatic Cholangiography. Aneedle is inserted through the skin and places a thin tube into a duct in the liver. Dye is injected through the tube and x rays are taken. • Magnetic Resonance Cholangiopancreatography (MRCP). MRCP uses Magnetic Resonance Imaging (MRI) to take pictures of the bile ducts. MRI machines use radio waves and magnets. MRCP does not involve using x rays or inserting devices into the body. This safe and painless test is increasingly used for diagnosis. • Other testing may include ultrasound exams and a liver biopsy. • Ultrasound uses sound waves to create images of organs inside the body. • A biopsy involves removal of a small piece of tissue for analysis with a microscope.

11. Vitamins A, D, E & K • Vitamin A: Acts as an Antioxidant, Anti-Infection, Immunity Booster, helps avoid night blindness, helps to preserve eyesight(prevents cataracts), can decrease your chances of certain cancers, heart disease & stroke. • Vitamin E: Acts as a Skin Protectant, Cancer Prevention, Metabolism Booster, Prevention of Alzheimer's. • Vitamin K: Acts as a Blood clot reducer, reduce risk of Osteoporosis, Reduces the threat of bleeding in liver, & blood sugar regulator. • Vitamin D:Acts as a Calcium and Phosphorus Absorber, Arthritis Preventer, Blood Pressure Regulator, & Tension and Stress Reducer.

12. Vitamin D Deficiency • Vitamin D is one of four vitamins that lacks presence in the body; without Vitamin D in the body problems in the Skeletal & Muscular System could begin to develop. • Vitamin D allows the body to absorb Calcium(Ca2+) ; without this vitamin these two systems could not function properly thus bone growth, & the ability to contract muscles would not be possible because the natural processes would be interrupted.

13. Skeletal System Low Levels of Ca2+ Lack of Vitamin D Resorption(the breaking down of old bone) occurs when there is low Ca2+ in the body; osteoclasts break down the bone in order to put Ca2+ into the body. If Vitamin D is lacking in the body then it would be difficult for the body to collect Ca2+ from the small intestine; resulting in the breaking down of more bone to provide the body with more Ca2+. This excessive breaking down of bone leads to brittle bones which causes easy damage to the skeletal system. Causes pain for the person & constant visits to the hospital due to broken bones. • 1.Blood circulates through Parathyroid Gland, Ca2+below “set point” is detected. • 2.Parathyroid Gland releases Parathyroid Hormone(PTH) and PTH travels through blood. • 3.PTH travels through Haversian Canal to stimulate osteoclasts to work and break down the bone to release Ca2+ in the blood. • 4.Kidneys release Vitamin D to the small intestine and more Ca2+ is absorbed from digested foods(more Ca2+ in blood). • 5.Kidneys are also signaled to not release Ca2+ in urine which causes more Ca2+ in the blood.

14. Muscular System Steps to Muscle Contraction: Lack of Ca2+ If Vitamin D is not present in the body Calcium becomes low and affects the contraction of muscles. If the Ca2+ is not present then the Troponin cannot change its shape and it would not allow the Myosin to bind to the Actin. The body to becomes hard to control because of muscles are not being able to contract; causes the body to either become randomly or permanently limp. • 1.Brain sends nerve signals to the skeletal muscle releasing Aretylcholine to muscles, Ach then causes Ca2+ to be released to muscles. • 2.Ca2+ then binds to troponin, causing a change in shape, Tropomyosin then changes shape, rotating and revealing Actin binding site. • 3.This allows myosin to bind to Actin and pull Actin. ATP then releases Actin/Myosin so Myosin can bind to another Actin and this repeats. • 4. Ca2+ is then released from Troponin causing all molecules to go back to its original shape. Then Myosin cannot bind to Actin so the muscle is relaxed.

15. Works Cited • http://digestive.niddk.nih.gov/ddiseases/pubs/primarysclerosingcholangitis/index.aspx#complications • http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001330/ • http://www.antioxidantsdetective.com/benefits-of-vitamin-a.html • http://www.newsmax.com/fastfeatures/health-benefits-of-vitamin/2010/11/12/id/370865 • http://www.newsmax.com/FastFeatures/Health-benefits-vitamin-E/2011/02/01/id/369888 • http://www.healthdiaries.com/eatthis/6-health-benefits-of-vitamin-k.html