Primary Sclerosing Cholangitis

Treatment of Children

The goal of Mayo Clinic's team of pediatric specialists is to find the most appropriate treatment for children with primary sclerosing cholangitis (PSC). Mayo Clinic gastrointestinal specialists treat many patients with this disease every year, and have made numerous contributions to PSC research, including one of the largest case studies of children with PSC ever reported.

Due to increased awareness about the disease, and the growing use of invasive cholangiography (used to view the bile ducts, which transport harmful toxins out of the liver), PSC has become more frequently diagnosed in the past few years. However, symptoms of PSC can often mimic other diseases, and children with PSC have experienced overlap with other diseases such as inflammatory bowel disease (IBD), or autoimmune hepatitis (AIH). If not diagnosed properly, or left undiagnosed, this disease will progress and can eventually cause liver damage. Treatment of PSC focuses on the use of medications to slow disease progression and prevent further liver damage.

Diagnosis

Physicians in Pediatric Gastroenterology and Hepatology evaluate each child's condition, utilizing blood, tissue and X-ray tests as appropriate. These tests help determine how advanced the disease is, and whether related diseases may be present (disease overlap). This information allows physicians to pursue appropriate treatment options.

Treatment Options

Treatment options for PSC are generally the same for children and adults.

PSC is typically treated with medications to control disease symptoms, such as itching, as well as vitamin and nutrient deficiencies. These medications are designed to stop disease progression and other harmful conditions that PSC can generate, such as bone disease.

If PSC has caused moderate liver damage, endoscopic procedures, such as complex endoscopy, may be used to un-plug bile ducts and restore bile flow from the liver. Stents (short, narrow tubes) may also be placed inside the bile ducts to keep open previously blocked passageways.

If the bile ducts cannot be reopened, and the patient is at risk of liver failure, a referral to the Pediatric Liver Transplant Program may be necessary; however, recent studies have shown that PSC in children may progress despite all treatment, including transplants.

Appointments

For appointments or more information, call the Central Appointment Office at (507) 538-3270 7 a.m. to 7 p.m. Central time Monday through Thursday, 7 a.m. to 5 p.m. Friday.