Expanding Options for Optimal Pediatric Liver Disease Management
Children from all over the country come to the Hepatology Program at Children’s Hospital of Pittsburgh of UPMC because of its wide spectrum of available services and treatment options. Benjamin L. Shneider, MD, program director, works closely with every family to determine each child’s best treatment approach.
For decades, physicians and researchers at Children's Hospital of Pittsburgh of UPMC have been dedicated to helping children with liver disease by exploring all treatment options —maximizing liver function, minimizing complications and maintaining growth and development—all to improve the quality of life for each child and family.
Careful assessment of the clinical status of children with liver disease is essential, particularly if the disease is something that is not commonly seen in the referring doctor's office, and not easily recognized.
Since biliary atresia affects only one in every 15,000 infants, in many cases, diagnosis is not made soon enough. (For optimal results, children with biliary atresia should undergo surgery before they reach 2 months of age.) Due to its infrequency, many physicians are not familiar with biliary atresia's warning signs. Symptoms occur between 2 weeks and 2 months of life and may include:
- Jaundice that persists beyond 3 weeks of age
- Dark urine
- Light-colored stools
- Distended abdomen
- Failure to thrive
The common finding of physiologic jaundice often makes early recognition of biliary atresia difficult.
Children's Hospital's Pediatric Hepatology Program features several of the country's leading experts focusing on the management of liver disease, including biliary atresia, that explore all treatment alternatives of medical and surgical care.
Children's Hepatology Program offers two of the nation's most highly respected pediatric hepatologists, both of whom also have extensive backgrounds in pediatric transplant hepatology. Benjamin L. Shneider, MD, director of the program, and Robert Squires Jr., MD, the clinical director, have more than 40 years of combined hepatology experience. They are members of the National Institutes of Health-funded Biliary Atresia Research Consortium, Cholestatic Liver Disease Consortium and the multicenter group to study acute liver failure in children.
Robert H. Squires Jr., MD, has considerable expertise in pediatric transplant hepatology, pediatric
gastroenterology and pediatric liver disease, as well as an interest in helping children with
liver disease avoid transplantation.
Drs. Shneider and Squires have a special interest in developing and utilizing novel therapies to help children with liver disease delay or avoid the need for transplantation.
Biliary atresia is an irreversible problem that is fatal without treatment. However, surgical intervention (the Kasai hepatoportoenterostomy) may allow a child with biliary atresia to live longer and have a better quality of life. Early referral to a pediatric liver specialist is essential.
"Jaundice is usually the first sign, and sometimes the only sign, of liver disease," said Dr. Shneider. "No medications can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Until that happens, biliary atresia will not be curable. However, there are things we can do that will allow the child with biliary atresia to live longer and have a better quality of life."
Partnering With Referring Physicians Is Essential
Children's Hospital's hepatologists can play a critical role in providing comprehensive therapeutic plans, consultations and second opinions as needed, on a short- or longterm basis.
"We believe a vital aspect to a child's recovery involves maintaining close, lifetime relationships with patients, families and referring physicians," said Dr. Shneider, who is board certified as a pediatric transplant hepatologist. "The benefits of a multidisciplinary, comprehensive team approach are vital to patient care. Working with referring physicians to provide the best long-term follow-up care and quality of life for patients is our top priority."
Children's offers easy accessibility to experts for any questions, a variety of referral resources to assist in the process, and a pledge to keep referring physicians informed throughout each stage of treatment.
Research Helping Improve Quality of Life
Children's Hospital of Pittsburgh of UPMC maintains an active hepatology research program that is focused on continuing to achieve advancements in understanding and treating liver problems, including developing safer and more effective therapies for the management of liver disease.
Some of the country's leading National Institutes of Health funded research grants are being conducted at Children's, with areas of focus including: biliary atresia and neonatal hepatitis, acute liver failure, alpha-1 antitrypsin deficiency, familial intrahepatic cholestasis and sclerosing cholangitis, and autoimmune hepatitis. Click here for a partial list of publications.
"Research and clinical care truly go hand in hand," Dr. Shneider said. "Some of the most important discoveries in the lab are the result of exploring ideas born from patient care experiences."
For more information about Children’s Hepatology Program, please visit www.chp.edu, or contact Dr. Shneider at benjamin.shneider@chp.edu.
