Research

• Estella Alonso, MD, is studying the outcomes in transplantation treatments in order to improve patients’ care. Her plan is enroll every single transplant recipient into the outcomes data collection and analysis system. Ideally, every patient’s care would follow a critical pathway, or treatment plan, that had been designed according to the best prevailing practices. The outcomes of a series of patients following a pathway would be analyzed to determine if the design is effective or could be improved upon. The pathway would then be altered accordingly and the outcomes of patients following the new protocol analyzed for improvement related to the change. Repeating this process would ideally result in fine-tuning and eventually perfecting the treatment plan. She is examining issues such as the variables affecting length of hospitalization, the impact of transplantation on a child’s long-term school performance, the value of retransplantation, and the effects of aggressive nutritional rehabilitation on a child’s physical and intellectual development. Another study involves psychologist Lisa Sorensen, PhD, who is working with Dr. Alonso on neurobehavioral outcomes in adolescents post-liver transplantation. They are exploring questions, such as whether there are different outcomes in relation to the particular disease process that may have caused hepatic encephalopathy that led to transplant, and the timing of that insult to the brain. They are also measuring the effects of the complex drug regimen, including immunosuppressants, on neurobehavioral functioning, as well as the effects of acute and chronic rejection. The study will compare patients between 10 and 16 years of age two-years post-transplant to healthy "best-friend" controls, and measure functions such as IQ, behavioral, mood, attention and executive skills, problem solving and organization. Sorensen also participates in research protocols in the stem-cell transplant program, testing patients for baseline neuropsychological functioning pre-transplant, with follow up as needed. Because these children often receive chemotherapy and / or radiation treatment in preparation for stem cell transplantation, they are at increased risk for neuropsychological deficits that usually do not become apparent until two or more years following treatment. Additional research studies on neurocognitive outcomes are being planned in other areas of organ transplant, including heart and kidney, as well.
• Dr. Karan Emerick’s special interest is the relationship of growth to cholestasis in children with Alagille syndrome (a rare inherited liver disorder) and other chronic cholestatic disorders. During her training, she helped examine the largest collection of Alagille patients in North America and created a national research database for their clinical information. Her work has culminated in a manuscript describing the largest, most complete group of Alagille patients in the literature to date (Hepatology, March 1999). She has also designed a study of the growth of children with Progressive Familial Intrahepatic Cholestasis (PFIC) in collaboration with colleagues at the Children’s Memorial Institute in Warsaw. The study involves the mechanism of growth in a large population of children with PFIC before and after treatment with partial cutaneous biliary diversion, which reverses liver disease and growth failure in many patients. Finally, she has begun the investigation of the effect of cholestasis on human chondrocytes in cell culture in collaboration with Dr. Humberto Soriano. These studies should provide invaluable insight into the reasons for growth failure in children with liver disease and approaches to treatment.
• Research by Dr. Riccardo Superina has focused on various aspects of pediatric liver disease, including outcomes of children following surgical therapy for biliary atresia and the surgical management of malignancies of the liver biliary ducts, an area in which he has received international recognition. His basic science research has focused on the field of transplantation tolerance. Using an animal model of transplantation, he is studying ways in which rejection can be modified without using conventional immunosuppressive medications.
• Dr. Peter Whitington’s curriculum vitae lists over 200 publications related to gastroenterology, liver disease and liver transplantation in children. He is a member of several editorial boards and national and international committees. He lectures worldwide on the subjects of liver disease in children and pediatric transplantation.