POSTER SESSION I POSTERS OF DISTINCTION THURSDAY, OCTOBER 24, 2002 5:30 PM–7:30 PM

FULMINANT HEPATIC FAILURE AND LIVER TRANSPLANTATION IN THE PEDIATRIC POPULATION Chris Rhee, Martin Martin, Ricardo Molina, Pediatric Gastroenterology and Nutrition, University of California, Los Angeles, Los Angeles, CA; Pediatric Gastroenterology and Nutrition, University of California, Los Angeles, Los Angeles, CA; UCLA Medical School, University of California, Los Angeles, Los Angeles, CA Background: In contrast to the adult population, the single most common cause of fulminant hepatic failure in the pediatric population is of unknown etiology and characteristically has a much poorer prognosis when compared to other causes. Aim: The aim of this study is to describe the clinical experience with pediatric patients presenting with fulminant hepatic failure at a major liver transplant program in order to further define the clinical characteristics and outcome of patients with this disorder. Methods: The experience with fulminant hepatic failure in the pediatric population (0-18 years of age) of 270 patients at UCLA from May 1985 to the present was reviewed as a historical cohort study. Factors studied include outcome, etiology, and seasonality. Results: Of the 270 patients, the majority of the causes were of unknown etiology (40%), acetaminophen (14%), hepatitis A (5.5%). 48% of all the patients went on to require liver transplantation. Of the group that were diagnosed with fulminant hepatic failure of unknown etiology, 48% of these patients progressed to liver transplantation and survived. 11% died status post transplantation. Without transplantation, 18% of the group survived and 23% died. This is a marked difference in contrast to those who had fulminant hepatic failure secondary to acetaminophen where 73% of the patients survived without requiring transplantation. 12% of these patients underwent transplantation with a mortality rate of 50%. Of the group with fulminant hepatic failure due to hepatitis A, 53% went on to transplantation and survived versus 20% who died post-transplant. In this group, there were no deaths in those without transplant. There is clearly an increased prevalence of cases of fulminant hepatic failure of unknown etiology in the winter months as compared to the summer months, which seem to support the theory of an underlying infectious viral etiology. The three months in which the greatest number of cases occurred were December, January, and November, respectively. A COMPARISON OF THE DEVELOPMENTAL OUTCOME OF CHILDREN TRANSPLANTED BETWEEN 1 AND 7 MONTHS OF AGE WITH CHILDREN TRANSPLANTED BETWEEN 7 AND 15 MONTHS OF AGE Karen I Wayman, Carlos O Esquivel, Kenneth L Cox, Pediatrics, Stanford University, Palo Alto, CA This study is a retrospective analysis of clinical data from 60 children diagnosed with liver disease in the neonatal period who received a liver transplantation. Thirty children were transplanted prior to 7 months of age and 30 children were transplanted between 7 and 15 months of age. Groups were matched for SES using the Hollingshead four-factor index. Children were excluded from the study if they were born prematurely (1 patient), were diagnosed with a neurological disorder or coexisting disease prior to transplantation (2 patients) or received a multiple organ transplant (4 patients). These study criteria controlled for age of disease onset, complications of premature birth and the effects of neurological disorders and other disease variables on developmental functioning. The Bayley Scales of Infant Development were used to measure motor and mental development for all children prior to transplantation and at 1 and 2 years following transplantation. Growth parameters were obtained routinely at admission to the liver transplant program and at standard intervals pre and post transplant. Additional data was collected on the total number of days of hospitalization in the period prior to and 3 months after transplantation. Results: For children transplanted prior to 7 months, there were no significant differences between the group means for cognitive development. Psychomotor development, however, was very different. For children transplanted prior to 7 months, the group mean for motor skills remained constant over time at −2D, dropping to almost −3D at 3 months post transplant. On the other hand children transplanted after 7 months of age showed a significant improvement over time functioning at −1SD at 1 year post transplant (78 +14) and low-normal development (87 +11.6) by 2 years post transplant. There are significant differences between the two groups for psychomotor development at 2 years post transplant (P < .05). Growth and development variables were correlated with developmental functioning at 1 and 2 years post transplant for children transplanted prior to 7 months of age. Weight and head circumference were found to be positively correlated to both mental and motor functioning (P < .05) while length of hospital stay was inversely related to motor development (P < .01) but not mental development. GASTROINTESTINAL COMPLICATIONS IN PEDIATRIC BONE MARROW TRANSPLANT PATIENTS Collin C Barker, J Decker Butzner, Ron A Anderson, Reginald S Sauve, Departments of Pediatrics and Community Health Sciences, University of Calgary, Calgary, AB, Canada Aim: To determine the frequency of biliary, hepatic, pancreatic and intestinal complications in pediatric bone marrow transplant (BMT) patients in the first 100 days post-transplant. Methods: A heterogeneous group of 142 pediatric BMT transplants, transplanted from January 1993 to June 2000 was assessed by chart review. Descriptive analysis was performed to identify the incidences and characteristics of biliary disease, veno-occlusive disease (VOD), acute graft-versus-host disease (GVHD), hepatic and intestinal infections, pancreatitis, diarrhea and non-specific gastrointestinal symptoms. Results: Biliary complications consisted of acalculus cholecystitis in 1 transplant and biliary sludging in 33. Biliary sludging was associated with jaundice, pancreatitis, vomiting requiring increased anti-nauseant therapy, right upper quadrant pain, generalized abdominal pain and TPN pre-transplant. VOD occurred in 26/142 transplants with a related mortality of 10/26 of which 3 were directly attributable to VOD. Acute GVHD occurred in a total of 65/142 transplants, with liver involvement in 25, intestinal involvement in 39 and skin involvement in 59. No episodes of infectious hepatitis were found. There were 4 episodes of clinical pancreatitis of which 2 were associated with biliary sludging. Diarrhea occurred in 95 transplants with viral infections accounting for 10, Clostridium difficile infections accounting for 11, GVHD diarrhea accounting for 39 and no specific cause was found in 35. Typhilitis complicated 5 transplants. All were treated successfully with conservative medical therapy. Nonspecific gastrointestinal symptoms including abdominal pain (101), vomiting (121) and mucositis (128) were common in this cohort. Conclusions: Biliary sludging occurred in 33% of transplants. Hepatic complications were primarily accounted for by VOD and acute GVHD. Diarrhea is a common finding in this cohort with GVHD in 41% but an infectious agent was identified in only 15% of cases. Clinical pancreatitis was uncommon but associated with biliary sludging. Nonspecific gastrointestinal symptoms were common and accounted for the vast majority of morbidity in this patient population. SIROLIMUS AS IMMUNE SUPPRESSIVE THERAPY IN CHILDREN WITH MALIGNANCY AFTER LIVER TRANSPLANTATION Carolina Jimenez, Yaron Avitzur, Annie Fecteau, Nicola Jones, Vicky Ng, Pediatric Academic Multi-Organ Transplant Program, The Hospital for Sick Children, Toronto, ON, Canada Background: Sirolimus is a new immunesuppressant with proven anti-proliferative effects in vitro and renal sparing effects in the clinical setting. Its properties provide a rationale for the use of sirolimus in selected children after liver transplantation. Objective: To review the use of sirolimus in pediatric liver transplant recipients at the Hospital for Sick Children in Toronto. Methods: Charts of all pediatric liver transplant recipients started on sirolimus between October 2000 and March 2002 were reviewed. Results: Eight patients received sirolimus, mean age 8.75 yr (range: 1yr and 13 yr). Seven children had confirmed evidence of malignancy with histologically documented post-transplantation lymphoproliferative disease (PTLD) (N=6) and hepatoblastoma (N=1). Two of the six patients with PTLD also had documented renal impairment and chronic rejection. The eighth patient received sirolimus for treatment of chronic rejection and renal impairment. Sirolimus dosages ranged between 1.5 mg and 5 mg (0.10 to 0.14 mg/kg/d) once daily. Mean duration of follow up was 12.3 months (4–17 mo). To date, the patient transplanted for hepatoblastoma has not had a recurrence and there was recurrence of PTLD in one patient. Children with chronic rejection showed a trend towards improvement in liver enzyme tests, with a reduction in aspartate aminotransferase from 323+/−123 U/L to 165+/−115 U/L (p=NS) and alanine aminotransferase from 402+/−151 U/L to 175+/−118 U/L (p=NS). Two patients were diagnosed with acute rejection while on sirolimus. Side effects occurred in 6 patients including mouth ulcer, leg swelling, dyslipidemia and bone marrow suppression. Side effects resolved either spontaneously or with dose reduction in most of the patients. Sirolimus was discontinued in two patients for persistent bone marrow suppression and for interstitial pneumonitis. Conclusions: Pediatric liver transplant recipients with malignancy may benefit from the inclusion of sirolimus in their immune suppression regimen. However, careful monitoring for refractory bone marrow suppression and interstitial pneumonitis is mandatory. Randomized controlled multi-center pediatric trials are now required to delineate long-term efficacy, safety and tolerability of sirolimus in the pediatric transplant population. CATATONIA PRESENTING AFTER LIVER TRANSPLANTATION IN A TEENAGER PATIENT-SUCCESSFUL MANAGEMENT WITH ELECTROCONVULSIVE THERAPY Robyn Gutman, Marla Gerrek, David Seaman, Christopher Siegel, Nora Mc Namara, Steve Czinn, Samra S Blanchard, Pediatric Gastroenterology, University Hospitals of Cleveland, Cleveland, OH; Transplant Surgery, University Hospitals of Cleveland, Cleveland, OH; Child and Adolescent Psychiatry, University Hospitals of Cleveland, Cleveland, OH There have been multiple reports in the literature regarding neurological complications following liver transplantation. We report a teenager girl who developed psychosis and catatonia following orthotopic liver transplantation and only responded to electroconvulsive therapy (ECT) to return to her normal neurological baseline. The patient was a 12-year-old girl who presented with a chief complaint of jaundice and lower extremity edema. Her physical exam revealed an icteric sclera, jaundice, lower extremity edema, mild abdominal distention with diffuse tenderness. She was alert and oriented with normal neurological exam. She had no significant past medical or psychiatric history. Her laboratory evaluation on admission showed a prothrombin time of 23 seconds, total bilirubin 12 mg/dl, direct bilirubin 6 mg/dl, albumin 2.2 gm/dl, alanine transferase 41U/L, aspartate transferase 109 U/L and gammaglutamyl transferase 129 U/L and alkaline phosphatase 162 U/L, low ceruloplasmin and significantly elevated 24 hour urine collection for copper. Her eye exam was normal. She was diagnosed with presumed fulminant Wilson disease and transplanted 12 days after initial presentation. She did not develop hepatic encephalopathy prior to transplantation. Her intraoperative course was unremarkable. She was placed on tapering dose of solumedrol, tacrolimus, mycophenolate, ranitidine, ursodeoxycholic acid, flucanazole,gancyclovir, vitamin K and pipracillin/tazobactam. She was extubated on post-transplant day of 1 and she had normal neurological exam. On post-operative day 9, she became combative with screaming and verbalizing suicidal ideations. She also had symptoms of paranoia. She was initially diagnosed with depression secondary to her medical condition. She was treated with antianxiety and different antipscyhotic drugs with no improvement. She had normal electrolytes and negative infectious disease work-up. She became catatonic with no spontaneous movement or speech. An EEG and MRI were performed and both were normal. Tacrolimus was discontinued with no change in her status. Because of no improvement of her neurologic status on postoperative day 32, the decision was made to proceed with ECT. She immediately became responsive and was able to answer questions. She received her second ECT within 3 days and was able to perform her daily activities with no residual symptoms. She had third session 3 days later, and she was back to her normal baseline. This is the first report of successful use of ECT in children for catatonia after solid organ transplantation. ECT is rarely used in pediatric population. It is as effective in treating catatonia caused by organic illness as due to the primary psychiatric disease. It should be considered as a treatment option in children with catatonia who is not responsive to medical management. USE OF RIBAVIRIN IN THE TREATMENT OF ADENOVIRUS CHOLANGITIS IN A CHILD AFTER LIVER TRANSPLANTATION Norberto Rodriguez-Baez, Arthur Weinburg, Jay Roden, Robert H Squires, Children's Medical Center & Southwestern Medical School, Dallas, TX Background: Adenovirus is an uncommon cause of infection after liver transplantation. It can lead to severe invasive disease and graft loss with 40–60% mortality rate. No uniformly effective therapy is available. Methods: Medical record was reviewed. Diagnosis of adenovirus infection in liver was made using immunohistochemical staining. Result: A 15-month-old female with biliary atresia received liver transplantation at 11 months of age. Initial immunosuppression consisted of Lymphocyte Immune Globulin for 5 days, FK 506, prednisone and oral acyclovir. One month after transplantation, acute rejection was treated with methylprednisolone (10 mg/kg/day) for 3 days. Mycophenolate mofetil was added with clinical response. Four months after transplantation, she was hospitalized with a 2-day history of fever, cough and abnormal liver tests (Table). Liver biopsy revealed expanded portal tracts with a lymphocytic infiltrate and eosinophils, necrosis and hystiocytic infiltrate in the bile ducts and large numbers of viral inclusions within the biliary epithelial cells. The infected cells were positive for adenovirus and negative for herpes simplex virus and cytomegalovirus. Adenovirus was cultured from stool and nasopharynx. Treatment included decrease in immunosuppression, Respiratory Syncytial Virus Immune Globin (RSV IG) for 2 days and a 7-day course of intravenous ribavirin: loading dose of 33 mg/kg followed by 16 mg/kg every 6 hours for 4 days, and then 8 mg/kg every 8 hours for 3 days. No adverse reactions were noted. Liver enzymes improved. Liver biopsy one month later revealed moderate cellular rejection without cholangiolar necrosis or adenovirus inclusions. Her immunosuppression regimen was adjusted and 2 months after her illness she had normal aminotransferase levels.TableConclusions: Treatment with RSV IG and ribavirin along with reduction in immunosuppression was associated with clearance of adenovirus from liver and biliary tree in a child after liver transplantation. WHEN SHOULD CHILDREN WITH ACETAMINOPHEN INDUCED LIVER TOXICITY BE TRANSFERRED TO A TRANSPLANT CENTER? Joel R Lim, Joseph M Croffie, Joseph F Fitzgerald, Sandeep K Gupta, Jean P Molleston, Mark R Corkins, Marian D Pfefferkorn, Division of Pediatric Gastroenterology/Hepatology/Nutrition, Indiana University/James Whitcomb Riley Hospital for Children, Indianapolis, IN Background: Acetaminophen induced liver toxicity accounts for a number of admissions or tansfer of patients to a liver transplant center. Most of these patients survive and never require a liver transplant. Aim: To determine what factors were predictive of increased risk of mortality in children with acetaminophen induced hepatotoxicity who were transferred to a liver transplant center. Methods: We reviewed the charts of children who were transferred to Indiana University Hospitals for possible liver transplant secondary to acetaminophen induced liver toxicity.The age, dose of ingestion, time of presentation and laboratory data (i.e. transaminases, coagulation studies, ammonia level and acetaminophen level) and final outcome were evaluated. Results: Fourteen patients were transferred over a period of 4 years (1998 to 2001) for possible liver transplant. Age range was 14 months to 18 years. There were 9 females and 5 males. All patients were seen within 48 hours of ingestion and were treated with N-acetylcysteine prior to transfer. The dosage ingested ranged from 115 − 692 mg/kg of acetaminophen. None of the patients received a liver transplant. Thirteen were discharged to home and one patient was listed for transplantation but eventually died without the transplant. When the patient that died was compared with the 13 survivors, a much greater elevation of the coagulation studies specifically the INR (5.3) and ammonia level (150 mcmol/L) were noted. The highest level of INR among the survivors was 3.7 while the highest level of ammonia was109 mcmol/L. Conclusion: Patients with acetaminophen toxicity can be managed at a peripheral hospital. Transfer to a transplant center should be considered if the ammonia is greater than 100 mcmol/L or if the INR is greater than 3.5. TABLETableIDIOPATHIC COLITIS FOLLOWING CARDIAC TRANSPLANTATION: TWO PEDIATRIC CASES Ghassan Wahbeh, Vera Hupertz, Maryanne Kichuk-Chrisant, Pediatric Gastroenterology & Pediatric The Cleveland, OH Pediatric patients who a transplant may have a of gastrointestinal symptoms. are to common common pediatric and infections secondary to We describe two cases where was documented with and biopsy following transplantation. 1 is a 7 year female with who had chronic vomiting and diarrhea that and 5 months after transplant. showed and positive She was treated with a course of with improvement and of on Her course was significant for related post transplant lymphoproliferative disorder and graft disease requiring two years and diarrhea and showed with no infectious etiology. 2 is an with and developed diarrhea 3 years after transplantation and was treated for Clostridium difficile without of showed and right with No infectious etiology was can from intestinal infections, disease, or graft versus disease of the cases to have symptoms therapy for identified presumed infection and significant immunosuppression mycophenolate, and post transplantation. The of an infection versus a be Conclusion: following pediatric transplantation is a cause of significant further study is to identify etiology and A IN TRANSPLANTATION S L K S C S M Pediatrics, & Surgery, of Virus & of An underwent transplant at age 11 months of inclusion disease with TPN associated biliary of 115 days after revealed chronic without rejection. adenovirus in the of disease. Diarrhea resolved by days after The patient was days after when diarrhea much severe than 100 Diarrhea for the days which time 3 and 7 graft persistent epithelial chronic increased in and but not and no viral inclusions. but no viral and and U/L, liver biopsy that showed portal and necrosis but no viral were In a for infectious etiology of and and were for by all were was from to < graft showed marked reduction in and had diarrhea and by 3 later days after had to and has that cause in children and second in frequency only to The present that may cause chronic with diarrhea in intestinal a The clinical and presentation and may be graft rejection. of in patients, including intestinal and frequency of liver involvement to be OF IN PEDIATRIC LIVER AND TRANSPLANT PATIENTS A J P Pediatric Gastroenterology, University of Medical Surgery, University of Medical are which when ingested provide and are as for use as however, to be is a which has in gastrointestinal disorders as viral and It is to and the immune Liver and transplant patients have after transplantation due to factors including use of following a provide a of normal is in transplant patients. The of safety of to patients with Methods: To the safety of in patients, patients in therapy was We identified transplant patients, mean age months, who received of on a daily for a of 3 Seven had liver transplants, 13 transplants, and 5 transplants. were performed the course of therapy for a of Patients for months following transplantation. Results: No episodes of or related infections were in of the patients. Conclusion: to be to to post-transplant patients their of controlled studies should be to the of therapy in the of and further following liver and intestinal transplantation in

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