Karen I. Wayman

Neurodevelopmental outcome of young children with extrahepatic biliary atresia 1 year after liver transplantation

STUDY DESIGN Forty children < 2 years of age receiving extrahepatic liver transplantation were tested with the Bayley Scales of Infant Development before transplantation and again at 3 and 12 months after transplantation. Neurodevelopmental status 1 year after transplantation was organized by a descriptive statistic of normal, suspect, or delayed. Disease and transplantation variables were investigated for association with delayed neurodevelopmental outcome. RESULTS Before transplantation mental development was in the low-average range (92 +/- 13.2) with psychomotor development 1 SD below the norm (82.5 +/- 13). Three months after transplantation both mental (80.1 +/- 12.6) and psychomotor (69 +/- 16.1) scores dropped 1 SD, but 1 year after transplantation mental and psychomotor scores recovered to the pretransplantation level of functioning. One year after transplantation 35% of the study group was diagnosed as developmentally delayed. Delayed development was associated with decreased weight (p < 0.04), low albumin (p < 0.02), length of hospital stay (p < 0.04), and age at transplantation (p < 0.05). CONCLUSION Young children undergoing liver transplantation are at risk for developmental delay. Aggressive nutritional support before transplantation and timing of transplantation before malnutrition develops may reduce developmental delays.

One hundred percent patient and kidney allograft survival with simultaneous liver and kidney transplantation in infants with primary hyperoxaluria: A single-center experience

Background. Combined liver-kidney transplantation is the definitive treatment for end-stage renal disease caused by primary hyperoxaluria type I (PH1). The infantile form is characterized by renal failure early in life, advanced systemic oxalosis, and a formidable mortality rate. Although others have reported on overall results of transplantation for PH1 covering a wide age spectrum, none has specifically addressed the high-risk infantile form of the disease. Methods. Six infants with PH1 underwent simultaneous liver-kidney transplantation at our center between May 1994 and August 1998. Diagnosis was made at 5.2±3.3 months of age, they were on dialysis for 11.8±2.3 months, and they underwent transplantation at 14.8±3.0 months of age when they weighed 10.6±1.7 kg. Results. At a mean follow-up of 6.4±1.7 years (range, 3.9–8.1 years), we report 100% patient and kidney allograft survival. There were no cases of acute tubular necrosis. Long-term kidney allograft function remained stable in all patients, with serum creatinine values of less than 1.1 mg/dL and a mean creatinine clearance of 99 mL/min/1.73 m2 at follow-up. Those who received combined hemodialysis and peritoneal dialysis pretransplant had lower posttransplant urinary oxalate values than those receiving peritoneal dialysis alone. There was improvement in growth and psychomotor and mental developmental scores after transplantation. Conclusions. Combined liver-kidney transplantation for the infantile presentation of PH1 is associated with excellent outcome when the approach includes early diagnosis and early combined transplantation, aggressive pretransplant dialysis, and avoidance of posttransplant renal dysfunction.

Non-adherence to post-transplant care: prevalence, risk factors and outcomes in adolescent liver transplant recipients.

Abstract:  This study examined the prevalence, demographic variables and adverse outcomes associated with non‐adherence to post‐transplant care in adolescent liver transplant recipients. We conducted a retrospective chart review of 111 adolescent patients (age 12–21 yr) greater than six months post‐transplantation and defined non‐adherence as not taking the immunosuppressive(s) or not attending any clinic visit in 2005. Fifty subjects (45.0%) were non‐adherent and 61 (55.0%) were adherent. Twenty percent of the subjects did not attend clinic and 10.9% did not complete laboratory tests. Non‐adherence was significantly associated with fewer completed laboratory tests (p < 0.0001), single parent status (p < 0.0186), and older age and greater years post‐transplantation by both univariate and multivariate analyses (p < 0.008, p < 0.0141 and p < 0.0012, p < 0.0174, respectively). Non‐adherence to medication was significantly associated with a rejection episode in 31 patients (p < 0.0069) but not in the subgroup of seven patients who stopped their immunosuppression completely. Non‐adherence to post‐transplant care is a prevalent problem in adolescents particularly of an older age and greater years post‐transplantation. Rejection was a significant consequence of medication non‐adherence except in a subgroup with presumed graft tolerance who discontinued their immunosuppression. These results emphasize the need for strict monitoring of adherence to post‐transplant care to improve long‐term survival and quality of life in adolescent transplant patients.

Adolescent non‐adherence: Prevalence and consequences in liver transplant recipients

Abstract: Few studies have examined the prevalence, demographic variables and adverse consequences associated with non‐adherence to immunosuppressive therapy in the adolescent liver transplant population. Our hypothesis is that a significant proportion of adolescent liver transplant recipients exhibit non‐adherence to medical regimens and that certain demographic and medical condition‐related characteristics can be identified as potential predictors of non‐adherent behavior. Furthermore, non‐adherence leads to a greater incidence of morbidity and mortality in this population as compared with the adherent subset of adolescent patients. We reviewed the charts of 97 patients from 1987 to 2002 who by December of 2002 had survived at least 1 yr post‐transplant and were followed by the Pediatric Liver Transplant Service at any point during their adolescent period (ages of 12–21). Non‐adherence was defined as documentation of a report of non‐adherence by a patient, parent or healthcare provider that was recorded in the patients legal medical record. Descriptive statistics were used to determine the prevalence, demographic variables and adverse outcomes associated with non‐adherence to immunosuppressive therapy. Categorical variables were analyzed using the chi‐square test or the Fisher exact probability test. The unpaired Students t‐test was used to analyze the continuous variable of age at transplant. Using the inclusion criteria, a total of 97 patients represented the study sample of whom 37 subjects (38.1%) were defined as non‐adherent and 60 (61.8%) were adherent. Non‐adherent subjects were more likely to be female, older (>18 yr) and from a single‐parent household. There was no significant difference in immunosuppressive regimen between non‐adherent and adherent patients. Non‐adherence was significantly (p<0.025) associated with lower socioeconomic status (SES), older age at transplant (p<0.005, 95% CI: −5.5 to −.99, Students t‐test) and episodes of late acute rejection (p<.001). Non‐adherence was also significantly associated with re‐transplantation and death secondary to chronic rejection by the Fisher exact test (p<0.006 and p<0.05, respectively). Non‐adherence to immunosuppressive therapy is a prevalent problem that is correlated with certain demographic and medical condition‐related risk factors and more frequent adverse consequences in the adolescent liver transplant population. The greater incidence of late acute rejection, death and re‐transplantation owing to chronic rejection in non‐adherent patients suggests that non‐adherence is significantly associated with an increased risk of morbidity and mortality. Further investigation to identify patients at greatest risk for non‐adherence is necessary to design the most effective intervention to increase patient survival and well being.

Long‐term outcome following pediatric liver transplantation for metabolic disorders

Stevenson T, Millan MT, Wayman K, Berquist WE, Sarwal M, Johnston EE, Esquivel CO, Enns GM. Long‐term outcome following pediatric liver transplantation for metabolic disorders.
Pediatr Transplant 2010:14:268–275.

Improved pain management in pediatric postoperative liver transplant patients using parental education and non-pharmacologic interventions.

Abstract:  A pain management intervention, consisting of pretransplant parental education and support, pre‐ and postoperative behavioral pediatrics consultation, postoperative physical and occupational therapy consultation, and implementation of non‐pharmacologic pain management strategies, was introduced to all pediatrics patients receiving liver transplants at Lucile Packard Childrens Hospital beginning August 2001. Children receiving transplants pre‐intervention (May, 2000 to February, 2001) and post‐intervention (August, 2001 to March, 2002) were compared using pain scores, parent perception of pain ratings, length of stay, ventilator days, total cost, and opioid use. A total of 27 children were evaluated (13 historical control, 14 intervention). The two populations did not differ on age at transplant (mean age 53.8 vs. 63.6 months), sex (46.1% vs. 50% male), ethnicity (53.8% vs. 57.1% white, non‐Hispanic) weight at transplant (17.5 vs. 24.7 kg), percent with biliary atresia as the primary reason for transplant (42.9% vs. 69.2%), percent with status 1 transplant listing score (38.5% vs. 50.0%), or public insurance status (30.8 vs. 57.2% with Medicaid). No differences were found in mean pediatric intensive care unit (PICU) postoperative length of stay (6.7 vs. 5.3 days), total postoperative length of stay (17.5 vs. 17.5 days), total inpatient length of stay (27.0 vs. 24.4 days), time to extubation (30 vs. 24.3 h), total cost (

Simulation‐Based Medical Error Disclosure Training for Pediatric Healthcare Professionals

147 983 vs.

Impact of an EMR-Based Daily Patient Update Letter on Communication and Parent Engagement in a Neonatal Intensive Care Unit.

157 882) or opioid use through postoperative day (POD) 6 (0.24 vs. 0.25 mg/kg/day morphine equivalent). A decrease in mean pain score between POD 0 and 6 (2.82 vs. 2.12; p = 0.047), a decrease in mean parental pain perception score (3.1 vs. 2.1; p = 0.001), and an increase in number of pain assessments per 12 h shift (3.43 vs. 6.79; p < 0.005) were seen. A comprehensive non‐pharmacologic postoperative pain management program in children receiving a liver transplant was associated with decreased pain scores, improved parent perception of pain, and an increased number of pain assessments per 12 h shift. No increases in lengths of stay (PICU, postoperative, total), time to extubation, or total cost were found.