Su2069 Clinical Predictors of Esophageal and/or Gastric Varices Diagnosed by Endoscopy in Children With Portal Hypertension

Abstract

Introduction: Marked transient elevations of alkaline phosphatase (ALP) are sometimes seen in children. This entity, otherwise called transient hyperphosphatasemia of childhood or Ulysses syndrome can lead to extensive testing without identification of a specific etiology. Delayed renal clearance with elevated macro enzymes or viral infections are possible etiological factors. While the exact physiology is unclear, it typically resolves over a period of weeks to months and no intervention is required. We report a cohort of pediatric liver transplant patients with isolated elevations of ALP without any other clinical or biochemical abnormalities. Methods: Medical records of patients who underwent orthotopic liver transplant (OLT) at Texas Children's Hospital between 2006 2011 were reviewed retrospectively. All patients with marked elevations of ALP (>690, normal range 145-320) were included. The diagnosis, age of transplant, date of alkaline phosphatase increase and the time taken to normalize or steadily decrease were obtained. In addition, aminotransferases, EBV titers, immunosuppressive regimen and use of steroids to account for possible causes of ALP elevation were recorded. Results: We identified 10 patients with isolated marked elevation of ALP without any other biochemical abnormality. The average value of ALP levels was 4360 and the mean time to normalize was 7 months. The mean age of occurrence was 36 months (median 22) and the timing of this phenomenon after liver transplant varied between 7-38 months. There were no changes in EBV titers or immunosuppressive regimens around the time of ALP increase. None had any documented fevers or a positive infectious workup during the time of the episode. The characteristics of the patients are shown in the table. Discussion: We identified Ulysses syndrome in 7% of 142 patients transplanted since 2006, but children under age three were affected more often. Patients post-OLT are closely monitored for postoperative complications like graft rejection, biliary complications or infection which requires frequent lab tests including liver panel and immunosuppressant drug levels. However, there are instances where we see isolated elevations of ALP without other abnormalities as in our patient group. No infectious etiology or changes in medications were identified in these patients. However, it is possible that patients might have had associated viral syndromes during the time of these episodes that could not be tested due to lack of a comprehensive viral panel. All of the episodes resolved without any interventions. Starzl et al had previously reported a similar phenomenon in the early liver transplant era that is still common in the tacrolimus era. It is essential for hepatologists to recognize this benign entity and thus avoid unnecessary and invasive testing. Table