- Email: [email protected]
Su2067 Ulysses Syndrome Revisited in Pediatric Liver Transplant Patients
Su2067
(p < 0.25) in univariate analysis were subsequently included in a multivariate analysis using a stepwise(P < 0.05) forward Cox regression procedure. This study was approved by the Ethics Committee of our Hospital. Results: The mean follow-up was 7,45 months. The average of PMN was 2610.3 ± 3258.9 cells/ μL.. Culture was positive in 8 cases and E. coli was the most frequent organism cultured. All patients were treated with cefotaxime. Fourteen (73%) patients died and 8 (42%) underwent liver transplantation. Recurrence of ascites infection was observed in 5 patients. The cumulative probability of survival was 68% at 1 month, 29% at 3 months, 25% at 6 months and 10% at 12 months. Nineteen variables were selected for univariate analysis. Positive ascites culture, prolonged international normalized ratio (INR), low concentration of serum albumin and PMN count cell up to 900 cells/ μL were found to be related to the native liver survival. Cox regression analysis has showed that, for each increase in 100% in the INR value, loss of native liver has increased 242% and for each increase of 100% in serum albumin, the loss of the native liver has decreased 65% In the multivariate analysis, positive ascitic fluid culture was the most predictive factor of loss of native liver, followed by INR and serum albumin concentration. Conclusion: native liver survival after the first episode of SBP in pediatric patients is short and probably related with advanced liver dysfunction such as occurs in adult patients.
Ulysses Syndrome Revisited in Pediatric Liver Transplant Patients Ramya Ramraj, Julie M. Economides, Diesa Samp, Jacqueline M. Mackey, Kimberly A. Mackey, John A. Goss, Christine O'Mahony, Douglas S. Fishman 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 post- operative 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
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Introduction: rupture of esophageal and/or gastric varices (EV/GV) is a severe complication of portal hypertension and can be fatal. The standard diagnostic screening tool for EV/GV is endoscopy which is considered an invasive procedure in children. Aim: to evaluate clinical and laboratory parameters in predicting the presence of EV/GV in children with portal hypertension. Methods: eighty two children (46% female, media of age: 9.9 ± 4,6 years), 78 with chronic liver disease (25% biliary atresia) and 4 with extra-hepatic obstruction of portal vein (EOPV) were retrospectively recruited. All patients had no bleeding history and the indication of endoscopy was screening for EV/GV in all of them. The varices were classified in 3 degrees according to the caliber (small, medium and large). Patients who had EV/GV were divided in 2 groups: those with small EV/GV and those with medium and large EV/GV (patients who need prophylactic therapy).The clinical and laboratory parameters evaluated were severity of liver disease (PELD, Child-Pugh), liver function tests, APRI, platelet count, detection of splenomegaly by ultrasound followed by assessment of spleen length z score assessed by the same method. In addition, the Clinical Prediction Rule proposed by Gana et al ((0,75 x platelets / spleen length z score + 5) + 2.5 x albumin) was analyzed. Results: fifty nine children had esophageal varices in the first endoscopy (3 with EOPV). The presence of esophageal varices was correlated with two levels of platelet count: <100,000 (P=0.013 OR=4.27 IC95%: 1.4 - 13.0) and <130,000 (P=0.007 OR=4.14 IC95%: 1.5 - 11.7) and presence of splenomegaly (P=0.02 OR=12.5 IC95%: 1.3 - 119.3). The variceal caliber was not associated with clinical nor laboratory parameters. Conclusions: Presence of splenomegaly and platelet count <130,000 were associated with the presence of EV/GV but were not able to indicate which patients needed prophylactic therapy. Su2070 Patients With Cirrhosis Have a Higher Risk of Cholangitis and Related Mortality: Review of Nationwide Inpatient Sample Nilay Kumar, Muhammad Ali, Shahryar Ahmad, Gagan Kumar, Young Oh Introduction - Acute cholangitis poses a significant stress on the hepatobiliary system. Patients with cirrhosis have a significantly diminished hepatic reserve and also have decreased ability to handle severe systemic diseases. We looked at the frequency of cholangitis in patients with cirrhosis and their outcomes when compared to patients without cirrhosis. Methods We utilized the National Inpatient Sample (NIS) Database for this study. Primary diagnosis of acute cholangitis was identified using the appropriate International Classification of Diseases (ICD-9CM) codes. Patients with any secondary diagnosis of cirrhosis were also identified by the appropriate ICD-9CM codes. We calculated the frequency of acute cholangitis in these populations. Primary outcome evaluated was the mortality in these two groups. Statistical analysis was done using the Stata software using the Chi Square and multiple logistic regression analysis. A Wilcoxon rank-sum test was also performed. Results - There were total of 97,476 estimated patients with acute cholangitis. Out of these 8125 patients also had cirrhosis. The frequency of acute cholangitis in patients with cirrhosis was 1.16% as compared to 0.26% in controls (p=0.000). Also the mortality related to acute cholangitis was 7% as compared to 3% in controls (p=0.000). After logistic regression and adjusting for confounders cirrhotic patients had an Odds Ratio OR of 2.14; Confidence Interval CI 1.68-2.71. The mean length of stay LOS was 7.8 days in patients with cirrhosis as compared to 6.3 days in controls with (p-0.000) on Wilcoxon rank-sum test. Conclusion - Patients with cirrhosis not only have a higher frequency of acute cholangitis but also have a significantly higher mortality and length of stay associated with it. The pathophysiologic and anatomical changes associated with cirrhosis are likely the contributing factors in this observed difference. Patients with cirrhosis should be considered high risk population for acute cholangitis. The factors responsible for these differences warrant further investigation.
T- Tacrolimus, M- Mycophenolate, P- Prednisone Su2068 Native Liver Survival After Spontaneous Bacterial Peritonitis in Pediatric Patients With Cirrhosis Fernando P. Schwengber, Melina U. Melere, Carlos O. Kieling, Cristina Targa Ferreira, Jorge L. Santos, Marília R. Ceza, Maria Lúcia L. Zanotelli, Themis R. Silveira, Sandra M. Vieira Introduction: Spontaneous bacterial peritonitis (SBP) is a severe complication of cirrhosis and is related with a poor prognosis in adults. Presently there are no studies evaluating prognosis after SBP in the pediatric population. Aim: to determine the native liver survival after the first episode of SBP in pediatric patients with cirrhosis. Material and Methods: 19 patients were retrospectively studied. Eleven (58%) female, median age:1.0 years (range, 0.38-16.95 years);12(63%) biliary atresia; all patients presented Child-Pugh C. The diagnosis of cirrhosis was based on clinical, biochemical, ultrasound and/or histology findings. SBP was defined as an ascites fluid with polymorphonuclear (PMN) cell count>250 cells/μL in the absence of secondary peritonitis. Loss of the native liver was defined as patient death or liver transplantation. The cumulative probability of survival was calculated using the Kaplan-Meier method. A P equal to or smaller than 0.05 was considered significant. To identify independent predictors of survival, variables with a minimum 25% of significance
Mo1527 Defects of Cytochrome C Oxidase in Hepatitis B Virus Related Liver Cirrhosis Wey-Ran Lin, Chau-Ting Yeh, Cheng-Tang Chiu Background/aims: Defects of the respiratory chain, a typical feature of mitochondrial diseases, are caused by a loss of enzyme protein involving both nuclearly and mitochondrially coded subunits. It has been demonstrated that randomly distributed defects of complex IV (cytochrome c oxidase: COX) of the respiratory chain are expressed in human liver cirrhosis, however, the role of COX defects remains unclear. We aim to evaluate the effects of COX
S-987
AASLD Abstracts
AASLD Abstracts
Clinical Predictors of Esophageal and/or Gastric Varices Diagnosed by Endoscopy in Children With Portal Hypertension Marina R. Adami, Cristina Targa Ferreira, Carlos O. Kieling, Jorge L. Santos, Sandra M. Vieira
(p < 0.25) in univariate analysis were subsequently included in a multivariate analysis using a stepwise(P < 0.05) forward Cox regression procedure. This study was approved by the Ethics Committee of our Hospital. Results: The mean follow-up was 7,45 months. The average of PMN was 2610.3 ± 3258.9 cells/ μL.. Culture was positive in 8 cases and E. coli was the most frequent organism cultured. All patients were treated with cefotaxime. Fourteen (73%) patients died and 8 (42%) underwent liver transplantation. Recurrence of ascites infection was observed in 5 patients. The cumulative probability of survival was 68% at 1 month, 29% at 3 months, 25% at 6 months and 10% at 12 months. Nineteen variables were selected for univariate analysis. Positive ascites culture, prolonged international normalized ratio (INR), low concentration of serum albumin and PMN count cell up to 900 cells/ μL were found to be related to the native liver survival. Cox regression analysis has showed that, for each increase in 100% in the INR value, loss of native liver has increased 242% and for each increase of 100% in serum albumin, the loss of the native liver has decreased 65% In the multivariate analysis, positive ascitic fluid culture was the most predictive factor of loss of native liver, followed by INR and serum albumin concentration. Conclusion: native liver survival after the first episode of SBP in pediatric patients is short and probably related with advanced liver dysfunction such as occurs in adult patients.
Ulysses Syndrome Revisited in Pediatric Liver Transplant Patients Ramya Ramraj, Julie M. Economides, Diesa Samp, Jacqueline M. Mackey, Kimberly A. Mackey, John A. Goss, Christine O'Mahony, Douglas S. Fishman 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 post- operative 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
Su2069
Introduction: rupture of esophageal and/or gastric varices (EV/GV) is a severe complication of portal hypertension and can be fatal. The standard diagnostic screening tool for EV/GV is endoscopy which is considered an invasive procedure in children. Aim: to evaluate clinical and laboratory parameters in predicting the presence of EV/GV in children with portal hypertension. Methods: eighty two children (46% female, media of age: 9.9 ± 4,6 years), 78 with chronic liver disease (25% biliary atresia) and 4 with extra-hepatic obstruction of portal vein (EOPV) were retrospectively recruited. All patients had no bleeding history and the indication of endoscopy was screening for EV/GV in all of them. The varices were classified in 3 degrees according to the caliber (small, medium and large). Patients who had EV/GV were divided in 2 groups: those with small EV/GV and those with medium and large EV/GV (patients who need prophylactic therapy).The clinical and laboratory parameters evaluated were severity of liver disease (PELD, Child-Pugh), liver function tests, APRI, platelet count, detection of splenomegaly by ultrasound followed by assessment of spleen length z score assessed by the same method. In addition, the Clinical Prediction Rule proposed by Gana et al ((0,75 x platelets / spleen length z score + 5) + 2.5 x albumin) was analyzed. Results: fifty nine children had esophageal varices in the first endoscopy (3 with EOPV). The presence of esophageal varices was correlated with two levels of platelet count: <100,000 (P=0.013 OR=4.27 IC95%: 1.4 - 13.0) and <130,000 (P=0.007 OR=4.14 IC95%: 1.5 - 11.7) and presence of splenomegaly (P=0.02 OR=12.5 IC95%: 1.3 - 119.3). The variceal caliber was not associated with clinical nor laboratory parameters. Conclusions: Presence of splenomegaly and platelet count <130,000 were associated with the presence of EV/GV but were not able to indicate which patients needed prophylactic therapy. Su2070 Patients With Cirrhosis Have a Higher Risk of Cholangitis and Related Mortality: Review of Nationwide Inpatient Sample Nilay Kumar, Muhammad Ali, Shahryar Ahmad, Gagan Kumar, Young Oh Introduction - Acute cholangitis poses a significant stress on the hepatobiliary system. Patients with cirrhosis have a significantly diminished hepatic reserve and also have decreased ability to handle severe systemic diseases. We looked at the frequency of cholangitis in patients with cirrhosis and their outcomes when compared to patients without cirrhosis. Methods We utilized the National Inpatient Sample (NIS) Database for this study. Primary diagnosis of acute cholangitis was identified using the appropriate International Classification of Diseases (ICD-9CM) codes. Patients with any secondary diagnosis of cirrhosis were also identified by the appropriate ICD-9CM codes. We calculated the frequency of acute cholangitis in these populations. Primary outcome evaluated was the mortality in these two groups. Statistical analysis was done using the Stata software using the Chi Square and multiple logistic regression analysis. A Wilcoxon rank-sum test was also performed. Results - There were total of 97,476 estimated patients with acute cholangitis. Out of these 8125 patients also had cirrhosis. The frequency of acute cholangitis in patients with cirrhosis was 1.16% as compared to 0.26% in controls (p=0.000). Also the mortality related to acute cholangitis was 7% as compared to 3% in controls (p=0.000). After logistic regression and adjusting for confounders cirrhotic patients had an Odds Ratio OR of 2.14; Confidence Interval CI 1.68-2.71. The mean length of stay LOS was 7.8 days in patients with cirrhosis as compared to 6.3 days in controls with (p-0.000) on Wilcoxon rank-sum test. Conclusion - Patients with cirrhosis not only have a higher frequency of acute cholangitis but also have a significantly higher mortality and length of stay associated with it. The pathophysiologic and anatomical changes associated with cirrhosis are likely the contributing factors in this observed difference. Patients with cirrhosis should be considered high risk population for acute cholangitis. The factors responsible for these differences warrant further investigation.
T- Tacrolimus, M- Mycophenolate, P- Prednisone Su2068 Native Liver Survival After Spontaneous Bacterial Peritonitis in Pediatric Patients With Cirrhosis Fernando P. Schwengber, Melina U. Melere, Carlos O. Kieling, Cristina Targa Ferreira, Jorge L. Santos, Marília R. Ceza, Maria Lúcia L. Zanotelli, Themis R. Silveira, Sandra M. Vieira Introduction: Spontaneous bacterial peritonitis (SBP) is a severe complication of cirrhosis and is related with a poor prognosis in adults. Presently there are no studies evaluating prognosis after SBP in the pediatric population. Aim: to determine the native liver survival after the first episode of SBP in pediatric patients with cirrhosis. Material and Methods: 19 patients were retrospectively studied. Eleven (58%) female, median age:1.0 years (range, 0.38-16.95 years);12(63%) biliary atresia; all patients presented Child-Pugh C. The diagnosis of cirrhosis was based on clinical, biochemical, ultrasound and/or histology findings. SBP was defined as an ascites fluid with polymorphonuclear (PMN) cell count>250 cells/μL in the absence of secondary peritonitis. Loss of the native liver was defined as patient death or liver transplantation. The cumulative probability of survival was calculated using the Kaplan-Meier method. A P equal to or smaller than 0.05 was considered significant. To identify independent predictors of survival, variables with a minimum 25% of significance
Mo1527 Defects of Cytochrome C Oxidase in Hepatitis B Virus Related Liver Cirrhosis Wey-Ran Lin, Chau-Ting Yeh, Cheng-Tang Chiu Background/aims: Defects of the respiratory chain, a typical feature of mitochondrial diseases, are caused by a loss of enzyme protein involving both nuclearly and mitochondrially coded subunits. It has been demonstrated that randomly distributed defects of complex IV (cytochrome c oxidase: COX) of the respiratory chain are expressed in human liver cirrhosis, however, the role of COX defects remains unclear. We aim to evaluate the effects of COX
S-987
AASLD Abstracts
AASLD Abstracts
Clinical Predictors of Esophageal and/or Gastric Varices Diagnosed by Endoscopy in Children With Portal Hypertension Marina R. Adami, Cristina Targa Ferreira, Carlos O. Kieling, Jorge L. Santos, Sandra M. Vieira