Meeting News Coverage

Ulysses syndrome common in pediatric liver transplant patients

SAN DIEGO — Ulysses syndrome was identified in 7% of 142 patients who underwent orthotopic liver transplant since 2006; however, children aged younger than 3 years were affected more frequently, according to findings presented here at the 2012 Digestive Disease Week Annual Meeting.

According to background information in the study abstract, marked transient elevations of alkaline phosphatase — otherwise known as transient hyperphosphatasemia of childhood or Ulysses syndrome — are sometimes observed in children and can lead to extensive testing without identification of a specific etiology. Although delayed renal clearance with elevated macro enzymes or viral infections are possible etiological factors, the exact cause is unclear because the condition typically resolves during a period of weeks to months, and no intervention is required.

“Many pediatricians often encounter this abnormal elevation of alkaline phosphatase when they send liver panels for various reasons, and occasionally they come back with isolated elevations of alkaline phosphatase yet the rest of the liver panel remains completely normal,” Ramya Ramraj, MD, pediatrician in Houston, told Infectious Disease News. “This elevation causes some pediatricians, and obviously parents, to become quite concerned. While we do not know exactly why this elevation occurs in growing children, especially after viral infections, it is important to note that this is a commonly observed entity.”

To investigate possible factors involved in isolated elevations of alkaline phosphatase without any other clinical or biochemical abnormalities, Ramraj and colleagues reviewed medical records of children who underwent orthotopic liver transplant at Texas Children’s Hospital between 2006 and 2011.

Children with marked elevations of alkaline phosphatase (.690 IU/L, normal range 145-320 IU/L) were included in the present study. Additionally, aminotransferases, immunosuppressive regimen and use of steroids to account for possible causes of alkaline phosphatase elevation were recorded.

The researchers identified 10 patients with isolated marked elevation of alkaline phosphatase without any other biochemical abnormality. The average value of alkaline phosphatase levels was 4,360 IU/L and the mean time to normalize was 7 months. The mean age of occurrence was 36 months, and the timing of this phenomenon after liver transplant varied between 7 months and 38 months. No noted changes were observed in immunosuppressive regimens around the time of alkaline phosphatase increase. No children had any documented fevers or positive infectious workup during the time of the episode.

“We thought we could observe this phenomenon better in liver transplant patients because there is always a concern regarding postoperative complications, such as graft rejection, biliary complications or infection that require lab tests,” Ramraj said. “However, there are instances, like in our study group, where we see isolated elevations of alkaline phosphatase without other abnormalities. It is important for pediatricians to recognize this entity when the rest of the liver panel is normal and there is no other cause for the elevation. At this point, it is essential to avoid invasive testing and intervention and simply observe the patient until the episode resolves.”

References:

  • Ramraj R. #Su2067: Presented at: the 2012 Digestive Disease Week Annual Meeting; May 19-22; San Diego.

Disclosures:

  • The researchers report no relevant financial disclosures.

SAN DIEGO — Ulysses syndrome was identified in 7% of 142 patients who underwent orthotopic liver transplant since 2006; however, children aged younger than 3 years were affected more frequently, according to findings presented here at the 2012 Digestive Disease Week Annual Meeting.

According to background information in the study abstract, marked transient elevations of alkaline phosphatase — otherwise known as transient hyperphosphatasemia of childhood or Ulysses syndrome — are sometimes observed in children and can lead to extensive testing without identification of a specific etiology. Although delayed renal clearance with elevated macro enzymes or viral infections are possible etiological factors, the exact cause is unclear because the condition typically resolves during a period of weeks to months, and no intervention is required.

“Many pediatricians often encounter this abnormal elevation of alkaline phosphatase when they send liver panels for various reasons, and occasionally they come back with isolated elevations of alkaline phosphatase yet the rest of the liver panel remains completely normal,” Ramya Ramraj, MD, pediatrician in Houston, told Infectious Disease News. “This elevation causes some pediatricians, and obviously parents, to become quite concerned. While we do not know exactly why this elevation occurs in growing children, especially after viral infections, it is important to note that this is a commonly observed entity.”

To investigate possible factors involved in isolated elevations of alkaline phosphatase without any other clinical or biochemical abnormalities, Ramraj and colleagues reviewed medical records of children who underwent orthotopic liver transplant at Texas Children’s Hospital between 2006 and 2011.

Children with marked elevations of alkaline phosphatase (.690 IU/L, normal range 145-320 IU/L) were included in the present study. Additionally, aminotransferases, immunosuppressive regimen and use of steroids to account for possible causes of alkaline phosphatase elevation were recorded.

The researchers identified 10 patients with isolated marked elevation of alkaline phosphatase without any other biochemical abnormality. The average value of alkaline phosphatase levels was 4,360 IU/L and the mean time to normalize was 7 months. The mean age of occurrence was 36 months, and the timing of this phenomenon after liver transplant varied between 7 months and 38 months. No noted changes were observed in immunosuppressive regimens around the time of alkaline phosphatase increase. No children had any documented fevers or positive infectious workup during the time of the episode.

“We thought we could observe this phenomenon better in liver transplant patients because there is always a concern regarding postoperative complications, such as graft rejection, biliary complications or infection that require lab tests,” Ramraj said. “However, there are instances, like in our study group, where we see isolated elevations of alkaline phosphatase without other abnormalities. It is important for pediatricians to recognize this entity when the rest of the liver panel is normal and there is no other cause for the elevation. At this point, it is essential to avoid invasive testing and intervention and simply observe the patient until the episode resolves.”

References:

  • Ramraj R. #Su2067: Presented at: the 2012 Digestive Disease Week Annual Meeting; May 19-22; San Diego.

Disclosures:

  • The researchers report no relevant financial disclosures.

    See more from Digestive Disease Week