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Long-term complications of arteriohepatic dysplasia
S J Schwarzenberg1, R M Grothe, H L Sharp
1Department of Pediatrics, University of Minnesota, Minneapolis 55455.
Insights
Arteriohepatic dysplasia, a form of biliary paucity, can lead to severe long-term complications and mortality in adulthood. Patients may experience hepatic failure, renal failure, and cancer, even after appearing stable in childhood.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Genetics
Background:
- Arteriohepatic dysplasia is characterized as a form of biliary paucity.
- Historically, it has been associated with a favorable prognosis.
Purpose of the Study:
- To investigate the long-term morbidity and mortality associated with arteriohepatic dysplasia.
- To assess the clinical course of patients with arteriohepatic dysplasia into adulthood.
Main Methods:
- Retrospective chart review of patients with arteriohepatic dysplasia followed into adulthood.
- Analysis of long-term outcomes, including complications, morbidity, and mortality.
Main Results:
- Six of 16 patients with syndromic paucity survived beyond 18 years.
- Five of these six patients developed severe complications after age 16, including hepatic failure, renal failure, cerebellar herniation, and hepatocellular carcinoma.
- Two patients died due to complications, and three experienced severe morbidity.
Conclusions:
- Arteriohepatic dysplasia is associated with significant long-term manifestations extending beyond childhood.
- Physicians must be aware of potential new abnormalities and hepatic disease deterioration in adulthood.
- Close monitoring is crucial for patients with arteriohepatic dysplasia as they transition to adult care.
Purpose:
It has been stated that arteriohepatic dysplasia is a form of biliary paucity with a good prognosis. We wished to determine the long-term morbidity and mortality associated with arteriohepatic dysplasia.
Patients And Methods:
The charts of all patients with arteriohepatic dysplasia followed by the pediatric gastroenterologists of the University of Minnesota into adulthood were reviewed.
Results:
Over the last 33 years, the pediatric gastroenterologists have followed 16 children with syndromic paucity, six of whom are now beyond age 18 years. Although five of six patients responded to medical therapy with improvement in their cholestasis and appeared stable clinically through childhood, five of six patients had complications of arteriohepatic dysplasia after age 16 years that resulted in severe morbidity (three) or death (two). These complications included hepatic failure (two), renal failure (one), cerebellar herniation (one), and hepatocellular carcinoma (one). In only one patient were symptoms of the complications present prior to the age of 18 years.
Conclusion:
As more patients with arteriohepatic dysplasia reach adulthood, it appears that this syndrome may be accompanied by long-term manifestations extending beyond childhood. It is important that physicians assuming management of these patients from pediatricians be aware that new abnormalities may appear without warning and that the hepatic disease may deteriorate despite apparent stability through childhood.