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Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Paucity of intrahepatic bile ducts in neonates: the first case series from iran
Zahmatkeshan Mozhgan1, Geramizadeh Bita, Haghighat Mahmood
1Shiraz University of Medical Sciences, Shiraz, Iran.
Insights
Paucity of interlobular bile ducts (PIBD) causes neonatal cholestasis. Liver transplantation is crucial for survival in infants with intractable cholestasis and Alagille syndrome (AGS), which can also cause kidney issues.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Neonatal Medicine
Background:
- Paucity of interlobular bile ducts (PIBD) is a significant cause of neonatal cholestasis.
- Persistent cholestasis leads to intractable pruritus, requiring management.
- PIBD can be a component of Alagille syndrome (AGS), a familial cholestasis disorder.
Purpose of the Study:
- To describe the clinical status of Iranian patients diagnosed with PIBD.
- To evaluate the outcomes of medical and surgical interventions for neonatal cholestasis due to PIBD.
Main Methods:
- Retrospective analysis of pediatric patients with cholestasis and liver biopsy diagnosis of PIBD.
- Evaluation of clinical and paraclinical data from January 2006 to January 2010 in Shiraz.
- Assessment of treatment responses and survival rates.
Main Results:
- The study included jaundiced neonates presenting at an average age of 3 days.
- Seven patients met the criteria for Alagille syndrome (AGS).
- Cholestasis persisted in 28.6% despite medical treatment; liver transplantation improved survival in 5 patients. One patient with renal insufficiency died at 9 months. Overall mortality was 9.5% after 1-5 years.
Conclusions:
- Liver transplantation is essential for survival in neonatal PIBD patients with intractable cholestasis.
- Intractable cholestasis is the primary indication for liver transplantation in neonatal PIBD.
- This study is the first to report neonatal-onset renal insufficiency associated with Alagille syndrome (AGS).
Objective:
Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of Iranian patients with PIBD.
Methods:
In this retrospective study, patients with cholestasis admitted to the pediatric gastroenterology ward in a referral hospital in Shiraz from January 2006 to January 2010 and underwent liver biopsy were evaluated. Clinical and paraclinical status of children with the pathologic diagnosis of PIBD was assessed.
Findings:
Disease was presented in all jaundiced patients with aged in average 3 days at presentation. Seven patients had the criteria of AGS. Despite medical treatment, cholestasis was not controlled in 6 (28.6%) patients. Liver transplantation led to the survival of 5 patients while the other patient who did not undergo liver transplantation died at 2 months of age. One patient underwent peritoneal dialysis due to renal insufficiency and died at 9 months of age. After 1-5 years of follow-up, the mortality rate was 9.5%.
Conclusion:
In patients with intractable cholestasis, only patients that underwent liver transplantation survived. Thus, the most important criterion for liver transplantation in neonatal PIBD is intractable cholestasis. This is the first report that shows AGS can result in neonatal-onset renal insufficiency.
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