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2015 ; 18
(3
): 175-9
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Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation
during Infancy: A Single Center Study
#MMPMID26473137
Lee HP
; Kang B
; Choi SY
; Lee S
; Lee SK
; Choe YH
Pediatr Gastroenterol Hepatol Nutr
2015[Sep]; 18
(3
): 175-9
PMID26473137
show ga
PURPOSE: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as
biliary atresia and subsequently undergo Kasai operation. The purpose of this
study was to investigate the outcome of patients with AGS who had previously
received Kasai operation during infancy. METHODS: This retrospective study was
conducted at the Department of Pediatrics, Samsung Medical Center. We compared
the prognosis and mortality between those who had undergone Kasai operation
during infancy (Kasai group) and those who had not (non-Kasai group). RESULTS:
Among the 15 children with AGS, five had received Kasai operation, while 10 had
not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of
the non-Kasai group showed neonatal cholestasis. Liver transplantation was
performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the
non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai
group, and 10.0% (1/10) among the non-Kasai group (p=0.077). CONCLUSION: Although
overall mortality rate did not significantly differ between the two groups, the
proportion of patients receiving liver transplantation was significantly higher
in the non-Kasai group. The relatively worse outcome in AGS patients who had
received Kasai operation may be due to the unfavorable influences of Kasai
operation on the clinical course of AGS, or maybe due to neonatal cholestasis,
irrespective of the Kasai operation.