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MODERN EXPERIENCE WITH INFANTILE CHOLESTASIS. A 12 YEAR REVIEW
M Stormon, SFA Dorney, KR Kamath, EV O’Loughlin, KJ Gaskin
Department of Gastroenterology, Royal Alexandra Hospital for Children, Sydney, Australia
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CDDW Abstracts
MODERN EXPERIENCE WITH INFANTILE CHOLESTASIS. A 12 YEAR REVIEW M Stormon, SFA Dorney, KR Kamath, EV O’Loughlin, KJ Gaskin Department of Gastroenterology, Royal Alexandra Hospital for Children, Sydney,
Australia
090
Background
Cholestatic liver disease in infancy is caused by a wide range of conditions. This study documents the increasing number of specific diseases, particularly metabolic and genetic diseases, presenting as infantile cholestasis at a tertiary referral paediatric hospital in Sydney, Australia, over a twelve year period (1985-1996).
Methods
Infants less than six months of age were retrospectively identified from hospital records. Data were extracted using a newly established database.
Results
There were 157 infants identified as having cholestatic liver disease. An additional 45 infants with cholestasis due to total parenteral nutrition were not included in the study. The aetiology of the cholestasis was idiopathic in 31%, obstructive in 26% and metabolic in 24%. Other causes included infection (14%) and bile duct hypoplasia (5%).
Conclusions
Due to improvements in the investigation and identification of specific conditions causing infantile cholestasis, an increasing number of metabolic and genetic diseases are now recognised as presenting with cholestasis in infancy. This study reflects these improvements and also documents the wide range of disorders causing cholestatic liver disease.