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Congenital respiratory-biliary fistula

Disease definition

Congenital respiratory-biliary fistula (RBF) is a rare developmental defect characterized by an anomalous connection of trachea or bronchus with left hepatic duct presenting with respiratory distress, recurrent respiratory infections and biliary expectoration or vomitus.

ORPHA:2040

Classification level: Disorder

Synonym(s): -
Prevalence: <1 / 1 000 000
Inheritance: Not applicable
Age of onset: Childhood
ICD-10: Q32.4
OMIM: -
UMLS: -
MeSH: -
GARD: 1475
MedDRA: -

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Congenital respiratory-biliary fistula

ORPHA:2040

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