Cystic fibrosis associated liver disease in children
Joseph J Valamparampil1, Girish L Gupte2
1Liver Unit, Birmingham Children's Hospital, Birmingham B4 6NH, United Kingdom.
Abstract:
Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after pulmonary disease or lung transplant complications. We review the pathogenesis, clinical presentations, complications, diagnostic evaluation, effect of medical therapies especially CF transmembrane conductance regulator modulators and liver transplantation in CF associated liver disease.
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