Abstract

Children with cystic fibrosis-associated liver disease (CFALD) and portal hypertension may require supplemental feeding with gastrostomy; however, this could lead to the development of stomal varices. We assessed this risk and nutritional and pulmonary outcome in a series of 7 children with CFALD and portal hypertension. In 35.1 patient-years of follow-up, none developed stomal varices or had a gastrointestinal bleed attributable to a varix. There was significant improvement in nutrition and lung function 2 years postinsertion. We conclude that gastrostomy placement for poor nutrition in children with CFALD and portal hypertension is safe and contributes to improved nutritional and pulmonary outcome.

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