Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting.

Abstract:

:Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18-year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension.

journal_name

Gastroenterology

journal_title

Gastroenterology

authors

Stern RC,Stevens DP,Boat TF,Doershuk CF,Izant RJ Jr,Matthews LW

keywords:

subject

Has Abstract

pub_date

1976-05-01 00:00:00

pages

645-9

issue

5 PT.1

eissn

0016-5085

issn

1528-0012

pii

S001650857600098X

journal_volume

70

pub_type

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