A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent.

Abstract:

:Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to death. Treatment with interferon alpha might be effective.

journal_name

Eur J Pediatr

authors

Fonga-Djimi HS,Gottrand F,Bonnevalle M,Farriaux JP

doi

10.1007/BF01959789

subject

Has Abstract

pub_date

1995-10-01 00:00:00

pages

819-21

issue

10

eissn

0340-6199

issn

1432-1076

journal_volume

154

pub_type

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