Non-cirrhotic portal fibrosis in children.
Article
Dans En
| IMSEAR
| ID: sea-64312
ABSTRACT
BACKGROUND AND OBJECTIVES:
Non-cirrhotic portal fibrosis (NCPF) is an infrequent cause of portal hypertension in children. We report 11 children with NCPF, from among 338 with portal hypertension, seen over 6.5 years.METHODS:
The diagnosis was based on patent splenoportal axis on ultrasonography and/or splenoportal venography and liver biopsy showing no evidence of cirrhosis or other diagnosis, in children with portal hypertension. Those with variceal bleed were managed with endoscopic sclerotherapy and/or shunt surgery.RESULTS:
The median age was 11 years (range 5 to 14), and 8 were boys. Presentation was with variceal bleed in 6, lump in left upper abdomen in 5 (though all children had splenomegaly) and esophageal varices on endoscopy. The median spleen enlargement was 8.5 cm; 8 also had hepatomegaly. Hypersplenism was present in 7, and two had developed ascites after bleed. Of 6 children presenting with bleed, variceal obliteration was achieved on sclerotherapy (average 5.6 sessions) in 4 while two underwent shunt surgery for associated hypersplenism. After median follow up of 57.5 months (range 12-78) all are alive and well.CONCLUSION:
NCPF is an uncommon cause of portal hypertension in Indian children. Presentation with variceal bleed is less common than in adults; sclerotherapy is effective.
Texte intégral:
1
Indice:
IMSEAR
Sujet Principal:
Système porte
/
Fibrose
/
Femelle
/
Humains
/
Mâle
/
Enfant
/
Enfant d'âge préscolaire
/
Études rétrospectives
/
Adolescent
/
Hypersplénisme
Type d'étude:
Observational_studies
langue:
En
Année:
2000
Type:
Article