Document Detail

Combined portal and vena caval hypertension in Gaucher disease: The value of preoperative venography.
MedLine Citation:
PMID:  1185338     Owner:  NLM     Status:  MEDLINE    
Portal hypertension in Gaucher disease is unusual; the seventh known patient with this complication is reported. Prior to portacaval shunting in this child, a localized obstruction of the inferior vena cava at the subdiaphragmatic level was demonstrated by caval manometry and inferior vena cavography. At autopsy, centrilobular hepatic fibrosis seemed to be responsible for the portal hypertension. Nodular enlargement of the right and caudate lobes of the liver was the cause of the caval obstruction; elevated caval resistance may have contributed to the portal hypertension and possibly was responsible for failure of a portacaval anastomosis. The value of preoperative inferior vena cavography in addition to arterial portography in children with portal hypertension is stressed.
K E Fellows; R J Grand; A H Colodny; E N Orsini; A C Crocker
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  87     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1975 Nov 
Date Detail:
Created Date:  1976-01-16     Completed Date:  1976-01-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  739-43     Citation Subset:  AIM; IM    
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MeSH Terms
Child, Preschool
Gaucher Disease / complications*,  physiopathology
Hypertension / complications*,  physiopathology
Hypertension, Portal / complications*,  physiopathology,  surgery
Portacaval Shunt, Surgical
Portal Vein / radiography
Vena Cava, Inferior* / physiopathology,  radiography

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