Document Detail

Hepatic manifestations of familial patent ductus venosus in adults.
MedLine Citation:
PMID:  10446116     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The ductus venosus connects the umbilical vein to the inferior vena cava during fetal life and subsequently closes rapidly after birth. It is known as patent ductus venosus when it remains patent in adulthood. PATIENTS: A 43 year old man with a history of panhypopituitarism presented with recurrent bouts of pedal oedema associated with fatigue, hypoalbuminaemia, and elevated prothrombin time. An ultrasound examination of his abdomen with Doppler revealed notable attenuation of the main portal vein with diminished intrahepatic branches; a computed tomography scan with angiography revealed a large collateral vein within the liver consistent with a patent ductus venosus. Sequential liver biopsies showed a considerable reduction in the calibre and number of the portal veins. His younger brother, who was diagnosed with alcohol related cirrhosis, suffered from intermittent bouts of encephalopathy and was found to have the same vascular lesion. A third brother was found to have a patent ductus venosus as well as two large hepatic masses consistent with focal nodular hyperplasia. CONCLUSION: The syndrome of familial patent ductus venosus has only previously been described in three infant brothers who presented with hepatic encephalopathy and fatty degeneration of the liver. This report documents three brothers with a patent ductus venosus presenting in adulthood with different manifestations of liver disease. The presence of the same vascular anomaly in three brothers is highly suggestive of a recessive genetic trait with an anatomical manifestation of patent ductus venosus.
S Jacob; G Farr; D De Vun; H Takiff; A Mason
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Gut     Volume:  45     ISSN:  0017-5749     ISO Abbreviation:  Gut     Publication Date:  1999 Sep 
Date Detail:
Created Date:  1999-09-28     Completed Date:  1999-09-28     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  2985108R     Medline TA:  Gut     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  442-5     Citation Subset:  AIM; IM    
Section of Gastroenterology and Hepatology, Ochsner Medical Institutions, 1520 Jefferson Highway, New Orleans, LA 70121, USA.
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MeSH Terms
Liver Diseases / etiology,  genetics*
Portal System / abnormalities*,  radiography
Portal Vein / abnormalities
Tomography, X-Ray Computed
Vena Cava, Inferior / abnormalities
Comment In:
Gut. 1999 Sep;45(3):329-30   [PMID:  10446096 ]

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