Document Detail

Severe hypoplasia of the abdominal aorta and its branches in a patient and his daughter.
MedLine Citation:
PMID:  14687249     Owner:  NLM     Status:  MEDLINE    
We report the history of a patient and his daughter, both affected with hypoplasia of the abdominal aorta and its branches, leading to early and dramatic complications. In the index patient, renal ischaemia as a result of severe hypoplasia of the abdominal aorta and the origin of renal arteries led to progressive renal failure and end-stage renal disease at the age of 32 years. Other vascular abnormalities included hypoplasia of the celiac trunk (CT) and superior mesenteric artery (SMA). After a successful kidney transplantation at the age of 40 years, he eventually deceased following an episode of possibly ischaemic acute pancreatitis at 47 years. The patient's daughter suffered from an haemorrhagic stroke at the age of 7 years, which led to the discovery of severe hypertension caused by bilateral narrowing of renal arteries, as well as hypoplasia of CT, SMA, subclavian and pulmonary arteries. Biopsy of the narrowed renal artery of the daughter showed a particular form of fibrodysplasia characterized by an unusual fibrosis of the inner part of the media, just beneath the internal elastic lamina. To our knowledge, this is the first report of familial hypoplasia of the abdominal aorta. It might be the cardinal manifestation of a familial form of fibromuscular dysplasia (FMD). Interestingly, the histological lesions described in the daughter's renal artery differ from the classical form of medial FMD.
A Dejardin; P Goffette; P Moulin; R Verhelst; G Cornu; J F De Plaen; A Persu
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of internal medicine     Volume:  255     ISSN:  0954-6820     ISO Abbreviation:  J. Intern. Med.     Publication Date:  2004 Jan 
Date Detail:
Created Date:  2003-12-22     Completed Date:  2004-03-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8904841     Medline TA:  J Intern Med     Country:  England    
Other Details:
Languages:  eng     Pagination:  130-6     Citation Subset:  IM    
Department of Nephrology, Cliniques Universitaires St Luc (UCL), Brussels, Belgium.
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MeSH Terms
Abnormalities, Multiple / radiography*
Aorta, Abdominal / abnormalities*,  radiography
Aortic Coarctation / etiology,  radiography
Fatal Outcome
Fibromuscular Dysplasia / complications
Renal Artery / pathology

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