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- Title
Extracranial Aneurysms in 2 Patients with Autosomal Recessive Polycystic Kidney Disease.
- Authors
Elchediak, Daniel S.; Cahill, Anne Marie; Furth, Emma E.; Kaplan, Bernard S.; Hartung, Erum A.
- Abstract
Unlike autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD) is not generally known to be associated with vascular abnormalities. Only 4 cases of ARPKD patients with intracranial aneurysms have been reported previously. We present 2 ARPKD patients with extracranial vascular abnormalities: a young man with infrarenal aortic and iliac artery aneurysms complicated by dissection and a teenage girl with multiple splenic and gastric artery aneurysms and arterial vascular malformations. These cases raise the question of whether vascular integrity and development may be impaired in ARPKD, perhaps through molecular mechanisms overlapping with ADPKD. This possibility is supported by studies in mice that show ARPKD gene expression in the walls of large blood vessels.
- Subjects
ANEURYSMS; POLYCYSTIC kidney disease; HEPATIC fibrosis; PORTAL hypertension; GENE expression; LABORATORY mice; PATIENTS
- Publication
Case Reports in Nephrology & Dialysis, 2017, Vol 7, Issue 2, p34
- ISSN
2296-9705
- Publication type
Case Study
- DOI
10.1159/000475492