春田 昭二
   Department   School of Medicine(Yachiyo Medical Center), School of Medicine
   Position   Clinical Professor
Article types Case report
Language English
Peer review Peer reviewed
Title Generalized Vascular Dissection on Pathological Examination in a Patient With Polycystic Kidney Disease and Acute Aortic Dissection.
Journal Formal name:Journal of the National Medical Association
Abbreviation:J Natl Med Assoc
ISSN code:19434693/00279684
Domestic / ForeginForegin
Volume, Issue, Page 111(5),pp.563-568
Author and coauthor Haruta Shoji, Gunji Kazue, Kawamura Toshiharu, Hiroshima Kenzo
Publication date 2019/10
Summary Polycystic kidney disease (PKD) is a multiple cystic disease involving both the kidneys. Some studies have reported cases of patients with PKD and concurrent aortic dissection; however, autopsy has been performed in only few of these cases. Here, we present the case of a 62-year-old male patient with PKD who showed generalized vascular degeneration, including aortic dissection. The patient had a family history of autosomal dominant PKD and was brought to our hospital because of cardiopulmonary arrest. He was diagnosed with Stanford type A aortic dissection and died on the same day, despite being under cardiopulmonary resuscitation. Autopsy detected multiple cysts in the kidneys, liver, pancreas, and testes. Moreover, multiple tears in the vascular wall of the splenic artery and superior mesenteric artery, including the aorta, were observed. The case findings indicate that patients with PKD may develop associated generalized vascular disease; however, development of cerebral aneurysms and aortic dissections with PKD is particularly serious. Therefore, suitable screening tests must be developed for the early diagnosis and disease characterization, thus, ensuring that the appropriate treatment is administered to the patients.
DOI 10.1016/j.jnma.2019.04.008
PMID 31147097