Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease.

نویسندگان

  • Mariusz Niemczyk
  • Monika Gradzik
  • Magda Fliszkiewicz
  • Andrzej Kulesza
  • Marek Gołębiowski
  • Leszek Pączek
چکیده

Autosomal-dominant polycystic kidney disease (ADPKD) is a relatively frequent genetic disorder that is associated with increased prevalence of intracranial aneurysms (IAs). However, evidence on the natural history of IAs in ADPKD is suboptimal. That leads to difficulties in development of recommendations on surveillance on patients with IAs in their medical history, or the need for repeat imaging for IAs in those with a negative result of the initial screening. The aim of the article is to present our experience on the natural history of IAs in ADPKD patients. MATERIAL AND METHODS Thirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis. RESULTS Among 8 patients with an IA in their medical history, no new IA was observed during 93 patient-years of follow-up. In 6 patients with untreated, unruptured IAs, IA growth was observed in 2 cases during 32 patient-years of follow-up. Finally, among 20 patients with a negative result of initial screening, 2 new IAs were noticed during 115 patient-years of follow-up, including 1 patient with a positive family history for an IA, and 1 patient without a family history. CONCLUSIONS Our observations support repeat imaging for IAs in patients with ADPKD, positive family history of IA, and negative result of initial screening. Additionally, efforts should be made to develop clinical and/or laboratory risk factors for IAs development in ADPKD patients without family history of IA, which enable to identify patients who should undergo repeat imaging for IAs.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

On the role of screening for intracranial aneurysms in autosomal dominant polycystic kidney disease.

Screening patients with autosomal dominant polycystic kidney disease (ADPKD) for asymptomatic intracranial aneurysms has been proposed as a method of reducing the morbidity and mortality associated with aneurysm rupture. However, recent studies have shown lower spontaneous rupture rates of small aneurysms and higher risks of significant complications with interventions than previously reported....

متن کامل

Is Regular Screening for Intracranial Aneurysm Necessary in Patients with Autosomal Dominant Polycystic Kidney Disease? A Systematic Review and Meta-analysis.

BACKGROUND The prevalence of intracranial aneurysm in patients with autosomal dominant polycystic kidney disease (ADPKD) is higher than that among the general population. We performed a systematic review and meta-analysis on the prevalence and natural history of intracranial aneurysm among patients with ADPKD. METHODS Medline, Embase, Web of Science and Scopus, from inception to July 2016, we...

متن کامل

Should patients with autosomal dominant polycystic kidney disease be screened for cerebral aneurysms?

Autosomal dominant polycystic kidney disease is a genetic disorder affecting 1 in 1000 people worldwide and is associated with an increased risk of intracranial aneurysms. It remains unclear whether there is sufficient net benefit to screening this patient population for IA, considering recent developments in imaging and treatment and our evolving understanding of the natural history of unruptu...

متن کامل

Polycystic Kidney Disease, Autosomal Dominant - GeneReviews® - NCBI Bookshelf

Autosomal dominant polycystic kidney disease (ADPKD) is generally a late-onset multisystem disorder characterized by: bilateral renal cysts; cysts in other organs including the liver, seminal vesicles, pancreas, and arachnoid membrane; vascular abnormalities including intracranial aneurysms, dilatation of the aortic root, and dissection of the thoracic aorta; mitral valve prolapse; and abdomina...

متن کامل

Comments, Opinions, and Reviews Neurovascular Manifestations of Heritable Connective Tissue Disorders A Review

262. Huston J III, Torres VE, Sulivan PP, Offord KP, Wiebers DO. Value of magnetic resonance angiography for the detection of intracranial aneurysms in autosomal dominant polycystic kidney disease. JAm Soc Nephrol. 1993;3:1871-1877. 263. Kaehny W, Bell P, Earnest M, Stears J, Gabow P. Family clustering of intracranial aneurysms (ICA) in autosomal dominant polycystic kidney disease (ADPKD). ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • The New England journal of medicine

دوره 327 13  شماره 

صفحات  -

تاریخ انتشار 1990