Priority 10 from the Autosomal Dominant Polycystic Kidney Disease Project

UNCERTAINTY: What causes enlarged blood vessels (aneurysms) in some people with ADPKD and what is the most effective way to screen for and treat aneurysms? (Priority 10)
Overall ranking	10
JLA question ID	0103/10
Explanatory note	Not available for this PSP
Evidence	Cagnazzo, F., Gambacciani, C., Morganti, R. et al. Intracranial aneurysms in patients with autosomal dominant polycystic kidney disease: prevalence, risk of rupture, and management. A systematic review. Acta Neurochir 159, 811–821 (2017). https://doi.org/10.1007/s00701-017-3142-z ~ Zhou Z.a · Xu Y.b, c · Delcourt C.b, d · Shan J.e · Li Q.b · Xu J.a · Hackett M.L.b, c (2017) Is Regular Screening for Intracranial Aneurysm Necessary in Patients with Autosomal Dominant Polycystic Kidney Disease? A Systematic Review and Meta-analysis. Cerebrovasc Dis 44:75-82 https://doi.org/10.1159/000476073
Health Research Classification System category	Renal and urogenital

Extra information provided by this project
Original uncertainty examples	What is the natural history, including gene mutation, of intracranial aneurysm in people with ADPKD? ~ Development of a decision strategy addressing cost, safety and efficacy of magnetic resonance angiography (MRA) screening, for intracranial aneurysm in people with ADPKD, and for therapeutic approaches. ~ Which anti-hypertensive therapy is most effective for the prevention of intracranial aneurysm (ICA) in people with ADPKD? ~ Is systematic screening for intracranial aneurysm effective and efficient, in people with ADPKD, undergoing pre-transplant evaluation or major surgery? ~ Which therapeutic interventions including endovascular coiling are most effective for intracranial aneurysm in people with ADPKD, and how do the morbidity and mortality rates compare in the ADPKD and non-ADPKD population? ~ In children and young people with ADPKD what is the incidence of sub-arachnoid haemorrhage and the prevalence of intracranial aneurysm? ~ In adults, children and young people with ADPKD with a family history of intracranial aneurysm or sub-arachnoid haemorrhage does Intracranial Magnetic Resonance (MR) imaging reduce the risk of intracranial events?
Submitted by	2 x research recommendations and Kidney Disease Improving Global Outcomes (KDIGO)

Extra information provided by this project

Original uncertainty examples

What is the natural history, including gene mutation, of intracranial aneurysm in people with ADPKD? ~ Development of a decision strategy addressing cost, safety and efficacy of magnetic resonance angiography (MRA) screening, for intracranial aneurysm in people with ADPKD, and for therapeutic approaches. ~ Which anti-hypertensive therapy is most effective for the prevention of intracranial aneurysm (ICA) in people with ADPKD? ~ Is systematic screening for intracranial aneurysm effective and efficient, in people with ADPKD, undergoing pre-transplant evaluation or major surgery? ~ Which therapeutic interventions including endovascular coiling are most effective for intracranial aneurysm in people with ADPKD, and how do the morbidity and mortality rates compare in the ADPKD and non-ADPKD population? ~ In children and young people with ADPKD what is the incidence of sub-arachnoid haemorrhage and the prevalence of intracranial aneurysm? ~ In adults, children and young people with ADPKD with a family history of intracranial aneurysm or sub-arachnoid haemorrhage does Intracranial Magnetic Resonance (MR) imaging reduce the risk of intracranial events?

Submitted by

2 x research recommendations and Kidney Disease Improving Global Outcomes (KDIGO)

Project information
Project unique ID	0103
Project name	Autosomal Dominant Polycystic Kidney Disease
Total number of uncertainties identified by this project.	35 (To see a full list of all uncertainties identified, please see the detailed spreadsheet held on the JLA website)
Date of priority setting workshop	15 December 2020