Settembre Ottobre 2021

ADPKD and intracranial aneurysms: indications for screening, follow-up and clinical management


Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary nephropathy and is the fourth most common cause for end-stage renal disease in Europe. ADPKD is a systemic disease; besides the typical renal involvement, characterized by progressive cyst expansion leading to massive enlargement and distortion of the kidney architecture and, ultimately, to end-stage renal disease, multiple extrarenal manifestations can be observed included cysts in other organs, diverticulosis, cardiac valvulopathies, abdominal and inguinal hernias, vascular anomalies. The rupture of an intracranial aneurysm is one of the most serious complications in ADPKD patients. Aim of this review is to provide useful indications for the clinician to define the risk of intracranial aneurysms in ADPKD population, to identify screening criteria (which patients to screen, how often and with which diagnostic methods), to estimate the risk of rupture of intracranial aneurysms, which may require intervention.

Keywords: ADPKD, intracranial aneurysms, screening, risk of rupture, treatment

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La malattia renale policistica autosomica dominante dell’adulto (ADPKD) è la più comune nefropatia ereditaria, con una prevalenza stimata tra 1/1000 e 1/2500 individui [1,2]. L’ADPKD rappresenta la quarta causa di end-stage renal disease (ESRD) per incidenza e prevalenza [3] e si stima che in Europa un paziente in dialisi su 10 sia affetto da ADPKD [4]. In Italia vi sono almeno 32000 pazienti affetti da ADPKD [5,6].

Oltre al noto coinvolgimento renale, in pazienti ADPKD si possono osservare con frequenza variabile coinvolgimenti extrarenali: cisti in altri organi (fegato, pancreas, milza, vesciche seminali, membrana aracnoidea), diverticolosi, valvulopatie cardiache (es. prolasso mitralico), ernie della parete addominale ed inguinali, anomalie vascolari (es. aneurismi intracranici, dilatazione della radice aortica e dissezione dell’aorta toracica, occlusioni dell’arteria centrale retinica) [79].


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