The Prevalence of Cerebral Aneurysms in Patients with Polycystic Liver Disease

Abstract

"Background: It is well known that patients with polycystic kidney disease (PKD) are at increased risk of developing cerebral aneurysms, however, this association has not been well studied for patients with polycystic liver disease (PLD). Material and methods: Cross-sectional descriptive study, which included 15 adult patients diagnosed with polycystic liver disease at the Gastroenterology and Hepatology Department of Fundeni Clinical Institute. Standard neurological exam and brain MRI were performed in all patients on a 1.5 Tesla MRI. Brain imaging protocol included T1/T2, T1SE, T2-FLAIR, DWI, SWI, 2D-TOF, 3D-TOF. Results: The majority of patients (93%) were females. The mean age was 53 ± 5 years old. Patients with AD-PKD and polycystic hepatic disease predominated (60%). Aneurysms were found in only one patient diagnosed with AD-PKD (in whom three aneurysms were described). The overall prevalence of cerebral aneurysms in our patient group was 7%. However, none of the patients with isolated PLD was found to have intracranial aneurysms. Other brain imaging abnormalities were frequent but nonspecific (mostly attributable to vascular-degenerative changes). Conclusions: Given these results, and due to the small number of patients in our study, it is hard to appreciate if polycystic liver disease is indeed associated with an increased risk of cerebral aneurysms. "

Transient D-Penicillamine-Induced Nephrogenic Diabetes Insipidus during Treatment of a Patient with Cystinuria <br/>—D-Penicillamine-Induced Nephrogenic Diabetes Insipidus

“…For many years, d-Penicillamine (d-P) has been used to treat rheumatoid arthritis, Wilson's disease and cystinuria without significant renal disease except for a few cases of transient membranous nephropathy and rare ones with crescentic glomerulopathy [2]. Transient DI, induced by d-P has been reported once in a patient, with Wilson's disease, yet did not recur on re-challenge [8]. In this case presentation, we report the second case of DI induced by d-P and further define its characteristics.…”