Intracranial cysts in autosomal dominant polycystic kidney disease

1995 ◽  
Vol 83 (6) ◽  
pp. 1004-1007 ◽  
Author(s):  
Wouter I. Schievink ◽  
John Huston ◽  
Vicente E. Torres ◽  
W. Richard Marsh
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Arachnoid Cysts ◽  
Control Group ◽  
Polycystic Kidney ◽  
Content Type ◽  
Intracranial Cysts ◽  
Autosomal Dominant Polycystic Kidney ◽  
Fine Print

✓ Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well known for its association with intracranial aneurysms. A series of patients with ADPKD who were screened for the presence of an intracranial aneurysm were reviewed and found to include an unexpectedly high number with intracranial arachnoid cysts. Among 247 patients with ADPKD who underwent magnetic resonance imaging (180 cases) or high-resolution contrastenhanced computerized tomography (67 cases), there were 151 women and 96 men with a mean age of 44 years. Intracranial arachnoid cysts were found in 20 patients (8.1%) with ADPKD compared to two (0.8%) in a control group without ADPKD matched for age, sex, and method of imaging (p < 0.0001). Multiple intracranial arachnoid cysts were found in two patients. Polycystic liver disease was present in 17 (85.0%) of the 20 patients with intracranial arachnoid cysts compared to 119 (52.4%) of the 227 patients without (p < 0.004). Pineal cysts were found in two patients (0.8%) and choroid plexus cysts were found in three patients (1.2%) but this was not different from the control population. None of the intracranial cysts was symptomatic and none was treated surgically. Intracranial arachnoid cysts are a relatively frequent incidental finding in patients with ADPKD, providing further support for the systemic nature of this disease. In the authors' experience with approximately 1500 patients with ADPKD, no complication has been encountered from an intracranial arachnoid cyst, suggesting that asymptomatic intracranial arachnoid cysts in patients with ADPKD require no treatment.

scholarly journals PKD1-Associated Arachnoid Cysts in Autosomal Dominant Polycystic Kidney Disease

2021 ◽  
Vol 30 (9) ◽  
pp. 105943
Author(s):  
Kaori Shigemori ◽  
Eiji Higashihara ◽  
Masayuki Itoh ◽  
Hiroki Yoshida ◽  
Kouji Yamamoto ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Arachnoid Cysts ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

scholarly journals Effect of Statins on Renal Function and Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease

2020 ◽  
Vol 6 (6) ◽  
pp. 407-413
Author(s):  
Cheng Xue ◽  
Li-Ming Zhang ◽  
Chenchen Zhou ◽  
Chang-Lin Mei ◽  
Sheng-Qiang Yu
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Lipid Lowering ◽  
Control Group ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Kidney Volume ◽  
Yearly Change ◽  
Autosomal Dominant Polycystic Kidney

<b><i>Background:</i></b> Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary nephropathy with few treatments to slow renal progression. The evidence on the effect of lipid-lowering agents (statins) on ADPKD progression remains inconclusive. <b><i>Methods:</i></b> We performed a systematic review and meta-analysis by searching the PubMed, Embase, Web of Science, and Cochrane databases (up to November 2019). Changes in estimated glomerular filtration rate (eGFR) and total kidney volume (TKV) were the primary outcomes. Mean differences (MDs) for continuous outcomes and 95% confidence intervals (CIs) were calculated by a random-effects model. <b><i>Results:</i></b> Five clinical studies with 648 participants were included. Statins did not show significant benefits in the yearly change in eGFR (4 studies, MD = −0.13 mL/min/m<sup>2</sup>, 95% CI: −0.78 to 0.52, <i>p</i> = 0.70) and the yearly change in TKV (3 studies, MD = −1.17%, 95% CI: −3.40 to 1.05, <i>p</i> = 0.30) compared with the control group. However, statins significantly decreased urinary protein excretion (−0.10 g/day, 95% CI: −0.16 to -0.03, <i>p</i> = 0.004) and serum low-density lipoprotein level (−0.34 mmol/L, 95% CI: −0.58 to −0.10, <i>p</i> = 0.006). <b><i>Conclusion:</i></b> Despite these proteinuria and lipid-lowering benefits, the effect of statins on ADPKD progression was uncertain.

P0031IS OXIDATIVE STRESS ASSOCIATED WITH TOTAL KIDNEY VOLUME IN PATIENTS WITH AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE?

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ibrahim Dogan ◽  
Baris Eser ◽  
Nihal Aydemir ◽  
Huseyin Kayadibi ◽  
Oguzhan Ozcan ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Kidney Disease ◽  
Correlation Analysis ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Control Group ◽  
Kidney Volume ◽  
Renal Volume ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

Abstract Background and Aims Ischemia Modified Albumin (IMA) is a marker of cardiovascular risk factor associated with oxidative stress. In this study, our aim was to determine oxidative stress status in autosomal dominant polycystic kidney disease (ADPKD) and to evaluate its relationship with total renal volume. Method Seventy-two ADPKD (mean age 52 (41-64) years) and 33 healthy controls (mean age 54 (42-62) years) (P= 0.372) were included in the study. Total Oxidant Level (TOS) and Total Antioxidant Level (TAS) were measured using the colorimetric method. Oxidative Stress Index (OSI) was calculated. IMA level was measured by ELISA. Total renal volume was assessed by magnetic resonance imaging. The relationship between oxidative stress, inflammation and renal volume was evaluated by correlation analysis. P values &lt;0.05 were considered significant for statistical analyses. Results Serum IMA level was higher in the patient group (158.46 ng/ml (85.98-246.70)) than in the control group (70.04 ng/ml (50.40-80.02); (P&lt; 0.001). TOS level and OSI rate were significantly higher in the patient group compared to the control group, and TAS level was not different between the two groups (P&lt; 0.001, P&lt; 0.001, P= 0.576, respectively).Correlation analysis was performed between IMA, TAS and TOS levels, OSI rate and kidney volume. IMA level correlated with TOS level and OSI positively (P&lt; 0.001), but there was no correlation between IMA level and TAS level and renal volume (P= 0.550, P= 0.286, respectively). IMA level was negatively correlated with GFR and positively correlated with proteinuria (P= 0.040, P&lt; 0.001, respectively). There was no correlation between total renal volume and IMA, TAS, CRP and proteinuria. The correlation between volume and GFR and TOS level was borderline negative (P= 0.054, P= 0.051, respectively). Conclusion There is an increased level of oxidative stress in ADPKD. Increased IMA level may show oxidative stress in this population. Total renal volume in ADPKD is associated with loss of renal function but not sufficient to show oxidative stress.

Ferroptosis Promotes Cyst Growth in Autosomal Dominant Polycystic Kidney Disease Mouse Models

2021 ◽  
Vol 32 (11) ◽  
pp. 2759-2776
Author(s):  
Xiaoqin Zhang ◽  
Linda Xiaoyan Li ◽  
Hao Ding ◽  
Vicente E. Torres ◽  
Chen Yu ◽  
...  
Keyword(s):  
Lipid Peroxidation ◽  
Cell Death ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Mouse Models ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Content Type ◽  
Autosomal Dominant Polycystic Kidney ◽  
Cyst Growth

BackgroundAutosomal dominant polycystic kidney disease (ADPKD), the most common inherited kidney disease, is regulated by different forms of cell death, including apoptosis and autophagy. However, the role in ADPKD of ferroptosis, a recently discovered form of cell death mediated by iron and lipid metabolism, remains elusive.MethodsTo determine a pathophysiologic role of ferroptosis in ADPKD, we investigated whether the absence of Pkd1 (encoding polycystin-1) affected the expression of key factors involved in the process of ferroptosis, using Western blot and qRT-PCR analysis in Pkd1 mutant renal cells and tissues. We also examined whether treatment with erastin, a ferroptosis inducer, and ferrostain-1, a ferroptosis inhibitor, affected cyst growth in Pkd1 mutant mouse models.ResultsWe found that kidney cells and tissues lacking Pkd1 exhibit extensive metabolic abnormalities, including reduced expression of the system Xc− amino acid antiporter (critical for import of cystine), of iron exporter (ferroportin), and of GPX4 (a key and negative regulator of ferroptosis). The abnormalities also include increased expression of iron importers (TfR1, DMT1) and HO-1, which in turn result in high iron levels, low GSH and GPX4 activity, increased lipid peroxidation, and propensity to ferroptosis. We further found that erastin increased, and ferrostatin-1 inhibited ferroptotic cell death and proliferation of Pkd1-deficient cells in kidneys from Pkd1 mutant mice. A lipid peroxidation product increased in Pkd1-deficient cells, 4HNE, promoted the proliferation of survived Pkd1 mutant cells via activation of Akt, S6, Stat3, and Rb during the ferroptotic process, contributing to cyst growth.ConclusionThese findings indicate that ferroptosis contributes to ADPKD progression and management of ferroptosis may be a novel strategy for ADPKD treatment.

Rupture of a previously documented small asymptomatic intracranial aneurysm in a patient with autosomal dominant polycystic kidney disease

1998 ◽  
Vol 89 (3) ◽  
pp. 479-482 ◽  
Author(s):  
Wouter I. Schievink ◽  
Virginia Prendergast ◽  
Joseph M. Zabramski
Keyword(s):  
Kidney Disease ◽  
Intracranial Aneurysm ◽  
Intracranial Aneurysms ◽  
Mr Angiography ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Content Type ◽  
Autosomal Dominant Polycystic Kidney ◽  
Mca Aneurysm ◽  
Fine Print

✓ Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.

High serum soluble CD200 levels in patients with autosomal dominant polycystic kidney disease

2017 ◽  
Vol 65 (4) ◽  
pp. 784-786 ◽  
Author(s):  
Funda Sari ◽  
Saadet Gumuslu ◽  
Ramazan Cetinkaya ◽  
Metin Sarikaya ◽  
Arzu Didem Yalcin
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
High Serum ◽  
Soluble Form ◽  
Control Group ◽  
Polycystic Kidney ◽  
Serum Samples ◽  
Autosomal Dominant Polycystic Kidney

CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD. Serum soluble CD200 levels were measured in 44 patients with ADPKD and 24 healthy volunteers. Concentrations of soluble CD200 in the serum samples were quantified using an ELISA kit. The mean serum soluble CD200 levels were higher in patients with ADPKD than in the control group (71.4±29.2 and 21.4±5.6 pg/mL, p<0.001). Positive correlation was detected between serum soluble CD200 levels and glomerular filtration rate (r=0.772, p<0.001), and serum albumin level (r=0.466, p=0.001). Negative correlation was detected between serum soluble CD200 levels and serum creatinine levels (r=−0.761, p<0.001), and C reactive protein levels (r=−0.364, p=0.015). In the ADPKD patients group, serum soluble CD200 levels were lower in patients with stage 5 chronic kidney disease (CKD) than in patients with stages 1–2 (p<0.001), 3 (p=0.005) and 4 CKD (p=0.006). Serum soluble CD200 levels were similar in patients with stages 1–2, 3, and 4 CKD (p>0.05). Our results show that patients with ADPKD have activated soluble CD200 levels which were related to renal function and inflammation.

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