Imaging Classification of Autosomal Dominant Polycystic Kidney Disease

Irazabal, María V.; Rangel, Laureano J.; Bergstralh, Eric J.; Osborn, Sara L.; Harmon, Amber J.; Sundsbak, Jamie L.; Bae, Kyongtae T.; Chapman, Arlene B.; Grantham, Jared J.; Mrug, Michal; Hogan, Marie C.; El-Zoghby, Ziad M.; Harris, Peter C.; Erickson, Bradley J.; King, Bernard F.; Torres, Vicente E.

doi:10.1681/asn.2013101138

Cited by 465 publications

(701 citation statements)

References 33 publications

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“…It was reported that total kidney volume calculated using the ellipsoid equation (TKVe) is strongly correlated with total kidney volume measured by stereology (TKVs) without systematic underestimation or overestimation. 36 It was also reported that the difference between TKVe and TKVs exceeded 10% in ,30% of their patients and exceeded 20% in ,6%. TKVe might be less accurate in patients with large cysts than in patients without large cysts, but the difference between TKVe and TKVs did not exceed 15% in our 12 patients who had large cysts with wall thickening.…”

Section: Discussionmentioning

confidence: 95%

Suitability of Patients with Autosomal Dominant Polycystic Kidney Disease for Renal Transcatheter Arterial Embolization

Suwabe

Ubara

Mise

et al. 2015

JASN

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In patients with autosomal dominant polycystic kidney disease (ADPKD), massive renal enlargement is a serious problem. Renal transcatheter arterial embolization (TAE) can reduce renal volume (RV), but effectiveness varies widely, and the reasons remain unclear. We investigated factors affecting renal volume reduction rate (RVRR) after renal TAE in all 449 patients with ADPKD who received renal TAE at Toranomon Hospital from January of 2006 to July of 2013, including 228 men and 221 women (mean age =57.069.1 years old). One year after renal TAE, the RVRR ranged from 3.9% to 84.8%, and the least squares mean RVRR calculated using a linear mixed model was 45.5% (95% confidence interval [95% CI], 44.2% to 46.8%). Multivariate analysis using the linear mixed model revealed that RVRR was affected by the presence of large cysts with wall thickening (regression coefficient [RC], 26.10; 95% CI, 29.04 to 23.16; P,0.001), age (RC, 20.82; 95% CI, 21.03 to 20.60; P,0.001), dialysis duration (RC, 20.10; 95% CI, 20.18 to 20.03; P,0.01), systolic BP (RC, 0.39; 95% CI, 0.19 to 0.59; P,0.001), and the number of microcoils used for renal TAE (RC, 1.35; 95% CI, 0.83 to 1.86; P,0.001). Significantly more microcoils were needed to achieve renal TAE in patients with younger age and shorter dialysis duration. In conclusion, cyst wall thickening had an important effect on cyst volume reduction. Renal TAE was more effective in patients who were younger, had shorter dialysis duration, or had hypertension, parameters that might associate with cyst wall stiffness and renal artery blood flow.

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Section: Discussionmentioning

confidence: 95%

Suitability of Patients with Autosomal Dominant Polycystic Kidney Disease for Renal Transcatheter Arterial Embolization

Suwabe

Ubara

Mise

et al. 2015

JASN

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“…Although the reasons for this favorable prognosis are unclear, some of them may have atypical PKD with unilateral, segmental, or asymmetric cystic involvement, which has been reported to be associated with stable kidney function. 22 Other patients with de novo disease and markedly asymmetric cystic kidney involvement may have somatic mosaicism. The hallmark of mosaicism is the presence of more than one genetically distinct cell line in the affected subject whose disease is invariably mild or focal because of dilution of the mutant gene dosage at the tissue level.…”

Section: Discussionmentioning

confidence: 99%

Refining Genotype-Phenotype Correlation in Autosomal Dominant Polycystic Kidney Disease

Hwang

Conklin

Chan

et al. 2015

JASN

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Renal disease variability in autosomal dominant polycystic kidney disease (ADPKD) is strongly influenced by the gene locus (PKD1 versus PKD2). Recent studies identified nontruncating PKD1 mutations in approximately 30% of patients who underwent comprehensive mutation screening, but the clinical significance of these mutations is not well defined. We examined the genotype-renal function correlation in a prospective cohort of 220 unrelated ADPKD families ascertained through probands with serum creatinine #1.4 mg/dl at recruitment. We screened these families for PKD1 and PKD2 mutations and reviewed the clinical outcomes of the probands and affected family members. Height-adjusted total kidney volume (htTKV) was obtained in 161 affected subjects. Multivariate Cox proportional hazard modeling for renal and patient survival was performed in 707 affected probands and family members. Overall, we identified pathogenic mutations in 84.5% of our families, in which the prevalence of PKD1 truncating, PKD1 in-frame insertion/deletion, PKD1 nontruncating, and PKD2 mutations was 38.3%, 4.3%, 27.1%, and 30.3%, respectively. Compared with patients with PKD1 truncating mutations, patients with PKD1 in-frame insertion/deletion, PKD1 nontruncating, or PKD2 mutations have smaller htTKV and reduced risks (hazard ratio Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide, responsible for 5%-10% of ESRD. 1,2 Mutations in two genes (PKD1 and PKD2) account for most patients with ADPKD. 2,3 Previous studies of European families ascertained through probands enriched with renal failure reported that approximately 85% and approximately 15% of ADPKD families were linked to the PKD1 and PKD2 loci, respectively. 3 However, a higher prevalence of PKD2 of 26% has been recently reported in a population-based study. 4 Disease progression of ADPKD is highly variable, in part because of a strong gene locus effect. [5][6][7][8] Adjusted for age, patients with PKD1 have larger kidneys and earlier onset of ESRD than patients with PKD2 (mean age at ESRD, 53.4 versus 72.7 years old, respectively). 5,6,8 Additionally, significant intrafamilial renal disease variability in ADPKD suggests a modifier effect. [9][10][11] [

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“…However, this cross-sectional imaging approach is more difficult and requires more timeconsuming processes in image analysis than using ultrasound imaging. A simple but less precise approach of approximating kidney volume from CT or MR images uses the ellipsoid formula that is on the basis of the multiplication of three orthogonally measured longest dimensions of kidney (Supplemental Appendix 2) (22). In a typical clinical setting, the kidney volume from CT or MR images is measured manually by a trained expert who delineates the kidney border over the CT or MR images.…”

Section: Discussionmentioning

confidence: 99%

Automated Segmentation of Kidneys from MR Images in Patients with Autosomal Dominant Polycystic Kidney Disease

Kim¹,

Ge²,

Tao³

et al. 2016

Clinical Journal of the American Society of Nephrology

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Background and objectives Our study developed a fully automated method for segmentation and volumetric measurements of kidneys from magnetic resonance images in patients with autosomal dominant polycystic kidney disease and assessed the performance of the automated method with the reference manual segmentation method.Design, setting, participants, & measurements Study patients were selected from the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease. At the enrollment of the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Study in 2000, patients with autosomal dominant polycystic kidney disease were between 15 and 46 years of age with relatively preserved GFRs. Our fully automated segmentation method was on the basis of a spatial prior probability map of the location of kidneys in abdominal magnetic resonance images and regional mapping with total variation regularization and propagated shape constraints that were formulated into a level set framework. T2-weighted magnetic resonance image sets of 120 kidneys were selected from 60 patients with autosomal dominant polycystic kidney disease and divided into the training and test datasets. The performance of the automated method in reference to the manual method was assessed by means of two metrics: Dice similarity coefficient and intraclass correlation coefficient of segmented kidney volume. The training and test sets were swapped for crossvalidation and reanalyzed.Results Successful segmentation of kidneys was performed with the automated method in all test patients. The segmented kidney volumes ranged from 177.2 to 2634 ml (mean, 885.46569.7 ml). The mean Dice similarity coefficient 6SD between the automated and manual methods was 0.8860.08. The mean correlation coefficient between the two segmentation methods for the segmented volume measurements was 0.97 (P,0.001 for each crossvalidation set). The results from the crossvalidation sets were highly comparable.Conclusions We have developed a fully automated method for segmentation of kidneys from abdominal magnetic resonance images in patients with autosomal dominant polycystic kidney disease with varying kidney volumes. The performance of the automated method was in good agreement with that of manual method.

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Imaging Classification of Autosomal Dominant Polycystic Kidney Disease

Cited by 465 publications

References 33 publications

Suitability of Patients with Autosomal Dominant Polycystic Kidney Disease for Renal Transcatheter Arterial Embolization

Suitability of Patients with Autosomal Dominant Polycystic Kidney Disease for Renal Transcatheter Arterial Embolization

Refining Genotype-Phenotype Correlation in Autosomal Dominant Polycystic Kidney Disease

Automated Segmentation of Kidneys from MR Images in Patients with Autosomal Dominant Polycystic Kidney Disease

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