Common bile duct dilatation on MRI in autosomal dominant polycystic kidney disease.

Purpose: Polycystic liver disease is the most prevalent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Non-obstructive asymptomatic common bile duct (CBD) dilatation has been observed anecdotally on CT scans, but CT is not optimal for biliary visualization. Here, we measured CBD diameter on T2-weighted MRI in ADPKD subjects to determine if CBD dilatation is associated with ADPKD.

Effects of Pregnancy on Liver and Kidney Cyst Growth Rates in Autosomal Dominant Polycystic Kidney Disease: A Pilot Study.

: Polycystic liver disease (PLD) is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). PLD is more prevalent in women, and women have larger liver cysts, possibly due to estrogen-related mechanisms. Maternal estrogen levels normally increase during pregnancy.

The Role of Baseline Total Kidney Volume Growth Rate in Predicting Tolvaptan Efficacy for ADPKD Patients: A Feasibility Study.

: Although tolvaptan efficacy in ADPKD has been demonstrated in randomized clinical trials, there is no definitive method for assessing its efficacy in the individual patient in the clinical setting. In this exploratory feasibility study, we report a method to quantify the change in total kidney volume (TKV) growth rate to retrospectively evaluate tolvaptan efficacy for individual patients. Treatment-related changes in estimated glomerular filtration rate (eGFR) are also assessed.

Hypothesis: Reactive increases in plasma renin activity attenuate the fall in blood pressure caused by salt depletion and renin-angiotensin system inhibition.

There are inconsistencies in the effect of raising or lowering body salt on blood pressure (BP). We hypothesize that they are caused in part by differences in plasma renin activity (PRA). PRA changes reciprocally with body salt.

Automatically Detecting Pancreatic Cysts in Autosomal Dominant Polycystic Kidney Disease on MRI Using Deep Learning.

Background: Pancreatic cysts in autosomal dominant polycystic kidney disease (ADPKD) correlate with PKD2 mutations, which have a different phenotype than PKD1 mutations. However, pancreatic cysts are commonly overlooked by radiologists. Here, we automate the detection of pancreatic cysts on abdominal MRI in ADPKD.

Prevalence of Spinal Meningeal Diverticula in Autosomal Dominant Polycystic Kidney Disease.

Background And Purpose: Patients with autosomal dominant polycystic kidney disease (ADPKD) develop cysts in the kidneys, liver, spleen, pancreas, prostate, and arachnoid spaces. In addition, spinal meningeal diverticula have been reported. To determine whether spinal meningeal diverticula are associated with ADPKD, we compared their prevalence in subjects with ADPKD with a control cohort without ADPKD.

Improved predictions of total kidney volume growth rate in ADPKD using two-parameter least squares fitting.

Mayo Imaging Classification (MIC) for predicting future kidney growth in autosomal dominant polycystic kidney disease (ADPKD) patients is calculated from a single MRI/CT scan assuming exponential kidney volume growth and height-adjusted total kidney volume at birth to be 150 mL/m. However, when multiple scans are available, how this information should be combined to improve prediction accuracy is unclear. Herein, we studied ADPKD subjects ( ) with 8+ years imaging follow-up (mean = 11 years) to establish ground truth kidney growth trajectory.

A Primer for Utilizing Deep Learning and Abdominal MRI Imaging Features to Monitor Autosomal Dominant Polycystic Kidney Disease Progression.

Abdominal imaging of autosomal dominant polycystic kidney disease (ADPKD) has historically focused on detecting complications such as cyst rupture, cyst infection, obstructing renal calculi, and pyelonephritis; discriminating complex cysts from renal cell carcinoma; and identifying sources of abdominal pain. Many imaging features of ADPKD are incompletely evaluated or not deemed to be clinically significant, and because of this, treatment options are limited. However, total kidney volume (TKV) measurement has become important for assessing the risk of disease progression (i.

Feasibility of Water Therapy for Slowing Autosomal Dominant Polycystic Kidney Disease Progression.

Key Points: Water therapy in autosomal dominant polycystic kidney disease (ADPKD) reduces urine osmolality and serum copeptin level, a marker of vasopressin activity. Water therapy reduces the ADPKD kidney growth rate indicating it is slowing disease progression. Patients with ADPKD are less likely to report pain on water therapy.

Quantitative susceptibility mapping for detection of kidney stones, hemorrhage differentiation, and cyst classification in ADPKD.

Background And Purpose: The objective is to demonstrate feasibility of quantitative susceptibility mapping (QSM) in autosomal dominant polycystic kidney disease (ADPKD) patients and to compare imaging findings with traditional T1/T2w magnetic resonance imaging (MRI).

Methods: Thirty-three consecutive patients (11 male, 22 female) diagnosed with ADPKD were initially selected. QSM images were reconstructed from the multiecho gradient echo data and compared to co-registered T2w, T1w, and CT images.

Test Retest Reproducibility of Organ Volume Measurements in ADPKD Using 3D Multimodality Deep Learning.

Rationale And Objectives: Following autosomal dominant polycystic kidney disease (ADPKD) progression by measuring organ volumes requires low measurement variability. The objective of this study is to reduce organ volume measurement variability on MRI of ADPKD patients by utilizing all pulse sequences to obtain multiple measurements which allows outlier analysis to find errors and averaging to reduce variability.

Materials And Methods: In order to make measurements on multiple pulse sequences practical, a 3D multi-modality multi-class segmentation model based on nnU-net was trained/validated using T1, T2, SSFP, DWI and CT from 413 subjects.

Size Matters: How to Characterize ADPKD Severity by Measuring Total Kidney Volume.

Following patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD) has been challenging because serum biomarkers such as creatinine often remain normal until relatively late in the disease [...

Clinical Quality Control of MRI Total Kidney Volume Measurements in Autosomal Dominant Polycystic Kidney Disease.

Total kidney volume measured on MRI is an important biomarker for assessing the progression of autosomal dominant polycystic kidney disease and response to treatment. However, we have noticed that there can be substantial differences in the kidney volume measurements obtained from the various pulse sequences commonly included in an MRI exam. Here we examine kidney volume measurement variability among five commonly acquired MRI pulse sequences in abdominal MRI exams in 105 patients with ADPKD.

Effect of Averaging Measurements From Multiple MRI Pulse Sequences on Kidney Volume Reproducibility in Autosomal Dominant Polycystic Kidney Disease.

Background: Total kidney volume (TKV) is an important biomarker for assessing kidney function, especially for autosomal dominant polycystic kidney disease (ADPKD). However, TKV measurements from a single MRI pulse sequence have limited reproducibility, ± ~5%, similar to ADPKD annual kidney growth rates.

Purpose: To improve TKV measurement reproducibility on MRI by extending artificial intelligence algorithms to automatically segment kidneys on T1-weighted, T2-weighted, and steady state free precession (SSFP) sequences in axial and coronal planes and averaging measurements.

Pleural Effusions on MRI in Autosomal Dominant Polycystic Kidney Disease.

Autosomal dominant polycystic kidney disease (ADPKD) has cystic fluid accumulations in the kidneys, liver, pancreas, arachnoid spaces as well as non-cystic fluid accumulations including pericardial effusions, dural ectasia and free fluid in the male pelvis. Here, we investigate the possible association of ADPKD with pleural effusion. ADPKD subjects (n = 268) and age-gender matched controls without ADPKD (n = 268) undergoing body magnetic resonance imaging from mid-thorax down into the pelvis were independently evaluated for pleural effusion by 3 blinded expert observers.

Deep Learning Automation of Kidney, Liver, and Spleen Segmentation for Organ Volume Measurements in Autosomal Dominant Polycystic Kidney Disease.

Organ volume measurements are a key metric for managing ADPKD (the most common inherited renal disease). However, measuring organ volumes is tedious and involves manually contouring organ outlines on multiple cross-sectional MRI or CT images. The automation of kidney contouring using deep learning has been proposed, as it has small errors compared to manual contouring.

Deployed Deep Learning Kidney Segmentation for Polycystic Kidney Disease MRI.

This study develops, validates, and deploys deep learning for automated total kidney volume (TKV) measurement (a marker of disease severity) on T2-weighted MRI studies of autosomal dominant polycystic kidney disease (ADPKD). The model was based on the U-Net architecture with an EfficientNet encoder, developed using 213 abdominal MRI studies in 129 patients with ADPKD. Patients were randomly divided into 70% training, 15% validation, and 15% test sets for model development.

Prevalence of Inferior Vena Cava Compression in ADPKD.

Introduction: Kidney and liver cysts in autosomal dominant polycystic kidney disease (ADPKD) can compress the inferior vena cava (IVC), but IVC compression prevalence and its risk factors are unknown.

Methods: Patients who have ADPKD (n = 216) with abdominal magnetic resonance imaging (MRI) studies and age-/sex-matched controls (n = 216) were evaluated for IVC compression as well as azygous vein diameter (a marker of collateral blood flow) and IVC aspect ratio (left-to-right dimension divided by anterior-to-posterior dimension with a value of 1 corresponding to a circular (high pressure) IVC caudal to compression.

Results: Severe IVC compression (≥70%) was observed in 33 (15%) ADPKD subjects and mild compression (≥50% to <70%) was observed in 33 (15%) subjects; whereas controls had no IVC compression ( < 0.

Hemorrhagic Cysts and Other MR Biomarkers for Predicting Renal Dysfunction Progression in Autosomal Dominant Polycystic Kidney Disease.

Background: Screening for rapidly progressing autosomal dominant polycystic kidney disease (ADPKD) is necessary for assigning and monitoring therapies. Height-adjusted total kidney volume (ht-TKV) is an accepted biomarker for clinical prognostication, but represents only a small fraction of information on abdominal MRI.

Purpose: To investigate the utility of other MR features of ADPKD to predict progression.

Hypertension: evolving from standardized to individualized care.

: The hypertension paradigm has contributed to a dramatic reduction in CVD mortality. This has been achieved by applying average results of population studies to identify a target population and design a common intervention to achieve a BP goal. Progressive lowering of the BP threshold has expanded the fraction of persons at risk who have access to treatment.

MRI in autosomal dominant polycystic kidney disease.

Magnetic resonance imaging (MRI) is increasingly used in autosomal dominant polycystic kidney disease (ADPKD) for diagnosis, classification, assessment of disease progression and treatment response, and for identifying complications. Herein we review the role of MRI in the management of patients with ADPKD. We show how MRI-derived total kidney volume is a biomarker for assessing ADPKD severity and predicting decline in renal function.

Relationship of Seminal Megavesicles, Prostate Median Cysts, and Genotype in Autosomal Dominant Polycystic Kidney Disease.

Background: Autosomal dominant polycystic kidney disease (ADPKD) can involve prostate and seminal vesicles but the potential interrelationship of these findings and associations with PKD gene mutation locus and type is unknown.

Purpose: To determine the interrelationship of seminal megavesicles (seminal vesicles with lumen diameter > 10mm) and prostatic cysts in ADPKD and to determine whether there are associations with PKD gene mutations.

Study Type: Retrospective, case control.

Bladder diverticuli following injection of onabotulinum toxin A in a patient with multiple sclerosis and autosomal dominant polycystic kidney disease.

Urinary incontinence due to neurogenic detrusor overactivity is common in patients with disorders of lower motor neurons controlling the bladder. Multiple sclerosis is a major cause of neurogenic detrusor overactivity, which negatively impacts quality of life. Bladder wall injection of onabotulinum toxin A can diminish spontaneous bladder contraction, urinary urgency, and urge incontinence.