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Rare-MR-urography--a new diagnostic method in autosomal recessive polycystic kidney disease
S Kern1, L B Zimmerhackl, F Hildebrandt
1Department of Diagnostic Radiology, University Hospital, Freiburg, Germany.
Purpose:
To describe the appearance of autosomal recessive polycystic kidney disease (ARPKD) by using a new diagnostic method: RARE-MR-urography.
Material And Methods:
Eight children were evaluated using MR images from 0.23 T and 1.5 T MR units, using T1-weighted spin-echo and T2-weighted turbo spin-echo sequences and RARE-MR-urography. Signal intensities, morphological appearance of the affected kidneys and, specifically, the picture of the urinary tract in RARE-MR-urography, were evaluated.
Results:
All children showed enlargement, reniform but humpy kidney shape, homogeneous-grainy renal parenchyma, normal renal pelvis and calyces. Although ARPKD is always associated with some degree of congenital hepatic fibrosis, there was no bile duct dilatation or liver fibrosis at the time of examination. Signal intensity was hyperintense in T2-weighted images in all cases. In 5 cases, T1-weighted images were hypointense. In RARE-MR-urography, hyperintense, linear, radial patterns in cortex and medulla were seen, which represent microcystic dilatation of collecting ducts and are therefore characteristic of ARPKD. Four patients presented with a few circumscribed small subcapsular cysts.
Conclusion:
RARE-MR-urography is a noninvasive method which demonstrates the pathognomonic water-filled cystic structures throughout the kindeys in ARPKD.
Insights
RARE-MR-urography effectively visualizes microcystic changes in autosomal recessive polycystic kidney disease (ARPKD). This noninvasive MRI technique highlights characteristic radial patterns, aiding in ARPKD diagnosis.
Area of Science:
- Radiology
- Pediatric Nephrology
- Medical Imaging
Background:
- Autosomal recessive polycystic kidney disease (ARPKD) is a genetic disorder affecting the kidneys and liver.
- Early and accurate diagnosis is crucial for managing ARPKD and its complications.
Purpose of the Study:
- To evaluate the utility of RARE-MR-urography in characterizing the renal and urinary tract appearance in pediatric ARPKD patients.
- To describe the specific imaging findings of ARPKD using this novel MRI technique.
Main Methods:
- Eight children diagnosed with ARPKD were scanned using 0.23 T and 1.5 T MRI units.
- T1-weighted spin-echo, T2-weighted turbo spin-echo, and RARE-MR-urography sequences were employed.
- Analysis focused on signal intensities, kidney morphology, and urinary tract visualization.
Main Results:
- All patients exhibited enlarged kidneys with a humpy shape and homogeneous-grainy parenchyma.
- RARE-MR-urography revealed characteristic hyperintense, linear, radial patterns in the renal cortex and medulla, indicative of microcystic collecting duct dilatation.
- Normal renal pelvis and calyces were observed; no significant liver fibrosis or bile duct dilatation was evident at the time of examination.
Conclusions:
- RARE-MR-urography is a noninvasive and effective method for diagnosing ARPKD.
- The technique clearly demonstrates pathognomonic water-filled cystic structures within the kidneys.
- This imaging modality aids in the characteristic visualization of microcystic changes in ARPKD.