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Updated: Mar 12, 2026

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
Published on: June 23, 2015
[Clinical Manifestations in Autosomal Dominant Polycystic Kidney Disease (ADPKD)]
Insights
Autosomal dominant polycystic kidney disease (ADPKD) is a systemic condition with significant extrarenal issues like polycystic liver disease. Early recognition and management of ADPKD complications are crucial for patient outcomes.
Area of Science:
- Nephrology
- Genetics
- Systemic Diseases
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a systemic genetic disorder.
- ADPKD presents with numerous extrarenal manifestations, impacting quality of life and disease burden.
- Key extrarenal manifestations include polycystic liver disease (PCLD), gastrointestinal issues, pancreatic cysts, hernias, and diverticular disease.
Purpose of the Study:
- To highlight the significant role of extrarenal manifestations in ADPKD.
- To emphasize the importance of early diagnosis and prevention of ADPKD-related complications.
- To inform nephrologists about recognizing and managing diverse clinical manifestations of ADPKD.
Main Methods:
- Review of clinical manifestations and natural history of ADPKD.
- Analysis of factors influencing renal function decline.
- Discussion of nephrologist's role in managing systemic ADPKD.
Main Results:
- ADPKD involves a long stable period followed by progressive renal function decline.
- Hypertension, cyst infections, and nephrolithiasis accelerate kidney failure.
- Extrarenal conditions like PCLD and hernias significantly contribute to disease burden.
Conclusions:
- Early diagnosis and prevention of ADPKD complications, including extrarenal manifestations, are fundamental.
- Nephrologists must be adept at recognizing and managing diverse ADPKD-related conditions.
- Comprehensive management addressing both renal and extrarenal aspects is vital for ADPKD patients.
Abstract:
ADPKD is a systemic disorder, associated with numerous extrarenal manifestations, including polycystic liver disease (PCLD) and other gastrointestinal manifestations, as well as pancreatic cysts, diverticular disease, inguinal and ventral hernias which play a significant role in disease burden, particularly in the advanced stage of ADPKD. In most cases the natural history of ADPKD goes through a long period of stability followed by a progressive decline in renal function. The coexistence of hypertension, cyst infections and nephrolithiasis can influence and accelerate the progression of kidney failure. The early diagnosis and prevention of these conditions are of foundamental importance. Nephrologists should know how to recognize and handle other clinical manifestations related to ADPKD like haematuria, renal cell carcinoma and intracranial aneurysms (ICA).
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