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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
Developments in the management of autosomal dominant polycystic kidney disease
Amirali Masoumi1, Berenice Reed-Gitomer, Catherine Kelleher
1Department of Medicine, Health Sciences Center, University of Colorado School of Medicine Denver, CO 80262, USA.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder causing kidney cysts and enlargement. Early diagnosis and treatment of hypertension may slow disease progression and prevent complications.
Area of Science:
- Nephrology
- Genetics
- Hereditary Diseases
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a prevalent, life-threatening genetic disorder.
- It is characterized by progressive cyst development, leading to renal enlargement and functional decline.
- ADPKD is a leading cause of end-stage renal disease (ESRD) and involves multisystemic abnormalities.
Purpose of the Study:
- To review prognostic factors for ADPKD progression.
- To highlight the importance of early diagnosis and management.
- To emphasize the role of hypertension control in mitigating complications.
Main Methods:
- Review of recent studies on ADPKD.
- Identification of prognostic factors for renal impairment.
- Analysis of therapeutic strategies for hypertension management.
Main Results:
- Key prognostic factors include gender, race, age, proteinuria, hematuria, hypertension, and left ventricular mass index (LVMI).
- Renin-angiotensin-aldosterone system inhibitors are effective in managing hypertension.
- Treatment may reduce LVMI, cardiovascular risks, and slow renal disease progression.
Conclusions:
- Early identification of prognostic factors is crucial for managing ADPKD.
- Aggressive hypertension management is vital for slowing renal disease progression.
- Targeted therapies can improve outcomes and reduce morbidity/mortality in ADPKD patients.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent life- threatening, hereditary disease. ADPKD is more common than sickle cell anemia, cystic fibrosis, muscular dystrophy, hemophilia, Down's syndrome, and Huntington's disease combined. ADPKD is a multisystemic disorder characterized by the progressive development of renal cysts and marked renal enlargement. Structural and functional renal deterioration occurs in ADPKD patients and is the fourth leading cause of end-stage renal disease (ESRD) in adults. Aside from the renal manifestations, extrarenal structural abnormalities, such as liver cysts, cardiovascular abnormalities, and intracranial aneurysms may lead to morbidity and mortality. Recent studies have identified prognostic factors for progressive renal impairment including gender, race, age, proteinuria, hematuria, hypertension and increased left ventricular mass index (LVMI). Early diagnosis and better understanding of the pathophysiology of the disease provides the opportunity to aggressivly treat hypertension with renin-angiotensin-aldosterone system inhibitors and thereby potentially reduce LVMI, prevent cardiovascular morbidity and mortality and slow progression of the renal disease.
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