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Metabolic abnormalities in autosomal dominant polycystic kidney disease
Zhiguo Mao1, Guoqiang Xie1, Albert C M Ong2
1Division of Nephrology, Kidney Institute of CPLA, Changzheng Hospital Second Military Medical University, Shanghai 200003, China.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) involves kidney cyst formation. This review highlights metabolic abnormalities as potentially significant, under-recognized complications in ADPKD patients.
Area of Science:
- Nephrology
- Genetics
- Metabolic Disorders
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent inherited kidney disease.
- ADPKD is characterized by progressive kidney cyst development, leading to organ damage.
- Non-cystic manifestations include intracranial aneurysms and cardiac valve defects.
Purpose of the Study:
- To review the literature on metabolic abnormalities in ADPKD.
- To emphasize the potential role of metabolic dysfunction in disease progression.
- To highlight these abnormalities as under-recognized and under-treated aspects of ADPKD.
Main Methods:
- Comprehensive literature review.
- Synthesis of existing research on ADPKD and associated metabolic changes.
- Analysis of studies investigating cystogenesis and systemic complications.
Main Results:
- ADPKD kidneys exhibit numerous expanding cysts, impairing function.
- Metabolic abnormalities are frequently observed in ADPKD patients.
- These metabolic issues may influence cyst progression and other ADPKD complications.
Conclusions:
- Metabolic abnormalities are a significant, yet often overlooked, feature of ADPKD.
- Addressing metabolic dysfunction could be crucial for managing ADPKD.
- Further research and clinical attention are warranted for these under-treated aspects.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder and is known to affect all ethnic groups with a prevalence of 1:400-1:1000 live births. The kidney in ADKPD is characterized by the formation of numerous cysts which progressively expand and eventually destroy normal kidney structure and function. Cysts occur in other organs outside the kidney, most commonly in the liver, pancreas and spleen. Important non-cystic features include intracranial aneurysms and cardiac valve defects. Less well recognized are a range of metabolic abnormalities, which could be involved in cystic disease progression or be associated with other disease complications. In this review, we summarize the literature suggesting that metabolic abnormalities could be important under-recognised and under-treated features in ADPKD.
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