The screening, diagnosis, and management of patients with autosomal dominant polycystic kidney disease: A national consensus statement from Taiwan.

Chun-Liang Lin, Ming-Ju Wu, Yi-Wen Chiu, Mai-Szu Wu, Der-Cherng Tarng, Chih-Kang Chiang, Jenq-Wen Huang, Kuan-Yu Hung, Yung-Chang Chen, Chia-Ter Chao, Shih-Hua Lin, Wei Wang, Pao-Wen Yen, Yung-An Chen, Fang-Sheng Mao, Juliana Tze-Wah Kao

Journal: Nephrology (Carlton, Vic.) 2024;29(5):245-258

PMID: 38462235

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Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of end-stage kidney disease (ESKD) worldwide. Guidelines for the diagnosis and management of ADPKD in Taiwan remains unavailable. In this consensus statement, we summarize updated information on clinical features of international and domestic patients with ADPKD, followed by suggestions for optimal diagnosis and care in Taiwan. Specifically, counselling for at-risk minors and reproductive issues can be important, including ethical dilemmas surrounding prenatal diagnosis and pre-implantation genetic diagnosis. Studies reveal that ADPKD typically remains asymptomatic until the fourth decade of life, with symptoms resulting from cystic expansion with visceral compression, or rupture. The diagnosis can be made based on a detailed family history, followed by imaging studies (ultrasound, computed tomography, or magnetic resonance imaging). Genetic testing is reserved for atypical cases mostly. Common tools for prognosis prediction include total kidney volume, Mayo classification and PROPKD/genetic score. Screening and management of complications such as hypertension, proteinuria, urological infections, intracranial aneurysms, are also crucial for improving outcome. We suggest that the optimal management strategies of patients with ADPKD include general medical care, dietary recommendations and ADPKD-specific treatments. Key points include rigorous blood pressure control, dietary sodium restriction and Tolvaptan use, whereas the evidence for somatostatin analogues and mammalian target of rapamycin (mTOR) inhibitors remains limited. In summary, we outline an individualized care plan emphasizing careful monitoring of disease progression and highlight the need for shared decision-making among these patients.

Address: Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, Min-Sheng General Hospital, Taoyuan City, Taiwan.; Division of Nephrology, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.; Division of Nephrology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung City, Taiwan.; Kidney Research Center, Department of Nephrology, Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.; Division of Nephrology, Department of Internal Medicine, Taipei Medical University-Shuang-Ho Hospital, Ministry of Health and Welfare, New Taipei City, Taiwan.; Division of Nephrology, Department of Internal Medicine, Fu-Jen Catholic University Hospital, Fu-Jen Catholic University, New Taipei City, Taiwan.; Division of Nephrology, Department of Internal Medicine, Taipei Medical University-Shuang-Ho Hospital, Ministry of Health and Welfare, New Taipei City, Taiwan.; Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, Chia-Yi Chang Gung Memorial Hospital, Chia-Yi County, Taiwan.; Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan.; Division of Nephrology, Department of Internal Medicine, Taipei Medical University-Shuang-Ho Hospital, Ministry of Health and Welfare, New Taipei City, Taiwan.

DOI: 10.1111/nep.14287

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MeSH Terms: Humans, Polycystic Kidney, Autosomal Dominant, Taiwan, Tolvaptan, Kidney, Kidney Failure, Chronic