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

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

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Pages (from-to)245-258
Number of pages14
JournalNephrology
Volume29
Issue number5
DOIs
Publication statusAccepted/In press - 2024

Keywords

  • autosomal dominant polycystic kidney disease
  • end-stage kidney disease
  • nephrology
  • tolvaptan
  • total kidney volume

ASJC Scopus subject areas

  • Nephrology

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