https://scholars.lib.ntu.edu.tw/handle/123456789/641460
標題: | The screening, diagnosis, and management of patients with autosomal dominant polycystic kidney disease: A national consensus statement from Taiwan | 作者: | Yen, Pao-Wen Chen, Yung-An Wang, Wei Mao, Fang-Sheng Chao, Chia-Ter CHIH-KANG CHIANG Lin, Shih-Hua Tarng, Der-Cherng Chiu, Yi-Wen Wu, Ming-Ju Chen, Yung-Chang Kao, Juliana Tze-Wah Wu, Mai-Szu Lin, Chun-Liang Huang, Jenq-Wen Hung, Kuan-Yu |
關鍵字: | autosomal dominant polycystic kidney disease; end-stage kidney disease; nephrology; tolvaptan; total kidney volume | 公開日期: | 10-三月-2024 | 出版社: | John Wiley and Sons Inc | 來源出版物: | Nephrology | 摘要: | 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. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/641460 | ISSN: | 13205358 | DOI: | 10.1111/nep.14287 |
顯示於: | 毒理學研究所 |
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