A risk score to predict kidney survival in patients with autosomal recessive polycystic kidney disease at the age of two months.

• Tác giả: Klaus Arbeiter, Bahriye Atmis, Olivia Boyer, Anja Buescher, Kathrin Burgmaier, Jörg Dötsch, Ismail Dursun, Florian Erger, Marc Fila, Matthias Galiano, Ibrahim Gokce, Karsten Haeffner, Dieter Haffner, Nakysa Hooman, Samuel Kilian, Guenter Klaus, Jens König, Bärbel Lange-Sperandio, Max Christoph Liebau, Matko Marlais, Laura Massella, Djalila Mekahli, Monika Miklaszewska, Gordana Miloševski-Lomić, Lukasz Obrycki, Bruno Ranchin, Franz Schaefer, Barbara Seitz, Stella Stabouli, Yilmaz Tabel, Katarzyna Taranta-Janusz, Lutz Thorsten Weber, Marcus Weitz, Elke Wühl, Alev Yilmaz
• Ngôn ngữ: eng
• Thông tin xuất bản: United States : Kidney international , 2025
• Bộ sưu tập: NCBI
• ID: 701988

Tóm tắt

Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal fibrocystic disorder. Its rareness and the variability of disease courses have been major obstacles for the establishment of clinical trials on treatment of kidney disease in ARPKD. In this observational study we characterized kidney disease progression in a very large cohort of up to 658 patients with the clinical diagnosis of ARPKD and identified risk factors associated with rapid kidney disease progression. The estimated probability of kidney failure by the age of 20 years was 50.1% (95% confidence interval 42.2%‒57.0%), with earlier kidney failure in specific subgroups. Mean yearly estimated glomerular filtration rate decline after the first year of life was 1.3 ml/min per 1.73 m