Rate of functional deterioration in polycystic kidney disease

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Hyperuricemia and deterioration of renal function in autosomal dominant polycystic kidney disease

BACKGROUND The role of hyperuricemia in disease progression of autosomal dominant polycystic kidney disease (ADPKD) has not been defined well. We investigated the association of serum uric acid (sUA) with renal function and the effect of hypouricemic treatment on the rate of renal function decline. METHODS This is a single-center, retrospective, observational cohort study. A total of 365 pati...

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Polycystic kidney disease.

A number of inherited disorders result in renal cyst development. The most common form, autosomal dominant polycystic kidney disease (ADPKD), is a disorder most often diagnosed in adults and caused by mutation in PKD1 or PKD2. The PKD1 protein, polycystin-1, is a large receptor-like protein, whereas polycystin-2 is a transient receptor potential channel. The polycystin complex localizes to prim...

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Beyond polycystic kidney disease

Tuberous sclerosis(TS) is an autosomal dominant disease caused by mutations in TSC1 and TSC2 genes. TSC2 gene is located in chromosome 16p13.3, adjacent to PKD1 gene, responsible for the autosomal dominant polycystic kidney disease. In a rare subgroup of patients, the presence of a deletion which simultaneously affects the TSC2 and PKD1 genes has been confirmed. TSC2/PKD1-Contiguous Gene Syndro...

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[Polycystic kidney disease].

要旨: 常染色体優性遺伝嚢胞腎(ADPKD)は 進行性の腎機能低下が主要な病態であるが, その予後は従 来いわれているように 「診断後10年で腎不全に到る」ものでもなく, 腎不全が不可避でもない. 本邦で の透析導入時平均年齢は52~56歳 であるが, 透析に移行しない者も含めると, おおよそ平均73歳で終末 期腎不全に到る. 60歳代で透析を受ける割合は約40%で あり, 本邦のADPKDの 予後は欧米よりも若干 良好である可能性がある. ADPKDの 遺伝子は第16染色体の短腕上のα-globin遺伝子の近くに存在することが確かめられてい る. この遺伝子(PKD1)に よるADPKDと, PKD1の 関与が証明されないADPKDで は, 腎機能の予 後が異なることが報告されている. 高血圧は約60%に 認められる. 嚢胞の圧迫によって腎動脈が狭細化し, レニン-ア ンギオテソシ...

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Tesevatinib ameliorates progression of polycystic kidney disease in rodent models of autosomal recessive polycystic kidney disease

AIM To investigate the therapeutic potential of tesevatinib (TSV), a unique multi-kinase inhibitor currently in Phase II clinical trials for autosomal dominant polycystic kidney disease (ADPKD), in well-defined rodent models of autosomal recessive polycystic kidney disease (ARPKD). METHODS We administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk mod...

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ژورنال

عنوان ژورنال: Kidney International

سال: 1983

ISSN: 0085-2538

DOI: 10.1038/ki.1983.51