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  1. Fulltext Congenital hepatic fibrosis in a child with autosomal dominant polycystic kidney disease
    Kanaheswari Y, Hamzaini AH, Wong SW
    Med J Malaysia, 2008 Aug;63(3):251-3.
    PMID: 19248702 MyJurnal
    The association of congenital hepatic fibrosis (CHF) with autosomal recessive polycystic kidney disease (ARPKD) is well known and occurs in approximately 50% of cases. However the association of CHF with autosomal dominant polycystic kidney disease (ADPKD) is less well known and less well documented. We report a child with neonatal onset of hypertension due to ADPKD who later develops portal hypertension due to CHF in childhood. A review of this rare association follows.
    Matched MeSH terms: Polycystic Kidney, Autosomal Dominant/complications*
  2. Fulltext Renin-angiotensin-aldosterone system antagonism and polycystic kidney disease progression
    Hian CK, Lee CL, Thomas W
    Nephron, 2016;134(2):59-63.
    PMID: 27476173 DOI: 10.1159/000448296
    Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a systemic disease characterised by the formation of multiple renal cysts that adversely affect renal function. ADPKD shows significant progression with age when complications due to hypertension are most significant. The activation of the renin-angiotensin-aldosterone system (RAAS) occurs in progressive kidney disease leading to hypertension. The RAAS system may also contribute to ADPKD progression by stimulating signalling pathways in the renal cyst cells to promote growth and deregulate epithelial transport. This mini review focuses on the contribution of the RAAS system to renal cyst enlargement and the potential for antagonists of the RAAS system to suppress cyst enlargement as well as control ADPKD-associated hypertension.
    Matched MeSH terms: Polycystic Kidney, Autosomal Dominant/complications
  3. International comparison of trends in patients commencing renal replacement therapy by primary renal disease
    Stel VS, Awadhpersad R, Pippias M, Ferrer-Alamar M, Finne P, Fraser SD, et al.
    Nephrology (Carlton), 2019 Oct;24(10):1064-1076.
    PMID: 30456883 DOI: 10.1111/nep.13531
    AIM: To examine international time trends in the incidence of renal replacement therapy (RRT) for end-stage renal disease (ESRD) by primary renal disease (PRD).

    METHODS: Renal registries reporting on patients starting RRT per million population for ESRD by PRD from 2005 to 2014, were identified by internet search and literature review. The average annual percentage change (AAPC) with a 95% confidence interval (CI) of the time trends was computed using Joinpoint regression.

    RESULTS: There was a significant decrease in the incidence of RRT for ESRD due to diabetes mellitus (DM) in Europe (AAPC = -0.9; 95%CI -1.3; -0.5) and to hypertension/renal vascular disease (HT/RVD) in Australia (AAPC = -1.8; 95%CI -3.3; -0.3), Canada (AAPC = -2.9; 95%CI -4.4; -1.5) and Europe (AAPC = -1.1; 95%CI -2.1; -0.0). A decrease or stabilization was observed for glomerulonephritis in all regions and for autosomal dominant polycystic kidney disease (ADPKD) in all regions except for Malaysia and the Republic of Korea. An increase of 5.2-16.3% was observed for DM, HT/RVD and ADPKD in Malaysia and the Republic of Korea.

    CONCLUSION: Large international differences exist in the trends in incidence of RRT by primary renal disease. Mapping of these international trends is the first step in defining the causes and successful preventative measures of CKD.

    Matched MeSH terms: Polycystic Kidney, Autosomal Dominant/complications*
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