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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*; Polycystic Kidney, Autosomal Dominant/diagnosis; Polycystic Kidney, Autosomal Dominant/therapy
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; Polycystic Kidney, Autosomal Dominant/drug therapy*; Polycystic Kidney, Autosomal Dominant/pathology
Increased psychosocial risk, depression and reduced quality of life living with autosomal dominant polycystic kidney disease

Simms RJ, Thong KM, Dworschak GC, Ong AC

Nephrol Dial Transplant, 2016 07;31(7):1130-40.
PMID: 26268712 DOI: 10.1093/ndt/gfv299

BACKGROUND: The psychosocial impact of living with autosomal dominant polycystic kidney disease (ADPKD) is poorly understood. In this study, we assessed the overall quality of life (QOL), mood, perceived social support and psychosocial risk of having a diagnosis of ADPKD in a patient cohort from a major UK nephrology centre serving a large catchment population.
METHODS: A postal questionnaire was sent to 349 patients registered at the Sheffield Kidney Institute with chronic kidney disease but not on renal replacement therapy (RRT). The questionnaire incorporated three validated forms: kidney disease quality-of-life short form (KDQOL SF1.3) to assess QOL; nine-item patient health questionnaire (PHQ9) to screen for depression; multidimensional scale of perceived social support (MSPSS) to evaluate perceived social support; as well as a novel genetic psychosocial risk instrument (GPRI-ADPKD) designed to study the specific psychosocial impact of coping with a diagnosis of ADPKD.
RESULTS: The overall response rate was 53%. Patients with a lower estimated glomerular filtration rate (<30 mL/min) or larger kidneys (mean length on ultrasound ≥17 cm) reported reduced QOL and increased psychosocial risk. Clinically significant depression was reported in 22% and 62% felt guilty about passing ADPKD on to their children. In multivariate analysis, female gender was associated with overall poorer psychosocial well-being, whereas increasing age, lower kidney function, larger kidneys and loss of a first degree relative from ADPKD were additional risk factors for QOL, depression or psychosocial risk, respectively.
CONCLUSIONS: Our results reveal a significantly poorer QOL and increasing psychosocial risk with markers of disease progression in patients, particularly women, with ADPKD prior to starting RRT. The future management strategy of ADPKD should address these issues and provide for better individual and family support throughout the patient journey.

Matched MeSH terms: Polycystic Kidney, Autosomal Dominant/epidemiology; Polycystic Kidney, Autosomal Dominant/physiopathology; Polycystic Kidney, Autosomal Dominant/psychology*
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*; Polycystic Kidney, Autosomal Dominant/epidemiology
Fulltext Intraductal papillary mucinous neoplasm (IPMN) in association with autosomal dominant polycystic kidney disease (ADPKD)

Ikhwan Sani Mohamad, Nishioka, Yujiro, Shindoh, Junichi, Hashimoto, Masaji

Malaysian Journal of Medicine and Health Sciences, 2017;13(2):71-74.
MyJurnal

Intraductal Papillary Mucinous Neoplasm (IPMN) of the pancreas in association with Autosomal Dominant Polycystic Kidney Disease (ADPKD) is extremely rare, even though 10% of ADPKD patients may develop simple pancreatic cyst. The first case report was published by Yasunori Sato from Japan in 2009. Since then less than 10 case reports are available worldwide to describe about this condition. We reported such a rare case of a 67-year-old man with ADPKD who was referred to our centre because of chronic abdominal pain and diagnosed as IPMN based on the serial imaging procedures. Despite of the high risk comorbidities, he successfully underwent pylorus preserving total pancreaticoduodenectomy with splenectomy.

Matched MeSH terms: Polycystic Kidney, Autosomal Dominant
Natural-derived compounds and their mechanisms in potential autosomal dominant polycystic kidney disease (ADPKD) treatment

Mahendran R, Lim SK, Ong KC, Chua KH, Chai HC

Clin Exp Nephrol, 2021 Nov;25(11):1163-1172.
PMID: 34254206 DOI: 10.1007/s10157-021-02111-x

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic kidney disorder that impairs renal functions progressively leading to kidney failure. The disease affects between 1:400 and 1:1000 ratio of the people worldwide. It is caused by the mutated PKD1 and PKD2 genes which encode for the defective polycystins. Polycystins mimic the receptor protein or protein channel and mediate aberrant cell signaling that causes cystic development in the renal parenchyma. The cystic development is driven by the increased cyclic AMP stimulating fluid secretion and infinite cell growth. In recent years, natural product-derived small molecules or drugs targeting specific signaling pathways have caught attention in the drug discovery discipline. The advantages of natural products over synthetic drugs enthusiast researchers to utilize the medicinal benefits in various diseases including ADPKD.
CONCLUSION: Overall, this review discusses some of the previously studied and reported natural products and their mechanisms of action which may potentially be redirected into ADPKD.

Matched MeSH terms: Polycystic Kidney, Autosomal Dominant/drug therapy*