Long-term safety profile of tolvaptan in autosomal dominant polycystic kidney disease patients: TEMPO Extension Japan Trial
CONCLUSIONS: Patients with ADPKD and preserved and reduced GFR demonstrate lower tubular secretory solute excretion compared with healthy controls and patients with non-ADPKD CKD. Our results suggest that tubular secretion is impaired in ADPKD independent of GFR. PMID: 31628117 [PubMed - as supplied by publisher]
AbstractBackgroundAlthough high-density lipoprotein (HDL) modulates many cell types in the cardiovascular system, little is known about HDL in the kidney. We assessed urinary excretion of apolipoprotein AI (apoAI), the main protein in HDL.MethodsWe enrolled 228 children with various kidney disorders and 40 controls. Urinary apoAI, albumin, and other markers of kidney damage were measured using ELISA, apoAI isoforms with Western blot, and renal biopsies stained for apoAI.ResultsPatients followed in nephrology clinic had elevated urinary apoAI vs. controls (median 0.074 μg/mg; interquartile range (IQR) 0.0160–...
CONCLUSION: Measurement of urinary β2MG and MCP-1 excretion allows selection of ADPKD patients with rapidly progressive disease, with a predictive value comparable to or even higher than that of TKV or PKD mutation. Easy and inexpensive to measure urinary markers therefore hold promise to help predict prognosis in ADPKD. PMID: 31600749 [PubMed - as supplied by publisher]
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited, progressive nephropathy accounting for 4-10% of end stage renal disease worldwide. PKD1 and PKD2 are the most common disease loci, but even accounting for other genetic causes, about 7% of families remain unresolved. Typically, these unsolved cases have relatively mild kidney disease and often have a negative family history. Mosaicism, due to de novo mutation in the early embryo, has rarely been identified by conventional genetic analysis of ADPKD families.
CONCLUSIONS: While other disease associations and characteristic liver histomorphology are helpful clues to suspect the diagnosis of CHF in adult patients, other differential diagnoses should be excluded clinically and radiologically. This study highlights the importance of a multidisciplinary diagnostic approach by pathologists, radiologists, and hepatologists for the accurate diagnosis of CHF during adulthood. PMID: 31584623 [PubMed - as supplied by publisher]
AbstractSeveral animal- and human-derived models are used in autosomal dominant polycystic kidney disease (ADPKD) research to gain insight in the disease mechanism. However, a consistent correlation between animal and human ADPKD models is lacking. Therefore, established human-derived models are relevant to affirm research results and translate findings into a clinical set-up. In this review, we give an extensive overview of the existing human-based cell models. We discuss their source (urine, nephrectomy and stem cell), immortalisation procedures, genetic engineering, kidney segmental origin and characterisation with neph...
ConclusionsmTOR inhibitors did not significantly influence renal progression in patients with ADPKD, but were associated with a higher risk of complications. Whether mTOR inhibitors can be an add-on option or second-line agents remain undetermined.
Abstract INTRODUCTION: While medically induced end-stage renal disease (m-ESRD) has been well-studied, outcomes in patients with surgically induced ESRD (s-ESRD) are unknown. We sought to quantitatively compare the non-oncological outcomes for s-ESRD and m-ESRD in a large population-based cohort. METHODS: Medicare patients>65 years old initiating hemodialysis were identified using the U.S. Renal Data System database (2000‒2012). Metastatic cancer, prior transplant history, and nephrectomy for polycystic kidney disease were exclusion criteria. Patients were classified as having s-ESRD or m-ESRD based on h...
To evaluate splenic phenotype in autosomal dominant polycystic kidney disease (ADPKD) including presence of cysts and splenomegaly to determine if these are ADPKD related or represent unrelated incidental findings.
The objective of this study was to evaluate the technique of embolization of polycystic kidneys to reduce their volume before heterotopic renal transplantation.