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Introduzione: La malattia policistica autosomica dominante (ADPKD) è una causa comune di malattia renale terminale (ESKD). È caratterizzata dallo sviluppo di cisti renali bilaterali che aumentano progressivamente di volume. Il Tolvaptan viene prescritto in base a 3 criteri: volume renale totale (HtTKV) e Mayo Clinic Imaging Class (MCIC), tasso di declino dell'eGFR e al Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD), che combina variabili cliniche e genetiche. In questa coorte multicentrica retrospettiva, l'obiettivo era di valutare e migliorare la concordanza di sensibilità e specificità predittive di MCIC e PROPKD. Metodi: I dati di pazienti adulti affetti da ADPKD sono stati ottenuti da 2 centri di Bologna (B) e Dublino (D). Abbiamo definito RP un calo dell'eGFR ≥3 mL/min/1,73m2/anno su 4 anni (Clinical Score), o classi MCIC 1C-D-E, o punteggio PROPKD da 7 a 9. Per i parametri clinici sono state utilizzate statistiche descrittive. La concordanza tra i punteggi è stata valutata tramite la statistica Kappa. Nelle varianti missenso di PKD1, il punteggio REVEL è stato trattato come una variabile continua; (>0,65 patogeno'). Risultati: Abbiamo valutato 201 pazienti con ADPKD. Il Propkd e il MCIC erano rispettivamente: 90% specifico e 31,3% sensibile; 89,6% sensibile e 28,6% specifico per identificare il calo dell'eGFR. Kappa di Cohen era di 0,025. Il 47,9% (n=143) è risultato concorde. Il punteggio Revel applicato alle mutazioni PKD1NT identifica da 15 a 19 pazienti che potrebbero avere una RP. L'analisi multivariata mostra dati statisticamente significativi per HB (p:0,016), eventi urologici (p: 0,005) e MCIC (p: 0,074). Conclusioni: La concordanza tra i punteggi risulta bassa. Il PROPKD è più selettivo rispetto al Mayo. Tuttavia, il PROPKD permette di identificare alcune RP escluse dall'uso del solo MCIC. L'uso combinato dei punteggi può aumentare la capacità di identificare le RP. Il punteggio REVEL potrebbe migliorare questa concordanza, Background: Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of end-stage kidney disease (ESKD). It is characterized by the progressive development of bilateral renal cysts, resulting in enlargement of the kidney volume and ESKD. Tolvaptan therapy is prescribed according to 3 criteria: total kidney volume (HtTKV) and Mayo Clinic Imaging Class (MCIC), rate of decline in eGFR, and the Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD) score, combining clinical and genetic variables. In this retrospective multicentric cohort, the aim was evaluate and improve the concordance of sensitivity and specificity of MCIC and PROPKD predictive abilities for rapid progressor (RP). Method: Data from adult ADPKD patients were obtained from 2 centers Bologna (B), Dublin(D). We defined RP an eGFR slope ≥3 mL/min/1.73m2/ yearly over 4 years (Clinical Score), or MCIC classes 1C-D-E, or PROPKD score 7 to 9. Descriptive statistics were used to clinical parameters. The concordance between scores was assessed using Kappa statistics. In PKD1 missense variants, the REVEL score was treated as a continuous variable; (>0.65 pathogenic’). Results: We evaluated 201 ADPKD patients. The Propkd and MCIC were respectively: 90% specific and 31,3% sensitive; 89.6% sensitive and 28.6% specific in order to identify a eGFR slope. Assessment of RP using PROPKD, and Mayo scores yielded Kappa Cohen of 0,025 47.9% (n=143) were concordant. The Revel score applied to PKD1NT mutations identifies 15 to 19 patients who may have a RP. Multivariate analysis shows statistically significant data for HB (p:0.016), events urological (p: 0.005) and MCIC (p: 0.074). Conclusion: Concordance between scores results low. The PROPKD is more selective compared to the Mayo. Nevertheless, PROPKD allows the identification of some PR excluded from using the MCIC only. The combined use of scoring may increase the ability to identify RP. REVEL score could improve this agreement.

Olga Baraldi,1 Francesca Bianchi,2,3 Viola Menghi,1 Andrea Angeletti,1 Anna Laura Croci Chiocchini,1 Maria Cappuccilli,1 Valeria Aiello,1 Giorgia Comai,1 Gaetano La Manna1 1Department of Experimental, Diagnostic and Specialty Medicine, Nephrology, Dialysis and Renal Transplant Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna, 2Stem Wave Institute for Tissue Healing, Gruppo Villa Maria Care & Research – Ettore Sansavini Health Science Foundation, Lugo, Ravenna, 3National Institute of Biostructures and Biosystems at the Department of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy Background: Acute kidney injury, known as a major trigger for organ fibrosis and independent predictor of chronic kidney disease, is characterized by mesangial cell proliferation, inflammation and unbalance between biosynthesis and degradation of extracellular matrix. Therapeutic approaches targeting the inhibition of mesangial cell proliferation and matrix expansion may represent a promising opportunity for the treatment of kidney injury. An ester of hyaluronic acid and butyric acid (HB) has shown vasculogenic and regenerative properties in renal ischemic-damaged tissues, resulting in enhanced function recovery and minor degree of inflammation in vivo. This study evaluated the effect of HB treatment in mesangial cell cultures exposed to H2O2-induced oxidative stress.Materials and methods: Lactate dehydrogenase release and caspase-3 activation were measured using mesangial cells prepared from rat kidneys to assess necrosis and apoptosis. Akt and p38 phosphorylation was analyzed to identify the possible mechanism underlying cell response to HB treatment. The relative expressions of matrix metallopeptidase 9 (MPP-9) and collagen type 1 alpha genes were also analyzed by quantitative real-time polymerase chain reaction. Cell proliferation rate and viability were measured

Maria Cappuccilli,1 Gabriele Donati,1 Giorgia Comai,1 Olga Baraldi,1 Diletta Conte,1 Irene Capelli,1 Valeria Aiello,1 Andrea Pession,2 Gaetano La Manna1 1Department of Experimental Diagnostic and Specialty Medicine (DIMES), Nephrology, Dialysis, and Renal Transplant Unit, 2Molecular Laboratory of Pediatrics, Hematology–Oncology Unit, St Orsola Hospital, University of Bologna, Bologna, Italy Background: The aim of this study was the application of complementarity-determining region-3 spectratyping analysis to determine T-cell-repertoire complexity and to detect T-cell-clone expansion, as a measure of immune response in nonfunctioning kidney transplants (group hemodialysis-transplant [HD-Tx]), nontransplanted dialysis patients (group hemodialysis [HD]), and normal subjects as controls (group C).Patients and methods: Analysis of T-cell receptor (TCR) diversity by spectratyping was applied to peripheral blood samples collected from 21 subjects: eight in group HD-Tx, seven in group HD, and six in group C.Results: Considering the extent of the skew in TCR variable region repertoires as a measure of clonal T cells, we found that the number of altered spectra showed a progressive increase from normal subjects to dialysis patients and to nonfunctioning kidney transplants, respectively. Healthy subjects had the lowest number of altered spectra, and patients with nonfunctioning kidney transplants the highest. Differences were significant for group HD-Tx vs group C (P=0.017) and group HD vs group C (P=0.015), but not between nonfunctioning kidney-transplant recipients and dialysis patients (group HD-Tx vs group HD).Conclusion: Although dialysis appears to be a weaker trigger for clonal expansion of T cells, our data suggest that the utilization of complementarity-determining region-3 spectratyping analysis of the TCR repertoire might be useful to monitor specific immunoactivation in patients before and after kidney transplantation. Keywords: dialysis, nonfunctioning k