Renal Volume in ADPKD Patient Evaluation

Abstract

The clinical manifestations of ADPKD are related to the growth of renal cysts. Renal volume has been recognised as the biomarker that is able to identify those patients at risk of complications (hypertension and haematuria) and at risk of progression to End Stage Renal Disease (ESRD). Recently, several scores have been introduced to predict the evolution of ADPKD. The Mayo Clinic Group developed a classification based on renal volume as measured by CT or MRI and corrected for age and height (Ht-TKV); this allowed predicting the evolution of the disease, but it has not been fully validated so far. In addition, it is used to identify patients labelled as “fast progressors” and eligible for Tolvaptan therapy according to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendations. We studied 80 patients who underwent MRI and had been classified as ADPKD typical form (class 1A-1E). A significant correlation between renal volume, hypertension, and low GFR was found (p<0.005). A progressive increase in disease severity has been found across the different Mayo classes; 41.2% were eligible for Tolvaptan therapy. The results demonstrate that the Mayo method is easy to perform and provides valid information in order to identify with rapidly progressing disease.

Safety and feasibility of synchronous unilateral nephrectomy and contralateral heminephrectomy for extremely severe autosomal dominant polycystic kidney disease: Techniques and outcome

“…However, most of the studies demonstrating minimally invasive approaches are limited to the lowest (class 1A) and intermediate (class 1B and 1C) severity groups of patients with a relatively small size of affected kidney and with low morbidity. The highest severity group (class 1D and 1E) tended to have a higher risk of hypertension, low GFR, hematuria, and greater morbidity and mortality during surgical resection [ 13 14 ]. In fact, Seoul National University Hospital (Seoul, Korea) previously experienced a 20% rate of immediate or delayed mortality due to acute hypotension in this group of patients.…”

“…Thus, there are a couple of factors to consider in surgical treatment planning for these patients. First, the laparoscopic approach is not feasible because of the extremely large kidney volume [ 13 14 ]. Second, these patients require a surgical approach that satisfies the conditions of maximal resection of the kidney while at the same time safely preserving a portion of the kidney.…”