The influence of renal manifestations to the progression of autosomal dominant polycystic kidney disease.

BACKGROUND: Renal stones, urinary tract infections (UTI) and gross hematuria (GH) are the most important renal manifestations of autosomal dominant polycystic kidney disease (ADPKD). They are not only common, but are also frequent cause of morbidity, influencing renal dysfunction. The aim of this study was to evaluate the frequency of these manifestations in our patients with ADPKD and their impact on renal function. METHODS: One hundred eighty ADPKD patients were included in the study. Subjects were studied for the presence of UTI, gross hematuria frequency and responsible factors of nephrolithiasis. Survival times were calculated as the time to renal replacement therapy or time of serum creatinine value up to 10 mg/dl. Kaplan-Meier product-limit survival curves were constructed, and log rank test was used to compare the survival curves. RESULTS: Kidney stones were present in 76/180 (42% of pts). The stones were composed of urate (47%) calcium oxalate (39%), and other compounds 14%. UTI was observed in 60% (108 patients). Patients treated with urinary disinfectants had a significant lower frequency of urinary infection (p<0.001) and hematuria (p<0.001) after one year than untreated patients. Gross hematuria was present in 113 patients (63%). In 43 patients hematuria was diagnosed before age 30 (38%), while in 70 patients it was diagnosed after age 30 (62%). CONCLUSIONS: UTI is frequent in our ADPKD patients. The correct treatment of UTI decreases its frequency and has beneficial role in the rate of progression to renal failure in ADPKD patients. Patients with recurrent episodes of gross hematuria may be at risk for more severe renal disease.

[The relation of hypertension, renal function and cardiovascular events in autosomal dominant polycystic kidney disease]. / La relazione tra l'ipertensione arteriosa, la funzione renale e gli eventi cardiovascolari nei pazienti con malattia policistica autosomica dominante.

BACKGROUND: The presence of hypertension in autosomal dominant polycystic kidney disease (ADPKD) appear to be related with progressive renal failure and end-organ damage, including left ventricular hypertrophy (LVH) in these patients. The aim of this study was to evaluate the frequency of hypertension in ADPKD patients and its correlation with renal function, renal structure and its influence in left ventricular wall. METHODS: Two hundred patients were included in the study. The patients were divided in two groups: first group of 92 patients with normal renal function, and second group of 108 patients with chronic renal failure. All patients performed an abdominal ultrasound and a M-mode echocardiography. RESULTS: Hypertension was observed in 140 ADPKD patients (70%). Subjects who developed hypertension before age 35 had worse renal survival than those who remained normotensive after age 35 (50 years vs. 62 years; p<0.0001; risk ratio = 4.3). Hypertensive patients had significantly higher serum creatinine concentration than those without hypertension (p<0.001). LVH was present in 56 patients with hypertension (40%) and in 9 normotensive patients (16%) (p<0.005). Patients with LVH had a worse renal survival than those without LVH (p<0.001). CONCLUSIONS: These findings suggest that hypertension is a serious complication in ADPKD that may lead to both an increased incidence of cardiovascular complications and more rapid progression of renal functional impairment.

Secondary retroperitoneal fibrosis associated with generalized atherosclerosis.

Retroperitoneal fibrosis is an uncommon disease that often presents in a subtle manner. Only a few cases of the combined association of generalized atherosclerosis and retroperitoneal fibrosis are reported in the recent literature, supporting the view that the condition is probably an autoimmune periaortitis. We describe a typical case of retroperitoneal fibrosis associated with generalized atherosclerosis with clinical presentation of progressive renal insufficiency, and claudication from arterial compromise.

The epidemiology of chronic renal failure and provision of renal services in Albania.

Tirana, the only dialysis facility in Albania (pop 4 million), has a stock of 12 patients and three haemodialysis machines. To determine the need for renal services in Albania we studied the incidence and outcome of patients with chronic renal failure (CRF) referred to the renal service in Tirana (pop 300 000) over 1 year. Case-notes of all patients with a serum creatinine concentration > or = 300 mumol/l during the study period (1992) were examined and outcome at 2 years recorded for each patient. In all, 84 patients (mean age 41.6 +/- 17.5 years, 56% male) were referred to nephrologists of whom 35 (42%) came from Tirana, giving an annual incidence of 116 per million pop. 77% were under 40 and had no co-morbid illness. Glomerulonephritis, the most common renal diagnosis, affected 26% patients. 22% patients (mean age 38 +/- 18.1) died within 2 years and only 5% received dialysis. The mean age of those who received dialysis was 29 +/- 8.3 compared with those who were not dialysed (42 +/- 18.0). The 59 patients (24 from Tirana i.e. 80 per million) who were alive with advanced CRF (creatinine > 500) had a mean creatine of 623 +/- 93 mumol/l and would be candidates for dialysis. Patients with progressive renal failure in Albania are regularly followed and treated with antihypertensives and dietary modification. The need for RRT, however, is not being met even for young patients with no co-morbidity.