Despite a widespread preconception that HD should be reserved for cases in which PD is not feasible, in Europe, we found 1 in 8 infants in need of maintenance dialysis to be initiated on HD therapy. Patient characteristics at dialysis therapy initiation, prospective survival, and time to transplantation were very similar for infants initiated on PD or HD therapy.

The epidemiology of glomerular disease in our single-center report is similar to that in data from adjacent Croatia and Greece. Focal segmental glomerulosclerosis was the dominant histopathological finding, followed by mesangioproliferative GN and IgA nephropathy.

Arterial hypertension is a risk factor affecting graft function in pediatric kidney transplants. Recent pediatric studies reported a high prevalence of hypertension, especially nocturnal hypertension in this population. Data regarding the prevalence of masked hypertension in pediatric patients with kidney transplants are still scarce. The aim of this cross-sectional study was to assess the prevalence of masked and hidden uncontrolled hypertension after renal transplantation. A total of 41 patients (25 males) with stable functioning renal graft were included in the study. Their median age was 14.5 years with the median interval since transplantation of 2.5 years (range 0.3 to 20.6). Spacelabs 90207 was used to measure ambulatory blood pressure (BP) during a 24-h period. Ambulatory hypertension was defined as mean systolic and/or diastolic BP index at day-time or nighttime >or=1. Masked hypertension was defined as normal office BP but daytime ambulatory hypertension in patients without antihypertensive medications. Hidden uncontrolled hypertension was defined as daytime ambulatory hypertension undetected by office BP measurements in treated patients. Antihypertensive medications were prescribed to 58%. Prevalence of nocturnal hypertension was 68%. On the basis of combination of office and ABPM masked hypertension and hidden uncontrolled hypertension was detected in 24% and 21% of the study population, respectively. Regular use of ambulatory blood pressure monitoring in transplanted patients enables detection of masked and hidden uncontrolled hypertension.

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenal involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.

The objective of this study was to investigate whether DNA polymorphisms of the renin-angiotensin system (RAS) genes were associated with renal scar formation in pediatric patients with bladder dysfunction (BD). Although these children are born healthy, due to persistence of immature voiding habits and evolution of BD, some develop progressive renal damage. It has been suggested that the DD genotype of the angiotensin I-converting enzyme (ACE) gene might be an adverse renal prognostic factor. The insertion/deletion (I/D) polymorphism of the ACE gene and the A1166C polymorphism of the angiotensin II type 1 receptor (ATR1) gene were identified by polymerase chain reaction amplification in 42 children with BD (aged 5-14 years) and 198 healthy adult controls. Twelve children had urgency syndrome and 30 had dysfunctional voiding. Renal scarring was found in 16 patients, while 26 patients had normal kidneys on dimercaptosuccinic acid scan. In children with renal lesions there was significant over-representation of the DD genotype compared with either controls or patients without renal damage ( P<0.05). On multivariate analysis, the DD genotype was the only factor that had a significant impact on renal scar formation, introducing a 2.51-fold risk (odds ratio 2.51, 95% confidence interval 1.04-6.04, P=0.04). The A1166C gene polymorphism was not significantly associated with the development of parenchymal damage in children with BD. Our findings introduce ACE I/D gene polymorphism as an independent risk factor for parenchymal destruction in pediatric patients with BD.