Search

Background: Primary focal segmental glomerulosclerosis (FSGS) is a glomerular disease, characterized by progressive renal function deterioration, nephrotic proteinuria, and risk of chronic renal failure. We present long-term results of 5 patients with primary FSGS and recurrence of nephrotic proteinuria after renal transplantation treated with plasma exchange (PE) and immunoadsorption (IA)., Methods: We retrospectively investigated the relationship between the delay in initiation of the therapy and treatment outcomes, particularly achievement of remission of proteinuria., Results: Remission occurred in all three patients who started PE/IA in interval 3-7 days after diagnosis of recurrence of FSGS. Remission was achieved after 3-4 weeks in two patients with 3 days of delay to the start of PE. The third patient (PE started with 7 days of delay) reached complete remission after 6 months of PE/IA treatment. All these patients had remission sustainable for a long time. The remaining two patients with 14 and 406 days of delay to PE treatment did not achieve remission sustainable for a long time. The two patients who did not achieve remission developed end-stage renal disease with graft loss (1 and 6.7 years after transplantation). Patients who achieved remission of proteinuria during PE/IA treatment have still functioning grafts (2.8, 9.7 and 3.8 years after renal transplantation). All these patients are still treated with PE/IA., Conclusions: The present 5 cases suggest that if recurrence of FSGS occurs, the probability of achieving remission is dependent on the early initiation of PE/IA therapy. Therefore, we suggest that PE/IA treatment might be started as soon as possible after recurrence of FSGS.

Background: Proteinuria is a common manifestation of chronic kidney disease (CKD), and there is a high incidence of CDK and its complications following renal transplantation. However, little data are available on the association between proteinuria and graft/patient survival in the paediatric transplant population. The primary aim of this study was to investigate the associations between posttransplant proteinuria and graft/patient survival in children after renal transplantation., Methods: In this retrospective study, we screened all 91 children receiving renal allografts at a single institution between 1997 and 2007. The inclusion criteria were a functioning graft at 1 year posttransplant, data availability and no recurrence of focal-segmental glomerulosclerosis. The final cohort included 75 patients. Proteinuria was considered to be pathologic if the urinary protein/creatinine ratio was >30 mg/mmol. Donor and recipient characteristics, data on proteinuria, estimated glomerular filtration rate (eGFR) and rejection episodes were analysed. The most recent of the biopsies performed during the follow-up after 1 year posttransplant were analysed separately in the proteinuric group and the non-proteinuric group., Results: Proteinuria at 1-year posttransplant was pathologic in 35 % of patients. The 5-year graft survival rate was significantly lower in the proteinuric group than in the non-proteinuric group (77 vs. 100 %; p < 0.001). Proteinuria at 1 year posttransplant was associated with reduced long-term graft survival independent of other risk factors, including decreased eGFR or episodes of acute corticosensitive and corticoresistant rejection. The most frequent histologic finding in the proteinuric group was chronic rejection. There was no significant difference in the 5-year patient survival rate between the proteinuric group and the non-proteinuric group., Conclusion: This study emphasizes the importance of proteinuria as a prognostic factor of renal allograft survival in children.

Seeman T, Dušek J, Vondrák K, Janda J. Ramipril in the treatment of proteinuria in children after renal transplantation. Pediatr Transplantation 2010:14:283–287. © 2009 John Wiley & Sons A/S. The efficacy and safety of ACEI in adult patients with hypertension and proteinuria after renal transplantation is proven however data on the effectiveness of ACEI in transplanted children are rare. The aim of the present study was to investigate the effect of ramipril on proteinuria and BP in children after R-Tx. Twelve transplanted children (median age 15.3 yr, median time after R-Tx 4.5 yr) with proteinuria with or without hypertension were prospectively treated with ramipril for six months. Proteinuria was assessed as protein/creatinine ratio. Office BP was evaluated and hypertension defined as BP ≥95th centile. Graft function was assessed (Schwartz formula). The starting dose of ramipril was 1.5 mg/m2/24-h. Proteinuria declined in 92% of children from a median 39 to 22 mg/mmol creatinine (p < 0.01). The median decline of proteinuria was 9 mg/mmol creatinine, it reached 23% of the initial values. The prevalence of hypertension did not change significantly (50% initially vs. 33% after six months). Graft function and serum potassium level did not change significantly, two children developed mild hyperkalemia. Ramipril can reduce proteinuria in most transplanted children; its antiproteinuric effect is exhibited even without BP lowering effect. [ABSTRACT FROM AUTHOR]

Background/Aims: In adults, nighttime hypertension and hyperuricemia are new risk factors for progression of IgA nephropathy (IgAN). In children, nighttime blood pressure (BP) and serum uric acid have never been investigated. The aim of the study was to investigate nighttime BP and uric acid in children with IgAN. Methods: Data on children with IgAN from two pediatric nephrology centers were retrospectively reviewed (renal biopsy – subclasses according Hass I–V, ambulatory blood pressure monitoring ABPM, serum uric acid, proteinuria). Results: Twenty-eight untreated children with IgAN were included. Hypertension was diagnosed on the basis of ABPM in 54% of children, 50% were nondippers and 25% have isolated nighttime hypertension. The mean ambulatory BP was higher at nighttime than during daytime (systolic nighttime BP 1.11 ± 0.79 SDS vs. daytime 0.59 ± 0.79, diastolic nighttime BP 1.16 ± 0.95 vs. daytime 0.52 ± 1.10, p < 0.01 for systolic and p = 0.01 for diastolic). Children with severe histological subclasses (III–IV) tended to have higher prevalence of hypertension than children with mild subclasses (I–II), 67% vs. 38%, p = 0.13. Hyperuricemia was diagnosed in 14% of children. A significant correlation was found between proteinuria and histopathological subclasses (r = 0.44, p < 0.05). Conclusion: Children with IgAN have often nighttime hypertension. Hypertension and proteinuria are associated with severe histopathological findings. Hyperuricemia is a rare finding in children. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background/Aim: Unilateral renal agenesis (URA) is a model for a reduced nephron number that is believed to be a risk factor for blood pressure (BP) elevation and reduced renal function. The aim of the study was to investigate BP and renal function in children with URA. Methods: Data on children with URA from two pediatric nephrology centers were firstly retrospectively reviewed (renal ultrasound and scintigraphy, clinical BP, creatinine clearance, urinalysis). Children with normal renal ultrasound and scintigraphy were thereafter investigated using ambulatory BP monitoring. Results: Twenty-nine children with URA were investigated – 14 children with an abnormal kidney (mostly scarring) and 15 children with healthy kidneys. Hypertension was diagnosed on the basis of clinical BP in 57% of the children with abnormal kidneys and on the basis of ambulatory BP monitoring in 1 child (7%) with healthy kidneys. The mean ambulatory BP in children with normal kidneys was not significantly different from that in controls. Forty-three percent of the children with abnormal kidneys had a reduced renal function, but none of children with normal kidneys. Conclusions: Children with abnormalities of a solitary kidney have often hypertension, proteinuria, or a reduced renal function. In contrast, children with healthy solitary kidneys have BP and renal function similar to those of healthy children. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

: BackgroundAngiotensin-converting enzyme inhibitors are the drugs of choice in renal hypertension. The efficacy and safety of ramipril in adults has been proved; however, data on effectiveness of ramipril in children are few. The aim of the present study was to investigate the effect of ramipril on blood pressure (BP) and proteinuria in children with chronic kidney diseases.: MethodsA total of 31 children (median age 11.3 years, range 1.9–19.8 years) with various chronic nephropathies and hypertension or proteinuria were prospectively treated with ramipril for 6 months. Blood pressure was evaluated using ambulatory BP monitoring and hypertension was defined as mean BP equal to or greater than the 95th percentile for healthy children. Proteinuria was defined as protein excretion ≥100 mg/m2/24 h. The starting dose of ramipril was 1.5 mg/m2/24 h once daily. In 27 children it was given as monotherapy.: ResultsThe median decrease in ambulatory BP was 11 mm Hg for daytime systolic, 10 mm Hg for daytime and nighttime diastolic, and 8 mm Hg for nighttime systolic BP. Hypertension normalized in 55% of the children. Proteinuria decreased in 84% of the children with pathologic proteinuria; the median decrease was 51%. A positive correlation was found between initial proteinuria and change of proteinuria (r = 0.95, P < .001). Glomerular filtration rate and serum potassium level did not change significantly. One child developed a cough that was believed to be related to ramipril.: ConclusionsRamipril is an effective and safe drug in children with chronic kidney diseases associated with hypertension, proteinuria, or both. [Copyright &y& Elsevier]

Objectives: Strict BP control can retard progression of CKD in children. This prospective 3‐year randomized controlled trial is aimed to investigate whether strict BP control can retard progression of chronic allograft dysfunction. Methods: Twenty‐three pediatric patients were randomly selected to the standard BP group (STAND, target 24‐hour MAP 50‐95th percentile, n = 11) or the intensified BP group (INTENS, target 24‐hour MAP <50th percentile, n = 12). The primary endpoint was an annual reduction in eGFR (Schwartz formula, mL/min/1.73 m2/y), secondary graft survival, BP, proteinuria, and safety. Results: A total of 21 children (age at entry 11.2 (range 6.2‐16.8) years) completed the study, with 73% of children in INTENS and 70% of children in STAND group reached their goal BP. Ambulatory indexed 24‐hour MAP decreased significantly in INTENS group (from 0.94 (range 0.86‐1.17) to 0.85 (range 0.79‐1.01, P < 0.01)) but not in STAND group (from 0.93 (range 0.85‐1.07) to 0.90 (range 0.84‐1.01)). Proteinuria did not change significantly in either group (22.1 mg/mmol creatinine to 15.3 in STAND group vs 25.7 to 11.8 in INTENS group). The annual reduction in eGFR did not differ between the INTENS and STAND groups (−1.9 mL/min/1.73 m2/y (range +6.4 to −14.3) vs −0.9 (range +4.0 to −8.5)). Conclusion: This first randomized controlled trial on strict BP control has demonstrated that strict BP control is feasible in 73% of children but the strict BP control does not lead to retardation of graft function decline in comparison with standard BP control. However, the results need to be interpreted with caution keeping the major limitation of the study, that is, small sample size in mind. [ABSTRACT FROM AUTHOR]