The Outcome of Childhood Immunoglobulin A Nephropathy with Acute Kidney Injury at the Onset of the Disease-National Study.

Introduction: IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. Decreased glomerular filtration rate is a known risk factor for disease progression. Aim: We aimed to examine factors that may contribute to disease progression in children that present with impaired eGFR at the onset of IgAN. Materials and methods: Of the 175 patients with IgAN from the Polish Registry of Children with IgAN and IgAVN, 54 (31%) patients with IgAN who had an onset of renal function impairment (GFR < 90 mL/min) were eligible for the study. All of them were analyzed for initial symptoms (GFR according to Schwartz formula, creatinine, proteinuria, IgA, C3), renal biopsy result with assessment by Oxford classification, treatment used (R-renoprotection, P-prednisone+R, Aza-azathioprine+P+R, Cyc-cyclophosphamide+P+R, CsA-cyclosporine+P+R, MMF-mycophenolate mofetil+P+R), and distant follow-up. Based on the GFR score obtained at the end, patients were divided into two groups: A-GFR > 90 mL/min and B-GFR < 90 mL/min. Results: In the study group, the mean age of onset was 12.87 ± 3.57 years, GFR was 66.1 ± 17.3 mL/min, and proteinuria was 18.1 (0-967) mg/kg/d. Renal biopsy was performed 0.2 (0-7) years after the onset of the disease, and MESTC score averaged 2.57 ± 1.6. Treatment was R only in 39% of children, P+R in 20%, Aza+P+R in 28%, Cyc+P+R in 9%, CsA+P+R in 7%, and MMF+P+R in 3%. The length of the observation period was 2.16 (0.05-11) years. At the follow-up, Group A had 30 patients (56%) and Group B had 24 patients (44%). There were no significant differences in any of the other biochemical parameters (except creatinine) or proteinuria values between the groups and the frequency of the MESTC score ≥ 2 and <2 was not significantly different between Groups A and B. Patients with normal GFR at the follow-up (Group A) were significantly more likely to have received prednisone and/or immunosuppressive treatment than those in Group B (p < 0.05) Conclusions: In a population of Polish children with IgAN and decreased renal function at the onset of the disease, 56% had normal GFR in remote observation. The use of immunosuppressive/corticosteroids treatment in children with IgAN and impaired glomerular filtration rate at the beginning of the disease may contribute to the normalization of GFR in the outcome, although this requires confirmation in a larger group of pediatric patients.

[Value of bioelectrical impedance analysis in the assessment of body water in children with nephrotic syndrome: initial results]. / Zastosowanie bioimpedancji do oceny przestrzeni plynowych u dzieci z zespolem nerczycowym--wyniki wstepne.

The study aims at assessing the value of bioelectrical impedance analysis in estimation of fluid compartments in children with nephrotic syndrome. The study included 23 children aged, 2 and 20.5 y. In 9 children in remission measured parametres were normal. In the group of 14 children with relapses and oedemas, significantly higher TBW and ECM/BCM were observed. These parameters decreased during the course of disease with clinical improvement, but did not reach normal levels at the disappearance of oedemas. BIA is a simple, non-invasive and valuable method for assessment of total body water and fluid compartments.

Progression to end-stage renal disease in children with posterior urethral valves.

Diagnostic and therapeutic strategies in boys with congenital posterior urethral valves (PUV) have much improved in past decades, but the impact of these changes on the progression to end-stage renal disease (ESRD) has rarely been investigated. We followed renal function in 20 boys with PUV from diagnosis to ESRD. From the first observation period (1969-1978) to the second period (1979-1992) we found a marked drop in age at diagnosis, at valve resection, at first increase of serum creatinine (SCr), and at onset of ESRD. The progression was analyzed by calculating the slope of 1/SCr and the probability of renal survival. In all patients combined, renal survival at the age of 10 years was 35%. In children undergoing valve resection in the 1st year of life, renal survival was worse than in those undergoing later surgery (15% vs. 65% after 10 years, P=0.006). Patients with a SCr>1.2 mg/dl before the age of 12 months progressed more rapidly to ESRD than those attaining this level later. The lower the minimum level of SCr observed after initial surgery, the older the patient at the onset of ESRD. The presence of renal dysplasia or hypoplasia, but not of vesicoureteric reflux, was associated with a more rapid progression. Mean body height at ESRD was -2.3+/-1.3 standard deviation score compared with controls, and was lower if PUV was diagnosed before the age of 6 months.

[Use of urea kinetic modeling for evaluating the efficacy of reusing capillary dialyzers]. / Zastosowanie modelowania kinetycznego mocznika do oceny skutecznosci reutylizacji dializatorów kapilarnych.

The aim of the study was to evaluate the influence of follow fiber dialyzers reprocessing procedure on urea kinetic modeling parameters, dialysis effectiveness for small molecules and effective clearance/predicted clearance ratio. Fifty patients (27 F, 23 M) aged 18-61 years (mean 47) being on maintenance hemodialysis due to end stage renal failure for 4-136 months were included into the study. Patients were treated 3 times a week with blood flow 160-290 ml/min and dialysate flow 500 ml/min. The dialysis time was calculated individually based on urea kinetic modeling parameters. Reprocessing of dialyzers was performed using Renatron RS 8300 and Renalin as sterilized agent. Dialysers with ppr below 80% were excluded from the use. Mean values of dialysis index Kt/V, TAC and dialysis effectiveness (Ct/C0) for urea, creatinine, uric acid and kalium as well as values of effective/predicted clearance ratio for consecutive reprocessing procedures were compared with the results obtained for the new dialyzers. There was no significant influence of reprocessing procedure on Kt/V, TAC and Ct/C0 for tested substances and studied clearances. The obtained results indicates that dialysers reprocessing procedure performed according to the described protocol was safe and didn't decrease dialysis effectiveness.

Long-term Cyclosporine A (Sandimmun) therapy for steroid resistant nephrotic syndrome in children.

The aim of the paper was to evaluate Cyclosporine A (CsA) treatment in children with steroid resistant nephrotic syndrome (diagnosed according to criteria established by ISKDC), in whom numerous attempts at other types of therapy had failed. CsA was used in 23 children aged 2-16 years. Renal biopsies revealed minimal changes (MCD) in 7 children, focal segmental glomerulosclerosis (FGS) in 11, mesangial glomerulonephritis (MES) in 4, and membrano-proliferative glomerulonephritis (MPGN) in 1. CsA was administered for a period of 6-41 months at a daily dose of 6 mg/kg, gradually decreased to 2.5 mg/kg. Blood CsA level was 40-200 ng/ml. In the course of the treatment, proteinuria disappeared or diminished and clinical improvement was seen in 21 patients. 13 children had relapses of nephrotic syndrome, showing very low blood CsA levels. Complete remissions were achieved in 16 children, partial remissions in 5, no remission in 2. Throughout the treatment and after its cessation 22 patients manifested no deterioration of renal function. In 1 child, repeated renal biopsies revealed some lesions that could suggest a nephrotoxic effect of CsA. Secondary steroid sensitivity observed in 3 children should be emphasized. Concluding, CsA is an effective and well tolerated agent in children with steroid resistant nephrotic syndrome, and the improvement noted in patients with FGS may reduce the risk of early terminal failure. The treatment requires monitoring blood CsA levels.

[Use of urea kinetic modeling in patients on maintenance hemodialysis]. / Zastosowanie modelowania kinetycznego mocznika u chorych leczonych powtarzanymi hemodializami.

The effect of urea kinetic modeling (u.k.m.) application on dialysis efficiency and metabolic status was evaluated in 50 maintenance dialyzed patients. U.k.m. sessions were performed once a month based on the self-developed computer program to control dialysis. The dialysis index (Kt/V), the time averaged concentration (TAC), protein catabolic rate (pcr) and dialysis effectiveness (Ct/Co) were evaluated and the results obtained at the beginning and after 2, 4, and 8 months of the study were compared. Kt/V had risen significantly in the modeled patients from 1.04 to 1.24 and was accompanied by 12% Ct/Co increase of urea removal after 8 months. The tendency of the moderate (non significant) decrease of TAC from 54.57 to 52.48 mg% BUN was observed during the study. According to the NCDS criteria the percentage of adequately dialyzed patients increased from 42% at the beginning to 64% after 80 months; underdialyzed patients decreased from 16% to 6% and malnourished also from 16% to 6%, respectively, after u.k.m. application. Dialysis effectiveness for creatinine and uric acid described by Ct/Co for the above after 4 and 8 months was significantly increased when compared with the results obtained at the beginning of the study. These results indicate that u.k.m. application allowed to take control over uremic toxemia and improved dialysis adequacy in patients on maintenance dialysis. Protein catabolic rate in studied patients increased from 1.18 to 1.24 g/kg/per day during the study and it was accompanied by a total blood protein and serum albumin increase. This could indicate improvement of the nutritional status of dialyzed subjects.