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.

Evaluation of bladder capacity in children with lower urinary tract symptoms: Comparison of 48-hour frequency/volume charts and uroflowmetry measurements.

INTRODUCTION: Objective evaluation of bladder capacity (BC) in children with lower urinary tract symptoms (LUTS) is important for recognizing types of bladder dysfunction. Bladder capacity is evaluated from 48-hour frequency/volume (48-h F/V) charts or by uroflowmetry with ultrasound post-void assessment. There are limited data on the reliability of both methods of assessment in children. OBJECTIVE: The aim of the study was to compare two modalities of assessment, (F/V chart and uroflowmetry) in cohorts of children with bladder dysfunctions. STUDY DESIGN: Maximum bladder capacity (MBC) obtained from 48-h F/V charts was compared with volumes calculated from uroflowmetry in a cohort of 86 children with different bladder dysfunctions. The BC obtained by the two modalities was compared for the three most frequent subtypes of bladder dysfunction: monosymptomatic nocturnal enuresis (MNE), overactive bladder (OAB), and dysfunctional voiding (DV). Considering a 48-h F/V chart as standard, the sensitivity, specificity, negative and positive predictive values of uroflowmetry measurements were calculated for detecting low bladder capacity. RESULTS: The mean maximal bladder capacity (188 ± 99.42 ml) obtained from home 48-h F/V chart measurement was 17 ml lower than the mean value obtained from uroflowmetry (205 ± 112.11 ml) (P = 0.58). The differences between bladder capacities estimated by 48-h F/V chart and uroflowmetry for subjects were not significant (Figure). Concordance between 48-h F/V chart and uroflowmetry categorization of BC was present in 64 (74%) subjects. The sensitivity and specificity of uroflowmetry, in comparison with 48-h F/V chart evaluation, for recognizing low bladder capacity were 75.5% and 73.17%. The sensitivity and specificity for the different types of LUTS achieved 68.42% and 58.83% for OAB, 80% and 83% for MNE, and 50% and 83.3% for DV. DISCUSSION: According to the International Children's Continence Society, the management of MNE in children can be made without uroflowmetry. History and MBC evaluation by 48-h F/V charts yields sufficient information. Nevertheless, in situations where F/V charts are unreliable or unavailable, uroflowmetry can be used as an alternative method. The highest discrepancy between both methods of BC evaluation was found in DV; this was mainly due to the mean PVR of 31 ml. CONCLUSION: For children with MNE, both 48-hour frequency/volume charts and triplicate urine flow measurement with PVR evaluation are reliable methods of maximum bladder capacity evaluation. For children with OAB or DV, both methods may be necessary for accurate evaluation of decreased BC, as F/V chart and uroflow results may not be comparable.

Prevention and treatment of renal osteodystrophy in children on chronic renal failure: European guidelines.

Childhood renal osteodystrophy (ROD) is the consequence of disturbances of the calcium-regulating hormones vitamin D and parathyroid hormone (PTH) as well as of the somatotroph hormone axis associated with local modulation of bone and growth cartilage function. The resulting growth retardation and the potentially rapid onset of ROD in children are different from ROD in adults. The biochemical changes of ROD as well as its prevention and treatment affect calcium and phosphorus homeostasis and are directly associated with the development of cardiovascular disease in pediatric renal patients. The aims of the clinical and biochemical surveillance of pediatric patients with CRF or on dialysis are prevention of hyperphosphatemia, avoidance of hypercalcemia and keeping the calcium phosphorus product below 5 mmol(2)/l(2). The PTH levels should be within the normal range in chronic renal failure (CRF) and up to 2-3 times the upper limit of normal levels in dialysed children. Prevention of ROD is expected to result in improved growth and less vascular calcification.

[Peritonitis in children treated with automated peritoneal dialysis]. / Zapalenie otrzewnej u dzieci leczonych automatyczna dializa otrzewnowa.

The rate of peritonitis, its etiology and efficacy of treatment was compared between 28 children treated with APD for a total of 439 dialysis months and 24 children on CAPD treated for 328 dialysis months. The peritonitis rate was significantly lower in children on APD (1 episode per 27 months) than in children on CAPD (1 episode per 17 months) (p = 0.005). The etiology of peritonitis (68% v. 62% Gram + organisms), efficacy of treatment (100% v. 100%) and relapse rate did not differ between the machine operated and manual methods of peritoneal dialysis.

[Results of treatment with recombinant human growth hormone in children with growth retardation in end-stage renal disease]. / Wyniki leczenia rekombinowanym ludzkim hormonem wzrostu niskoroslosci dzieci ze schylkowa niewydolnoscia nerek.

The aim of the study was to estimate the results of recombinant human growth hormone (rhGH) treatment in children with end-stage renal disease (ESRD). 60 growth retarded children with ESRD (mean age 11.2 +/- 7.2 years) were treated with rhGH at a dose of 1-1.1 IU/kg/week. The time of observation was 24 months. Thirty children completed first year, 18--second year of treatment. The mean growth velocity prior to the treatment was 3.03 +/- 1.9, during first year of the study--7.52 +/- 2.42, during second year 6.68 +/- 2.87 cm/year. The negative correlation between growth velocity and patient's age (r = -0.39; p < 0.05) suggest the better growth results in younger children during rhGH treatment. The rhGH therapy is effective method of treatment in growth retarded children with ESRD. Side effects are rare.

[Epidemiology of HBV infections and possibilities for therapeutic actions in children and adolescents with end-stage renal failure treated with dialysis]. / Epidemiologia zakazen HBV i mozliwosci dzialan terapeutycznych u dzieci i mlodziezy ze schylkowa niewydolnoscia nerek leczonych nerkozastepczo.

Wide spreading of prophylaxis principles of HBV infections in dialysis centers decreased the HBV infection rate in general population of dialyzed patients in Poland last years. There is neither data concerned with HBV infection epidemiology in children and adolescents, nor data about anti-viral treatment possibilities and effects in this group of dialyzed patients. The aim of the study was evaluating of HBV infection rate in patients of pediatric dialysis centers and analysis of causes of infection and efficacy of treatment. Study was based on data sent in a query-answer by 8 biggest pediatric dialysis centers, all of them treating 210 patients. HBV infection was found much more often (16.6%) than in population of all hemodialyzed patients in Poland. More than 75% non-vaccinated patients was infected before dialysis therapy, remaining were infected during vaccination, before the protecting level of antibodies was gained. Big differences in HBV infection rate among centers are observed. Nowadays HCV infections (more than 40% patients infected) are a bigger issue. Only 10 patients in 5 centers had anti-viral treatment (5 with isolated HBV infection, 5 with mixed HBV/HCV infection). In 9 patients interferon-alpha and in 1 patient lamivudine was administered. Efficacy of interferon-alpha treatment was similar to the population of non-uremic children (33.3% vs. 50% of HBeAg elimination). Majority of patients quite well tolerated the drug. Only in 1 case interferon-alpha treatment had to be ceased because of side effects. In a boy treated with lamivudine, after 3 months elimination of viremia and decrease of ALAT activity was observed. HBV infection in patients of pediatric dialysis centers is still a serious matter. More strict applying of vaccination against hepatitis B before dialysis treatment is needed. The possibility of HBV infections therapy is limited, mostly for economical reasons.

[Efficacy and safety of renal biopsy in children performed with an automatic biopsy device under control of ultrasonographic imaging]. / Skutecznosc i bezpieczenstwo biopsji nerki u dzieci wykonanej aparatem automatycznym pod kontrola obrazu ultrasonograficznego.

UNLABELLED: The efficacy and safety of kidney biopsy in children performed with an automatic Biopty Gun device under real time ultrasound was analysed. The procedure was performed under sedation during short hospitalisation with ultrasound assessment of complications performed after 24 hours. 98% of 131 biopsies yielded representative material with an average 16 glomeruli obtained per biopsy. Symptomatic perirenal haematomas were observed in 80%, subcapsular haematomas in 3.8% and arteriovenous fistulas in 4.6% of children on post biopsy ultrasound. They resolved spontaneously in all patients. CONCLUSIONS: Real time ultrasound guidance with the use of automatic biopsy devices should become the standard practice for kidney biopsy in children due to its efficacy and safety. The procedure should be performed during short hospitalization to enable ultrasound monitoring of early complications.

[Bilateral hydrothorax in a 13-year old girl treated with continuous ambulatory peritoneal dialysis renal replacement therapy]. / Obustronny przezprzeponowy przeciek plynu dializacyjnego do jam oplucnowych u 13-letniej dziewczynki leczonej nerkozastepczo metoda ciaglej cyklicznej dializy otrzewnowej.

Bilateral hydrothorax is a very rare complication of ambulatory peritoneal dialysis in children. We present a case of a 13-year-old girl who developed bilateral acute hydrothorax during after seven months of CCPD. Peritoneal dialysis was temporarily discontinued and the child was transferred to haemodialysis. Six weeks later the patient was successfully resumed CCPD.

[Changing clinical course of hemolytic uremic syndrome in children]. / Zmieniajacy sie obraz zespolu hemolityczno-mocznicowego u dzieci.

An incidence of hemolytic-uraemic syndrome as a cause of the acute renal failure in childhood, its early survival rate and long-term sequela quences were compared in the 1970s, 1980s, and 1990s in the same geographical area. 196 children with HUS were treated between 1972-1999. 94% had a typical D+ HUS. The majority were of patients severely ill with 87% requiring peritoneal dialysis of an average 14 days duration. The children were grouped into three 9-year periods for comparison (1972-1980, 1981-1989, 1990-1999). 105 patients were followed for 2-27 years (mean 1 years). An incidence of HUS as a cause of ARF in childhood ranged from 58% and 50% in the 1970s and 1980s to 30% in the past decade. With time survival rate has improved dramatically over the years from 80% and 75% to 97% in the 1990s. Deo spite better short term outcome the late consequences of HUS are serious. The longer the follow-up, the greater percentage of patients with severe outcome. At last follow-up only 32% children showed full recovery. 33% are on renal replacement therapy or have impaired renal function. The severity of the initial lesions to the kidney measured by the duration of anuria is a marker of poor late prognosis. With improving early outcome the clinical significance of HUS is focusing on its severe long-term consequences.

[Hypertension in children with chronic renal failure]. / Nadcisnienie tetnicze u dzieci z przewlekla niewydolnoscia nerek.

Hypertension is one of the main risk factors of mortality for children on renal replacement therapy. It has also been recognised as one of the major risk factors for progression of renal failure. The aim of the study was to define the prevalence of hypertension in children with chronic renal failure, treated in a single centre, and to assess the efficacy of its diagnosis and management. Hypertension was present in 27% of 40 children before the onset of chronic renal failure, increasing to 57% with the development of CRF, and reaching 86% at onset of dialysis. Reflux nephropathy, hemolytic-uraemic syndrome and glomerular disease were most frequently associated with severe hypertension. ACE inhibitors (70%), diuretics (52%), and calcium channel blockers were the most frequently used antihypertensives with 49% of the children being on monotherapy. Despite therapy 43% of children had elevated blood pressure levels and 16% had echocardiographic signs of LVH. A 24 hour ambulatory blood pressure measurements were more sensitive in diagnosing hypertension and assessing adequacy of blood pressure control. Early and intensified treatment should prevent end organ damage though optimal blood pressure values to aim obtain are still to be defined.

[Bone metabolism markers and bone histomorphometry in children with chronic renal failure in predialysis period during growth hormone treatment]. / Markery metabolizmu kostnego u dzieci z przewlekla niewydolnoscia nerek leczonych hormonem wzrostu w okresie przeddializacyjnym.

The aim of the study was to estimate biochemical bone metabolism markers and bone histomorphometric parameters in children with chronic renal failure (CRF) treated with recombinant human growth hormone (rhGH). Twelve children with CRF aged 2-13.4 years were treated with rhGH 1-1.1 IU/kg per week and alfacalcidol. Bone biopsies were performed before and after 12 months of therapy. An increase in the biochemical markers of bone formation and bone resorption were noted. A statistically significant increase in mineral apposition rate (MAR) was observed in bone histomorphometry. The administration of active vitamin D metabolites enable proper bone mineralization in fast growing children with CRF during rhGH treatment.

[Bilateral renal obstruction caused by cystine stones in a child with Henoch-Schönlein syndrome]. / Przypadek dziewczynki z zespolem Schönleina-Henocha i ostra zanerkowa niewydolnoscia nerek z powodu kamicy cystynowej.

Haematuria is a typical symptom of glomerular involvement in Henoch-Schoenlein syndrome in children. The authors present an unusual case of haematuria and deterioration of renal function caused by cystine stones in a child with anaphylactic purpura. This 6 year old girl was hospitalized because of severe gastrointestinal symptoms in the course of a typical Henoch-Schoenlein syndrome. After 4 weeks of illness--the child demonstrated haematuria and oliguria with severe loin pain and a rise in serum creatinine to 5.8 mg%. Urine findings of mainly non-glomerular erythrocytes and slight proteinuria were suggestive of a non-glomerular cause of renal failure. Ultrasound examination revealed the unexpected finding of bilateral kidney obstruction caused by multiple renal stones. Cystine crystals observed under microscopic examination of the urinary sediment and a positive cyanide-nitroprusside test suggested cystinuria as the cause of nephrolithiasis. High water intake and alkalization of urine and captopril for cystine excretion reduction resulted in fast improvement of renal function and and with time complete dissolution of calculi. The symptoms of Henoch-Schoenlein syndrome resolved over several weeks, and the child did not develop any signs of glomerular involvement.

[Ultrasound of exit-site and tunnel infections in children on peritoneal dialysis]. / Ocena ultrasonograficzna zapalenia ujscia i kanalu cewnika Tenckhoffa u dzieci dializowanych otrzewnowo.

Ultrasound diagnosis of catheter related infections in patients on peritoneal dialysis is easy to perform diagnostic procedure which enables more precise diagnosis and better follow-up of therapy. The authors present three cases of exit site and tunnel infections in children, illustrating the value of ultrasound evaluation in their diagnosis and in decisions on conservative treatment or catheter removal.

HLA-DRB and -DQB1 alleles in Polish patients with hepatitis B associated membranous nephropathy.

We have studied the HLA-DRB and -DQB1 alleles of 42 paediatric patients who have suffered from membranous nephropathy associated with a hepatitis B infection (HBVMN). These patients were all from the Gdansk area of Northern Poland and the disease was diagnosed by light and electron microscopy. The control population consisted of 55 healthy children, approximately age matched, from schools in Gdansk. In addition we have also analysed 40 patients chronically infected with hepatitis B, without any renal involvement, as hepatitis B disease controls. The HLA alleles were defined using PCR/SSP. As idiopathic membranous nephropathy and low responsiveness to hepatitis B vaccine have been found to be associated with DR3 in Caucasoids, our hypothesis was that the HBVMN patients would show an increase in DR3. Our results indicate that, although there is a small increase in the frequency of DRBl*0301 in the HBVMN patients (16/42 38%) when compared to the healthy controls (15/55 31%), this does not approach significance. There is a significant increase in the frequency of DQB1*0303 in the HBVMN patients vs the healthy controls, after correction for the number of antigens detected (Pc)(13/42 vs 2/55, RR=11.6, P=0.0007, Pc=0.02). A similar increase in DQB1*0303 is seen in the HBVMN patients when compared to the hepatitis controls (13/42 vs 4/40) but this is only significant before correction (RR=4.3, P=0.04).

[Acute renal insufficiency during Candida albicans candidiasis in a 2-month old infant]. / Ostra niedomoga nerek w przebiegu zakazenia grzybiczego Candida albicans u dwumiesiecznego niemowlecia.

In this paper has been described the case of 2-month old infant with acute renal insufficiency caused by mechanical obstacle of fungal bezoars. Bilateral pyelostomy has been performed and fungal masses have been washed out from kidney pelvis. The applied treatment including administration of Fluconazole intravenously caused disappearance of acute renal insufficiency. In this paper authors described pathogenesis, clinical symptoms, and treatment of urinary tract fungal infection.

A specific glomerular lesion of the graft: allograft glomerulopathy.

During the period from January 1973 to December 1970, 774 renal transplantations in 698 children have been performed in our Renal Unit. A total of 540 grafts have been examined both by light and immunofluorescence microscopy at least once. Recurrent glomerulonephritis was diagnosed in 62 grafts, de novo glomerulonephritis in 68 and allograft glomerulopathy (AGP) in 38. AGP was defined as a lesion affecting all glomeruli and characterized by widespread reduplication of the GBM with widening of the subendothelial space and interposition of mesangial matrix and without significant deposits by immunofluorescence. The aim of the current study is to describe the natural history of AGP and to delinate its clinical significance. At time of biopsy, an increase in serum creatinine was present in 30 patients associated with a proteinuria > or = 1 g/day in 21. During the post-transplantation course, proteinuria was present in 29 patients and associated with a nephrotic syndrome in 10 of them. With a mean follow-up of eight years seven months, two patients died, 23 lost their grafts and 13 have a functioning graft. The lesions of AGP recurred in three of the nine children who received a second graft. Thirteen of the 33 patients in whom earlier biopsies were performed showed a different pattern of involvement characterized by a prominent swelling of active endothelial and mesangial cells and a hypercellularity related to the presence of mononuclear cells both in the lumens and in the mesangial areas.(ABSTRACT TRUNCATED AT 250 WORDS)

Hepatitis B virus-associated glomerulonephritis: electron microscopic studies in 98 children.

Ninety-eight children with glomerulonephritis concomitant with hepatitis B surface HBs antigenemia were studied, the antigenemia being first documented at the clinical onset of glomerulopathy. Initial diagnoses, based on examination of the paraffin sections, varied, membrano-proliferative, mesangial, and membranous glomerulonephritis being most frequently considered. However, electron microscopic examination showed that 77 children had a uniform type of glomerulopathy, irrespective of the light microscopic appearance. This type was diagnosed as secondary membranous glomerulonephritis. The clinical course of this nephropathy was relatively indolent and short. Moreover, in many children, elimination of some hepatitis B virus (HBV) antigens from the circulation was also associated with clinical remission of glomerulopathy. The remaining 21 children with HBs antigenemia had various morphological forms of glomerulonephritis, these being similar to their idiopathic counterparts in both morphology and clinical course. The distinct clinical and morphological picture of secondary membranous glomerulonephritis with HBs antigenemia occurring in 77 of 98 children supports the hypothesis that HBsV-associated glomerulonephritis is of the secondary membranous type. Thus, we conclude that in children HBV antigenemia associated with glomerulonephritis other than secondary membranous is coincidental.

Prognostic factors in the hemolytic-uremic syndrome.

During 1972-1986, 142 children with the hemolytic-uremic syndrome were treated. Most of them were infants (73%). The total mortality rate reached 25.4%. Computer analysis revealed the following risk factors of a fatal outcome: severe gastrointestinal symptoms during the prodromal period, coma, convulsions, malignant hypertension, persistence of prodromal symptoms over 7 days, hyperkalemia over 7 mmol/l, acidosis with bicarbonate level less than 15 mmol/l, a delay of over 5 days in starting dialysis, and transport to dialysis unit of over 100 km. The greatest risk of death existed during the first 3 weeks from onset. Among 142 children, 106 survived the acute phase. They were followed up from 2 to 16 years. Nine were lost to follow-up. Twelve children developed chronic renal failure.

Henoch-Schönlein nephritis in children--a clinicopathological study.

36 children with Henoch-Schönlein nephritis had their renal biopsy specimens studied by light and electron microscopic and immunofluorescence antibody techniques. Though no pathognomic changes were found the histological picture was characteristic. The severity of histological changes correlated well with the clinical manifestation and disease persistence. IMF studies showed characteristic mesangial IgA staining. The most prominent ultrastructural feature was segmental mesangial and subendothelial deposits with basement membrane changes. The amount of deposits was a good exponent of disease activity-children with few deposits recovering shortly. In spite of long duration, the outcome after an average 4 year follow-up was good, the majority of children having improved and renal insufficiency developing rarely. Renal biopsy is essential for an estimation of severity of renal disease and enables prognosis of disease persistence and long term outlook.