Predictors of Progression in IgA Nephropathy in Childhood.

The aim of this retrospective study was to assess the usefulness of potential predictors of poor prognosis in IgA nephropathy in children. The study population consisted of 55 children aged 11 ± 4 years, diagnosed on the basis of the Oxford classification and MEST score of kidney biopsy findings. Proteinuria, glomerular filtration rate (GFR), and the IgA/C3 serum ratio were assessed in all patients twice: at onset and at follow-up. The patients were treated with steroids, immunosuppressive drugs, and/or angiotensin-converting enzyme inhibitors. Follow-up was at 3.9 ± 2.9 (median 2.7) years. The patients were subdivided into two groups: with GFR <90 and ≥90 mL/min at follow-up. ROC AUC curves and logistic regression were used to evaluate the power of prognostic factors. The two groups did not differ regarding the level of proteinuria, MEST score, and the IgA/C3 ratio at onset of disease. There was a significant association between GFR reductions at onset and follow-up (AUC = 0.660; p < 0.05). In patients with nephrotic range proteinuria at onset, proteinuria at follow-up was more frequent compared with other patients (AUC = 0.760; p < 0.05), MEST score ≥3 tended to be associated with reduced GFR (AUC = 0.650; p = 0.07) but not with proteinuria (AUC = 0.608; p = 0.47), and the IgA/C3 ratio was higher (p < 0.05) at follow-up. No significant associations were found between the IgA/C3 ratio at onset and reduced GFR (AUC = 0.565; p = 0.46) or proteinuria at follow-up (AUC = 0.263; p = 0.20). We conclude that predictors of poor outcome in childhood IgAN include the following: GFR reduction, nephrotic range proteinuria at onset of disease, and high MEST score in Oxford classification of kidney biopsy. Despite a higher serum IgA/C3 ratio in children with impaired renal function in long-term follow-up, we failed to demonstrate a significant association between this ratio at onset of disease and reduced GFR or persistent proteinuria at follow-up. Thus, IgA/C3 ratio is not a good foreteller of progression of IgA nephropathy in childhood.

Increased Serum IgA in Children with IgA Nephropathy, Severity of Kidney Biopsy Findings and Long-Term Outcomes.

The aim of the study was to determine whether an elevated IgA level at the time of the diagnosis of IgA nephropathy has an effect on the severity of kidney biopsy findings and long-term outcomes in children. We retrospectively studied 89 children with IgA nephropathy who were stratified into Group 1- elevated serum IgA and Group 2 - normal serum IgA at baseline. The level of IgA, proteinuria, hematuria, glomerular filtration rate (GFR) and hypertension (HTN) were compared at baseline and after the end of the follow-up period of 4.0 ± 3.1 years. Kidney biopsy findings were evaluated using the Oxford classification. The evaluation of treatment included immunosuppressive therapy and renoprotection with angiotensin converting-enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB), or no treatment. The elevated serum IgA was found in 46 (52 %) patients and normal serum IgA level was found in 43 (48 %) patients. No differences were found between the two groups regarding the mean age of patients, proteinuria, and the number of patients with reduced GFR or HTN at baseline. In kidney biopsy, mesangial proliferation and segmental sclerosis were significantly more common in Group 1 compared with Group 2 (p < 0.05). Immunosuppressive therapy was used in 67 % children in Group 1 and 75 % children in Group 2. The Kaplan-Meier survival curves for renal function (with normal GFR) and persistent proteinuria did not differ significantly depending on the serum IgA level at baseline. We conclude that in IgA nephropathy the elevated serum IgA at baseline may be associated with mesangial proliferation and segmental sclerosis contribute to glomerulosclerosis, but has no effect on the presence of proteinuria or on the worsening of kidney function during several years of disease course.

Imaging of bone in the diagnostics of renal osteodystrophy in children with chronic renal failure.

BACKGROUND: In the last two decades considerable advances have been made in the development of imaging tests of the skeletal system. This progress in diagnostic techniques, along with the growing availability of the tests, renders it necessary to review and evaluate their suitability for daily clinical practice. The aim of this article is to compare the results of radiological testing of bone with densitometrical, histomorphometric, and biochemical tests in children with chronic renal failure. MATERIAL AND METHODS: The research involved 31 children with renal failure, of whom 10 were being treated conservatively, 17 by continuous ambulatory peritoneal dialysis (CADO), and 4 by hemodialysis (HD). In all these children, radiological examinations of bone were performed in the arms, knees, and hips, along with tests for the serum concentration of parathormone (iPTH), calcium (Ca), and phosphates (P), and for the activity of alkaline phosphatase (AP). Bone density tests by the DXA method and bone biopsies were also performed. On the basis of radiological evaluation, the patients were divided into two groups: Group I, consisting of 14 children with a normal bone structure image, and Group II, consisting of 17 children with bone atrophy. RESULTS: No statistically significant differences were discovered in the mean values of the tested biochemical parameters between the two groups. The mineral density of total body was normal in 9 of the 14 patients in Group I (64%), and in 7 of 17 (41%) from Group II. The mineral density of total lumbar spine gave similar results. Lower bone density results were obtained in Group II than in Group I, though only in the case of the lumbar spine were the differences statistically significant. In Group I, 5 cases were discovered of chronic osteodystrophy without osteomalacia and hyperparathyroidism (NB), 2 cases of adynamic bone disease (ABD), 4 cases of hyperparathyroidism (HP), 2 cases of moderate hyperparathyroidism (MHP), and one mixed form (Mix); in Group II, there were 6 NBs, 2 ABDs, 1 case of osteomalacia (OM), 5 HPs, and 3 mixed. Radiological examinations revealed one male in Group I with features of prior Perthes's disease, one with fibrous cortical defect, and four cases of valgity of the coxa valga. In Group II, there were 3 children with radiological changes typical for osteomalacia, and in 1 case typical radiological signs of hyperparathyroidism. CONCLUSIONS: Given the lack of consistency in the results of the tests here presented, an entire panel of available tests should be performed for the comprehensive evaluation of the status of the skeleton.

[Vesicoureteral refluxes in children during the first two years of life]. / Odplywy pecherzowo-moczowodowe u dzieci w pierwszych dwóch latach zycia.

Ninety-six children with vesicoureteral reflux (VUR) diagnosed by age 2 were studied. The most common indication for radiological visualization of the urinary tract was a urinary tract infection (UTI). Among 143 cases of VUR, 51% were unilateral and 49% were bilateral. Other urinary tract abnormalities coexisted with VUR in 14.6% of children. Abnormal USG result was found in 24% of children, including only 13.4% with VUR and other urinary tract abnormalities. Conservative treatment was chosen in 126 (88%) cases of VUR in 87.5% of children. Follow-up voiding cystourethrography was performed in 60.7% of children. Conservative treatment was successful in 68.6% of children. VUR disappeared in 70.9% of cases after mean follow-up of 13.7 months (including 76.2% cases of VUR grade II, 54.5% cases of VUR grade III and 50% cases of VUR grade I). Recurrent UTI and lack of improvement after conservative treatment were indications for surgical treatment in 9.8% of children. Recurrent UTI were observed in 15.6% of children (including 16.7% among those treated conservatively and 8.3% among those treated surgically) and their course was febrile in most cases.

[Evaluation of prednisone treatment in children with IgA nephropathy and Schönlein-Henoch nephropathy according to the Waldo protocol with regard to pathomorphologic changes in renal biopsy]. / Ocena leczenia prednisonem dzieci z nefropatia IgA i nefropatia Schönleina-Henocha wg protokolu Waldo z uwzglednieniem zmian patomorfologicznych w biopsji nerki.

UNLABELLED: The aim of the study was to analyse effectiveness of long term alternate-day prednisone treatment according the protocol of Waldo (Pediatr. Nephrol. 1993, 7, 529) in children with IgA nephropathy (IgAN) and Schönlein-Henoch nephritis (HSN). Eight pts: 6 with IgAN, mean age 10.1 yrs and 2 with HSN aged 10.3 and 14.3 yrs were treated with use of alternate-day prednisone for 2.3 to 3.92 (mean 2.90 yrs). Renal biopsies were performed in all patients 2 to 72 (mean 16 mths) after onset and were graded according the classification of WHO. All pts had normal serum creatinine concentrations at presentation. 3 pts had proteinuria > 1 g/1.73 m2 per day at onset and 5 pts had macroscopic or microscopic haematuria and/or increased proteinuria and/or III grade WHO in renal biopsy. After treatment haematuria and proteinuria disappeared in 2 pts and decreased in 6 pts. Hypertension (2/8), hypercalciuria (2/8), mild weight gain (6/8) and low bone density in lumbar region (5/8) were observed during the treatment. CONCLUSIONS: 1. Long term alternate-day prednisone treatment according the protocol of Waldo allows to decrease haematuria and proteinuria in children with IgAN and HSN 2. Side effects of therapy were observed in 62.5% pts.

[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.

[Bone mineral density in children with end-stage renal disease]. / Badanie gestosci kosci u dzieci ze schylkowa niewydolnoscia nerek.

The aim of the study was to evaluate bone mineralisation in comparison to chronological age, bone age and high age in children with end-stage renal disease. Fourty-four patients (16 female, 28 male) aged 7-16 years were examined. DXA bone densitometry of total body, bone age evaluated by Greulich-Pyle method and high age were performed in all patients. In our patients bone, and high age were significantly decreased in comparison to chronological age. In contrast mean value of Z-score TG BMD bone and high age compared to mean value of Z-score TB BMD for chronological age were increased significantly. We conclude that bone mineralisation should be compared with or high age not to chronological age in patients with end-stage renal disease.

[Risk factors of adynamic bone disease in children with end-stage renal failure: personal experience]. / Czynniki ryzyka adynamicznej choroby kosci u dzieci ze schylkowa niewydolnoscia nerek--doswiadczenia wlasne.

The aim of the study was to estimate predisposing factors which can cause adynamic bone disease (ABD) and biochemical markers, bone densitometry results, bone histomorphometry in 17 children with this from of the renal osteodystrophy. Half of these of patients were treated with alphacalcidol pulses. In 47% of patients hypercalcemic episodes were noted, 76% had PTH level < 50 pg/ml. Four patients with osteoporosis (low bone volume at histological analysis) were distinguished. Two of them were treated with corticosteroids, 1 was immobilized for a long time.

[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.

[Effect of lipid lowering diet on serum lipids in peritoneal dialysis patients]. / Wplyw diety hipolipemizujacej na profil lipidowy surowicy chorych ze schylkowa niewydolnoscia nerek leczonych dializa otrzewnowa.

The aim of the study was to assess an effect of lipid lowering diet on serum lipids in peritoneal dialysis patients. Total cholesterol (TC) decreased after 3 months of low-fat diet from 203.7 mg/dl to 181 mg/dl, probably due to increased P/S ratio (PUFA/SFA) from 0.4 to 0.57. After another 3 months of the diet, a decrease in P/S ratio and concomitant increase in TC and LDL-C levels were found. Nutritional status of patients during lipid lowering diet was stable.

[CAPD treatment in children with renal amyloidosis]. / Leczenie dializa otrzewnowa dzieci z amyloidoza nerek.

The aim of the study was to summarize the experience in the treatment of chronic renal failure due to secondary amyloidosis in the course of juvenile rheumatoid arthritis. Fourteen children aged 7.5-17.7 years were treated with dialysis; 12 with CAPD, 2 with HD. Our results indicate that CAPD is a proper dialysis technique for children with amyloidosis, despite a high rate of complications in early period of CAPD, such as: bleeding, leaks, hernias, and impaired wound healing.

[Generalized tuberculosis as a cause of renal amyloidosis in a 9-year- old girl]. / Uogólniona gruzlica jako przyczyna wtórnej amyloidozy nerek u 9-letniej dziewczynki.

We present a case of a 9 years old girl with generalized tuberculosis diagnosed at the age of 5. Renal amyloidosis was diagnosed 21 months later. Clinically amyloidosis has been presented with steroid-resistant nephrotic syndrome, which within 15 months led to end stage renal failure. The girl is on automatic peritoneal dialysis with no signs of active tuberculosis up to now.

[Index of histopathological changes in rapidly progressive glomerulonephritis: a preliminary report]. / Indeks zmian histopatologicznych w gwaltownie postepujacym klebuszkowym zapaleniu nerek--doniesienie wstepne.

We present the case of an 8-year-old girl with rapidly progressive glomerulonephritis. The activity and chronicity index of histopathological changes in this patient was determined in 2 renal biopsies performed in a 25-month interval. The determination of this index in renal biopsies may be useful in the choice of glomerulonephritis with bad prognosis therapy.

Antibody response to influenza immunization in two consecutive epidemic seasons in patients with renal diseases.

The aim of this study was to assess antihemagglutinin and antineuraminidase antibody kinetics in 26 patients with renal diseases vaccinated against influenza in two consecutive epidemic seasons. Antibody responses were measured before immunization and 1, 3 and 6 months after immunization. Antihemagglutinin (HI) antibodies were determined by the hemagglutinin inhibition test and antineuraminidase (NI) antibody levels by the neuraminidase inhibition test. After vaccination HI and NI antibody titers significantly increased when compared with the pre-vaccination levels. Three months after vaccination the protection rates ranged from 50 to 61.5% in the 1995/96 season and 100% for all antigens in the 1996/97 season. Response rates ranged from 50 to 57.7% and 93.8 to 100% respectively. Significantly higher humoral response was recorded in the 1996/97 season than in the 1995/96 season. No serious adverse reactions were observed in the vaccinated patients and no symptoms of influenza or influenza-like infection were noted. In spite of some doubts about the safety and efficacy of influenza vaccination in patients from high-risk groups, the results of this study showed that many of them are able to produce HI antibodies in titers which are sufficient to protect against the influenza infection.

Swan neck presternal peritoneal dialysis catheter: five-year experience in children.

OBJECTIVES: To reduce the incidence of exit-site infection (ESI) a new peritoneal dialysis (PD) catheter, the Swan neck presternal catheter (SNPC), composed of abdominal and presternal parts joined by a titanium connector, with the exit site located on the chest wall, was designed. DESIGN: A prospective study was undertaken to estimate the usefulness of the SNPC for continuous ambulatory peritoneal dialysis (CAPD) in children. SETTING: University Children's Hospital, Medical Academy, Warsaw, Poland. PATIENTS: From December 1991 to June 1997, 11 SNPCs were implanted in 10 children for the following reasons: recurrent ESI in 3, the presence of ureterocutaneostomies in 3, obesity in 3, the use of diapers in 2, young age in 1, and fecal incontinence in 1. More than one indication was present in some patients. In 7 patients the SNPC was the first PD catheter inserted. INTERVENTION: In all children the presternal catheter was implanted surgically by the modification of the technique described by Twardowski et al. RESULTS: The observation period ranged from 1-60 months. The rate of ESI was 1/162 patient-months. The major complication was trauma of the exit site (4 times in 3 of 10 patients). In spite of leaving an extra length of the catheter in the entire subcutaneous tunnel at the time of implantation, the two parts of the SNPC became disconnected in 2 children (after 7 and 33 months respectively). CONCLUSION: Our results achieved with the SNPC in children are very good. The presternal catheter reduces the risk of ESI. However, the chest localization of the exit site does not help to prevent trauma in children. This type of PD catheter should be reserved for patients with specific indications.

Recombinant human erythropoietin dosage in children undergoing hemodialysis and continuous ambulatory peritoneal dialysis.

The efficacy of erythropoietin (EPO) in 11 children on hemodialysis (HD) and 8 on continuous ambulatory peritoneal dialysis (CAPD) (mean age 11.8 years) was compared. The initial EPO dose was 50 U/kg s.c. once a week; the time of observation was 24 weeks. In the CAPD group, the mean hemoglobin (Hb) level increased from 7.7 +/- 0.2 to 11.2 +/- 0.6 g/dl (P < 0.001) and hematocrit (Hct) from 22.3 +/- 1.0 to 32.6 +/- 1.4% (P < 0.001), while in the HD group the mean Hb rose from 7.7 +/- 0.6 to 9.3 +/- 0.8 g/dl (P < 0.001) and mean Hct from 22.7 +/- 2.3 to 27.6 +/- 2.8% (P < 0.001) after 12 weeks of observation. An increase in Hb to over 10 g/dl was obtained in 87.5% of children on CAPD but in only 10% on HD after 8 weeks of EPO treatment. After 12 weeks of treatment, all children on CAPD had the target Hb level of more than 10 g/dl, while 7 children on HD required increased doses of EPO (100 U/kg per week). We conclude that the EPO dose of 50 u/kg given s.c. once a week is effective for children with anemia on CAPD but is insufficient for children on HD.

Dialysis treatment in children with amyloidosis due to juvenile rheumatoid arthritis.

Rapid deterioration of renal function in amyloidotic children may be due to progression of amyloidosis or exacerbation of chronic renal failure (CRF). Continuous ambulatory peritoneal dialysis (CAPD) is superior to hemodialysis (HD) both in end-stage renal disease and in exacerbations of CRF in amyloidosis. A better supply of protein and easier control of hypertension are possible on CAPD, while HD is associated with the risk of hypovolemia episodes, cardiovascular disturbances and bleeding connected with heparinization.

The influence of dialysate calcium concentration on the PTH level in children undergoing CAPD.

In 12 children aged four-and-a-half to 18 years (mean 11 +/- 4.2) undergoing continuous ambulatory peritoneal dialysis (CAPD), serum intact parathyroid hormone (iPTH), ionized calcium (iCa) levels, and calcium mass transfer (CaMT) were measured on three consecutive days: day 1, after a four-hour interval between dialyses; on day 2, after four hours dwell time with peritoneal dialysis (PD) Ca 3.5 mEq/L; and on day 3, after four hours dwell time with PD Ca 2.5 mEq/L. A significantly more negative CaMT was found when PD Ca 2.5 mEq/L was used, as compared with values obtained using PD Ca 3.5 mEq/L. Significantly lower parathyroid hormone (PTH) values were found after the interval between exchanges. We conclude that in order to properly evaluate parathyroid gland function and to decide whether or not to give vitamin D metabolites, a protocol for determining PTH should be standardized.