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Background: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy. Methods: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites. Results: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0–2.2) and 2.8 (IQR 0.2–18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9–35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2–7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis. Conclusion: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential. [ABSTRACT FROM AUTHOR]

Background. Lowe syndrome (LS) and Dent-2 disease (DD2) are disorders associated with mutations in the OCRL gene and characterized by progressive chronic kidney disease (CKD). Here, we aimed to investigate the long-term renal outcome and identify potential determinants of CKD and its progression in children with these tubulopathies. Methods. Retrospective analyses were conducted of clinical and genetic data in a cohort of 106 boys (LS: 88 and DD2: 18). For genotype-phenotype analysis, we grouped mutations according to their type and localization. To investigate progression of CKD we used survival analysis by Kaplan-Meier method using stage 3 CKD as the end-point. Results. Median estimated glomerular filtration rate (eGFR) was lower in the LS group compared with DD2 (58.8 versus 87.4 mL/min/1.73 m², P < 0.01). CKD stage II-V was found in 82% of patients, of these 58% and 28% had moderate-to-severe CKD in LS and DD2, respectively. Three patients (3%), all with LS, developed stage 5 of CKD. Survival analysis showed that LS was also associated with a faster CKD progression than DD2 (P < 0.01). On multivariate analysis, eGFR was dependent only on age (b=-0.46, P < 0.001). Localization, but not type of mutations, tended to correlate with eGFR. There was also no significant association between presence of nephrocalcinosis, hypercalciuria, proteinuria and number of adverse clinical events and CKD. Conclusions. CKD is commonly found in children with OCRL mutations. CKD progression was strongly related to the underlying diagnosis but did not associate with clinical parameters, such as nephrocalcinosis or proteinuria. [ABSTRACT FROM AUTHOR]

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Introduction: The proportion of glomerulopathies as a cause of end-stage renal failure has been increasing in several regions of the world in recent years. Aim of study: An analysis of the contribution of glomerulopathies in 803 children (362 girls and 441 boys) on renal replacement therapy recorded between 2000-2007 in the Polish Renal Replacement Therapy Registry. Results: Among 803 cases, 37.5% were diagnosed as glomerulopathy. The majority were classified as primary glomerulonephritis (22.8%). Secondary glomerulonephritis due to systemic disease was diagnosed in 8.5% of cases and congenital glomerulopathy in 6.2%. The most frequent diagnosis among primary glomerulonephritis was FSGS (35%) and RPGN (13%). There was no differences in sex distribution between glomerulopathy groups. The mean age at start of RRT in glomerulopathy group was 11.4±5 years and did not differ from the whole population (10.4±5.5 years). Patients with congenital nephrotic syndrome started RRT at younger age (7.1±6.4 years, p <0.05). Time to transplant in children with glomerulopathy did not differ significantly compared to the whole population: median observation time was 24.2 months vs 26 months. During 8 years of follow-up 59 deaths were observed: 6 deaths in 181 children with primary glomerulopathies, 4 deaths in 38 children with congenital nephrotic syndrome and 11 deaths in 37 children with secondary glomerulonephrities. Conclusions: Glomerulopathies are the second cause of end-stage renal failure in children after congenital urinary disorders. The worst prognosis and highest case-fatality rate was observed in secondary glomerulopathy group. [ABSTRACT FROM AUTHOR]

Classic xanthinuria is a rare metabolic defect concerning the final reactions of purine catabolism. There are two types of the disorder: type I results from xanthine dehydrogenase (XDH) deficiency, while type II is characterized by lack of both XDH and aldehyde oxidase activity. Both types are clinically similar and are characterized by elevated xanthine concentration in body fluids that can lead to xanthine crystallisation. The most common manifestation of the disease is urolithiasis, but in most cases xanthinuria remains asymptomatic and the diagnosis is accidental. In the paper we report the first case study of xanthinuria in Poland in a child presenting with urolithiasis. 17-years old female patient was diagnosed because of recurrent urinary lithiasis and hypouricemia was detected during routine tests. Plasma and urine concentrations of oxypurines were measured by high-performance liquid chromatography (HPLC) and showed typical features of xanthinuria: hypouricemia, hypouricosuria, xanthinuria and elevated plasma xanthine. The allopurinol loading test demonstrated type I xanthinuria. The presented case report supports that first symptoms of xanthinuria can appear at any age and this disorder should be considered during diagnosing urolithiasis.

Unlabelled: The medical history and treatment of 2.5 years old girl with chronic kidney disease and heavy hyperparathyreoidism was presented in this case report. This girl was treated by peritoneal dialysis (APD) due to chronic kidney disease and congenital nephrotic syndrome. The secondary paraidothyreoidism was a reason of the epiphysial of the upper end of the femur, which appeared despite of the treatment by the Vitamin D3 (Calciphediol and Alphacalcidol) and phosphate binding drugs like Calcium Carbonate and Sevelamer. The primary laboratory tests were: Ca 9.32 mg/dl, Ca jon. 1.21 mmol/l, PO4 7.29 mg/dl, BE +4.9 mmol/l, ALP 1425 U/l, PTH 3774 pg/ml, Albumins 3.23 g/l, Hgb 8.2 g/l. The treatment of cinacalcet (Mimpara, 30 mg, Amgen) in dose of 15 mg was started because of lack of the standard therapy results. The laboratory tests were controlled after 2 weeks and then every week to 5th and every 2 weeks to the end. This therapy was finished after 30 weeks and only the standard therapy of hyperphosphatemia was continued. The laboratory tests 3 month after stopped the treatment were: Ca 10.5 mg/dl, Ca jon. 1.36 mmol/l, PO4 4.0 mg/dl, BE +4.9 mmol/l, ALP 312 U/l, PTH 134 pg/ml, Albumins 3.23 g/l, Hgb 9.9 g/l. There were any adverse events observed during the treatment., Conclusion: It seems, the cinacalcet may be an alternate treatment to paraidectomy in children with chronic kidney disease and heavy secondary hyperparathyreoidism.

Unlabelled: The aim of study was the estimation of efficacy and safety of sevelamer treatment in children with chronic kidney disease with resistant hyperphsphatemia., Material and Methods: The study group was 7 patients (hemodialysed--2 boys, mean age 7, 7+/-0.1; peritoneal dialysis--5: 3 boys and 2 girls, mean age 5, 6+/-5.0). Sevelamer (Renagel, 800 mg, Genzyme) was used in all patients in mean dosis of 117 mg/kgm.c./d due to lack of effect of the typical treatment (vitamin D, diet, calcium carbonate). Results of treatment by sevelamer were estimated by measuring of calcium, phosphates, albumins, parathormone and alkalic phosphate concentration in the serum and blood gases after 6 and 12 month., Results: There were found that sevelamer treatment significantly decrease the phosphates concentration and CaxP product in the serum without influence on concentration of PTH or calcium. Any adverse events were observed during the study and any drug intolerance., Conclusion: Sevelamer treatment may be usefulness in children with resistant hyperphosphatemia.

Unlabelled: End-stage renal disease (ESRD) patients are subjected to enhanced oxidative stress. Excess of reactive oxygen species (ROS) may lead to the functional disabilities of lymphocytes. The aim of the study was to investigate the effect of vitamin E and N-acetylcysteine (NAC) on antioxidant status and intracellular oxidative stress in T-cells in children treated with dialysis., Material and Methods: 18 children treated with dialysis (hemodialysis n = 5 and peritoneal dialysis n = 13) were enrolled into the study. The age range was 2-20 ys. with a mean of 10.94 +/-5.86 ys. Vitamin E and NAC were given for six months orally. Throughout the study total antioxidant status (TAS), superoxide dismutase (SOD) and glutathione peroxidase (GPx) activity, and intracellular oxidative stress in T lymphocytes was measured., Results: In children treated with dialysis, TAS was significantly reduced compared to the controls (p = 0.012). We found no differences in GPx and SOD activities between patient and control groups. Mean fluorescence intensity (MFI), which reflected intracellular oxidative stress, was significantly increased in: CD3+, CD3+CD4- and CD8+CD28-. After six months of antioxidant treatment, a significant reduction in MFI was noted in most T-cell subsets (p < 0.001). MFI in T-helper cells remained unchanged. Although there was a trend toward rise in TAS and GPx activity, only significant differences in SOD activity were found (p = 0.022)., Conclusions: In children with ESRD treated with dialysis reduced TAS coexists with enhanced intracellular oxidative stress in T lymphocytes. The combined treatment with vitamin E and NAC lead to the reduction in oxidative stress within T-cells that might be of therapeutic value in dialyzed patients.

Tubulointerstitial nephritis (TN) is a heterogenous disease, where disturbances of the interstitial tissue and renal tubules are found. Different immunological and nonimmunological mechanisms initiated by infectious and non-infectious factors may lead to TN. A case of 13-years-old girl with primary diagnosis of acute pyelonephritis is presented. The abdominal pain, headache, pain in lumbar region and intermittent fever with loss of appetite were observed in this girl a few weeks before admission. Microcytic anemia, proteinuria and glucosuria, azotemia and elevated markers of inflammatory response were found. In ultrasound examination heterogenous cortex echogenicity of both kidneys and disturbances in parenchymal blood flow were observed. In renal scintigraphy the discriminated catch index was found. Kidney biopsy revealed the edema of the interstitial space with mononuclear and lymphocyte infiltration. The diagnosis of TN was established upon the history, clinical examination, results of laboratory tests, kidney imaging and biopsy. After steroid and doxycycline treatment an improvement and normalization of the results of laboratory tests were observed. It seems to be justified to consider Yersinia infection as a cause of acute tubulointerstitial nephritis.

Background: Nephrotic Syndrome (NS) is a disease in which immune system function disorders play an important role. The aim of the study was an assessment of intracellular expression of cytokines in primary NS including assessment of intracellular expression of monokines (TNF-alpha, IL-8) in non-specific LPS stimulation system and intracellular expression of lymphokines (IL-2, INF-gamma, IL-4, IL-6) in experimental system with application of stimulation with ionomycin and PMA., Methods: The study included 47 children with NS aged 2-16 years. After activation of whole blood cells in the presence of brefeldin A the cells were labelled with monoclonal antibodies against surface antigens. In the second phase the cells underwent permeabilisation and were labelled with monoclonal antibodies against individual cytokines., Results: The studies performed demonstrated in children with NS relapse an increase of intracellular synthesis of cytokines characteristic of Th1 system (IL-2) and a decrease of synthesis of cytokines characteristic of Th2 system (IL-4, IL-6). In these patients an increase was also found of intracellular synthesis of proinflammatory monokines (TNF-alpha and IL-8). The studies performed, confirmed the earlier observations concerning abnormal cellular response in NS and demonstrated that differences in serum concentrations of individual cytokines may result from disturbances in their intracellular synthesis.