Colostomy in children on chronic peritoneal dialysis.

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.

Long-term renal outcome in children with OCRL mutations: retrospective analysis of a large international cohort.

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.

Anaemia treatment in chronically dialysed children: a multicentre nationwide observational study.

OBJECTIVE: Erythropoiesis-stimulating agents (ESAs) are applied as a standard therapy in children with anaemia in chronic kidney disease. The aim of this study was to describe the efficacy and details of ESA treatment in a population of dialysed children in Poland. MATERIAL AND METHODS: The study had a prospective observational design and was performed in 12 dialysis centres. The study group comprised 117 dialysed children with a mean age at enrolment of 165.33 (97.18-196.45) months. RESULTS: Dialysed children were treated mostly with epoietin beta and darbepoietin. The mean dose of ESA was 99 (68-147) U/kg/week with a significant difference between patients on peritoneal dialysis [83 (54-115)] and haemodialysis [134 (103-186)] (p < 0.0001). The mean haemoglobin of all the time-point tests during 6 months was 10.91 ± 1.18 g/dl. The efficacy of anaemia treatment was unsatisfactory in 52% of subjects. In multivariate analysis, initial haemoglobin level <10 g/l, any infection, younger age at first dialysis, malnutrition and inadequate ESA dosage remained significant predictors of anaemia. CONCLUSIONS: The study revealed that anaemia treatment in Polish children is unsatisfactory. Late commencement of the treatment, inadequate dosing, malnutrition and infections could constitute risk factors for therapy failure.

[Xanthinuria type I as the cause of nephrolithiasis in 17-years old girl]. / Ksantynuria typu I jako przyczyna kamicy nerkowej u 17-letniej dziewczynki.

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.

[Cinacalcet in the treatment of secondary hyperparathyreoidism in a child with chronic kidney disease--case report]. / Zastosowanie cinakalcetu w leczeniu wtónej nadczynnosci przytarczyc u dziecka z przewlekla niewydoinoscia nerek--opis przypadku.

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.

[The use of sevelamer in the treatment of resistant hyperphosphataemia in children with chronic renal disease]. / Zastosowanie sewelameru w leczeniu opornej hiperfosfatemii u dzieci z przewlekla niewydolnoscia nerek.

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.

Evaluation of the effect of 3-month bladder basic advice in children with monosymptomatic nocturnal enuresis.

INTRODUCTION: Advice (BBA) into the standards of patients' care in both monosymptomatic and non-monosymptomatic nocturnal enuresis. Although the idea of this recommendation was clear and reflects clinical experience, duration and efficacy have not been definitely established. Recent data have demonstrated the lack of efficacy of BBA and a fierce discussion has ensued. The present study was aimed to assess the efficacy of BBA in a group of previously untreated children with primary monosymptomatic nocturnal enuresis (MNE). STUDY DESIGN: The study was a prospective interventional multicenter trial in a cohort of previously untreated MNE patients. Forty-nine children (36 males, 13 females, mean age 7.2 years) were included in the analysis. The treatment efficacy was assessed at the 30th, 60th, and 90th days of BBA. RESULTS: We discovered that the mean number of wet nights decreased significantly (p < 0.001) only after 3 months of BBA from 8.9 to 5.9 episodes every 2 weeks. BBA was fully successful in 2% o the children after 30 day, 12% after 60 days, and 18% after 90 days (Figure). Partial response (by ICCS) was assessed for 8%, 20%, and 34% of the patients. We noted a relatively high rate of non-responders that decreased from 90% to 47% after 90 days. We detected no differences in BBA efficacy between children with night-time polyuria or decreased maximal voided volume. A lower number of wet nights initially predicted the response to the BBA. DISCUSSION: Our study confirmed rather limited efficacy of BBA, similarly to previous observations, but provided more information on isolated MNE, because of a more specific study group and longer period of observation. The limitation of the study was lack of randomization. CONCLUSION: Our study revealed that in treatment-naïve children with monosymptomatic enuresis basic bladder training had a low (18%) and late effect, mostly pronounced after the third month of therapy. It seems that only if the patient presents with a favorable profile of bedwetting, occasionally and with a high maximum voided volume, it is worth maintaining BBA for a longer period of up to 3 months before initiating second-line therapy. In an unfavorable initial profile desmopressin or an alarm may be introduced much earlier.

Abnormal cytokine synthesis as a consequence of increased intracellular oxidative stress in children treated with dialysis.

BACKGROUND/AIM: End-stage renal disease (ESRD) induces a clinical state of immunodeficiency with a higher incidence of infections and higher mortality due to infectious complications compared with the normal population. The definite mechanism responsible for the host defense alterations is not well understood. The aim of the study was to investigate intracellularly the relationship between cytokine synthesis and oxidative stress in peripheral blood lymphocytes in children with ESRD. METHODS: Twenty-one children (age 11.7 +/- 5.8 years) with ESRD treated with hemodialysis (HD; n = 10) and peritoneal dialysis (PD; n = 11) were studied. Nine healthy children of comparable age formed the control group. To determine intracellular oxidative stress we used dihydrorhodamine-123 (DHR), which after oxidation to rhodamine-123 (RHO) emitted a bright fluorescent signal. Intracellular oxidation of DHR in T lymphocytes reflected intracellular oxidative stress. The intracellular synthesis of cytokines (IL-2, IFN-gamma, IL-4, IL-6) was also measured. Both parameters were detected at a single-cell level by flow cytometry. Lymphocyte subsets were evaluated using the monoclonal antibodies conjugated with fluorochromes. RESULTS: We found that in T lymphocytes the mean fluorescence intensity (MFI), which reflected intracellular oxidative stress, was increased in ESRD patients compared to the controls (CD3+: 34.77 +/- 11.55 vs. 22.55 +/- 4.97, p < 0.01; CD3+CD8+: 34.31 +/- 12.17 vs. 20.77 +/- 4.89, p < 0.01; CD3+CD4+: 36.06 +/- 6.98 vs. 24.44 +/- 7.68, p < 0.001). HD patients showed slightly higher MFI compared to PD patients in CD3+ cells (39.32 +/- 11.70 vs. 30.63 +/- 10.20, NS), in CD3+CD8+ cells (37.90 +/- 14.32 vs. 31.06 +/- 9.34, NS) and in CD3+CD4+ cells (40.10 +/- 2.28 vs. 29.33 +/- 7.06, p < 0.001). The intracellular synthesis of IL-2 was higher in ESRD patients compared to the controls, both in CD3+ cells (31.34 +/- 9.80 vs. 20.49 +/- 15.26%, p < 0.05) and in CD3+CD4+ cells (36.10 +/- 8.69 vs. 24.03 +/- 16.95%, p < 0.05). The intracellular synthesis of IFN-gamma, IL-4 and IL-6 was significantly lower in the ESRD group compared to the controls. Interestingly, in patients treated with HD, negative correlations between the degree of intracellular oxidative stress and intracellular cytokine synthesis in CD3+ lymphocytes were found. CONCLUSION: Our results show that patients with ESRD, especially those treated with HD, present increased oxidative stress in T lymphocytes, which may lead to decreased cytokine synthesis and abnormal immune response.

[Influence of vitamin E and N-acetylcysteine on intracellular oxidative stress in T lymphocytes in children treated with dialysis]. / Wplyw witaminy E oraz N-acetylocysteiny na wewnatrzkomórkowy stres oksydacyjny limfocytów T u dzieci leczonych dializami.

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.

[Yersiniosis as a cause of acute tubulointerstitial nephritis and acute renal failure--case report]. / Jersinioza jako rzadka przyczyna ostrego sródmiazszowego zapalenia nerek i niewydolnosci nerek--opis przypadku.

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.

[Intracellular synthesis of cytokines as the index of function of peripheral blood lymphocytes and monocytes in children with first relapse of nephrotic syndrome]. / Wewnatrzkomórkowa synteza cytokin jako wykladnik funkcji limfocytów i monocytów u dzieci z I rzutem zespolu nerczycowego.

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.

Hypertension in dialysed children: the prevalence and therapeutic approach in Poland--a nationwide survey.

BACKGROUND: The aim of this nationwide analysis was to assess the incidence and current treatment profile of arterial hypertension in children undergoing chronic haemodialysis or peritoneal dialysis and attitudes of paediatric nephrologists towards the choice of antihypertensive drugs in their patients. METHODS: The study group consisted of 134 children (89 males, 45 females, mean age 10.7+/-5 years) from all 13 paediatric dialysis centres in Poland. The data were gathered through a questionnaire for each patient dialysed in November 2004. RESULTS: The overall incidence of hypertension in the study group was 55% (74 of 134 patients; 47 males, 27 females). The incidence rate was similar in boys and girls (53 vs 60%) and in those on haemodialysis and peritoneal dialysis (56 vs 54%). Chronic glomerulonephritis as an underlying renal disease was significantly more frequent in the hypertensive than in the normotensive subjects (37 vs 10%, P = 0.004). Residual urine output was higher in normotensives (41 vs 10 ml/kg body weight; P < 0.001). Among those treated with antihypertensives: 32% were treated by monotherapy, 36% received two drugs, 22% received three drugs, while 7% received > or = 4 drugs. The therapy was effective in only 57% of subjects. We observed no differences in biochemical and clinical parameters between those who responded to the therapy and those who failed to do so. Calcium channel blockers constituted the most frequently administered class of drugs [73% of children; in 43 out of 48 (90%) combined with other drugs, but in 11 out of 24 (46%) as a monotherapy]. In monotherapy, angiotensin-converting enzyme inhibitors and calcium channel blockers were administered most frequently. CONCLUSION: We conclude that the incidence of hypertension in dialysis children in Poland is high (55%). The effectiveness of antihypertensive treatment is rather low (58%) and the choice of drugs is limited.