We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.

Neutrophil gelatinase associated lipocalin (NGAL) is a protein identified in human neutrophil granules. The aim of the study was to assess whether urine level of NGAL (uNGAL) could represent a novel, reliable marker of urinary tract infection (UTI) and to determine the optimal cutoff level for uNGAL to predict UTI in children. Sixty patients with symptomatic UTI and 29 healthy controls were enrolled the study. Urine NGAL was measured by enzyme-linked immunosorbent assay. A dimercaptosuccinic acid (DMSA) radionuclide scan was performed within 7 days in the patients with UTI in an attempt to distinguish pyelonephritis from cystitis. Mean uNGAL level was significantly higher in the UTI group than in the controls (91.02 ng/ml vs 14.29 ng/ml, p = 0.0001) and using a cutoff 20 ng/ml for uNGAL for diagnosis of UTI, sensitivity, and specificity were 97% and 76%, respectively [area under the curve (AUC): 0.979]. Mean uNGAL/creatinine ratio (uNGAL/Cr) was also significantly higher in the UTI group [201.81 ng/mg creatinine (Cr) vs 18.08 ng/mg Cr; p = 0.0001], and using a cutoff 30 ng/mg Cr for uNGAL/Cr for diagnosis of UTI, sensitivity and specificity were 98% and 76%, respectively (AUC: 0.992). In conclusion, both uNGAL and uNGAL/Cr can be used as a novel, sensitive marker for early prediction of UTI in the absence of acute kidney injury and chronic kidney disease, and the optimal cutoff value for prediction of UTI is lower than the values determined for acute kidney injury. Further investigations with larger patient groups are required to confirm our results.

Recognition of the risk factors for ESBL-producing bacteria may be helpful to determine new policies in the management of UTI. Recurrence of UTI should be prevented especially in the first year of life, and prophylactic cephalosporins should be avoided.

Object The purpose of this study was to compare the methods of posterior lumbar interbody fusion (PLIF) and posterolateral fusion (PLF) in cases of isthmic Grades 1 and 2 lumbar spondylolisthesis, and to evaluate the clinical efficacy of the procedures. Methods Operations were performed in 50 patients with lumbar spondylolisthesis in the authors' clinics between 2001 and 2007. Indications for surgery were low-back pain with or without sciatica and neurogenic claudication that had not improved after at least 6 months of conservative treatment. The study included 33 female and 17 male patients, with mean ages of 50.6 years in the PLIF group and 47.3 years in the PLF group. These patients were randomly allocated into 2 groups: decompression, posterior transpedicular instrumentation, and PLF (Group 1; 25 patients) and decompression, posterior transpedicular instrumentation, and PLIF (Group 2; 25 patients). In the PLIF group, titanium cages were used, and autograft material was obtained from the decompression. In the PLF group, bone fragments collected from the iliac crest were used as autografts. A minimum 18-month follow-up was available in all patients. For clinical evaluation, a visual analog scale, Oswestry Disability Index, and the 36-Item Short Form Health Survey were used. Improvements in pre- and postoperative spondylolisthesis, segmental angles, fusion ratios, and postoperative complications were evaluated radiologically. Results The average follow-up period was 3.3 years. Based on the etiologies, isthmic spondylolisthesis was detected in all patients. The spondylolisthesis levels in the patients who underwent PLIF were located at L3–4 (5 patients, 20%); L4–5 (14, 56%); and L5–S1 (6, 24%), whereas the levels in the ones treated with PLF were located at L3–4 (4 patients, 16%); L4–5 (13, 52%); and L5–S1 (8, 32%). In the clinical evaluations, good or excellent results were obtained in 22 (88%) cases in the PLIF group and 19 (76%) cases in the PLF group. Fusion ratios were 100% in the PLIF group and 84% in the PLF group. Both lumbar lordosis and the segmental angle showed greater improvement in the PLIF group. There was no difference in the complication rates for each group. Conclusions Based on early clinical outcomes and the fusion ratios of adult isthmic spondylolisthesis, the authors found PLIF to be superior to PLF.

Background Hypertension and cardiovascular disease are common in children undergoing dialysis. Studies suggest that hemodiafiltration (HDF) may reduce cardiovascular mortality in adults, but data for children are scarce. Methods The HDF, Heart and Height study is a nonrandomized observational study comparing outcomes on conventional hemodialysis (HD) versus postdilution online HDF in children. Primary outcome measures were annualized changes in carotid intima-media thickness (cIMT) SD score and height SD score. Results We enrolled 190 children from 28 centers; 78 on HD and 55 on HDF completed 1-year follow-up. The groups were comparable for age, dialysis vintage, access type, dialysis frequency, blood flow, and residual renal function. At 1 year, cIMT SD score increased significantly in children on HD but remained static in the HDF cohort. On propensity score analysis, HD was associated with a +0.47 higher annualized cIMT SD score compared with HDF. Height SD score increased in HDF but remained static in HD. Mean arterial pressure SD score increased with HD only. Factors associated with higher cIMT and mean arterial pressure SD-scores were HD group, higher ultrafiltration rate, and higher b2-microglobulin. The HDF cohort had lower b2-microglobulin, parathyroid hormone, and high-sensitivity C-reactive protein at 1 year; fewer headaches, dizziness, or cramps; and shorter postdialysis recovery time. Conclusions HDF is associated with a lack of progression in vascular measures versus progression with HD, as well as an increase in height not seen in the HD cohort. Patient-related outcomes improved among children on HDF correlating with improved BP control and clearances. Confirmation through randomized trials is required.