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Introduction No data exist on the epidemiology of children incidentally diagnosed with advanced kidney failure (KF) during evaluation for non-specific symptoms. This is likely related to unrecognized symptoms and signs of CKD. The objective of our study was to evaluate incidentally diagnosed patients with advanced KF requiring long-term kidney replacement therapy (KRT). Methods An IRB-approved retrospective chart review of children who started KRT with dialysis (hemo- or peritoneal) was conducted. Included were children with no prior knowledge or diagnosis of underlying kidney disease with chronic kidney disease (CKD) disease stage 4 (GFR 15-29 mL/min/1.73 m.sup.2) or 5 (GFR < 15 mL/min/1.73 m.sup.2) at initial presentation and started on chronic KRT within 2 months of presentation. Results Of 177 patients initiating KRT during the study period, 26 (15%) were categorized as incidental advanced KF. This cohort with mean age 12.25 years consisted of 42% males, 54% African Americans included 46% with glomerular, and 54% with non-glomerular etiology for kidney failure. Vomiting (42%) and fatigue (39%) were most common, while growth failure (19%) and hyperkalemia (7%) were less frequent on initial presentation. Anemia (100%), hypertension (96%), hyperparathyroidism (96%), and hyperphosphatemia (92%) were the most frequently seen CKD comorbidities. Chronic KRT was started within 24 h in 62% and within 2 weeks in 88% of the cohort. Conclusion Under-diagnosis of patients with advanced KF is most likely related to milder non-specific clinical symptoms and normal growth in the majority of patients. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Wael M. Abukwaik [sup.1] , Rossana Baracco [sup.1] , Amrish Jain [sup.1] , Melissa Gregory [sup.1] , Rudolph P. Valentini [sup.1] , Gaurav Kapur [sup.1] Author Affiliations: (1) grid.253856.f, [...]

Background An internal permanent vascular access [arterio-venous fistula (AVF) or arteriovenous graft (AVG)] is preferred over central venous catheters (CVC) for chronic hemodialysis. However, CVC remain the most commonly used access in children. The objective of this study was to evaluate our experience with AVF. Methods We conducted a retrospective chart review of children aged 1-18 years on chronic hemodialysis from 2001 to 2012. Patients were divided into three time periods: 2001-2005, 2006-2009 and 2010-2012. A systematic approach to AVF placement was introduced in our department in 2006 which resulted in a greater number of AVF being placed and used, but the access failure rate was still higher than desired. In 2010, a more experienced vascular surgeon was contacted to perform AVF surgery in our most difficult AVF candidates. Results Sixty-five AVF were created in 55 patients (67.3 % male). The median age of the patients was 14 (3-18) years. Forty-one (63.1 %) AVF were used successfully, and this number increased from 52.6 to 57.6 to 92.3 % over the three time periods, respectively. Over time, AVF use rates increased and CVC use decreased. By 2012 only 7.7 % of our patients were using a CVC. The primary patency rate was 42.9 % at 1 year; secondary patency rates were 100 and 93.8 % at 1 and 2 years, respectively. Infection and hospitalization rates were higher for CVC than for AVF [0.8 vs. 0.1 infections per access-year (p < 0.001) and 0.9 vs. 0.2 hospitalizations per access-year (p < 0.001)]. Conclusions With a dedicated approach and vascular access team it is possible to decrease CVC and increase AVF use in children on hemodialysis. In our study, increased AVF use resulted in decreased access-related infection and hospitalization rates. Keywords Arteriovenous fistula * Central venous catheter * Chronic hemodialysis * Vascular access, Introduction The number of children with end-stage renal disease (ESRD) receiving hemodialysis (HD) is increasing. According to the 2013 United States Renal Data System Annual Report, the number of prevalent [...]

Background Low birth weight is associated with diminished renal function. However, despite African Americans being at increased risk of low birth weight and chronic kidney disease, little is known about the association between birth weight and renal function in diverse groups. We examined racial differences in the relationship of birth weight and renal function among healthy young children. Methods Birth weight and serum creatinine data were available on 152 children (61.8% African American; 47.4% female) from a birth cohort. Estimated glomerular filtration rate (eGFR) was calculated using the bedside Schwartz equation and gender- and gestational-age-adjusted birth weight Z-scores using the US population as a reference. Race-specific linear regression models were fit to estimate the association between birth weight Z-score and eGFR. Results Mean age was 1.5 [+ or -] 1.3 years at first eGFR measurement. African Americans had lower eGFR than non-African Americans (median eGFR = 82 vs. 95 ml/min per 1.73 [m.sup.2]; p = 0.06). Birth weight was significantly and positively associated with eGFR among African American (p = 0.012) but not non-African American children (p = 0.33). Conclusions We provide, for the first time, evidence suggesting that birth weight is associated with renal function in African American children. Future work is needed to determine if prenatal programming helps explain racial disparities in adult health. Keywords Prenatal programming * Race * Disparity * Birth weight * Renal function, Introduction Increasing evidence suggests that the predisposition to adult chronic disease may originate in the prenatal and gestational periods [1, 2]. Low birth weight is associated with smaller kidneys and [...]

Although prednisone is the treatment of choice for nephrotic syndrome (NS) in childhood, the dosing regimen varies between 60 mg/m.sup.2/day, as recommended in early studies, to the often prescribed 2 mg/kg/day dose, which is used in common practice. Mathematical models have demonstrated that weight-based dosing can be less than body surface area (BSA)-based dosing in smaller children. To test our hypothesis that weight-based dosing would result in altered treatment outcomes in children with NS, we analyzed a cohort of 56 children (mean age 5.4 ± 3.8 years) treated with a weight-based dosing regimen. Theoretical underdosing of corticosteroids was tested by calculating a relative underdosing percentage (RUP), which was defined as the dose difference between the theoretical BSA-based dose and the actual weight-based doses divided by the BSA-based dose x 100. We found that the mean 'actual' prednisone dose in our patients was 43.6 ± 19.3 mg/day; in contrast, the mean theoretical BSA-based dose was calculated to be 48.8 ± 16.7 mg/day. Among the 56 patients, 43 (76.7%) were initial responders, of whom 58% followed a frequently relapsing (FR) course. RUP was significantly higher in FR (16.6 ± 7.9%) than in infrequent relapsers (8.7 ± 9.8%) (P = 0.03). RUP was not significantly different among initial responders and nonresponders. Based on these results, we conclude that prednisone underdosing, when dosing is prescribed according to weight, does not affect the initial response to treatment, but it does increase the likelihood of a FR course in responders., Author(s): Sermin A. Saadeh [sup.1] , Rossana Baracco [sup.1] , Amrish Jain [sup.1] , Gaurav Kapur [sup.1] , Tej K. Mattoo [sup.1] , Rudolph P. Valentini [sup.1] Author Affiliations: (1) [...]

Iron deficiency (ID) contributes to the development of anemia in patients with chronic kidney disease (CKD). The frequency of ID in children with early CKD has not previously been reported. This was a retrospective chart review of children with CKD stages 2 and 3 followed at the CKD clinic of Children's Hospital of Michigan. ID was defined as low ferritin and transferrin saturation Keywords Iron deficiency * Anemia * Chronic kidney disease * Ferritin * Transferrin saturation, Introduction Anemia has been associated with increased mortality and increased risk of hospitalization in children with chronic kidney disease (CKD) [1]. Apart from the complications of anemia, iron deficiency (ID) [...]

The main aim of this study was to compare the response to trivalent inactivated influenza vaccine in children who received a kidney transplant and were on steroid-free versus steroid-based immunosuppression. Groups: 1. Kidney transplant recipients on steroid-free immunosuppression (n=27); 2. Kidney transplant recipients on steroid-based immunosuppression (n=39); 3. Healthy controls (n=21). Hemagglutination inhibition titers against 2007-2008 A/H1N1 and A/H3N2 and B strains were measured before and 8 weeks postvaccination. Postvaccination geometric mean titers to A/H1N1 were significantly lower among both transplant groups than controls (p=0.025 and 0.015, respectively). Postvaccination titers to H3N2 and B strains were not statistically different between groups. Proportions of participants developing seroprotection were not different among groups. Both kidney transplant groups seroconverted less than controls for A/H1N1 (p=0.0002) and were no different from controls for B. For A/H3N2, the steroid-free group had the weakest seroconversion (p=0.008), possibly due to mycophenolate-enhanced exposure and a younger age. Overall, children after kidney transplantation demonstrated a good serologic response to the inactivated influenza vaccine although somewhat lower than controls. Steroid-free immunosuppression did not seem to present an advantage in antibody response. Data on inactivated influenza vaccine safety and efficacy was collected and demonstrated absence of acute rejection or laboratory-proven influenza for 6 months postvaccination. Keywords Influenza * Vaccination * Kidney * Transplantation * Children, Introduction Influenza A and B are the two virus types that cause human disease [1]. Influenza A viruses are additionally categorized into subtypes based on two surface antigens: hemagglutinin and [...]

The approach to the pediatric patient with membranous nephropathy (MN) can be challenging to the practitioner. The clinical presentation of the child with this histologic entity usually involves some degree of proteinuria ranging from persistent, subnephrotic-ranged proteinuria to overt nephrotic syndrome. Patients often have accompanying microscopic hematuria and may have azotemia or mild hypertension. Children presenting with nephrotic syndrome are often steroid resistant; as such, their biopsy for steroid-resistant nephrotic syndrome results in the diagnosis of MN. The practitioner treating MN in the pediatric patient must weigh the risks of immunosuppressive therapy against the benefits. In general, the child with subnephrotic proteinuria and normal renal function can likely be treated conservatively with angiotensin blockade (angiotensin-converting enzyme inhibitors or angiotensin receptor blockers) without the need for immunosuppressive therapy. Those with nephrotic syndrome are usually treated with steroids initially and often followed by alkylating agents (cyclophosphamide or chlorambucil). Calcineurin inhibitors may also be useful, but the relapse rate after their discontinuation remains high. The absence of controlled studies in children with MN makes treatment recommendations difficult, but until they are available, using the patient's clinical presentation and risk of disease progression appears to be the most prudent approach. Keywords Membranous glomerulopathy * Membranous glomerulonephritis * Nephrotic syndrome * Cyclophosphamide * Cyclosporine * Pediatrics, Introduction Membranous nephropathy (MN) is a rare histologic entity in children, which usually presents as nephrotic syndrome or asymptomatic proteinuria [1, 2]. Whereas it is one of the most common [...]

Abstract Calciphylaxis is a rare, but life-threatening complication of end-stage renal disease (ESRD) that has been reported mostly in adult patients. The exact etiology is unknown, but the disease is [...]

Byline: Timothy E. Bunchman (1), Rulan S. Parekh (1), Joseph T. Flynn (1), William E. Smoyer (1), David B. Kershaw (1), Rudolph P. Valentini (1), Brenda J. Pontillo (1), Jill Sandvordenker (1), Catherine Brown (1), Aileen B. Sedman (1) Keywords: Key words: Pediatric kidney transplantation; Neoral; Sandimmun Abstract: Neoral was instituted in pediatric renal transplant patients with the hypothesis it would have more predictable kinetics than Sandimmun. However, significant questions have arisen concerning potential toxicity and dosing interval related to its rapid absorption with subsequent high initial peak. This is compounded by the fact that children appear to metabolize cyclosporine at a greater rate than adults. This combination of a rapid peak and rapid absorption may then result in lower trough levels at 12 h. We compared the trough cyclosporine levels of nine children who received Neoral with nine who received Sandimmun at the time of initial transplantation. More frequent dosing (every 8 h) was required in the Neoral population compared with the Sandimmun population for the 1st month in order to obtain comparable trough levels. Beyond the initial 4--6 weeks, trough levels were similar for Neoral and Sandimmun. Whereas 1-month creatinine levels and blood pressures were similar, the number of blood pressure medications was significantly higher in the Neoral group. At 5.5 +- 1.1 months&apos; followup, a single patient in the current Neoral group and in the retrospective Sandimmun group each experienced a single OKT3 allograft-treated rejection. We suggest that the area under the curve is different in Neoral than Sandimmun, and the initial dosing frequency may need to be adjusted accordingly. Author Affiliation: (1) Division of Pediatric Nephrology, University of Michigan, Michigan, USA, US Article note: Received August 21, 1996 received in revised form June 27, 1997 accepted June 30, 1997

Total parenteral nutrition (TPN) was started on hospital day 7 as the patient was still intubated and stuporous. Prior to initiation of TPN, serum thiamine and cobalamin levels were obtained [...]

Byline: Girish Deshpande (1), Kathleen L. Meert (1), R. P. Valentini (1) Keywords: Key wordsaCharcoal hemoperfusion; Carbamazepine; Poisoning; Ileus Abstract: A 16-month-old female experienced a massive carbamazepine ingestion resulting in a peak serum carbamazepine concentration of 55 Aug/ml. Clinical manifestations included generalized seizures, coma, shock, and gastrointestinal hypomotility. Gut decontamination was attempted using multiple-dose activated charcoal and cathartics. Because of the severity of illness, charcoal hemoperfusion was initiated. The patient underwent three sessions of charcoal hemoperfusion, each utilizing a fresh cartridge, with one session immediately following the other. Serum carbamazepine and carbamazepine-10,11-epoxide concentrations decreased from 54 Aug/ml to 23 Aug/ml, and 30 Aug/ml to 17 Aug/ml, respectively, during charcoal hemoperfusion. There were no complications. The patient recovered completely and was discharged on the 4th hospital day. Charcoal hemoperfusion should be considered for life-threatening carbamazepine intoxication, especially when drug-induced gastrointestinal hypomotility prevents elimination via the gut. Author Affiliation: (1) Department of Pediatrics, Children&apos;s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI 48201, USA Tel.: +1-313-745-5604, Fax: +1-313-966-0039, US Article note: Received: 12 October 1998 / Revised: 11 December 1998 / Accepted: 17 December 1998

Case summary A 16-year-old girl with a history of systemic lupus erythematosus (SLE), end-stage renal disease (ESRD) secondary to lupus nephritis on continuous cycling peritoneal dialysis (PD) presented to the [...]

Sir, We read with great interest the letter sent to your journal by Hirano and Fujinaga [1] in which they describe a retrospective review of children with idiopathic nephrotic syndrome [...]

Byline: Timothy E. Bunchman (1), Rudolph P. Valentini (1), John Gardner (1), Theresa Mottes (1), Timothy Kudelka (1), Norma J. Maxvold (1) Keywords: Key wordsaHigh-efficiency dialysis membranes; Vancomycin intoxication Abstract: Two children underwent acute hemodialysis using high-efficiency dialysis membranes for vancomycin intoxication (plasma levels 238 Aug/ml and 182 Aug/ml). During a 3-h treatment, plasma vancomycin removal was on average 60%, with a calculated vancomycin half-life (t .sub.1/2) of 2 h. This is in contrast to a recent report using charcoal hemoperfusion for vancomycin intoxication (plasma level of 137 Aug/ml), which resulted in a 40% relative plasma clearance and a calculated vancomycin t .sub.1/2 of 12.5 h for a 4-h treatment. The choice of optimal modality for clearing a toxin should take into account the availability of equipment, protein or lipid binding of the toxin, and inherent risks of charcoal hemofiltration (large extracorporeal circuit, reversible hypocalcemia, heat loss, reversible coagulation defects) versus risks of high-efficiency hemodialysis (large extracorporeal circuit). Author Affiliation: (1) Division of Pediatric Nephrology and Critical Care, C. S. Mott Children&apos;s Hospital, University of Michigan, Michigan, USA, US (2) Department of Pediatric Nephrology and Transplantation, University of Michigan, F6865 Box 0297, 1505 Simpson Road E., Ann Arbor, MI 48109, USA e-mail: bunchman@umich.edu Tel.: +1-734-9364210, Fax: +1-734-7636997, US Article note: Received: 6 July 1998 / Revised: 28 January 1999 / Accepted: 1 February 1999