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BACKGROUND: We evaluated the epidemiology of fluid balance (FB) over the first postnatal week and its impact on outcomes in a multi-center cohort of premature neonates from the AWAKEN study. METHODS: Retrospective analysis of infants

BACKGROUND: Measurement of glomerular filtration rate by iohexol disappearance (iGFR) has become a gold standard in the pediatric CKD population. The need for serial phlebotomy can be difficult and minimizing venipunctures would be beneficial. Furthermore, finger stick collection for dried blood spot (DBS) may be more tolerable in the pediatric population, and equivalence between these two methods may further simplify the process. METHODS: This was a cross-sectional study in children and adolescents 1 to 21 years with Stage I-IV CKD. Iohexol was infused and blood drawn 10, 30, 120, and 300 minutes later. Blood spots on filter paper were collected by finger-stick after each of the latter two blood draws. The rate of iohexol plasma disappearance was used to calculate GFR. Pearson’s correlation coefficient and bias, Students t-test and Bland Altman graphical representations were used to compare methods. RESULTS: Forty one patients were recruited. The mean creatinine was 1.13 mg/dL (SD 0.45), the mean 4-point iGFR was 73.2 ml/min/1.73m(2) (SD 27.5) and the mean 2-point iGFR was 75.6 ml/min/1.73m(2) (SD 27.3). Correlation between 2-point and 4-point venous GFR was r=0.97; p

Summary Background Acute kidney injury occurs in one in four children admitted to an intensive care unit (ICU) and its severity is independently associated with increased patient morbidity and mortality. Early prediction of acute kidney injury has the potential to improve outcomes. In smaller, single-centre trial populations, we have previously derived and validated the performance of a renal angina index, a context-driven risk stratification system, to predict severe acute kidney injury in children and adolescents. Here, we tested the predictive accuracy of this index for severe acute kidney injury in a large heterogeneous population. Methods We did a prospective, observational study (AWARE) that recruited patients in the ICUs of 32 hospitals in nine countries across Asia, Australia, Europe, and North America. All patients aged between 3 months and 25 years who were admitted to an ICU at least 48 h previously were eligible. Exclusion criteria were a history of stage 5 chronic kidney disease (ie, estimated glomerular filtration rate 2 or on maintenance dialysis) or kidney transplantation in the preceding 90 days. Patients' medical records were reviewed to collect data up to 3 months before (serum creatinine only), daily during the first 7 days, and on day 28 after ICU admission. For the assessment of the renal angina index, we included patients from the AWARE study who had full data from the day of ICU admission, day 3, and day 28, including serum creatinine concentrations and urine output measurements. The primary outcome was the presence of severe acute kidney injury (stage 2–3 acute kidney injury, according to Kidney Disease Improving Global Outcomes [KDIGO] guidelines) on the third day after ICU admission. We compared the performance of the renal angina index with changes in serum creatinine relative to baseline for prediction of the primary outcome. A score of eight points or more on the renal angina index defined fulfilment of renal angina; serum creatinine concentration relative to baseline was calculated using maximum serum creatinine concentration in the first 12 h of ICU admission). This trial is registered with ClinicalTrials.gov, number NCT01987921. Findings Between Jan 1 and Dec 31, 2014, we obtained data for 1590 patients. 286 patients (18%) had fulfilment of renal angina. At day 3, severe acute kidney injury occurred in 121 (42%) patients positive for renal angina and 247 (19%) patients negative for renal angina (relative risk [RR] 2·23, 95% CI 1·87–2.66, p vs 18 [2%], p vs 53 [4%], p=0·01). Fulfilment of renal angina showed better prediction for severe acute kidney injury than serum creatinine greater than baseline (RR 1.61, 95% CI 1·33–1·93; p vs serum creatinine greater than baseline 0·68, 0·49–4·94). Interpretation Earlier and better prediction of severe acute kidney injury has the potential to improve patient outcomes associated with acute kidney injury. Compared with isolated, context-free changes in serum creatinine, renal angina risk assessment improved accuracy for prediction of severe acute kidney injury in critically ill children and young people. Funding US National Institutes of Health.

The study utilized a case study approach to investigate the change in technology use of two teachers of elementary-aged students with moderate to severe disabilities during the first year of a multi-year professional development and research project. Participants in a larger multi-year professional development and research project focused on increasing educators’ capacity to provide comprehensive literacy instruction to their students; these teachers self-identified as interested in technology as a support for their students’ participation during instruction. Data sources included field notes and logs, interviews, classroom observations, photographs and video of instruction, and document review. The teachers made substantive changes in their literacy instruction as well as the technology tools they used to engage students instructionally. Two themes emerged from the data as influencers of technology integration: (a) the evolving nature of curriculum reform and (b) ongoing interdisciplinary collaboration. Implications for professional development and future research are shared.

Our intention of connecting professional development with self-study of practice reflects the idea that educators benefit from various forms of knowledge, including formal and practical, which relate to the what and how of teaching (LaBoskey, 2004). Since self-study of practice is intended to improve upon one’s immediate practice (LaBoskey) and to align theory to practice (Loughran, 2007), the use of self-study seemed like an effective way to connect the educators’ professional development work with their change in practice.

Objective: Elevated urine albumin to creatinine ratio (ACR) of >30 mg/gm is a widely agreed upon indicator of pathologic albuminuria in children. However, the most reliable specimen to measure ACR in children remains undefined. We assess the range and limits of upright and supine total albumin and ACR in healthy children. Methods: Healthy children age 6 - 18 years completed 24-hour and split upright and supine urine collections. Upright, supine and 24-hour protein, albumin and creatinine were measured. Primary outcomes are range and variation in urine albumin by diurnal status, age, gender, BMI percentile and Tanner stage. Results: In healthy children, with mean age 12.9 year (sd 3.2), upright ACR was 2-fold greater than supine (13.9 vs 6.8 mg/gm, p = 0.02). The range of ACR was much greater in the upright (2 - 323 mg/gm) compared to the supine (1.7 - 76 mg/gm) samples. The average total 24-hour urine albumin was 8.4 mg (sd 9.8) and the mean ACR was 8.9 mg/gm (sd 11.7). The 24-hour albumin increased with age and Tanner stage, but this relationship was not significant after adjusting for BSA or urine creatinine. A supine or upright ACR of >30 mg/gm was found in 5.4% of each group. However, in all subjects with an elevated ACR on an individual upright or supine sample, a second 1st am ACR sample was normal. Conclusions: In healthy children there is a marked diurnal variability in ACR with a higher value from a daytime sample compared to 1st morning specimen. Screening for pathologic albuminuria should always use a first morning urine specimen.

Background and objectives: Children with chronic kidney disease (CKD) have an increased risk of progression to ESRD. There is a need to identify treatments to slow the progression of CKD, yet there are limited data regarding clinical risk factors that may be suitable targets to slow progression. Design, setting, participants, & measurements: We performed a retrospective cohort study using the North American Pediatric Renal Trials and Cooperative Studies CKD database. There were 4166 pediatric subjects with CKD stages II to IV. Disease progression was defined as a GFR on follow-up of

Recently, Schwartz et al. (J Am Soc Nephrol 20:629-637, 2009) used data from the National Institutes of Health-funded Chronic Kidney Disease in Children (CKiD) study to generate new equations for estimating the glomerular filtration rate (eGFR), including an update of the commonly used bedside equation. However, it is unclear if the equation can be generalized to a broader pediatric population. We have used the updated equation on a sample of pediatric patients with less impaired renal function to evaluate the correlation between the new Schwartz equation and measured GFR by iothalamate clearance. We retrospectively analyzed 738 iothalamate clearance tests from 503 patients with a mean serum creatinine of 0.50 mg/dl whose ages ranged from 1 to 16 years. We measured bias, precision, and accuracy and performed a Bland-Altman plot to determine the measure of agreement between the two methods. The mean GFR by iothalamate clearance was 110.6 ml/min/1.73 m(2) and by the new Schwartz estimation 104.7 ml/min/1.73 m(2). The mean difference was 5.84 ml/min/1.73 m(2) (95% CI 4.00-7.67). The newly purposed bedside Schwartz equation therefore demonstrated good agreement with the iothalamate renal clearances in our patient population and appears to be a valid bedside estimating equation for GFR in this sample of children.

Case studies of three urban elementary schools were conducted to document the integration of technology given identical resources from a local university’s PT3 grant. Data sources for this qualitative study included participant observers’ field notes and journal entries, school personnel interviews, timeline and chronicle of technology-related priorities and events, and children’s and teachers’ technology artifacts. Cases were summarized with respect to prior technology context, agents of growth and development, and changes and future directions. The analysis identified three scaffolds that appear to have a significant influence on—and redefine the challenge of—technology integration: alignment with the curriculum/mission, teacher leadership, and public/private roles for technology recognition.

This article describes an integrated augmentative and alternative communication (AAC) and literacy intervention program developed for five adults with autism in a supported employment facility. Specific assessment and intervention strategies as well as general outcomes of the program are discussed. Three detailed case studies describe approaches used with project participants who had emerging, beginning, and more advanced levels of communication and literacy skill.

Introduction: Atypical HUS [aHUS] is a rare disease characterized by hemolytic anemia, thrombocytopenia and renal dysfunction due to genetic mutations that lead to uncontrolled activation of the alternative complement pathway. MCP [Membrane Co-Factor protein] or CD46 is a complement factor 3b (C3b) binding molecule which is a cofactor for complement factor I dependent cleavage of C3b. Pathogenic variants in MCP gene account for 5-9% of cases with aHUS. We report the case of a pediatric patient presenting with severe thrombocytopenia, anemia and renal dysfunction who was found to have a novel mutation in MCP and complete recovery with eculizumab therapy. Case report: A previously healthy 9-year-old girl presented with a 2-day history of fever, abdominal pain, emesis and oliguria. There was no history of diarrhea, sick contacts or infectious exposures. There was no known renal disease in her family. Her exam was remarkable for mild scleral icterus, epigastric tenderness but no peritoneal signs. Her labs showed renal failure; BUN 90mg/dl, Cr 4.69 mg/dl, anemia: hemoglobin of 8.4g/dl and severe thrombocytopenia with a platelet count of 4,000/uL. Her peripheral smear had schistocytes, the LDH was elevated at 2511 units/liter and haptoglobin was low at 14 mg/dl. Coagulation parameters were normal. All findings were consistent with severe thrombotic microangiopathy (TMA). A stool assay for Shiga toxin was negative, Her C3 and C4 levels were normal Renal ultrasound showed only increased echogenicity in the right kidney. Urinalysis showed hematuria and proteinuria, culture was negative. Based on triad of hemolytic anemia, thrombocytopenia and renal dysfunction microangiopathic hemolytic anemia (MAHA) was suspected. Pending ADAMTS13 results, plasmapheresis was performed daily (x4) without significant improvement in hemolysis or renal function- as a result. Eculizumab, a human anti-C5 monoclonal antibody was initiated: after the first 2 doses, there was significant improvement in renal function, her hemolysis stopped, and platelet counts began to recover. After 2 months of treatment, renal function, hemoglobin and platelets have all normalized. Her genetic renal panel tested for mutations in 10 genes implicated in aHUS revealed a heterozygous variant in Exon 2 of the CD46 gene (c.132G>A, pMet44Ile) predicted pathogenic by 5/6 algorithms. Based on the patient presentation, normal ADAMTS13 activity (69%), negative Shiga toxin, and response to eculizumab, this case of aHUS was most likely related to the novel CD46 mutation reported for the first time in this work.. Conclusion: Our patient had an unusual presentation of Hemolytic uremic syndrome characterized by severe thrombocytopenia and hemolytic anemia. This case is unique in that we saw moderately severe renal failure, but no other organ system involvement despite severe hematologic aberrations. In atypical HUS,we often see more multiorgan involvement when platelets counts are very low, consistent with more severe thombotoc microangipathy. Her genetic test revealed a novel mutation in the CD46 gene, a regulator of complement activation. She has had an excellent response to eculizumab therapy with complete recovery of renal function and resolution of all hematologic signs of thrombotic microangiopathy, suggesting that his gene variant can be causative of atypical HUS. Disclosures Aaron: Alexion - aHUS Regional Advisory Board: Consultancy, Membership on an entity's Board of Directors or advisory committees. Wong:UpToDate: Consultancy, Patents & Royalties.

David A. Koppenhaver is director of the Carolina Literacy Center for Persons with Developmental Disabilities. He is currently investigating the roles of augmentative communication and other assistive technologies in the literacy learning of children with developmental disabilities. Jane D. Steelman is associate director of technology at the Carolina Literacy Center. She is an instructional technologist who has taught in grades 1 through 12 and is currently developing literacy software for students with developmental disabilities. Patsy L. Pierce is associate director of education at the Carolina Literacy Center. She is a speech-language pathologist and special educator currently studying emergent literacy strategies and development in children with developmental disabilities. David E. Yoder is professor of speech and hearing sciences and chair of the Department of Medical Allied Health Professions at the University of North Carolina at Chapel Hill. He is interested in the role of augmentative and alternative communication in the acquisition of reading and writing. Amy Staples is a research associate at the Carolina Literacy Center who is currently coordinating a threeyear study of the role of technology in literacy learning of children with developmental disabilities. She is completing a doctorate in educational psychology at the University of North Carolina at Chapel Hill. David A. Koppenhaver, PhD Jane D. Steelman, EdD Patsy L. Pierce, PhD David E. Yoder, PhD Amy Staples, MEd

The aim of this study was to characterize the 24-h and diurnal variability of urinary protein excretion and identify the prevalence of orthostatic proteinuria (OP) in healthy children. Upright, supine, and 24-h total urinary protein (UrTP) and creatinine clearance (CrCl) were measured in 91 healthy children ages 6–19 years. Urinary protein and creatinine excretions were calculated and examined by gender, age, Tanner stage, and body mass index (BMI). Orthostatic proteinuria (OP) was defined as a 24-h UrTP >100 mg/m2 with a normal supine UrTP (10 years and BMI >85%. In children with OP, a first morning UPcr shows a value in the normal range, whereas a random daytime UPcr is elevated. There exists a diurnal variability in urinary protein excretion that is exaggerated in participants with OP. UPcr reliably estimates 24-h UrTP. Using current pediatric criteria, OP is very common, particularly in boys. A normal first morning UPcr ratio indicates that a child with elevated random urinary protein has OP.

The present review provides an overview of the identified risk factors for chronic kidney disease (CKD) progression emphasizing the pediatric population.Over the past 10 years, there have been significant changes to our understanding and study of preterminal kidney failure. Recent refinements in the measurement of glomerular filtration rate and glomerular filtration rate estimating equations are important tools for identification and association of risk factors for CKD progression in children. In pediatric CKD, lower level of kidney function at presentation, higher levels of proteinuria, and hypertension are known markers for a more rapid decline in glomerular filtration rate. Anemia and other reported risk factors from the pregenomic era require further study and validation. Genome-wide association studies have identified genetic loci that have provided novel genetic risk factors for CKD progression.With cohort studies of children with CKD becoming mature, they have started to yield important refinements to the assessment of CKD progression. Although many of the traditional risk factors for renal progression will certainly be assessed, such cohorts will be important for evaluating novel risk factors identified by genome-wide studies.

C1q nephropathy (C1qN) is an uncommon disorder seen in children and adults with nephrotic syndrome and non-specific urinary findings. It has been described with minimal change nephrotic syndrome (MCNS), focal segmental glomerulonephritis and isolated mesangial proliferative glomerulonephritis. We describe nine children with MCNS and mesangial C1q deposition. These children had a median age of 2.7 years at diagnosis (range 1.3–15 years), 56% were male and 78% were Hispanic. We compared these children to concurrent patients with nephrotic syndrome and biopsy-proven MCNS. We found that the C1qN patients were more likely than MCNS children to require chronic immunosuppression with calcineurin inhibitors or mycophenolate mofetil to maintain remission. However, all children were able to achieve and sustain clinical remission of nephrotic syndrome. Children with C1qN and minimal change histology have an increased frequency of frequently relapsing and steroid-unresponsive disease, but they can attain prolonged remission and stable renal function with calcineurin inhibitor or mycophenolate mofetil therapy.

School-age students who use AAC need access to communication, reading, and writing tools that can support them to actively engage in literacy learning. They also require access to core literacy learning opportunities across grade levels that foster development of conventional literacy skills. The importance of the acquisition of conventional literacy skills for students who use AAC cannot be overemphasized. And yet, one of the critical challenges in supporting the literacy learning of students who use AAC has been a lack of knowledge about literacy curricula and supports to literacy learning for these students. Most students who use AAC do not become conventionally literate and few of those who do achieve literacy skills beyond the second grade level. This article will provide an overview of the most frequent reading instructional activities in first and third grade classrooms. To better understand the foundational experiences important to literacy learning, the results of a survey project that examined the reading activities of general education students and teachers during primary grade instruction are presented, and critical shifts in instruction that occurred between first and third grade are highlighted. The primary instructional focus of core reading activities is also examined, along with adaptations for students who use AAC.