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The authors talk about the International Pediatric Nephrology Association (IPNA)-European Society for Paediatric Nephrology (ESPN) Junior Master Class program. Topics include purpose of launching the program in 2014, components of the program, and demographic characteristics of IPNA-ESPN Junior Master Class participants. Participants' feedback evaluation is also mentioned.

The effects of bone and mineral metabolism on skeletal formation, as well as vascular and soft tissue calcifications, define chronic kidney disease–metabolic bone disease (CKD-MBD). Treatment recommendations center on establishing adequate vitamin D status, phosphate control through diet restriction and phosphate binders, and the use of vitamin D analogs for specific indications. Several emerging bone-promoting therapies have now been studied in adults with CKD, including bisphosphonates and denosumab. These approaches are associated with improved bone mass and, in some cases, decreased fracture rates in adults with CKD-MBD and are of potential interest for some children with CKD-MBD. In children with CKD and immobilization and/or muscle weakness, bisphosphonates appear to be an effective treatment to increase bone mass; baseline assessment and careful monitoring of bone density and/or bone biopsy findings are important in consideration of any new bone therapies for children with CKD-MBD. [ABSTRACT FROM AUTHOR]

Background: To define the incidence of acute kidney injury (AKI), chronic kidney disease (CKD), and hypertension (HTN) in pediatric patients diagnosed with acute lymphoblastic leukemia (ALL) over a recent 9-year period. Methods: This study is a retrospective cohort study of all pediatric patients diagnosed with ALL at Nationwide Children's Hospital from January 1, 2008, to December 31, 2016. Patient demographic and clinical data including serum creatinine and blood pressure were collected at diagnosis up to 9 years post diagnosis. Results: A total of 222 patients were identified for this study. The overall incidence of AKI in our cohort was high, with 101 subjects (45.5%, CI 38.8–52.3%) developing AKI at least once. CKD status could only be determined in 214 patients due to limited later GFR data. The incidence of CKD was low with only 5 of 214 patients developing CKD (2.3%, CI: 0.8–5.4%). The overall incidence of HTN at diagnosis was 45.6% (95% CI: 59.1–72%), and at 1 month post diagnosis was 65.8% (95% CI: 59.1–72.0%). Chronic HTN could only be determined in 216 patients due to limited blood pressure data. Chronic HTN was noted in 34.3% of patients (74/216, 95% CI: 28–41%). Conclusions: Among children with ALL, the incidence of AKI is relatively high at the time of diagnosis. However, development of CKD is relatively rare, suggesting good mid-term kidney prognosis. There is a high incidence of HTN at the time of diagnosis, 1 month post diagnosis, and chronic HTN that often goes untreated. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Background: Data from adult and pediatric literature have shown an association between albumin levels and AKI. Whether hypoalbuminemia and neonatal AKI are associated has not been studied. Methods: We evaluated the association of albumin with early (during the first postnatal week) and late (after the first postnatal week) AKI for 531 neonates from the Assessment of Worldwide AKI Epidemiology in Neonates (AWAKEN) database and for 3 gestational age (GA) subgroups: < 29, 29 to < 36, and ≥ 36 weeks GA. Results: Low albumin levels were associated with increased odds of neonatal AKI; for every 0.1 g/dL decrease in albumin, the odds of late AKI increased by 12% on continuous analysis. After adjustment for potential confounders, neonates with albumin values in the lowest quartiles (< 2.2 g/dL) had an increased odds of early [Adjusted Odd Ratio (AdjOR) 2.5, 95% CI = 1.1–5.3, p < 0.03] and late AKI [AdjOR 13.4, 95% CI = 3.6–49.9, p < 0.0001] compared to those with albumin in the highest quartile (> 3.1 g/dL). This held true for albumin levels 2.3 to 2.6 g/dL for early [AdjOR 2.5, 95% CI = 1.2–5.5, p < 0.02] and late AKI [AdjOR 6.4, 95% CI = 1.9–21.6, p < 0.01]. Albumin quartiles of (2.7 to 3.0 g/dL) were associated with increased odds of late AKI. Albumin levels of 2.6 g/dL and 2.4 g/dL best predicted early (AUC = 0.59) and late AKI (AUC = 0.64), respectively. Analysis of albumin association with AKI by GA is described. Conclusions: Low albumin levels are independently associated with early and late neonatal AKI. Albumin could be a potential modifiable risk factor for neonatal AKI. [ABSTRACT FROM AUTHOR]

Background: Acute kidney injury (AKI) in preterm infants is associated with prolonged hospitalization and high mortality. Diuretic therapy has been used to enhance urine output in preterm infants with AKI. Treatment with diuretics, prescription patterns, and relationship with length of stay (LOS), mechanical ventilation (MV), and mortality in preterm infants who also had AKI have not been fully evaluated. Methods: This multicenter retrospective study used the Pediatric Hospital Information System database. We included 2121 preterm infants with AKI diagnosis from 46 hospital Neonatal Intensive Care Units (NICUs) born <37 weeks gestational age (GA). Treatment with diuretics, practice patterns across 46 NICUs in the USA, and associated outcomes including LOS, MV, and mortality were evaluated. Results: Seventy-six percent of infants received at least one dose of diuretics (median treatment 18 days). Diuretic prescription varied significantly across hospitals and ranged from 42 to 96%. Diuretics were used more frequently in infants with younger GA and smaller birth weight. Infants with older GA who received diuretics at or before 28 days postnatally had worse survival even after adjusting for known confounders. Conclusions: Preterm infants with AKI diagnosis were frequently treated with diuretics. Moreover, infants with younger GA and smaller birth weight were more likely to receive diuretics. Worse survival in infants with older GA who received diuretics could be the result of more underlying severe illness in these infants and not the cause of more severe illness. Prospective studies are needed to best determine patient safety and outcomes with diuretic treatment in preterm infants with AKI. [ABSTRACT FROM AUTHOR]

Edema is the abnormal accumulation of fluid in the interstitial compartment of tissues within the body. In nephrotic syndrome, edema is often seen in dependent areas such as the legs, but it can progress to cause significant accumulation in other areas leading to pulmonary edema, ascites, and/or anasarca. In this review, we focus on mechanisms and management of edema in children with nephrotic syndrome. We review the common mechanisms of edema, its burden in pediatric patients, and then present our approach and algorithm for management of edema in pediatric patients. The extensive body of experience accumulated over the last 5 decades means that there are many options, and clinicians may choose among these options based on their experience and careful monitoring of responses in individual patients. [ABSTRACT FROM AUTHOR]

A correction is presented to the article "Low Albumin Levels Are Independently Associated With Neonatal Acute Kidney Injury: A Report From AWAKEN Study Group" which appeared in the March 16, 2022 online issue of the magazine.

Background: Cross-sectional studies of children with prevalent nephrotic syndrome (NS) have shown 25-vitamin D (25(OH)D) deficiency rates of 20-100 %. Information on 25(OH)D status in incident patients or following remission is limited. This study aimed to assess 25(OH)D status of incident idiopathic NS children at presentation and longitudinally with short-term observation. Methods: Multicenter longitudinal study of children (2-18 years old) from 14 centers across the Midwest Pediatric Nephrology Consortium with incident idiopathic NS. 25(OH)D levels were assessed at diagnosis and 3 months later. Results: Sixty-one children, median age 5 (3, 11) years, completed baseline visit and 51 completed second visit labs. All 61 (100 %) had 25(OH)D < 20 ng/ml at diagnosis. Twenty-seven (53 %) had 25(OH)D < 20 ng/ml at follow-up. Fourteen (28 %) children were steroid resistant. Univariate analysis showed that children prescribed vitamin D supplements were less likely to have 25(OH)D deficiency at follow-up (OR 0.2, 95 % CI 0.04, 0.6). Steroid response, age, and season did not predict 25(OH)D deficiency. Multivariable linear regression modeling showed higher 25(OH)D levels at follow-up by 13.2 ng/ml (SE 4.6, p < 0.01) in children supplemented with vitamin D. Conclusions: In this incident idiopathic NS cohort, all children at diagnosis had 25(OH)D deficiency and the majority continued to have a deficiency at 2-4 months. Supplemental vitamin D decreased the odds of 25(OH)D deficiency at follow-up, supporting a role for supplementation in incident NS. [ABSTRACT FROM AUTHOR]

Osteogenesis imperfecta (OI) is an inherited disorder characterized by bone fragility and low bone mass. Low bone density and fracture is a cause of morbidity. Limited data exists on bisphosphonate treatment in patients under 24 months of age. The objective of the study was to examine the safety and efficacy of pamidronate in children under 24 months with OI. To do so, we carried out a retrospective chart review and analysis of OI patients started on intravenous pamidronate under 24 months of age. Pamidronate was administered in three-day cycles. Growth, the number of fractures, and lumbar bone mineral densities were recorded both prior to and after treatment initiation. A total of 18 patients were reviewed. Five were classified as OI type I, seven were type III, and six were type IV. The mean age at treatment initiation was 12 months (range 11 days to 23 months). The mean lumbar z score at baseline was −3.63, which improved to −1.53 at one year ( P < 0.01) and 0.79 ( P < 0.01) at the end of the study. The fracture rate improved from 68 fractures in 209 months (0.32 fractures/patient-month) before treatment to 41 fractures in 1,248 months (0.03 fractures/patient-month) post-treatment ( P < 0.05). Height standard deviation score (SDS) was conserved from baseline to end of study (−2.12 ± 2.45 vs. −2.45 ± 2.73) ( P = 0.05) with an average follow-up of 73 months. The only adverse effect recorded in six infants was fever during the initial pamidronate infusion. Treatment with intravenous pamidronate is safe, significantly improves lumbar bone mineral density (L-BMD), and reduces fracture rates in young infants with OI while preserving linear growth. [ABSTRACT FROM AUTHOR]

Background: The Patient Reported Outcomes Measurement Information System (PROMIS) II is a prospective study that evaluates patient reported outcomes in pediatric chronic diseases as a measure of health-related quality of life (HRQOL). We have evaluated the influence of disease duration on HRQOL and, for the first time, compared the findings of the PROMIS measures to those of the PedsQL™ 4.0 Generic Scales (PedsQL) from the PROMIS II nephrotic syndrome (NS) longitudinal cohort. Methods: This was a prospective study in which 127 children (age range 8-17 years) with active NS from 14 centers were enrolled. Children with active NS defined as the presence of nephrotic range proteinuria (>2+ urinalysis and edema or urine protein/creatinine ratio >2 g/g) were eligible. Comparisons were made between children with prevalent ( N = 67) and incident ( N = 60) disease at the study enrollment visit. Results: The PROMIS scores were worse in prevalent patients in the domains of peer relationship ( p = 0.01) and pain interference ( p < 0.01). The PedsQL showed worse scores in prevalent patients for social functioning ( p < 0.01) and school functioning ( p = 0.03). Multivariable analyses showed that prevalent patients had worse scores in PROMIS pain interference ( p = 0.02) and PedsQL social functioning ( p < 0.01). Conclusion: The PROMIS measures detected a significant impact of disease duration on HRQOL in children, such that peer relationships were worse and pain interfered with daily life to a greater degree among those with longer disease duration. These findings were in agreement with those for similar domains in the PedsQL legacy instrument. [ABSTRACT FROM AUTHOR]

Background and objectives: Chronic kidney disease is a persistent chronic health condition commonly seen in pediatric nephrology programs. Our study aims to evaluate the sensitivity of the Patient Reported Outcomes Measurement Information System (PROMIS) pediatric instrument to indicators of disease severity and activity in pediatric chronic kidney disease. Methods: This cross sectional study included 233 children 8-17 years old, with chronic kidney disease from 16 participating institutions in North America. Disease activity indicators, including hospitalization in the previous 6 months, edema, and number of medications consumed daily, as well as disease severity indicators of kidney function and coexisting medical conditions were captured. PROMIS domains, including depression, anxiety, social-peer relationships, pain interference, fatigue, mobility, and upper extremity function, were administered via web-based questionnaires. Absolute effect sizes (AES) were generated to demonstrate the impact of disease on domain scores. Four children were excluded because of missing glomerular filtration rate (GFR) estimations. Results: Of the 229 children included in the final analysis, 221 completed the entire PROMIS questionnaire. Unadjusted PROMIS domains were responsive to chronic kidney disease activity indicators and number of coexisting conditions. PROMIS domain scores were worse in the presence of recent hospitalizations (depression AES 0.33, anxiety AES 0.42, pain interference AES 0.46, fatigue AES 0.50, mobility AES 0.49), edema (depression AES 0.50, anxiety AES 0.60, pain interference AES 0.77, mobility AES 0.54) and coexisting medical conditions (social peer-relationships AES 0.66, fatigue AES 0.83, mobility AES 0.60, upper extremity function AES 0.48). Conclusions: The PROMIS pediatric domains of depression, anxiety, social-peer relationships, pain interference, and mobility were sensitive to the clinical status of children with chronic kidney disease in this multi-center cross sectional study. We demonstrated that a number of important clinical characteristics including recent history of hospitalization and edema, affected patient perceptions of depression, anxiety, pain interference, fatigue and mobility. The PROMIS instruments provide a potentially valuable tool to study the impact of chronic kidney disease. Additional studies will be required to assess responsiveness in PROMIS score with changes in disease status over time. [ABSTRACT FROM AUTHOR]

Although sleep disorders are common in adults with chronic kidney disease, little is known about the prevalence of sleep problems in children and adolescents with chronic kidney disease and their relationship to health-related quality of life measurements. We performed a clinic-based survey of sleep habits and common symptoms of sleep disturbances in 159 school-aged patients with chronic kidney disease. Three patient groups of chronic kidney disease were assessed: group 1, those not on dialysis and not transplanted; group 2, those on dialysis; and group 3, those with a functioning renal allograft. Four symptom domains for sleep disorders were assessed: excessive daytime sleepiness; sleep disordered breathing; restless legs syndrome symptoms; and insufficient sleep. Patients and the parent-proxy also completed the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales questionnaire. Ninety-three (93) patients (58.5%) had symptoms of a sleep disturbance. The presence of a sleep disturbance correlated with a decrease in health-related quality of life scores that was independent of the chronic kidney disease study group or estimated glomerular filtration rate. We conclude that sleep disturbances are common throughout the spectrum of chronic kidney disease in children and adolescents and are associated with diminished health-related quality of life scores. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A clear definition of the appropriate growth response during recombinant human growth hormone (rhGH) treatment has never been established in the pediatric chronic kidney disease (CKD) population. We present here data from Genentech’s National Cooperative Growth Study (NCGS) on the first-year growth response in prepubertal children with CKD. Using NCGS data, we constructed response curves for the first year of rhGH therapy in 270 (186 males, 84 females) naïve-to-treatment, prepubertal children with CKD prior to transplant or dialysis. Data from both genders were combined because gender was not significantly related to height velocity ( p = 0.51). Response to rhGH was expressed as height velocity (HV) in cm/year. Mean, mean ± 1SD, and mean − 2SD for HV during the first year of rhGH treatment as well as pretreatment HV were plotted versus age. Age-specific HV plots for rhGH-treated children with CKD are presented. At all ages, the first-year mean HV was greater than the mean pretreatment HV. The mean − 2SD for HV in children on rhGH treatment was similar to the mean pretreatment HV. These growth plots will be useful to clinicians for assessing a patient’s first-year growth response. We propose that a HV below the mean − 1SD is an inadequate response. These curves may help identify patients with a suboptimal growth response due to confounding medical factors and/or non-compliance. [ABSTRACT FROM AUTHOR]

As an initial effort to identify opportunities to improve the management of children with nephrotic syndrome, the goal of this study was to assess the present-day management of children with primary nephrotic syndrome. A web-based survey was designed to assess the current management styles of all pediatric nephrology faculties at ten participating institutions. Ninety-one percent completed the initial survey. The duration of initial glucocorticoid therapy ranged from 4 to 24 weeks. Physicians reported that the recommendation for kidney biopsy was dependent on the response to initial corticosteroid therapy, with the minority always recommending a biopsy for frequently relapsing or steroid-dependent cases. All responding physicians recommended a kidney biopsy in steroid-resistant cases. Treatment strategies were reported to vary based upon the steroid response pattern and, where available, kidney histopathology. Striking variations in therapeutic preferences were described when alternatives to glucocorticoids were considered. The variability of management practices among pediatric nephrologists in the USA combined with the changing characteristics of our pediatric population raise concerns about our future strategies for improving healthcare for children coping with nephrotic syndrome. This variability is not unique to children’s healthcare or to nephrology. However, a systematic approach to patient care and improvement in health outcomes has been shown to substantially improve morbidity and mortality outcomes in children with chronic health conditions. [ABSTRACT FROM AUTHOR]

The objective of this study was to identify how many children with hypercalciuria and/or nephrolithiasis have a low bone density and whether the risk of low bone density can be identified by 24-h urine stone-risk profiles and/or growth parameters. A retrospective chart review was performed on 110 idiopathic hypercalciuria and/or kidney stone patients who received both a 24-h urine for stone-risk profile and a dual-energy X-ray densitometry scan. Patients were divided into low bone density vs. normal bone density groups and hypercalcuria verus nephrolithiasis groups and analyzed for differences in growth parameters, urine stone-risk profiles, and bone densities. Overall, 47% had a bone density z score < −1, and 26% had a bone density z score < −2. Patients with a low bone density had a higher body mass index and lower urine creatinine and ammonium than those with a normal bone density. Patients with nephrolithiasis had a lower bone density z score than patients with hypercalcuria and no nephrolithiasis. Clinicians should be aware of the increased incidence of low bone density in children with hypercalciuria and nephrolithiasis. The effect of hypercalciuria and nephrolithiasis treatment on bone density and the natural progression of the bone density in the studied patient population warrants further investigation. [ABSTRACT FROM AUTHOR]

Important discoveries and studies that help inform us about the best methods to evaluate and manage children with end-stage renal disease (ESRD) continue to emerge. This review addresses a number of recent publications regarding important clinical issues for children with ESRD. Despite advances made in previous years, many clinical problems remain in the care of the pediatric dialysis patient. This review covers five topics of recent interest: three articles that address important patient outcome measures such as dialysis adequacy and hemoglobin; two articles that address growth failure in a chronic dialysis patients; five articles that address cardiovascular (CV) morbidity, mortality, and interventions to reduce CV risk in children; two articles that address mineral-bone disorder (MBD) and evidence that past strategies for MBD in children may have increased CV disease; and two articles that address nephrogenic systemic fibrosis, a recently described disorder in chronic kidney disease (CKD) patients that occurs in children as well as adults. Using a concise consistent format, each of the 14 key publications is summarized, and the “conclusion” for the practitioner is identified. The goal of this review is to highlight important work done in this area and focus attention on the important issues raised by each article. [ABSTRACT FROM AUTHOR]

Despite its effectiveness, recombinant human growth hormone (rhGH) is under-utilized in short children with chronic kidney disease (CKD). We conducted a multicenter study to explore the obstacles preventing children with CKD from receiving rhGH. We investigated the use of rhGH in 307 children with CKD from seven pediatric nephrology centers. Among the 110 patients who fell below the 5th percentile, 56 (51%) had not received rhGH. The most common reasons given for these children not receiving rhGH were family refusal, secondary hyperparathyroidism, and non-compliance. However, no explanation was apparent for 25% of the short children with CKD. Boys were more likely than girls to receive rhGH (65% vs 31%; P = 0.002). Use of rhGH was similar in African Americans and non-Hispanic Whites. Children who had received rhGH achieved a 0.5 increase in height z-score in the first year after the initiation of rhGH therapy. Children who had not received rhGH achieved a 0.03 increase in height z-score during the first year after falling below the 5th percentile ( P = 0.005 vs the children who had received rhGH). Waiting for insurance company approval led to a significant delay in the initiation of rhGH treatment in 18% of patients. The fact that more than 50% of short children with CKD did not receive rhGH is secondary to multiple factors, many of which may be amenable to intervention efforts. [ABSTRACT FROM AUTHOR]

Pediatric stem cell transplant (SCT) recipients commonly develop acute renal failure (ARF). We report the demographic and survival data of pediatric SCT patients enrolled in the Prospective Pediatric Continuous Renal Replacement Therapy (ppCRRT) Registry. Since 1 January 2001, 51/370 (13.8%) patients entered in the ppCRRT Registry had received a SCT. Median age was 13.63 (0.53–23.52) years. The primary reasons for the initiation of continuous renal replacement therapy (CRRT) were treatment of fluid overload (FO) and electrolyte imbalance (49%), FO only (39%), electrolyte imbalance only (8%) and other reasons (4%). The CRRT modalities included continuous veno-veno hemodialysis (CVVHD), 43%, continuous veno-veno hemofiltration (CVVH), 37% and continuous veno-veno hemodiafiltration (CVVHDF), 20%. Seventy-six percent had multi-organ dysfunction syndrome (MODS), 72% received ventilatory support and the mean FO was 12.41 ± 3.70%. Forty-five percent of patients survived. Patients receiving convective therapies had better survival rates (59% vs 27%, P < 0.05). Patients requiring ventilatory support had worse survival (35% vs 71%, P < 0.05). Mean airway pressure (Paw) at the end of CRRT was lower in survivors (8.7 ± 2.94 vs 25.76 ± 2.03 mmH2O, P < 0.05). Development of high mean airway pressure in non-survivors is likely related to non-fluid injury, as it was not prevented by early and aggressive fluid management by CRRT therapy. [ABSTRACT FROM AUTHOR]

Growth failure is a clinically important issue in children with chronic kidney disease (CKD) and is associated with significant morbidity and mortality. Many factors contribute to impaired growth in these children, including abnormalities in the growth hormone (GH)–insulin-like growth factor-I (IGF-I) axis, malnutrition, acidosis, and renal bone disease. The management of growth failure in children with CKD is complicated by the presence of other disease-related complications requiring medical intervention. Despite evidence of GH efficacy and safety in this population, some practitioners and families have been reluctant to institute GH therapy, citing an unwillingness to comply with daily injections, reimbursement difficulties, or impending renal transplantation. Suboptimal attention to growth failure management may be further compounded by a lack of clinical guidelines for the appropriate assessment and treatment of growth failure in these children. This review of growth failure in children with CKD concludes with an algorithm developed by members of the consensus committee, outlining their recommendations for appropriate steps to improve growth and overall health outcomes in children with CKD. [ABSTRACT FROM AUTHOR]

Improved recognition of the relationship between childhood and adult blood pressures and identification of end-organ damage in children, adolescents, and young adults with hypertension has led to increased focus by pediatricians and general practitioners on the detection, evaluation, and treatment of hypertension. Notably, detection, evaluation, and treatment of pediatric hypertension has increased significantly since the first Task Force Report on High Blood Pressure in Children and Adolescents in 1977 with advances in both nonpharmacologic and pharmacologic treatments. Angiotensin-converting enzyme inhibitors (e.g. captopril, enalapril, lisinopril, ramipril) and calcium channel antagonists (e.g. nifedipine, amlodipine, felodipine, isradipine) are the most commonly prescribed antihypertensive medications in children due to their low adverse-effect profiles. Diuretics (e.g. thiazide diuretics, loop diuretics, and potassium-sparing diuretics) are usually reserved as adjunct therapy. Newer agents, such as angiotensin receptor antagonists (e.g. irbesartan), are currently being studied in children and adolescents. These agents may be an option in children with chronic cough secondary to angiotensin-converting enzyme inhibitors. β-Adrenoreceptor antagonists (e.g. propranolol, atenolol, metoprolol, and labetalol), α-adrenoreceptor antagonists, α-adrenoreceptor agonists, direct vasodilators, peripheral adrenoreceptor neuron agonists, and combination products are less commonly used in pediatric patients because of adverse events but may be an option in children unresponsive to calcium channel blockers, angiotensin converting-enzyme inhibitors, or angiotensin receptor blockers. [ABSTRACT FROM AUTHOR]

There is ample evidence that the same pathophysiological processes that affect cardiovascular function in adults with end-stage renal disease (ESRD) also operate in children with ESRD. In adults undergoing hemodialysis (HD), a good correlation has been established between left ventricular mass (LVM) and aortic distensibility (AD) as markers of cardiovascular disease progression; however, this correlation has not been established in children. Therefore, in this retrospective study we investigated some aspects of cardiovascular damage (i.e., LVM, LVMI, and AD) in children with ESRD undergoing HD (n=9) or peritoneal dialysis (PD,n=9), and analyzed the relationship between AD, LVM, LVMI, pre-dialysis, post-dialysis blood pressure (BP), and demographic factors in children and adolescents with ESRD. Both LVM and AD were significantly greater in the dialysis population than in a control population derived from our institutional files (P=0.015,P=0.001). LVM and LVMI in children undergoing HD (92.9±83.7 g, 80.1±31.1 g/cm) were not statistically different from the values in children on PD (130.0±89.2 g, 89.6±35.9 g/cm), (P=0.3,P=0.5). AD in children on HD (2.2±0.55 cm2* dynes-1*10-6) was significantly lower than in children on PD (2.7±0.54 cm2* dynes-1*10-6), (P=0.01). The findings in this study confirm earlier studies that demonstrated that LVMI is greater in children on dialysis. This study also demonstrates that abnormal vascular stiffness, as defined by AD, is present in these children. The degree of vascular stiffness in children receiving HD is greater than in children receiving PD. However, further study is needed to address how control of BP, uremia, and other factors may affect these abnormalities in children with ESRD. [ABSTRACT FROM AUTHOR]

The objectives of this study were (1) to determine the relationship of body mass index (BMI) to primary or secondary hypertension in children and adolescents and (2) to assess BMI at the age of onset of hypertension in children and adolescents. Patient demographics, BMI, family history, presentation of disease, etiology of hypertension, medication, laboratory data, and findings from other procedures were recorded for all patients with hypertension followed in the Pediatric Nephrology Clinic at Children’s Hospital, Columbus, Ohio, over a 4-year period. In total, 314 patients were studied: 218 with primary hypertension and 96 with secondary hypertension. Our patient population (166 males, 148 females) was diverse in age (13±6.3 years) and ethnicity (237 Caucasians, 54 African-Americans, 23 other). BMI was greater in patients with primary (27.5±9.2 kg/m2) versus secondary (23.9±9.3 kg/m2) hypertension (P=0.002). Children with primary hypertension with an increased BMI presented at an earlier age than children with secondary hypertension and an increased BMI. The age of onset (10.5±2.6 years) in primary hypertension was related to increased BMI (r=0.12,P=0.001); however, there was no relationship between BMI and age of onset of secondary hypertension (P=0.21). Children whose family members had essential hypertension had increased BMI compared with children without a family history of essential hypertension. Based on the logistic regression model constructed from our data, the likelihood of primary versus secondary hypertension was influenced by the presence of family history of hypertension independent of presence of obesity in the child. In conclusion,increased BMI is more common in children with primary than secondary hypertension; earlier onset of primary hypertension in the pediatric population was associated with increased BMI; the assessment of BMI is important in the evaluation of secondary as well as primary hypertension; the role of obesity in the development of secondary as well as primary hypertension in children merits further study. [ABSTRACT FROM AUTHOR]

IgA nephropathy is the world's most common primary glomerulonephropathy. Recent evidence in a rat model implicated excessive production of oxygen-free radicals in the pathogenesis and suggested that vitamin E-treatment ameliorated progression. We studied this antioxidant therapy on the glomerular filtration rate (GFR), proteinuria and hematuria in biopsy-proven IgA nephropathy in children. The duration of treatment or placebo was 2 years, with vitamin E treatment consisting of 400 IU/day in children weighing <30 kg, and twice that dose for those >30 kg. We measured GFR at entry, midpoint and exit. At baseline and at 4-month intervals after randomization, urinary protein/creatinine ratios and urinalysis were examined. The mixed model procedure with log transformation was used in data analysis to test treatment difference as well as the potential time effect. Fifty-five patients were randomized and 38 completed at least 1 year of follow-up. At entry, the clinical characteristics were not different between the treatment and placebo groups. There was a trend toward better preservation of GFR in vitamin E-treated versus placebo patients, 127±50 vs. 112±31 ml/min/1.73 m[sup 2], respectively (P=0.09). The urinary protein/creatinine ratio was significantly lower in the vitamin E-treated group vs. placebo; 0.24±0.38 vs. 0.61±1.37 (P<0.013). However, there was no difference in the prevalence of hematuria between the groups. Vitamin E treatment in our study patients was associated with significantly lower proteinuria, but no effect on hematuria. While there was a trend toward stabilization of GFR in the vitamin E-treated patients, long-term treatment and follow-up are needed to determine whether antioxidant therapy is associated with preservation of renal function in IgA nephropathy. [ABSTRACT FROM AUTHOR]

This paper describes the development and implementation of a self-management program designed to address noncompliance in adolescents with renal transplants. Transplantation provides the best treatment alternative for End-Stage Renal Disease (ESRD), but is a procedure that subsequently demands recipients follow a life-long medication regimen. Nonadherance to medical therapy is a serious problem for adolescents; it is one of the most common causes of chronic graft rejection in this population. To improve compliance rates for this population, a self-management program was designed: (1) to provide social support; (2) to review information about medications and transplant management; and (3) to both teach and provide transplantation patients the opportunity to practice self-management skills. Details about the program, including recruitment issues and session content, are provided. Evaluations by participants indicated that the program was effective in creating a supportive environment for both patients and their parents, and in addressing health-related concerns. [ABSTRACT FROM AUTHOR]

Essential hypertension in pediatric patients may require pharmacological treatment. There is a need for efficacious, safe, and well-tolerated antihypertensive agents with a once-a-day dosing regimen in children and adolescents. The aim of the trial was to evaluate the dose-response and tolerability of the dihydropyridine calcium channel blocker, felodipine extended-release tablets (felodipine ER), given once daily to pediatric patients with essential hypertension. A randomized double-blind, parallel-group, multi-center clinical study comparing felodipine ER (2.5, 5, or 10 mg once daily) and placebo was performed on pediatric patients with a baseline systolic (SBP) or diastolic blood pressure (DBP) above the 95th percentile for age, sex, and height. Of 133 randomized patients, 128 (96.2%) completed the 3 weeks of double-blind treatment. The study population included 50% children 6-12 years of age or Tanner stage ≤3, 39% black, and 40% female patients. All doses of felodipine ER and placebo decreased both SBP and DBP. The 5-mg dose of felodipine ER resulted in a significant decline in trough sitting, standing, and supine DBP (placebo-adjusted,-4.6,-5.06, and-5.05 mmHg, respectively,P<0.05) compared with placebo. However, there was no significant decline in SBP. The 2.5- and 10-mg doses showed no significant differences compared with placebo in lowering BP. The effect of felodipine ER was comparable in patient subgroups by age, race, and gender. The drug was well tolerated. The study failed to show a dose response of felodipine ER in pediatric patients with essential hypertension. This finding might be explained in part by the limitations of study design. The spectrum of side effects in pediatric patients was comparable with that in adults, except for a lower incidence of peripheral edema. Further studies are needed to clarify the role of felodipine ER in the management of children and adolescents with essential hypertension. [ABSTRACT FROM AUTHOR]

Polycrystalline thin films of the most silicon-rich manganese silicide phase were grown on silicon substrates. X-ray diffraction indicates that the films are single phase tetragonal MnSi. Reflectance and transmittance measured as a function of wavelength were used to derive values of the optical constants and the absorption coefficient of the material. An analysis of the absorption coefficient data suggests that MnSi has a minimum direct energy gap of 0.68 eV. With such a direct bandgap, the material may be suitable for the development of both optical sources and detectors within the silicon microelectronics technology. [ABSTRACT FROM AUTHOR]

Presents a case of primary steroid-sensitive nephrotic syndrome (SSNS) and juvenile idiopathic arthritis (JIA) in which nephrosis preceded the development of JIA by years. Response to steroid therapy; Demonstration of a frequently relapsing NS; Maintenance of low doses of alternate-day prednisolone to prevent relapses of NS.