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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). 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 m2) or 5 (GFR

While the causes of chronic kidney disease (CKD) are different in children than their adult counterparts, many comorbidities are commonly encountered by both populations. Particularly concerning is that these entities contribute to the decrease in lifespan in children with CKD. Amongst these conditions are hypertension, anemia, and electrolyte abnormalities. While these can cause metabolic derangements individually, they each contribute to the development and progression of cardiovascular disease, which in turn, exponentially increases mortality (Vidi Curr Opin Pediatr 30 (2): 247-251, 2018). It has been estimated that the mortality rate of children with CKD is 30 times higher than their healthy peers (Ferris et al. Blood Purif 41 (1-3): 205-10, 2016). As these conditions are each treatable, optimization of medical management can lessen the risk of death in this high-risk population. Hypertension remains underdiagnosed in children with CKD despite a high prevalence. The American Academy of Pediatrics released the Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents in 2007, in which experts outline the need for aggressive diagnosis and treatment of hypertension. While the mainstay of anemia management remains correction of iron deficiency and erythropoiesis stimulating agents, long-acting erythropoietic agents offer an alternative option to traditional management. Electrolyte abnormalities including metabolic acidosis and hyperkalemia must be addressed in order to optimize clinical outcomes. The care of children with CKD remains complex with the need for a wholistic approach. Although the injury initiates within the kidneys, the associated comorbidities have systemic consequences. Some of these include hypertension, anemia, and electrolyte abnormalities. The early identification of these conditions as well as aggressive treatment can improve the health of these children.

An internal permanent vascular access [arteriovenous 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.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.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)].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.

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.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.Mean age was 1.5 ± 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(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).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.

Although prednisone is the treatment of choice for nephrotic syndrome (NS) in childhood, the dosing regimen varies between 60 mg/m(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 × 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.

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 saturation20%. Patients on iron supplements were considered as iron-deficient cases. There were 50 patients included in the study (72% male) with a mean age of 10.31 (±5.21). The mean glomerular filtration rate (GFR) was 55.4 ml/min/1.73 m(2) (±14.61). ID was present in 42% of patients, out of whom almost half (42.9%) presented with anemia. Females had a higher frequency of ID (64.3%). The frequency of ID with anemia increased from 4.3% to 29.6%, (p = 0.03) in stage 2 to stage 3 CKD, respectively. The frequency of ID without anemia also increased with progression of CKD from stage 2 to stage 3, however, the difference was not statistically significant. ID is frequent in patients with early CKD. Monitoring of iron tests and treatment of ID is important in this population of patients.

Arterial wall stiffness is a recognized complication in children with chronic kidney disease (CKD). Vascular abnormalities in these patients are shown to predate cardiac abnormalities such as left ventricular hypertrophy and diastolic dysfunction. The etiology of vascular abnormalities in these patients currently is not clear. This study explored the relationship between various parameters of calcium-phosphorus metabolism including 25-hydroxy vitamin D and arterial wall stiffness in pediatric patients with CKD. This study investigated a cohort of 43 children with CKD who had no history of underlying congenital or structural cardiac disease. The Augmentation Index (AI), a measure of peripheral arterial reflective properties using radial artery tonometry, was used as an indirect measure of central aortic stiffness. Serum biochemical markers of calcium-phosphorus metabolism were simultaneously measured. Univariate testing showed that AI correlated with worsening kidney function. Serum 25-hydroxy vitamin D levels were low and correlated negatively with AI (r = -0.39; p < 0.05). Multiple regression analysis showed that 25-hydroxy vitamin D was the only significant independent predictor of increased central arterial stiffness in the subgroup of children receiving hemodialysis. No association was observed between AI and any other measured biochemical parameter of calcium-phosphorus metabolism. This is the first study to investigate pediatric patients with CKD that suggests an association between nutritional vitamin D deficiency and increased arterial stiffness in children with CKD. The pathophysiologic mechanisms of vitamin D that regulate increased arterial stiffness need to be integrated further in pediatric CKD patients.

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.

The aim of this study was to characterize clinical features, treatment response, and outcome of idiopathic membranous glomerulonephritis (MGN) in a single-center cohort of children. A retrospective review of biopsy-proven idiopathic MGN in 12 children (mean age 11.9 years) was undertaken. Presentation was nephrotic syndrome (NS) (75%), hematuria/proteinuria (17%), and asymptomatic proteinuria (8%). Ten patients (83%) with NS and nephrotic range proteinuria (NRP) were treated with prednisone, and two patients with non-NRP were not treated with immunosuppressive medications. Steroid response in the treated patients was complete (10%), partial (40%), and absent (50%), respectively. Oral cyclophosphamide was used in seven patients of whom five were steroid resistant, one was steroid dependent, and one was partially responsive. At the mean follow up of 27 months, outcome parameters included an estimated glomerular filtration rate of 128 cc/min per 1.73 m(2), albumin of 4.2 gm/dL, and a urine protein/creatinine ratio of 0.87 [median 0.16 (range 0.02-6.52)]. Remission was complete in 75% of the patients and partial in 17%. One patient (8%) with chronic kidney disease (stage 2) was unresponsive to therapy. Complete remission was significantly associated with the absence of chronic histological changes (p = 0.03). In conclusion, children with NS and/or NRP associated with MGN appear to have a good prognosis when treated with a combination of corticosteroids and cyclophosphamide.

Chronic kidney disease (CKD) is associated with altered calcium-phosphate homeostasis and hyperparathyroidism due to decreased activity of 1alpha-hydroxylase and impaired activation of 25-hydroxyvitamin D3 [25(OH)D3]. In some patients these problems start earlier because of vitamin D deficiency. A retrospective review of patients followed in the chronic renal insufficiency clinic at Children's Hospital of Michigan assessed the prevalence of vitamin D deficiency in CKD stages 2-4 and evaluated the effect of treatment with ergocalciferol on serum parathormone (PTH). Blood levels of 1,25 dihydroxyvitamin D3, 25(OH)D3, and parathormone (PTH) were examined in 57 children (40 boys; mean age 10.6 years). Of 57 subjects, 44 (77.2%) had 25(OH)D3 levelsor =30 ng/ml, with overall mean of 26.4 +/- 14.3 ng/ml. PTH for patients with 25(OH)D3 levels30 ng/ml was 67.84 +/- 29.09 ng/ml and in the remaining patients was elevated, at 120.36 +/- 86.42 ng/ml (p = 0.05). Following ergocalciferol treatment (22), PTH decreased from 122.13 +/- 82.94 ng/ml to 80.14 +/- 59.24 ng/ml (p0.001) over a period of 3 months. We conclude that vitamin D deficiency is common in children with CKD stages 2-4 and is associated with hyperparathyroidism in the presence of normal 1,25 dihydroxyvitamin D3. Its occurrence before significant renal impairment is noteworthy. Early diagnosis and appropriate treatment is emphasized.

Central venous lines (CVL) continue to be the most commonly used vascular access device for children on hemodialysis (HD). Despite their frequent use, little is known regarding the frequency of CVL-related intradialytic complications that could interfere with delivery of effective dialysis. To better assess this, we conducted a cross-sectional study of ten HD centers within the Midwest Pediatric Nephrology Consortium. Vascular access was provided by CVL in 61 of the 83 patients (73%) included. CVL dysfunction (defined as reduced blood flows, need for reversed lines, or frequent intradialytic alarms) occurred in 46% in the prior month. Treatment for suspected clots occurred in 16 patients. Intraluminal tissue plasminogen activator (tPA) was the preferred treatment for a suspected clot. The survey also inquired about the preferred treatment for documented clots, and intraluminal tPA was most preferred, followed by CVL stripping, CVL removal, CVL brushing, and systemic tPA. As for preventative strategies, most HD centers locked the CVL with intraluminal heparin in concentrations ranging from 1,000 to 5,000 U/ml. In conclusion, catheter usage rates and complications were highly prevalent in pediatric HD units in this study. As treatment strategies varied greatly, future prospective studies are needed to determine the effectiveness of each individual therapy.

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 commonly associated with a high calcium-phosphorus product and elevated levels of parathyroid hormone (PTH). We herein review the published reports on calciphylaxis in ESRD patients less than 18 years old and report the case of a patient with severe calciphylaxis who presented with lower extremity pain, muscle tenderness and difficulty in walking. The serum PTH was low, and the calcium-phosphorus product was normal. The diagnosis of calciphylaxis was confirmed by a muscle biopsy. Treatment with low calcium peritoneal dialysate and substitution of calcium-based phosphorus binders with sevelamer (Renagel) was unsuccessful. The patient's clinical condition progressed to extensive soft tissue calcification and ulcerating skin lesions. Nine months after the onset of symptoms, the patient died of cardiopulmonary arrest.

A 17-year-old healthy girl was admitted to our hospital with diffuse abdominal pain and decreased oral intake of about 11 days duration. About a week prior to admission, she had taken naproxen, 250 mg four times a day for 4 days. Physical examination was normal except for diffuse abdominal tenderness on deep palpation. Investigations revealed high serum BUN (42 mg/dl) and creatinine (4.0 mg/dl). Serum electrolytes and complement (C3, C4) levels and urinalysis were normal. Antinuclear-antibody and anti-dsDNA were negative. Kidney biopsy revealed renal papillary necrosis, acute tubular necrosis, and focal interstitial nephritis. A diagnosis of nonoliguric acute renal failure due to naproxen nephrotoxicity was made. She received intravenous hydration, and oral steroids, which was gradually discontinued in 3 months. A follow-up at 4 months revealed normal renal function with a serum creatinine of 1.1 mg/dl, BUN 7 mg/dl, and normal urinalysis. The report highlights a need for caution while using naproxen or any other nonsteroidal anti-inflammatory drugs, even for a short duration.

Although patients with nephrotic syndrome and IgM or C1q mesangial labeling are sometimes said to have a high propensity to develop ESRD, there is controversy as to the specificity of such labeling.