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Common causes of pediatric ESRD are distinct from those seen in the adult population. In the pediatric population, the most common are congenital anomalies of the kidney and urinary tract (CAKUT), affecting approximately 30% of children with CKD. These structural anomalies often require coordinated care with the pediatric urology team to address voiding issues, bladder involvement, and the potential need for surgical intervention. For pediatric nephrologists and urologists, common CAKUT that are encountered include antenatal hydronephrosis, obstructive uropathies (eg, posterior urethral valves), and vesicoureteral reflux. As more pediatric patients with CAKUT, CKD, and ESRD transition to adult care, it is important for receiving adult nephrologists to understand the clinical presentation, natural history, and prognosis for these diagnoses. This review outlines the diagnosis and potential interventions for these conditions, including strategies to address bladder dysfunction that is often seen in children with CAKUT. A discussion of these management decisions (including surgical intervention) for CAKUT, which are quite common to pediatric nephrology and urology practices, may provide unique learning opportunities for adult nephrologists who lack familiarity with these pediatric conditions.

Background: Antifungal azoles are the first-line agents used to treat topical and, above all, systemic mycosis. The latter could be life-threating infections in immunocompromised patients. Chemotherapeutic antibiotics, including antifungal azoles, may induce hypersensitivity reactions; however, such immunologic adverse reactions have not been defined and carefully investigated. Objective: The study aims to provide an update on the evaluation and diagnosis of skin allergy to azole antifungal agents. Methods: This is a systematic review performed on PubMed and Google Schoolbarusing using the key terms “allergy, hypersensitivity, anaphylaxis, immediate-type reaction, delayed-type reaction, ketoconazole, fluconazole, posaconazole, voriconazole, itraconazole, triazoles, imidazoles, antifungals, antimycotics”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, reviews and case reports. Results: One hundred twenty-four articles matched our search terms. The most common adverse events reported were T-cell mediated delayed-type hypersensitivity reactions, fixed drug eruptions, exanthematous dermatitis, Steven-Johnson syndrome, toxic epidermal necrolysis and acute generalized exhanthematous pustulosis. Rarely a drug rash with eosinophilia systemic symptoms, has been described. Also, immediate-type reactions such as urticaria-angioedema or anaphylaxis have been reported following the administration of antifungal imidazoles, although not so frequently. Conclusion: Despite their widespread use, triazoles seem to induce rare cutaneous hypersensitivity reactions, but the pathomechanisms, risk factors, diagnostic and management strategies, including skin tests and challenge tests, are little known and poorly investigated.

To describe hypersensitivity reactions in patients receiving maintenance hemodialysis.

The objectives of this study were to explore pediatric undergraduate medical educators' understanding of reflective practice, the barriers they face in teaching this, the curricular activities they use, and the value they assign to reflective practice.

Renal allograft thrombosis is the leading cause of graft failure in pediatric transplant recipients in the early post-transplant period. Various factors at multiple levels- donor, recipient, surgical and therapy-related, come into play in contributing to this risk. While early detection and intervention are keys in limiting the adverse effect of graft thrombosis on graft survival, preventative strategies have the most to offer in reducing morbidity related to this devastating complication. This article reviews the epidemiology, risk factors, diagnostic and therapeutic modalities to assist the clinician in taking care of children who are in the process of receiving renal transplants. The authors also address various strategies to screen for and prevent thromboses in this population. Lastly, we identify gaps in our knowledge base related to allograft thrombosis, to bridge which, robust research is urgently needed.

OBJECTIVES To determine if interventions during the pre–hemolytic uremic syndrome (HUS) diarrhea phase are associated with maintenance of urine output during HUS. DESIGN Prospective observational cohort study. SETTINGS Eleven pediatric hospitals in the United States and Scotland. PARTICIPANTS Children younger than 18 years with diarrhea-associated HUS (hematocrit level <30% with smear evidence of intravascular erythrocyte destruction), thrombocytopenia (platelet count <150 × 103/mm3), and impaired renal function (serum creatinine concentration >> upper limit of reference range for age). INTERVENTIONS Intravenous fluid was given within the first 4 days of the onset of diarrhea. OUTCOME MEASURE Presence or absence of oligoanuria (urine output ≤0.5 mL/kg/h for >1 day). RESULTS The overall oligoanuric rate of the 50 participants was 68%, but was 84% among those who received no intravenous fluids in the first 4 days of illness. The relative risk of oligoanuria when fluids were not given in this interval was 1.6 (95% confidence interval, 1.1-2.4; P = .02). Children with oligoanuric HUS were given less total intravenous fluid (r = −0.32; P = .02) and sodium (r = −0.27; P = .05) in the first 4 days of illness than those without oligoanuria. In multivariable analysis, the most significant covariate was volume infused, but volume and sodium strongly covaried. CONCLUSIONS Intravenous volume expansion is an underused intervention that could decrease the frequency of oligoanuric renal failure in patients at risk of HUS.

Adults receiving preemptive renal transplants have better allograft survival. Our study investigated differences in graft and patient survival based on need for, and duration of, pretransplant dialysis in pediatric renal transplant recipients.

Cast nephropathy is the most common manifestation of renal injury in patients with multiple myeloma but is rarely reported in other conditions. We are reporting our experience in caring for a teenager with a metastatic neuroendocrine carcinoma who developed rapidly progressive kidney injury that advanced to end-stage renal disease. On renal biopsy extensive tubular necrosis and intratubular eosinophilic casts were noted. This previously unreported finding should prompt oncologists to closely monitor for such a complication in patients with secretory tumors. Whether early plasmapheresis could be of benefit, as has been tried in multiple myeloma, remains to be determined.

Renal involvement is the major factor determining the long-term outcome of children with Henoch-Schonlein Purpura (HSP) nephritis. The precise incidence of adverse outcomes is debatable; moreover, there are conflicting data on the validity of prognostic factors for predicting adverse outcome in children with HSP nephritis. Difficulties in prognostication are compounded by lack of non-invasive diagnostic methods in children suspected of having HSP. The purpose of this article is to review recent data evaluating the role of serum galactose-deficient IgA in the pathogenesis of HSP nephritis and its potential role as a non-invasive diagnostic tool. Recent studies evaluating the role of prognostic markers and the risk of adverse long-term outcomes in children with HSP nephritis are also described. HSP nephritis, although relatively benign in most children, can lead to significant chronic kidney disease in a subset of children, especially on very longterm follow-up. Health-care providers need to be aware of this and tailor appropriate follow-up and screening tests to identify early signs of renal functional deterioration, so that therapies that can retard the rate of progression can be instituted early.

Systemic lupus erythematosus (SLE) is a serious medical illness with frequent renal involvement at disease onset. Although predominantly affecting young women, SLE often first presents during childhood. Previous studies have suggested that patients with disease onset in childhood have a worse prognosis. Renal involvement in SLE is the major determinant of long-term outcome; the ten-year survival in children with lupus nephritis was only around 20% in late 60s, but outcomes have improved dramatically in recent years to around 94%. This article will review recent data on new approaches in the treatment of pediatric lupus nephritis by referencing the major studies over the last few years. We will also examine the validity of various factors that have been suggested by previous studies to have prognostic value in determining outcomes.

Intravenous Immunoglobulin (IVIG) has been used since the 1970's for the treatment of a variety of different rheumatologic and infectious diseases. While the therapeutic efficacy of IVIG has been definitively demonstrated in many different disorders, its mechanism(s) of action have remained poorly explained. This is complicated, in part by the difference in pathophysiology of the many diseases it has been used in the treatment of. Regardless of the types of diseases, understanding why transfusing pooled donor antibodies to unscreened patients fails to elicit a detrimental immunologic response, let alone benefit these patients, needs explanation. This review aims to explain how IVIG works by reviewing the medical literature and the relevant basic immunology involved.

Little is known about the incidence and evolution of proteinuria as a complication of sirolimus rescue in children. This study describes pediatric renal transplant (Tx) recipients who were treated with sirolimus and who developed heavy proteinuria. Risk factors for the development of proteinuria and its time course are explored.

Calcineurin-inhibitor nephrotoxicity plays a role in the pathogenesis of chronic allograft nephropathy by causing renal ischemia mediated by vasoconstrictive metabolites of the prostanoid pathway. The purpose of our study was to evaluate whether altering the prostanoid profile using juniper oil (JO) would afford renoprotection in rats treated with tacrolimus.

Background:Proteinuria is an important marker of kidney disease. Simple methods to determine the presence of proteinuria in a semiquantitative fashion require measurement of either a protein-creatinine or protein-osmolality ratio. Methods:Urine samples from 134 healthy infants and children and 150 children from the pediatric nephrology practice were analyzed to develop normative data for protein-osmolality ratios on random urine samples and compare protein-osmolality with protein-creatinine ratio as a predictor of 24-hour urine protein excretion. Children were grouped according to age. Three groups were established: infants (<2 years), younger children (2 to 8 years), and older children (9 to 18 years). An adult cohort was similarly analyzed. Results:For healthy children older than 2 years, the optimal value discriminating normal from abnormal protein excretion was determined to be a protein-osmolality ratio of 0.15 mg · kg H2O/mOsm · L; for children between 2 and 8 years old, 0.14; and for children older than 8 years, 0.17 (P= not significant between age groups). The corresponding optimal cutoff value for protein-creatinine ratio for the entire group of children older than 2 years is 0.20. Area under the curve analysis of receiver operator characteristic curves showed protein-creatinine ratio was superior to protein-osmolality ratio for predicting abnormal amounts of proteinuria in children and adolescents (P< 0.0001). In adults, both ratios are equally accurate. Conclusion:Given the superiority of protein-creatinine ratio in children, it would be appropriate to screen urine samples for proteinuria using protein-creatinine ratio rather than protein-osmolality ratio.

To review and present data on the prevalence, clinical manifestations, diagnostic techniques, and management options in patients with hypersensitivity reactions to corticosteroid preparations.

Long-term renal transplant function is limited primarily by a progressive scarring process loosely termed "chronic rejection, chronic allograft nephropathy, or allograft fibrosis." Although the etiology of transplant fibrosis is uncertain, several possible factors including chronic cyclosporin A (CsA) exposure may contribute to its pathogenesis. CsA stimulates renal fibrosis perhaps through the induction of the potent pro-sclerotic growth factor, transforming growth factor β (TGFβ). Previously, we demonstrated that, in human transplant biopsies, acute CsA toxicity but not acute tubular necrosis is associated with elevated levels of renal TGFβ protein. We now examine whether long-term CsA treatment (>1 year) is associated with elevated levels of intra-allograft TGFβ and whether heightened expression of TGFβ is clinically significant.

Mycophenolate mofetil (MMF) has been shown to be superior to azathioprine in reducing the incidence of acute rejection in adult renal transplant recipients. Although MMF is also being widely used in pediatric transplant patients, data documenting its safety are limited.

Reading this article will enable health care providers to recognize and to diagnose paroxysmal sneezing, sighing dyspnea, habit cough, and vocal cord dysfunction and to reinforce their knowledge of the epidemiology, etiopathology, clinical features, and treatment of these disorders.

Children with acute lymphoblastic leukemia (ALL) are at risk for serious electrolyte abnormalities. The authors report their experience in managing a child with ALL who developed severe hyperphosphatemia as a consequence of a large exogenous load of phosphorus from high-dose liposomal amphotericin B. Health care providers need to recognize this potentially life-threatening complication of liposomal amphotericin B, since early detection and intervention can prevent significant morbidity.

Our report concerns a neonate who was noted to have palpable kidneys soon after birth. Her clinical and radiologic findings were compatible with the diagnosis of autosomal recessive polycystic kidney disease. The patient eventually died of sepsis-related multiorgan failure, and on autopsy, surprisingly, was found to have leukemic infiltrates in the kidney. Healthcare providers need to be aware that congenital acute myelogenous leukemia, although rare, can present in the newborn period with nephromegaly.

Psychogenic polydipsia is a well-described phenomenon in those with a diagnosed psychiatric disorder such as schizophrenia and anxiety disorders. Primary polydipsia is differentiated from psychogenic polydipsia by the lack of a clear psychotic disturbance. We present a case of a 27-month-old boy who presented with polyuria and polydipsia. Laboratory studies, imaging, and an observed water deprivation test were consistent with primary polydipsia. Polydipsia resolved after family limited his fluid intake and began replacing water drinking with other transition objects and behaviors for self-soothing. This case highlights the importance of water deprivation testing to differentiate between causes of polyuria, thereby avoiding misdiagnosis and iatrogenic hyponatremia. Secondly, primary polydipsia can result during the normal stages of child development without overt psychiatric disturbances.