Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Hyponatremia is the most common electrolyte abnormality encountered in children. In the past decade, new advances have been made in understanding the pathogenesis of hyponatremic encephalopathy and in its prevention and treatment. Recent data have determined that hyponatremia is a more serious condition than previously believed. ⋯ It has also become apparent that both children and adult patients are dying from symptomatic hyponatremia due to inadequate therapy. We have proposed the use of intermittent intravenous bolus therapy with 3% sodium chloride, 2 cc/kg with a maximum of 100 cc, to rapidly reverse CNS symptoms and at the same time avoid the possibility of overcorrection of hyponatremia. In this review, we discuss how to recognize patients at risk for inadvertent overcorrection of hyponatremia and what measures should taken to prevent this, including the judicious use of 1-desamino-8d-arginine vasopressin (dDAVP).
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Recent years has seen an increasing use of regional citrate anticoagulation in pediatric dialysis. Several approaches have been described for monitoring anticoagulation in the extracorporeal circuit, such as serum citrate levels, post-filter ionized calcium (iCa), and activated coagulation time (ACT). However, no standard recommendations have yet been established for applying any of these parameters, especially for iCa. ⋯ Our citrate protocol [citrate 3% rate (ml/h) approximately blood flow rate (ml/min) x 2] meets the established criteria with a high sensitivity. Based on these results, we conclude that the post-filter iCa level can be reliably used for the management of extracorporeal anticoagulation with citrate in pediatric HD. We recommend the application of our citrate prescription protocol in the setting of pediatric intermittent hemodialysis.
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Although most patients with idiopathic nephrotic syndrome (NS) respond to steroid treatment, development of steroid dependency may require a long-term multidrug therapy including steroid and calcineurin inhibitor. Rituximab was shown to allow a reduction of the doses of steroid and immunosuppressive drugs in those patients. In the present series, 22 patients with steroid-sensitive, but steroid-dependent nephrotic syndrome were treated with rituximab. ⋯ Rituximab failed in 2 patients and 1 refused any additional treatment, despite B cell recovery and relapse. Toxicity of rituximab was limited to reversible cytokine shock in 2 patients and reversible neutropenia in 1 patient. No severe infection was observed.
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Case Reports
Development of renal and iliac aneurysms in a child with generalized infantile myofibromatosis.
Infantile myofibromatosis is a rare disorder characterized by the formation of tumors in the skin, soft tissues, bone, and viscera. We report the case of a 3-week-old girl who presented with severe hypertension due to generalized infantile myofibromatosis including renal involvement. ⋯ However, her evolution was marked by the development of aneurismal dilations of the renal and iliac arteries as observed in fibromuscular dysplasia. We discuss the possibility of a link between these two mesenchymal disorders.
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Recent data suggest that elevated levels of uric acid (UA) might contribute to the progression of renal disease. Rasburicase, recombinant urate oxidase, is a highly safe and efficacious hypo-uricosuric agent for treatment of elevated UA levels from tumor lysis. We adopted the use of rasburicase for management of hyperuricemia in infants with acute kidney injury (AKI) and, herein, report our experience. ⋯ We observed no treatment-related side effects. All patients demonstrated a normalization of uric acid level without need of renal replacement therapy. In conclusion, a single intravenously administered bolus of rasburicase appears to be a novel treatment for hyperuricemia in infants with AKI.