Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.
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Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). ⋯ Methionine synthase deficiency is very rare and characterized by megaloblastic anemia and neurological symptoms. We report the second case of MSD associated to TMA previously diagnosed as aHUS in which the patient had a poor response to eculizumab.
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In infants, oliguria is defined as a urine output of <1.5 mL/kg/h. The aim of our study was to assess the impact of oliguria on urinary neutrophil gelatinase-associated lipocalin (NGAL) and serum cystatin C (CysC) levels in very-low-birth-weight infants (VLBWIs) with a normal serum creatinine (Cr) level. ⋯ Urinary NGAL level and urinary NGAL/Cr ratio were more sensitive markers for the presence of oliguria in VLBWIs with normal serum Cr levels than serum CysC level.
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Low case volume has been associated with worse survival outcomes in solid organ transplantation. Our aim was to analyze wait-list outcomes in conjunction with posttransplant outcomes. ⋯ Pediatric transplant candidates are listed at low-volume transplant centers are transplanted less frequently and have a much greater risk of dying while on the wait list. Further studies are needed to elucidate the reasons behind the significant outcome differences.
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Observational Study
Regional citrate anticoagulation for continuous renal replacement therapy in children.
Anticoagulation of the continuous renal replacement therapy (CRRT) circuit is an important technical aspect of this medical procedure. Most studies evaluating the efficacy and safety of citrate use have been carried out in adults, and little evidence is available for the pediatric patient population. The aim of this study was to compare regional citrate anticoagulation versus systemic heparin anticoagulation in terms of the lifetime of hemofilters in a pediatric population receiving CRRT at a pediatric center in Bogota, Colombia. ⋯ Regional citrate anticoagulation could be more effective than heparin systemic anticoagulation in terms of prolonging the hemofilter lifetime in patients with acute renal injury who require CRRT.