Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Case Reports
Idiopathic membranous nephropathy in children treated with rituximab: report of two cases.
Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. ⋯ Rituximab seems to be a safe and effective treatment option in children with idiopathic membranous nephropathy due to anti-PLA2R. Further studies are needed to evaluate this effectiveness.
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Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. ⋯ To the best of our knowledge, our patient is the first to have Cbl C defect-HUS accompanied by complement dysregulation, who responded well to eculizumab therapy.
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Fluid overload (FO) is common after neonatal congenital heart surgery and may contribute to mortality and morbidity. It is unclear if the effects of FO are independent of acute kidney injury (AKI). ⋯ Fluid overload is an important independent contributor to outcomes in neonates following congenital heart surgery. Careful fluid management after cardiac surgery in neonates with and without AKI is warranted.
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Morbidity and mortality with necrotizing enterocolitis (NEC) remains a significant challenge. Acute kidney injury (AKI) has been shown to worsen survival in critically ill neonates. To our knowledge, this study is the first to evaluate the prevalence of AKI and its impact on outcomes in neonatal NEC. ⋯ This study shows that AKI not only occurs in over half of patients with NEC, but that it is also associated with more than a two-fold higher mortality, highlighting the importance of early recognition and potentially early intervention for AKI.
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Acute kidney injury (AKI) is a common complication of cardiopulmonary bypass surgery (CPB) in children. Several promising postoperative AKI biomarkers have been identified, but no preoperative biomarkers are available. We evaluated the association of urinary uromodulin (uUMOD) with postoperative AKI. ⋯ Children with lowest preoperative levels of uUMOD have greatly increased risk of AKI post-CPB. If uUMOD were used to risk-stratify patients undergoing CPB, clinical measures could be taken to minimize AKI development.