Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Inherited cystic kidney diseases, including autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), are the most common monogenetic causes of end-stage renal disease (ESRD) in children and adults. While ARPKD is a rare and usually severe pediatric disease, the more common ADPKD typically shows a slowly progressive course leading to ESRD in adulthood. At the present time there is no established disease-modifying treatment for either ARPKD or ADPKD. ⋯ Various studies have pointed out that total kidney volume (TKV) is a potential surrogate parameter for disease severity in ADPKD. Recent trials have therefore measured TKV by magnet resonance imaging (MRI) to monitor and to predict disease progression. Here, we discuss novel insights on polycystic kidney disease (PKD), the value of MRI, and the measurement of TKV in the diagnosis and follow-up of PKD, as well as novel emerging therapeutic strategies for ADPKD.
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Early acute kidney injury (AKI) diagnosis in critically ill children has been an important recent research focus because of the known association of AKI with poor outcomes and the requirement of early intervention to mitigate negative effects of AKI. In children having surgery, the preoperative period offers a unique opportunity to predict postoperative acute kidney injury (AKI), well before AKI occurs. Pediatric AKI epidemiologic studies have begun to identify which preoperative factors may predict development of postoperative cardiac surgery. ⋯ Developing risk scores requires rigorous methodology and validation before widespread use. There is little information currently on the use of preoperative biological or physiological biomarkers to predict postoperative AKI, representing an important area of future research. This review will provide an overview of methodology of preoperative risk score development, discuss pediatric-specific issues around deriving such risk scores, including the combination of preoperative clinical and biologic biomarkers for AKI prediction, and suggest future research avenues.
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Multicenter Study Observational Study
Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium.
Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown. ⋯ Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.
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The aim of this study was to investigate the association between the occurrence of acute kidney injury (AKI) according to pediatric RIFLE (pRIFLE) criteria and adverse outcomes in children after heart surgery. ⋯ The occurrence of AKI according to pRIFLE criteria is associated to adverse outcomes in children after heart surgery.
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End-stage renal disease (ESRD) during infancy has been associated with poor short-term neurocognitive outcomes. Limited information exists regarding long-term outcomes. ⋯ In summary, patients diagnosed with ESRD as infants had intellectual and metacognitive functioning significantly lower than sibling controls. Fewer months on dialysis and younger age at transplant were associated with better outcomes.