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      Renal Function in Children with Cyanotic Congenital Heart Disease: Pre- and Post-Cardiac Surgery Evaluation

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          Abstract

          Objective: Cyanotic congenital heart diseases (CCHDs) are a series of cardiac anomalies that have long been recognized as a potential cause of nephropathy. There have been few reports on renal impairment in patients with CCHD before and after corrective cardiac surgery. The aim of this study was to evaluate the prevalence of renal dysfunction before and after cardiac surgery and the impact of some risk factors on final renal outcome.

          Methods: Thirty children with CCHD who had done corrective cardiac surgery in the previous 6 months were enrolled in this study. All data prior to surgery were collected from the charts. Post-operation data including blood and spot urine samples were taken simultaneously for CBC, Cr, and uric acid and 24 hour urine was collected for microalbumin and Cr during the follow up visits. Pre- and post-operation parameters were compared to study the impact of cardiac surgery on renal function.

          Findings : Pre- and post-operative GFRs were not significantly different. Final GFR was significantly and inversely associated with pre- and post-operation age ( P=0.008 r=-0.48, P=0.03 r=-0.38). Three (10%) patients had microalbuminuria. The prevalence of microalbuminuria in children older than 10 years was 30%. There was no link between microalbuminuria and age, GFR, and hematocrit ( P=0.1, P=0.3, P=0.3, respectively). Patients with preoperation hematocrit >45 had a significantly lower final GFR compared to children with HCT <45 (83.7±6.5 vs 111.10.2, P=0.001). The mean uric acid fraction (FEua) excretion was 8.21±4.75. Pre-operative HCT was inversely associated to FEua ( P=0.01, r=-0.44). There was no relationship between FEua and age, serum uric acid, and GFR ( P=0.7, P=0.4, P=0.2).

          Conclusion: Children with CCHD are at increased risk of renal injury which is related more to the duration of cyanosis and higher degree of hematocrit level. To lower the risk, corrective cardiac surgery is recommended to be done as soon as possible to improve renal function and stop more renal impairment.

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          Glomerular hypertrophy in minimal change disease predicts subsequent progression to focal glomerular sclerosis.

          The study sought a diagnostic clue to identify the group of pediatric patients with apparent minimal change disease who subsequently develop focal glomerular sclerosis (FGS). Review of all renal biopsy material at our institutions identified 42 pediatric patients who met the standard criteria for minimal change disease (MCD) on initial biopsies. Of those, 10 deteriorated clinically and on rebiopsy showed focal glomerular sclerosis (FGS). The initial renal biopsies of these 10 patients were analyzed morphometrically to determine the mean glomerular tuft area (GA). The results were compared to those of the remaining 32 patients whose subsequent benign clinical course was consistent with MCD, and to randomly selected, age-matched autopsy controls without renal disease (CONT, N = 10). The mean age was comparable among the three groups studied. Separate groups of adult (N = 12) and pediatric (N = 18) patients with initial biopsies with FGS were also studied. The initial biopsy of pediatric patients who subsequently showed FGS (rebiopsy performed on average 3.3 years later) had an average GA of 13.5 x 10(-3) mm2, 76% larger than glomeruli from children with MCD (7.7 x 10(-3) mm2, P less than 0.0005) and 62% larger than CONT (8.4 x 10(-3) mm2, P less than 0.005). Patients with FGS on initial biopsy, whether adult or pediatric, also had significantly larger GA than the age-matched MCD or CONT groups. Evaluation of GA in all the 42 pediatric biopsies with initial MCD further showed that in 23 patients GA was equal to or smaller than the CONT average.(ABSTRACT TRUNCATED AT 250 WORDS)
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            Renal function and injury in infants and young children with congenital heart disease.

            The aim of this study was to investigate renal function and injury in infants and young children with congenital heart disease (CHD).
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              Mechanisms of development and progression of cyanotic nephropathy.

              Cyanotic nephropathy (CN) is often accompanied by congenital cyanotic heart diseases (CCHD). The purpose of this study was to clarify the risk factors and the mechanisms of involved in the development and progression of CN. Thirty patients with CCHD were examined. We analyzed the risk factors for the development of CN on the basis of the clinical and laboratory findings. We also examined ten renal biopsy specimens obtained from patients with CN. Patients with CN showed significantly higher hematocrit levels than those without CN (P=0.025), although there was no difference between the two groups in terms of oxygen saturation. The renal plasma flow (RPF) in patients both with and without CN was low. However, the filtration fraction (FF) was significantly lower in patients with CN than in those without CN (P=0.001). The glomeruli of biopsy specimens with significant proteinuria (n=7) were larger than those of biopsy specimens without significant proteinuria, and there were more capillaries per glomerulus in the former than in the latter (n=3) and the control specimens (n=6) (glomerular size: P<0.01; number of glomerular capillaries: P<0.01). In conclusion, hyperviscosity by polycythemia may be responsible for the development of CN. This pathological condition may induce an angiogenic increase in the glomerular capillary beds, in turn leading to glomerulomegaly. In addition, the failure of a compensatory mechanism to respond to reduced RPF by hyperfiltration may be accompanied by the development and progression of CN.
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                Author and article information

                Journal
                Iran J Pediatr
                Iran J Pediatr
                IJPD
                Iranian Journal of Pediatrics
                Tehran University of Medical Sciences (Tehran, Iran )
                2008-2142
                2008-2150
                February 2014
                25 January 2014
                : 24
                : 1
                : 81-86
                Affiliations
                [1 ]Department of Pediatric Cardiology, Nemazee Hospital
                [2 ]Shiraz Nephrology and Urology Research Center
                [3 ]Department of Pediatrics, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
                Author notes
                [* ]Corresponding Author: Address: Pediatric Nephrology Ward, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz-Iran m_basiratnia@ 123456yahoo.com
                Article
                IJPD-24-81
                4359609
                af6a2ef3-8107-40c1-970f-a40670d2f3a2
                Copyright © 2014 by Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, All rights reserved

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 6 July 2013
                : 11 January 2014
                Categories
                Original Article

                Pediatrics
                congenital heart disease,nephropathy,cardiac surgery,cyanosis,microalbuminuria
                Pediatrics
                congenital heart disease, nephropathy, cardiac surgery, cyanosis, microalbuminuria

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