Article
Renal allograft survival according to primary diagnosis: a report of the North American Pediatric Renal Transplant Cooperative Study.
Department of Pediatrics, University of Minnesota Medical School, Mineapolis, USA.
Pediatric Nephrology (impact factor:
2.52).
01/1996;
9(6):679-84.
DOI:10.1007/BF00868709
pp.679-84
Source: PubMed
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Citations (0)
- Cited In (7)
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Article: Familial hematuria.
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ABSTRACT: Hematuria is a common presenting complaint in pediatric nephrology clinics and often has a familial basis. This teaching article provides an overview of causes, diagnosis, and management of the major forms of familial hematuria, Alport syndrome, and thin basement membrane nephropathy.Pediatric Nephrology 11/2007; 24(10):1951-8. · 2.52 Impact Factor -
Article: Treatment of Alport syndrome: beyond animal models.
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ABSTRACT: Alport syndrome (AS) is a hereditary glomerulopathy due to abnormal composition of the glomerular basement membrane, leading to end-stage renal disease (ESRD). Studies of animal models of AS have suggested a variety of potentially effective therapies, but none of these has been definitely shown to prevent or delay ESRD in human AS. Studies in Alport mice suggest that angiotensin inhibition not only has antiproteinuric effects but suppresses cytokine and collagen production as well as tubulointerstitial fibrogenesis and inflammation. For these reasons, many Alport patients are treated empirically with angiotensin antagonists. Cyclosporine may reduce proteinuria in AS, but the risk of nephrotoxic side effects complicates long-term therapy in children. Current data on the role of HMG-CoA reductase inhibition are sparse, so therapy should be limited to adults with dyslipoproteinemia. Results of some, but not all, studies suggest that bone marrow-derived cells may ameliorate disease in Alport mice. However, until experimental doubts concerning the superiority of bone-marrow transplantation over other treatments are resolved by additional investigation, human research subjects should not be exposed to cell-based therapies that may carry substantial risks. In summary, all potential therapies are off-label use in children. As a consequence, initial therapeutic trials should focus on the safety and efficiency of medical treatment, as well as the optimal timing of therapy.Kidney International 07/2009; 76(6):599-603. · 6.61 Impact Factor -
Article: Cystinosis: practical tools for diagnosis and treatment.
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ABSTRACT: Cystinosis is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. In older patients cystinosis can mimic idiopathic nephrotic syndrome due to focal and segmental glomerulosclerosis. Measuring elevated white blood cell cystine content is the corner stone for the diagnosis. The diagnosis is confirmed by molecular analysis of the cystinosin gene. Corneal cystine crystals are invariably present in all patients with cystinosis after the age of 1 year. Treatment with the cystine depleting drug cysteamine should be initiated as soon as possible and continued lifelong to prolong renal function survival and protect extra-renal organs. This educational feature provides practical tools for the diagnosis and treatment of cystinosis.Pediatric Nephrology 02/2011; 26(2):205-15. · 2.52 Impact Factor
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Keywords
cadaver allograft recipients
cold ischemia time
data base
diagnostic groups
donor age
familial nephritis
focal segmental glomerulosclerosis
glomerulonephritis
graft failure
hemolytic uremic syndrome
highest failure rates
highest graft failure rates
highest risks
lowest graft failure rates
North American Pediatric Renal Transplant Cooperative Study
primary diagnoses
primary diagnosis
prior transplantation
renal transplantation
structural anomalies