Francesco Emma

Author PubWeight™ 77.55‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int 2009 7.45
2 The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int 2009 7.19
3 Validation of the Oxford classification of IgA nephropathy in cohorts with different presentations and treatments. Kidney Int 2014 3.40
4 COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement. J Am Soc Nephrol 2007 2.52
5 Growth hormone treatment started in the first year of life in infants with chronic renal failure. Pediatr Nephrol 2009 2.21
6 The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int 2010 2.14
7 Cranioectodermal Dysplasia, Sensenbrenner syndrome, is a ciliopathy caused by mutations in the IFT122 gene. Am J Hum Genet 2010 1.86
8 Eculizumab for the treatment of dense-deposit disease. N Engl J Med 2012 1.76
9 Short-term survival of hyperammonemic neonates treated with dialysis. Pediatr Nephrol 2014 1.54
10 MKS3/TMEM67 mutations are a major cause of COACH Syndrome, a Joubert Syndrome related disorder with liver involvement. Hum Mutat 2009 1.43
11 Inflammatory response to cardiac bypass in ewe fetuses: effects of steroid administration or continuous hemodiafiltration. J Thorac Cardiovasc Surg 2003 1.38
12 Broadening the spectrum of diseases related to podocin mutations. J Am Soc Nephrol 2003 1.33
13 The pathogenesis of cystinosis: mechanisms beyond cystine accumulation. Am J Physiol Renal Physiol 2010 1.25
14 Genetic screening in adolescents with steroid-resistant nephrotic syndrome. Kidney Int 2013 1.21
15 Cyclosporine in patients with steroid-resistant nephrotic syndrome: an open-label, nonrandomized, retrospective study. Clin Ther 2004 1.21
16 Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome. J Am Soc Nephrol 2014 1.20
17 Long-term outcome of nephropathic cystinosis: a 20-year single-center experience. Pediatr Nephrol 2010 1.17
18 Recurrence of focal segmental glomerulosclerosis after renal transplantation in patients with mutations of podocin. Am J Kidney Dis 2003 1.13
19 Improvement in the renal prognosis in nephropathic cystinosis. Clin J Am Soc Nephrol 2011 1.10
20 A patient with cubilin deficiency. N Engl J Med 2011 1.10
21 Stem cell microvesicles transfer cystinosin to human cystinotic cells and reduce cystine accumulation in vitro. PLoS One 2012 1.07
22 Phenotypic and genetic heterogeneity in Dent's disease--the results of an Italian collaborative study. Nephrol Dial Transplant 2006 1.07
23 Risk factors for cyclosporin A nephrotoxicity in children with steroid-dependant nephrotic syndrome. Clin J Am Soc Nephrol 2009 1.02
24 TRPC6 mutations in children with steroid-resistant nephrotic syndrome and atypical phenotype. Clin J Am Soc Nephrol 2011 0.97
25 Collapsing glomerulopathy associated with inherited mitochondrial injury. Kidney Int 2008 0.97
26 Cysteamine toxicity in patients with cystinosis. J Pediatr 2011 0.94
27 Identification and subcellular localization of a new cystinosin isoform. Am J Physiol Renal Physiol 2008 0.92
28 Serum creatinine levels are significantly influenced by renal size in the normal pediatric population. Clin J Am Soc Nephrol 2010 0.92
29 Clinical features and long-term outcome of nephrotic syndrome associated with heterozygous NPHS1 and NPHS2 mutations. Clin J Am Soc Nephrol 2009 0.92
30 Impaired activity of the gamma-glutamyl cycle in nephropathic cystinosis fibroblasts. Pediatr Res 2006 0.91
31 Sensenbrenner syndrome: a new member of the hepatorenal fibrocystic family. Am J Med Genet A 2006 0.90
32 Calcium-sensing receptor and aquaporin 2 interplay in hypercalciuria-associated renal concentrating defect in humans. An in vivo and in vitro study. PLoS One 2012 0.90
33 Genetic risk factors in typical haemolytic uraemic syndrome. Nephrol Dial Transplant 2008 0.90
34 Type IV Bartter syndrome: report of two new cases. Pediatr Nephrol 2006 0.90
35 Post-transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation. Pediatr Transplant 2013 0.89
36 Lupus nephritis in children and adolescents: results of the Italian Collaborative Study. Nephrol Dial Transplant 2013 0.88
37 Analysis of CTNS gene transcripts in nephropathic cystinosis. Pediatr Nephrol 2010 0.88
38 Idiopathic membranous nephropathy associated with polycystic kidney disease. Pediatr Nephrol 2009 0.87
39 A spectrum of LMX1B mutations in Nail-Patella syndrome: new point mutations, deletion, and evidence of mosaicism in unaffected parents. Genet Med 2010 0.86
40 A locus for familial skewed X chromosome inactivation maps to chromosome Xq25 in a family with a female manifesting Lowe syndrome. J Hum Genet 2006 0.85
41 Distribution of cystinosin-LKG in human tissues. Histochem Cell Biol 2012 0.84
42 Locus heterogeneity of Dent's disease: OCRL1 and TMEM27 genes in patients with no CLCN5 mutations. Pediatr Nephrol 2009 0.84
43 Cyclosporine A treatment in patients with Alport syndrome: a single-center experience. Pediatr Nephrol 2010 0.84
44 Inhibition of B-cell proliferation and antibody production by mesenchymal stromal cells is mediated by T cells. Stem Cells Dev 2015 0.84
45 Time for initial response to steroids is a major prognostic factor in idiopathic nephrotic syndrome. J Pediatr 2010 0.84
46 Modulation of CTNS gene expression by intracellular thiols. Free Radic Biol Med 2010 0.83
47 One-year angiotensin-converting enzyme inhibition plus mycophenolate mofetil immunosuppression in the course of early IgA nephropathy: a multicenter, randomised, controlled study. J Nephrol 2005 0.83
48 Induction of regulatory T cells after prophylactic treatment with photopheresis in renal transplant recipients. Transplantation 2007 0.83
49 Development of a score based on urinalysis to improve the management of urinary tract infection in children. Clin Chim Acta 2011 0.82
50 A novel interstitial deletion in Xq25, identified by array-CGH in a patient with Lowe syndrome. Eur J Med Genet 2006 0.82
51 Effect of carnitine supplementation on lipid profile and anemia in children on chronic dialysis. Pediatr Nephrol 2007 0.81
52 Association of the macrophage migration inhibitory factor -173*C allele with childhood nephrotic syndrome. Pediatr Nephrol 2008 0.81
53 Inappropriate left ventricular mass in children and young adults with chronic renal insufficiency. Pediatr Nephrol 2009 0.80
54 Lack of cardiac anomalies in children with NPHS2 mutations. Nephrol Dial Transplant 2007 0.80
55 Severe syncope and sudden death in children with inborn salt-losing hypokalaemic tubulopathies. Nephrol Dial Transplant 2005 0.79
56 Rare functional variants of podocin (NPHS2) promoter in patients with nephrotic syndrome. Gene Expr 2006 0.79
57 Angiotensin-converting enzyme (ACE) haplotypes and cyclosporine A (CsA) response: a model of the complex relationship between ACE quantitative trait locus and pathological phenotypes. Hum Mol Genet 2005 0.79
58 Stop codon at arginine 586 is the prevalent nephronopthisis type 1 mutation in Italy. Nephrol Dial Transplant 2006 0.79
59 Detailed investigations of proximal tubular function in Imerslund-Gräsbeck syndrome. BMC Med Genet 2013 0.77
60 Suggested revision of the National High Blood Pressure Education Program blood pressure standardization for use in severely growth retarded children. Pediatr Nephrol 2010 0.77
61 Permissive hypofiltration: an alternative view. Crit Care 2012 0.77
62 Prognostic value of glomerular collagen IV immunofluorescence studies in male patients with X-linked Alport syndrome. Clin J Am Soc Nephrol 2013 0.76
63 Copper deficiency in patients with cystinosis with cysteamine toxicity. J Pediatr 2013 0.76
64 The ERA-EDTA Working Group on inherited kidney disorders. Nephrol Dial Transplant 2012 0.76
65 Usefulness of skin immunofluorescence for distinguishing SLE from SLE-like renal lesions: a pilot study. Pediatr Nephrol 2010 0.76
66 Long-term cardiovascular effects of pre-transplant native kidney nephrectomy in children. Pediatr Nephrol 2010 0.75
67 Gender-related effects on urine L-cystine metastability. Amino Acids 2013 0.75
68 Glomerulocystic kidney disease in hypomelanosis of Ito. Pediatr Nephrol 2008 0.75
69 [Cystinuria]. G Ital Nefrol 2010 0.75
70 Combined split liver and kidney transplantation in a three-year-old child with primary hyperoxaluria type 1 and complete thrombosis of the inferior vena cava. Pediatr Transplant 2009 0.75
71 Transcriptional and posttranscriptional regulation of the CTNS gene. Pediatr Res 2011 0.75