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
|