Francesco Emma

Francesco Emma

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Atypical IgM on T cells predict relapse and steroid dependence in idiopathic nephrotic syndrome.

Kidney Int 2019 Oct 7;96(4):971-982. Epub 2019 May 7.

Laboratory of Nephrology, Department of Rare Diseases, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ospedale Pediatrico Bambino Gesù, Rome, Italy; Division of Nephrology, Department of Pediatric Subspecialties, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.

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http://dx.doi.org/10.1016/j.kint.2019.04.006DOI Listing
October 2019

Cardiac Abnormalities in Children with Autosomal Recessive Polycystic Kidney Disease.

Cardiorenal Med 2019 7;9(3):180-189. Epub 2019 Mar 7.

Nephrology and Dialysis Unit, Department of Pediatric Subspecialties, Bambino Gesù Children's Hospital - IRCSS, Rome, Italy.

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http://dx.doi.org/10.1159/000496473DOI Listing
August 2019

Intrinsic Bone Defects in Cystinotic Mice.

Am J Pathol 2019 May 19;189(5):1053-1064. Epub 2019 Feb 19.

Bone Physiopathology Group, Multifactorial Disease and Complex Phenotype Research Area, Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.1016/j.ajpath.2019.01.015DOI Listing
May 2019

Equations to estimate prednisone dose using body weight.

Pediatr Nephrol 2019 Apr 27;34(4):685-688. Epub 2018 Oct 27.

Department of Pediatric Subspecialties, Division of Nephrology, Bambino Gesù Children's Hospital - IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://link.springer.com/10.1007/s00467-018-4127-8
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http://dx.doi.org/10.1007/s00467-018-4127-8DOI Listing
April 2019

B cell phenotype in pediatric idiopathic nephrotic syndrome.

Pediatr Nephrol 2019 01 28;34(1):177-181. Epub 2018 Sep 28.

Laboratory of Nephrology, Department of Rare Diseases, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-018-4095-zDOI Listing
January 2019

Levamisole for children with nephrotic syndrome: new evidence for the use of an "old" drug.

Kidney Int 2019 01;95(1):25-28

Division of Nephrology, Department of Pediatric Subspecialties, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2018.10.008DOI Listing
January 2019

FGF23 blockade coming to clinical practice.

Kidney Int 2018 11;94(5):846-848

Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2018.08.022DOI Listing
November 2018

Impact of atypical mitochondrial cyclic-AMP level in nephropathic cystinosis.

Cell Mol Life Sci 2018 Sep 16;75(18):3411-3422. Epub 2018 Mar 16.

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Policlinico, Piazza G. Cesare, 11, 70124, Bari, Italy.

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http://dx.doi.org/10.1007/s00018-018-2800-5DOI Listing
September 2018

Immunology of idiopathic nephrotic syndrome.

Pediatr Nephrol 2018 04 27;33(4):573-584. Epub 2017 Apr 27.

Division of Nephrology and Dialysis, Ospedale Pediatrico Bambino Gesù-IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://link.springer.com/10.1007/s00467-017-3677-5
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http://dx.doi.org/10.1007/s00467-017-3677-5DOI Listing
April 2018

Phenotypic and functional characterization of mesenchymal stromal cells isolated from pediatric patients with severe idiopathic nephrotic syndrome.

Cytotherapy 2018 03 29;20(3):322-334. Epub 2017 Dec 29.

Department of Pediatric Subspecialties, Division of Nephrology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.jcyt.2017.12.001DOI Listing
March 2018

A randomized clinical trial indicates that levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome.

Kidney Int 2018 02 18;93(2):510-518. Epub 2017 Oct 18.

Department of Pediatric Nephrology, Emma Children's Hospital/Academic Medical Center, Amsterdam, The Netherlands; Department of Pediatric Nephrology, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.

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https://linkinghub.elsevier.com/retrieve/pii/S00852538173060
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http://dx.doi.org/10.1016/j.kint.2017.08.011DOI Listing
February 2018

Oligonephronia and Wolf-Hirschhorn syndrome: A further observation.

Am J Med Genet A 2018 02 28;176(2):409-414. Epub 2017 Nov 28.

Center for Rare Disease and Congenital Defects, Fondazione Policlinico Universitario A. Gemelli, Catholic University, Rome, Italy.

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http://dx.doi.org/10.1002/ajmg.a.38554DOI Listing
February 2018

The 5-phosphatase OCRL in Lowe syndrome and Dent disease 2.

Nat Rev Nephrol 2017 Aug 3;13(8):455-470. Epub 2017 Jul 3.

Institute of Physiology, University of Zurich, Winterthurerstrasse 190, CH-8057, Zurich, Switzerland.

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http://dx.doi.org/10.1038/nrneph.2017.83DOI Listing
August 2017

Outcome of childhood-onset full-house nephropathy.

Nephrol Dial Transplant 2017 Jul;32(7):1194-1204

Division of Nephrology and Dialysis, Children's Hospital Bambino Gesù, IRCCS, Rome, Italy.

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http://dx.doi.org/10.1093/ndt/gfw230DOI Listing
July 2017

Mitochondrial cytopathies and the kidney.

Nephrol Ther 2017 Apr;13 Suppl 1:S23-S28

Clinical Genetics Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.nephro.2017.01.014DOI Listing
April 2017

Minimal Change Disease.

Clin J Am Soc Nephrol 2017 02 9;12(2):332-345. Epub 2016 Dec 9.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; and.

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http://dx.doi.org/10.2215/CJN.05000516DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5293332PMC
February 2017

Ofatumumab in two pediatric nephrotic syndrome patients allergic to rituximab.

Pediatr Nephrol 2017 01 29;32(1):181-184. Epub 2016 Sep 29.

Division of Nephrology, Dialysis, and Transplantation, IRCCS-Istituto Giannina Gaslini, Genoa, Italy.

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http://dx.doi.org/10.1007/s00467-016-3498-yDOI Listing
January 2017

Cystinosin-LKG rescues cystine accumulation and decreases apoptosis rate in cystinotic proximal tubular epithelial cells.

Pediatr Res 2017 01 22;81(1-1):113-119. Epub 2016 Sep 22.

Department of Nephrology and Urology, Division of Nephrology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1038/pr.2016.184DOI Listing
January 2017

Nephrotic-Range Proteinuria and Peripheral Edema in a Child: Not Only Idiopathic Nephrotic Syndrome.

Case Rep Nephrol Dial 2016 Sep-Dec;6(3):120-127. Epub 2016 Nov 1.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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http://dx.doi.org/10.1159/000449423DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5121544PMC
November 2016

Skeletal implications and management of cystinosis: three case reports and literature review.

Bonekey Rep 2016 17;5:828. Epub 2016 Aug 17.

INSERM, Faculté de Médecine Lyon Est, site Laennec, Lyon, France; Université de Lyon, Lyon, France.

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http://dx.doi.org/10.1038/bonekey.2016.55DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4988218PMC
August 2016

Cellular immune profile of kidney transplant patients developing anti-HLA antibodies during childhood.

Pediatr Nephrol 2016 Jun 21;31(6):1001-10. Epub 2015 Dec 21.

University Department of Pediatrics, DPUO, Unit of Immune and Infectious Diseases, Bambino Gesù Children's Hospital and Research Institute (IRCCS), Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-015-3274-4DOI Listing
June 2016

Controversies and research agenda in nephropathic cystinosis: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

Kidney Int 2016 06;89(6):1192-203

Department of Pediatric Nephrology, University Hospitals Leuven, Belgium; Department of Development and Regeneration, Katholieke Universiteit Leuven, Belgium. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2016.01.033DOI Listing
June 2016

Mitochondrial dysfunction in inherited renal disease and acute kidney injury.

Nat Rev Nephrol 2016 May 25;12(5):267-80. Epub 2016 Jan 25.

Clinical Genetics Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy.

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http://dx.doi.org/10.1038/nrneph.2015.214DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5469549PMC
May 2016

Continuous renal replacement therapy in children: fluid overload does not always predict mortality.

Pediatr Nephrol 2016 Apr 12;31(4):651-9. Epub 2015 Nov 12.

Department of Nephrology and Urology, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-015-3248-6DOI Listing
April 2016

Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome.

Mol Immunol 2016 Mar 16;71:131-142. Epub 2016 Feb 16.

IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Ranica, Bergamo, Italy; Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy; Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy.

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http://dx.doi.org/10.1016/j.molimm.2016.01.010DOI Listing
March 2016

Mutation Update of the CLCN5 Gene Responsible for Dent Disease 1.

Hum Mutat 2015 Aug 11;36(8):743-52. Epub 2015 Jun 11.

Assistance Publique-Hôpitaux de Paris, Service de Génétique, Hôpital Européen Georges Pompidou, Paris, France.

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http://dx.doi.org/10.1002/humu.22804DOI Listing
August 2015

Short-term survival of hyperammonemic neonates treated with dialysis.

Pediatr Nephrol 2015 May 4;30(5):839-47. Epub 2014 Sep 4.

Nephrology and Dialysis Unit, Department of Nephrology-Urology, "Bambino Gesù" Children's Hospital-IRCCS, Rome, Italy,

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http://dx.doi.org/10.1007/s00467-014-2945-xDOI Listing
May 2015

Genetic homogeneity but IgG subclass-dependent clinical variability of alloimmune membranous nephropathy with anti-neutral endopeptidase antibodies.

Kidney Int 2015 Mar 7;87(3):602-9. Epub 2015 Jan 7.

1] Sorbonne Universités, UPMC Univ Paris 06, UMR_S 1155, Paris, France [2] INSERM, UMR_S 1155, Hôpital Tenon, Paris, France.

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http://dx.doi.org/10.1038/ki.2014.381DOI Listing
March 2015

Acute thiamine deficiency and refeeding syndrome: Similar findings but different pathogenesis.

Nutrition 2014 Jul-Aug;30(7-8):948-52. Epub 2014 Mar 13.

Division of Metabolism and Research Unit of Metabolic Biochemistry, Department of Pediatric Medicine, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://search.proquest.com/openview/d35d7f422a0899cd6bd704be
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http://linkinghub.elsevier.com/retrieve/pii/S089990071400124
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http://dx.doi.org/10.1016/j.nut.2014.02.019DOI Listing
February 2015

Sialylation of N-linked glycans influences the immunomodulatory effects of IgM on T cells.

J Immunol 2015 Jan 24;194(1):151-7. Epub 2014 Nov 24.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital-Scientific Institute, 00165 Rome, Italy;

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http://dx.doi.org/10.4049/jimmunol.1402025DOI Listing
January 2015

Treatment of C3 glomerulopathy with complement blockers.

Semin Thromb Hemost 2014 Jun 5;40(4):472-7. Epub 2014 May 5.

Division of Nephrology and Dialysis, Children's Hospital Bambino Gesù, IRCCS, Rome, Italy.

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1375299
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http://dx.doi.org/10.1055/s-0034-1375299DOI Listing
June 2014

Clinical utility gene card for: cystinosis.

Eur J Hum Genet 2014 May 18;22(5). Epub 2013 Sep 18.

1] Inserm U983, Institut Imagine, Université Paris Descartes, Paris, France [2] Department of Genetics, Assistance Publique, Hôpitaux de Paris, Necker Hospital, Paris, France.

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http://dx.doi.org/10.1038/ejhg.2013.204DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3992566PMC
May 2014

Gender-related effects on urine L-cystine metastability.

Amino Acids 2014 Feb 11;46(2):415-27. Epub 2013 Dec 11.

Gene Expression-Microarrays Laboratory, Bambino Gesù Children's Hospital - IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy,

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http://dx.doi.org/10.1007/s00726-013-1631-9DOI Listing
February 2014

Post-transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation.

Pediatr Transplant 2013 Dec 30;17(8):E177-81. Epub 2013 Sep 30.

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://doi.wiley.com/10.1111/petr.12151
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http://dx.doi.org/10.1111/petr.12151DOI Listing
December 2013

Lupus nephritis in children and adolescents: results of the Italian Collaborative Study.

Nephrol Dial Transplant 2013 Jun 22;28(6):1487-96. Epub 2013 Jan 22.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1093/ndt/gfs589DOI Listing
June 2013

Prognostic value of glomerular collagen IV immunofluorescence studies in male patients with X-linked Alport syndrome.

Clin J Am Soc Nephrol 2013 May 31;8(5):749-55. Epub 2013 Jan 31.

Nephrology and Dialysis Unit, Department of Nephrology and Urology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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http://dx.doi.org/10.2215/CJN.07510712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3641614PMC
May 2013

Distribution of cystinosin-LKG in human tissues.

Histochem Cell Biol 2012 Aug 29;138(2):351-63. Epub 2012 Apr 29.

Division of Nephrology, Department of Nephrology and Urology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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http://link.springer.com/content/pdf/10.1007/s00418-012-0958
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http://link.springer.com/10.1007/s00418-012-0958-8
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http://dx.doi.org/10.1007/s00418-012-0958-8DOI Listing
August 2012

Renal involvement in mitochondrial cytopathies.

Pediatr Nephrol 2012 Apr 9;27(4):539-50. Epub 2011 Jun 9.

Division of Nephrology and Dialysis, Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, piazza Sant'Onofrio 4, 00165 Rome, Italy.

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http://ndt.oxfordjournals.org/content/11/5/762.full.pdf
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http://link.springer.com/content/pdf/10.1007/BF00866789.pdf
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http://link.springer.com/10.1007/s00467-011-1926-6
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http://dx.doi.org/10.1007/s00467-011-1926-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3288375PMC
April 2012

Development of a score based on urinalysis to improve the management of urinary tract infection in children.

Clin Chim Acta 2012 Feb 19;413(3-4):478-82. Epub 2011 Nov 19.

Department of Laboratory Medicine, Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.1016/j.cca.2011.11.005DOI Listing
February 2012

Cysteamine toxicity in patients with cystinosis.

J Pediatr 2011 Dec 23;159(6):1004-11. Epub 2011 Jul 23.

Department of Pediatric Nephrology, Laboratory of Pediatrics, University Hospitals Leuven, Leuven, Belgium.

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http://dx.doi.org/10.1016/j.jpeds.2011.05.057DOI Listing
December 2011

Renal mitochondrial cytopathies.

Int J Nephrol 2011 27;2011:609213. Epub 2011 Jul 27.

Division of Nephrology and Dialysis, Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, piazza Sant'Onofrio 4, 00165 Rome, Italy.

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http://dx.doi.org/10.4061/2011/609213DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3146993PMC
November 2011

Improvement in the renal prognosis in nephropathic cystinosis.

Clin J Am Soc Nephrol 2011 Oct 25;6(10):2485-91. Epub 2011 Aug 25.

European Society for Paediatric Nephrology/European Renal Association and European Dialysis and Transplant Association Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, The Netherlands.

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http://dx.doi.org/10.2215/CJN.02000311DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3359556PMC
October 2011

Transcriptional and posttranscriptional regulation of the CTNS gene.

Pediatr Res 2011 Aug;70(2):130-5

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, 00165 Rome, Italy.

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http://www.nature.com/doifinder/10.1203/PDR.0b013e3182200187
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http://dx.doi.org/10.1203/PDR.0b013e3182200187DOI Listing
August 2011

Serum creatinine levels are significantly influenced by renal size in the normal pediatric population.

Clin J Am Soc Nephrol 2011 Jan 30;6(1):107-13. Epub 2010 Sep 30.

Department of Nephrology and Urology, Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.2215/CJN.00580110DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3022231PMC
January 2011

Usefulness of skin immunofluorescence for distinguishing SLE from SLE-like renal lesions: a pilot study.

Pediatr Nephrol 2011 Jan 13;26(1):77-83. Epub 2010 Oct 13.

Department of Nephrology and Urology, Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://link.springer.com/10.1007/s00467-010-1655-2
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http://dx.doi.org/10.1007/s00467-010-1655-2DOI Listing
January 2011

Long-term outcome of nephropathic cystinosis: a 20-year single-center experience.

Pediatr Nephrol 2010 Dec 29;25(12):2459-67. Epub 2010 Aug 29.

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-010-1641-8DOI Listing
December 2010

Long-term cardiovascular effects of pre-transplant native kidney nephrectomy in children.

Pediatr Nephrol 2010 Dec 25;25(12):2523-9. Epub 2010 Sep 25.

Division of Pediatric Nephrology, Bambino Gesú Hospital, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-010-1638-3DOI Listing
December 2010

The pathogenesis of cystinosis: mechanisms beyond cystine accumulation.

Am J Physiol Renal Physiol 2010 Nov 8;299(5):F905-16. Epub 2010 Sep 8.

Dept. of Pediatric Nephrology, Univ. Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium.

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http://www.physiology.org/doi/10.1152/ajprenal.00318.2010
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http://dx.doi.org/10.1152/ajprenal.00318.2010DOI Listing
November 2010

Cyclosporine A treatment in patients with Alport syndrome: a single-center experience.

Pediatr Nephrol 2010 Jul 18;25(7):1269-75. Epub 2010 Mar 18.

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Nephrology and Dialysis Unit, Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-010-1484-3DOI Listing
July 2010

Analysis of CTNS gene transcripts in nephropathic cystinosis.

Pediatr Nephrol 2010 Jul 30;25(7):1263-7. Epub 2010 Mar 30.

Department of Nephrology and Urology, Division of Nephrology, Bambino Gesù Children's Hospital and Research Institute, Piazza S. Onofrio, 4, 00165, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-010-1502-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2874020PMC
July 2010

Time for initial response to steroids is a major prognostic factor in idiopathic nephrotic syndrome.

J Pediatr 2010 Jun 10;156(6):965-971. Epub 2010 Mar 10.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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https://linkinghub.elsevier.com/retrieve/pii/S00223476090124
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http://dx.doi.org/10.1016/j.jpeds.2009.12.020DOI Listing
June 2010

Idiopathic membranous nephropathy associated with polycystic kidney disease.

Pediatr Nephrol 2010 May 22;25(5):961-3. Epub 2009 Dec 22.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1007/s00467-009-1398-0DOI Listing
May 2010

[Cystinuria].

G Ital Nefrol 2010 Jan-Feb;27(1):30-6

Dipartimento Nefrologia Urologia, Ospedale Pediatrico Bambino Gesù, Istituto di Ricerca Scientifica, Roma.

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May 2010

Modulation of CTNS gene expression by intracellular thiols.

Free Radic Biol Med 2010 Apr 14;48(7):865-72. Epub 2010 Jan 14.

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1016/j.freeradbiomed.2010.01.011DOI Listing
April 2010