Literature DB >> 17636340

Remission of steroid- and CyA-resistant nephrotic syndrome using multiple drug immunosuppression.

Tim Ulinski1, Laurence Perrin, Vincent Guigonis, Françoise Driss, Georges Deschênes, Albert Bensman.   

Abstract

Nephrotic proteinuria in minimal change disease (MCD) is supposed to be due to a circulating factor of immunologic origin. End-stage renal failure occurs if both steroids and immunosuppressive drugs remain ineffective. Three children (2 years, 3 years, and 6 years of age) with secondary steroid-resistant nephrotic syndrome (NS) were included, as they remained resistant to 30 days of treatment with prednisone (60 mg/m(2) per day), three pulses of methylprednisolone (1 g/1.73 m(2)) followed by oral administration of CyA 7.5 mg/kg per day over 2 months, and 1 month of intravenous (i.v.) administration of cyclosporine (blood level 500-600 ng/ml). All three patients were partially responsive to methylprednisolone pulses, with an increase of serum albumin by 100%. They were treated with plasma exchanges, cyclophosphamide and cyclosporine A, both given orally, pefloxacin and methylprednisolone pulses followed by orally administered prednisone. All three patients went into remission within 2 to 5 weeks. The character of their NS changed to a steroid-sensitive one. There were no significant side effects from the therapy. They had normal renal function, normal blood pressure and no residual proteinuria. A combination of plasmapheresis and multiple immunosuppressive medications was effective in producing remission of minimal change NS in three children who were previously resistant to glucocorticoids and cyclosporine.

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Year:  2007        PMID: 17636340     DOI: 10.1007/s00467-007-0551-x

Source DB:  PubMed          Journal:  Pediatr Nephrol        ISSN: 0931-041X            Impact factor:   3.714


  13 in total

1.  NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome.

Authors:  N Boute; O Gribouval; S Roselli; F Benessy; H Lee; A Fuchshuber; K Dahan; M C Gubler; P Niaudet; C Antignac
Journal:  Nat Genet       Date:  2000-04       Impact factor: 38.330

2.  Nephrotic syndrome and plasmapheresis.

Authors:  G E Russo; M Bonello; B Bauco; M Bosco; M Tedaldi; M F Bisciglia; E Vitaliano; R Russo; A DePaola
Journal:  Int J Artif Organs       Date:  2000-02       Impact factor: 1.595

3.  Pathogenesis of lipoid nephrosis: a disorder of T-cell function.

Authors:  R J Shalhoub
Journal:  Lancet       Date:  1974-09-07       Impact factor: 79.321

4.  Intravenous cyclosporine therapy in recurrent nephrotic syndrome after renal transplantation in children.

Authors:  Rémi Salomon; Marie-France Gagnadoux; Patrick Niaudet
Journal:  Transplantation       Date:  2003-03-27       Impact factor: 4.939

5.  Cyclosporine in patients with steroid-resistant nephrotic syndrome: an open-label, nonrandomized, retrospective study.

Authors:  Gian Marco Ghiggeri; Paolo Catarsi; Francesco Scolari; Gianluca Caridi; Roberta Bertelli; Alba Carrea; Simone Sanna-Cherchi; Francesco Emma; Landino Allegri; Giovanni Cancarini; Gian Franco Rizzoni; Francesco Perfumo
Journal:  Clin Ther       Date:  2004-09       Impact factor: 3.393

6.  Peritonitis in childhood nephrotic syndrome: 1970-1980.

Authors:  A M Krensky; J R Ingelfinger; W E Grupe
Journal:  Am J Dis Child       Date:  1982-08

7.  Recurrent nephrotic syndrome after transplantation: early treatment with plasmaphaeresis and cyclophosphamide.

Authors:  P Cochat; A Kassir; S Colon; C Glastre; B Tourniaire; B Parchoux; X Martin; L David
Journal:  Pediatr Nephrol       Date:  1993-02       Impact factor: 3.714

Review 8.  Nephrotic syndrome in children.

Authors:  P Niaudet
Journal:  Curr Opin Pediatr       Date:  1993-04       Impact factor: 2.856

9.  Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome.

Authors:  M Kestilä; U Lenkkeri; M Männikkö; J Lamerdin; P McCready; H Putaala; V Ruotsalainen; T Morita; M Nissinen; R Herva; C E Kashtan; L Peltonen; C Holmberg; A Olsen; K Tryggvason
Journal:  Mol Cell       Date:  1998-03       Impact factor: 17.970

10.  Enalapril dosage in steroid-resistant nephrotic syndrome.

Authors:  Arvind Bagga; Basanagoud D Mudigoudar; Pankaj Hari; Vandita Vasudev
Journal:  Pediatr Nephrol       Date:  2003-11-25       Impact factor: 3.714

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