Document Detail

Sequential maintenance therapy with cyclosporin A and mycophenolate mofetil for sustained remission of childhood steroid-resistant nephrotic syndrome.
MedLine Citation:
PMID:  21976740     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: There is currently no established standard for maintenance therapy of steroid-resistant nephrotic syndrome (SRNS). We report the long-term clinical course, medication, pharmacokinetic data, and renal function of 23 children with primary, non-familial SRNS with focal segmental glomerulosclerosis (FSGS).
METHODS: To achieve initial remission, patients were treated with high-dose intravenous (i. v.) methylprednisolone and oral cyclosporin A (CsA). Maintenance therapy included transient alternate day oral prednisolone, CsA and angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin receptor blockers. In 18 patients, mycophenolate mofetil (MMF) (adjusted to achieve blood mycophenolic acid trough concentrations > 2 μg/mL) was sequentially added, and 16 patients were converted to MMF monotherapy.
RESULTS: During a mean follow-up time of 7.0 years (1.7-16.5 years; cumulative observation time 161 patient-years), sustained remission could be achieved in all patients. Five of 23 patients (21%) experienced 10 relapses; all responded to relapse therapy. Maintenance therapy could be permanently discontinued in seven patients (30%). After conversion from CsA to MMF, renal function improved significantly; the eGFR at last follow-up was 137 (range 106-198) mL/min × 1.73 m(2). The mean number of anti-hypertensive drugs decreased from 1.86 per patient after initial remission to 0.57 on MMF monotherapy (P < 0.002).
CONCLUSIONS: The data of this uncontrolled retrospective study indicate that in children with SRNS/FSGS achieving initial remission, a sequential steroid-free therapy consisting of a combination of CsA and MMF followed by MMF alone (with the addition of ACE inhibitors and angiotensin receptor blockers), can provide sustained long-term remission, preservation of renal function and better control of blood pressure.
Jutta Gellermann; Jochen H H Ehrich; Uwe Querfeld
Publication Detail:
Type:  Journal Article     Date:  2011-10-04
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  27     ISSN:  1460-2385     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  2012 May 
Date Detail:
Created Date:  2012-05-01     Completed Date:  2012-12-07     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  1970-8     Citation Subset:  IM    
Department of Paediatric Nephrology, Charité Children’s Hospital, Berlin, Germany.
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MeSH Terms
Administration, Oral
Angiotensin Receptor Antagonists / pharmacology,  therapeutic use
Angiotensin-Converting Enzyme Inhibitors / pharmacology,  therapeutic use
Blood Pressure / drug effects
Child, Preschool
Cyclosporine / administration & dosage*,  therapeutic use*
Drug Administration Schedule
Drug Resistance*
Follow-Up Studies
Glomerulosclerosis, Focal Segmental / drug therapy*,  epidemiology
Immunosuppressive Agents / administration & dosage,  therapeutic use
Injections, Intravenous
Mycophenolic Acid / administration & dosage,  analogs & derivatives*,  therapeutic use
Nephrotic Syndrome / drug therapy*,  epidemiology
Prednisolone / therapeutic use
Remission Induction
Retrospective Studies
Steroids / therapeutic use*
Treatment Outcome
Reg. No./Substance:
0/Angiotensin Receptor Antagonists; 0/Angiotensin-Converting Enzyme Inhibitors; 0/Immunosuppressive Agents; 0/Steroids; 24280-93-1/Mycophenolic Acid; 50-24-8/Prednisolone; 59865-13-3/Cyclosporine; 9242ECW6R0/mycophenolate mofetil

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