Cochrane · systematic review · CD003594

Interventions for idiopathic steroid‐resistant nephrotic syndrome in children

Liu, ID, Willis, NS, Craig, JC, et al. · Cochrane Database of Systematic Reviews · 2019 · Cochrane Library

Kidney and Transplant3 versionsAMSTAR 2: not assessed
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
62
current version
GRADE rated
62
No studies
0
Included studies
NR
No studies 0 · 0%Ungraded 0 · 0%Very low 14 · 22.6%Low 42 · 67.7%Moderate 6 · 9.7%High 0 · 0%

Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.

Questions and results

Current version only

Cyclosporin versus placebo or no treatment for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Cyclosporin vs placebo or no treatment
RR 3.5 [1.09, 11.2]4 studies · 74 participantsLow
Downgraded: risk of bias
Complete remission: FSGS
Cyclosporin vs placebo or no treatment
RR 3.14 [0.97, 10.18]3 studies · 58 participantsLow
Downgraded: risk of bias
Complete or partial remission: all renal pathologies
Cyclosporin vs placebo or no treatment
RR 3.15 [1.04, 9.57]4 studies · 74 participantsLow
Downgraded: risk of bias
Complete or partial remission: FSGS
Cyclosporin vs placebo or no treatment
RR 2.66 [0.85, 8.31]2 studies · 49 participantsLow
Downgraded: risk of bias
Adverse events: worsening of hypertension
Cyclosporin vs placebo or no treatment
RR 1 [0.17, 5.98]1 study · 24 participantsVery low
Downgraded: risk of bias
Adverse events: infection
Cyclosporin vs placebo or no treatment
RR 0.7 [0.2, 2.51]1 study · 17 participantsVery low
Downgraded: risk of bias
Adverse events: ESKD
Cyclosporin vs placebo or no treatment
RR 0.23 [0.03, 1.79]1 study · 25 participantsVery low
Downgraded: risk of bias

Calcineurin inhibitor versus IV cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 3 to 6 months: complete or partial remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 1.98 [1.25, 3.13]2 studies · 156 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 3 to 6 months: complete remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 3.43 [1.84, 6.41]2 studies · 156 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 3 to 6 months: partial remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 1.68 [0.43, 6.56]2 studies · 156 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: treatment failure at 6 months (non response, serious infection, persistently elevated creatinine)
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.32 [0.18, 0.58]1 study · 124 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: medications ceased due to adverse events
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.2 [0.04, 0.86]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: serious infections
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.49 [0.16, 1.56]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: death
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.33 [0.01, 7.92]1 study · 131 participantsLow
Downgraded: risk of bias, imprecision, inconsistency

Tacrolimus versus cyclosporin for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 6 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months: complete remission
Tacrolimus vs cyclosporin
RR 1.14 [0.64, 2.03]1 study · 41 participantsLow
Treatment response at 6 months: complete and partial remission
Tacrolimus vs cyclosporin
RR 0.57 [0.16, 2.08]1 study · 41 participantsLow
Treatment response at 12 months: complete remission
Tacrolimus vs cyclosporin
RR 0.8 [0.45, 1.42]2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 12 months: complete and partial remission
Tacrolimus vs cyclosporin
RR 1.05 [0.87, 1.25]2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: persistent nephrotoxicity
Tacrolimus vs cyclosporin
RR 0.48 [0.05, 4.85]1 study · 41 participantsLow
Adverse events: worsening of hypertension
Tacrolimus vs cyclosporin
no estimate extracted2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision

Cyclosporin versus mycophenolate mofetil with pulse dexamethasone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 52 weeks: complete remission (primary outcome 1, 2)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 2.14 [0.87, 5.24]1 study · 138 participantsModerate
Treatment response at 52 weeks: partial remission (primary outcome 3)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.09 [0.61, 1.93]1 study · 138 participantsModerate
Sustainable remission between 52 and 78 weeks: complete or partial remission (primary outcome 1, 2, 3)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.38 [0.9, 2.1]1 study · 138 participantsModerate
CKD or death: death by 52 weeks
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 0.18 [0.01, 3.75]1 study · 138 participantsLow
CKD or death: 50% decline in GFR by 78 weeks
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 2.29 [0.46, 11.41]1 study · 138 participantsLow
Adverse events (weeks 0 to 26): serious infection requiring hospitalisation
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 0.65 [0.22, 1.96]1 study · 138 participantsLow
Adverse events (weeks 0 to 26): hypertension
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.68 [0.66, 4.29]1 study · 138 participantsLow

Tacrolimus versus mycophenolate mofetil to maintain remission for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Number with complete or partial response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 2.01 [1.32, 3.07]1 study · 60 participantsLow
Downgraded: risk of bias
Number with complete response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 1.79 [1.11, 2.9]1 study · 60 participantsLow
Downgraded: risk of bias
Number with partial response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 4.68 [0.58, 37.68]1 study · 60 participantsLow
Downgraded: risk of bias
Number with treatment failure by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.18 [0.06, 0.54]1 study · 60 participantsLow
Downgraded: risk of bias
Number with frequent relapses by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.28 [0.09, 0.92]1 study · 60 participantsLow
Downgraded: risk of bias
Number with steroid resistance by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.07 [0, 1.23]1 study · 60 participantsLow
Downgraded: risk of bias
Change in GFR
Tacrolimus vs mycophenolate mofetil to maintain remission
no estimate extracted1 study · 60 participantsLow
Downgraded: risk of bias

Oral cyclophosphamide versus prednisone or placebo for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Oral cyclophosphamide vs prednisone or placebo
RR 1.06 [0.61, 1.87]2 studies · 84 participantsLow
Downgraded: risk of bias
Complete remission: FSGS
Oral cyclophosphamide vs prednisone or placebo
RR 1.01 [0.43, 2.37]2 studies · 63 participantsLow
Downgraded: risk of bias
Complete or partial remission
Oral cyclophosphamide vs prednisone or placebo
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias
Complete or partial remission: FSGS
Oral cyclophosphamide vs prednisone or placebo
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias
Treatment failure
Oral cyclophosphamide vs prednisone or placebo
RR 1.59 [0.87, 2.88]1 study · 60 participantsLow
Downgraded: risk of bias
Adverse events: death (all causes)
Oral cyclophosphamide vs prednisone or placebo
RR 1.07 [0.19, 5.95]1 study · 60 participantsLow
Downgraded: risk of bias
Adverse events: hypertension with seizures
Oral cyclophosphamide vs prednisone or placebo
RR 0.71 [0.05, 10.89]1 study · 60 participantsVery low
Downgraded: risk of bias

IV versus oral cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission
IV vs oral cyclophosphamide
RR 1.58 [0.65, 3.85]2 studies · 61 participantsLow
Downgraded: risk of bias, imprecision
Partial remission
IV vs oral cyclophosphamide
RR 1 [0.15, 6.55]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Continuing remission at one year
IV vs oral cyclophosphamide
RR 0.75 [0.19, 3.01]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: renal insufficiency
IV vs oral cyclophosphamide
RR 0.33 [0.04, 2.99]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: bacterial infection
IV vs oral cyclophosphamide
RR 1.02 [0.1, 10.62]2 studies · 61 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: vomiting
IV vs oral cyclophosphamide
RR 2.38 [0.35, 16.17]2 studies · 61 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: alopecia
IV vs oral cyclophosphamide
RR 1.5 [0.27, 8.22]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision

IV cyclophosphamide versus oral cyclophosphamide plus IV dexamethasone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months: complete remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.13 [0.65, 1.96]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 6 months: partial remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.88 [0.14, 5.79]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 6 months: complete or partial remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.09 [0.68, 1.74]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 18 months: sustained remission/steroid‐sensitive relapses
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.13 [0.65, 1.96]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 18 months: CKD
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.88 [0.06, 13.35]1 study · 49 participantsLow
Downgraded: imprecision
Adverse events: hypertension
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.04 [0, 0.68]1 study · 49 participantsLow
Downgraded: imprecision
Adverse events: bacterial infections
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.66 [0.27, 1.63]1 study · 49 participantsLow
Downgraded: imprecision

Rituximab/cyclosporin/prednisolone versus cyclosporin/prednisolone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB6 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Number with complete remission: complete remission in initial steroid resistance
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
no estimate extracted1 study · 16 participantsVery low
Downgraded: risk of bias
Number with complete remission: complete remission in delayed steroid resistance
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 1.14 [0.33, 3.94]1 study · 15 participantsVery low
Downgraded: risk of bias
Number with complete remission: complete remission in all patients
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 0.94 [0.22, 3.94]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: bronchospasm/treatment discontinued
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 2.82 [0.12, 64.39]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: hypotension
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 2.82 [0.12, 64.39]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: skin rash
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 6.59 [0.37, 117.77]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: mild dyspnoea
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 4.71 [0.24, 90.69]1 study · 31 participantsVery low
Downgraded: risk of bias

Cyclosporin versus placebo/no treatment

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB5 · 6 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Cyclosporin vs placebo/no treatment
RR 7.66 [1.06, 55.34]3 studies · 49 participantsLow
Downgraded: imprecision
Complete remission: FSGS
Cyclosporin vs placebo/no treatment
RR 5.83 [0.75, 45.09]2 studies · 33 participantsLow
Downgraded: imprecision
Complete or partial remission: all renal pathologies
Cyclosporin vs placebo/no treatment
RR 5.48 [1.95, 15.44]3 studies · 49 participantsLow
Downgraded: imprecision
Complete or partial remission: FSGS
Cyclosporin vs placebo/no treatment
RR 5 [1.63, 15.31]1 study · 24 participantsLow
Downgraded: imprecision
Adverse events: worsening of hypertension
Cyclosporin vs placebo/no treatment
no estimate extracted1 study · 24 participantsLow
Downgraded: imprecision
Adverse events: infection
Cyclosporin vs placebo/no treatment
no estimate extracted1 study · 17 participantsVery low
Downgraded: imprecision

Calcineurin inhibitors versus IV cyclophosphamide

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB5 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Remission at 3 to 6 months: complete or partial remission
Calcineurin inhibitors vs IV cyclophosphamide
RR 1.98 [1.25, 3.13]2 studies · 156 participantsLow
Remission at 3 to 6 months: complete remission
Calcineurin inhibitors vs IV cyclophosphamide
RR 3.43 [1.84, 6.41]2 studies · 156 participantsLow
Remission at 3 to 6 months: partial remission
Calcineurin inhibitors vs IV cyclophosphamide
RR 1.68 [0.43, 6.56]2 studies · 156 participantsVery low
Adverse events: treatment failure (non response, serious infection, persistently elevated creatinine) at 6 months
Calcineurin inhibitors vs IV cyclophosphamide
RR 0.32 [0.18, 0.58]1 study · 124 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: medications ceased due to adverse events
Calcineurin inhibitors vs IV cyclophosphamide
RR 0.2 [0.04, 0.86]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: serious infections
Calcineurin inhibitors vs IV cyclophosphamide
RR 0.49 [0.16, 1.56]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: death
Calcineurin inhibitors vs IV cyclophosphamide
RR 0.33 [0.01, 7.92]1 study · 131 participantsLow
Downgraded: risk of bias, imprecision, inconsistency

Cyclosporin versus mycophenolate mofetil with dexamethasone

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB5 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Remission at 52 weeks: complete remission (primary outcome 1,2)
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 2.14 [0.87, 5.24]1 study · 138 participantsModerate
Remission at 52 weeks: complete or partial remission (primary outcome 1,2,3)
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 1.38 [0.9, 2.1]1 study · 138 participantsModerate
CKD or death: death by 52 weeks
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 0.18 [0.01, 3.75]1 study · 138 participantsModerate
CKD or death: 50% decline in GFR by 78 weeks
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 2.29 [0.46, 11.41]1 study · 138 participantsModerate
Adverse effects (weeks 0 to 26): serious infection requiring hospitalisation
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 0.65 [0.22, 1.96]1 study · 138 participantsModerate
Adverse effects (weeks 0 to 26): neuropsychiatric conditions
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 1.26 [0.73, 2.19]1 study · 138 participantsModerate
Adverse effects (weeks 0 to 26): hypertension
Cyclosporin vs mycophenolate mofetil with dexamethasone
RR 1.68 [0.66, 4.29]1 study · 138 participantsModerate

Oral cyclophosphamide versus prednisone/placebo

Population: idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB5 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Oral cyclophosphamide vs prednisone/placebo
RR 1.06 [0.61, 1.87]2 studies · 84 participantsLow
Downgraded: risk of bias, imprecision
Complete remission: FSGS
Oral cyclophosphamide vs prednisone/placebo
RR 1.01 [0.43, 2.37]2 studies · 63 participantsLow
Downgraded: risk of bias, imprecision
Complete or partial remission
Oral cyclophosphamide vs prednisone/placebo
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias, imprecision
Treatment failure
Oral cyclophosphamide vs prednisone/placebo
RR 1.59 [0.87, 2.88]1 study · 60 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: all‐cause mortality
Oral cyclophosphamide vs prednisone/placebo
no estimate extracted1 study · 60 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: hypertension with seizures
Oral cyclophosphamide vs prednisone/placebo
no estimate extracted1 study · 60 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: bone marrow suppression
Oral cyclophosphamide vs prednisone/placebo
no estimate extracted1 study · 60 participantsVery low
Downgraded: risk of bias, imprecision

Cyclosporin versus placebo/no treatment for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Number of patients with complete remission ‐ All renal pathologies Urine protein excretion
Cyclosporin vs placebo/no treatment
RR 7.66 [1.06, 55.34]3 studies · 49 participantsLow
Number of patients with complete remission ‐ FSGS urine protein excretion
Cyclosporin vs placebo/no treatment
RR 5.83 [0.75, 45.09]2 studies · 33 participantsLow
Number of patients with complete or partial remission ‐ All renal pathologies
Cyclosporin vs placebo/no treatment
RR 5.48 [1.95, 15.44]3 studies · 49 participantsLow
Downgraded: risk of bias
Number of patients with complete or partial remission ‐ FSGS Urine protein excretion
Cyclosporin vs placebo/no treatment
RR 5 [1.63, 15.31]1 study · 24 participantsLow
Adverse effects
Cyclosporin vs placebo/no treatment
no estimate extracted2 studies · 41 participantsUngraded
Adverse effects ‐ Worsening of hypertension Blood pressure measurement
Cyclosporin vs placebo/no treatment
no estimate extracted1 study · 24 participantsLow
Adverse effects ‐ Infection Patient report
Cyclosporin vs placebo/no treatment
no estimate extracted1 study · 17 participantsLow

Cyclosporin versus IV cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 2 questions
OutcomeEffectEvidenceStatus / GRADE
Number achieving complete remission at 12 weeks Urinary protein excretion
Cyclosporin vs IV cyclophosphamide
RR 1.13 [0.18, 7.09]1 study · 32 participantsLow
Downgraded: imprecision
Number achieving complete or partial remission at 12 weeks urine protein excretion
Cyclosporin vs IV cyclophosphamide
RR 3.4 [1.12, 10.28]1 study · 32 participantsLow
Downgraded: imprecision

Tacrolimus versus cyclosporin for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months ‐ Complete remission Urinary protein excretion
Tacrolimus vs cyclosporin
RR 0.86 [0.44, 1.66]1 study · 41 participantsLow
Downgraded: risk of bias
Treatment response at 6 months ‐ Complete and partial remission Urine protein excretion
Tacrolimus vs cyclosporin
RR 1.07 [0.81, 1.42]1 study · 41 participantsLow
Downgraded: risk of bias
Treatment response at 12 months ‐ Complete remission
Tacrolimus vs cyclosporin
RR 0.87 [0.48, 1.58]1 study · 41 participantsLow
Treatment response at 12 months ‐ Complete and partial remission Urinary protein excretion
Tacrolimus vs cyclosporin
RR 1.14 [0.84, 1.55]1 study · 41 participantsLow
Downgraded: risk of bias
Number with relapse following complete or partial remission Urine protein excretion
Tacrolimus vs cyclosporin
RR 0.22 [0.06, 0.9]1 study · 34 participantsLow
Downgraded: risk of bias
Change in estimated GFR over 12 months
Tacrolimus vs cyclosporin
no estimate extracted1 study · 35 participantsLow
Adverse effects ‐ Reversible nephrotoxicity Serum creatinine
Tacrolimus vs cyclosporin
no estimate extracted1 study · 41 participantsLow
Downgraded: risk of bias

Oral cyclophosphamide versus prednisone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Number of patients with complete remission ‐ All renal pathologies clinical assessment
Oral cyclophosphamide vs prednisone
RR 1.06 [0.61, 1.87]2 studies · 84 participantsLow
Downgraded: risk of bias
Number of patients with complete remission ‐ FSGS urine testing
Oral cyclophosphamide vs prednisone
RR 1.01 [0.43, 2.37]2 studies · 63 participantsLow
Downgraded: risk of bias
Number of patients with complete or partial remission clinical assessment
Oral cyclophosphamide vs prednisone
no estimate extracted1 study · 53 participantsLow
Downgraded: risk of bias
Number of patients with complete or partial remission ‐ FSGS urine testing
Oral cyclophosphamide vs prednisone
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias
Adverse events ‐ All cause mortality
Oral cyclophosphamide vs prednisone
no estimate extracted1 study · 60 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events ‐ Bone marrow suppression clinical assessment
Oral cyclophosphamide vs prednisone
no estimate extracted1 study · 60 participantsVery low
Downgraded: risk of bias, imprecision
Treatment failure clinical assessment
Oral cyclophosphamide vs prednisone
RR 1.59 [0.87, 2.88]1 study · 60 participantsVery low
Downgraded: risk of bias, imprecision

IV versus oral cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 3 questions
OutcomeEffectEvidenceStatus / GRADE
Number of patients with complete remission clinical assessment
IV vs oral cyclophosphamide
RR 3.12 [0.81, 12.06]1 study · 11 participantsVery low
Downgraded: imprecision
Adverse effects ‐ Bacterial infection clinical assessment
IV vs oral cyclophosphamide
no estimate extracted1 study · 11 participantsVery low
Downgraded: imprecision
Adverse effects ‐ Vomiting clinical assessment
IV vs oral cyclophosphamide
no estimate extracted1 study · 11 participantsVery low
Downgraded: imprecision

IV cyclophosphamide versus IV dexamethasone and oral cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months ‐ Complete remission clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 1.13 [0.65, 1.96]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 6 months ‐ Complete or partial remission Clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 1.09 [0.68, 1.74]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 18 months ‐ Sustained remission/steroid sensitive relapses Clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 1.27 [0.72, 2.26]1 study · 52 participantsLow
Downgraded: imprecision
Treatment response at 18 months ‐ CKD clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 0.88 [0.06, 13.35]1 study · 49 participantsLow
Downgraded: imprecision
Complete or partial resistance in subgroups ‐ Initial SRNS clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 0.96 [0.46, 2.01]1 study · 18 participantsLow
Downgraded: imprecision
Complete or partial resistance in subgroups ‐ Late SRNS clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
RR 1.17 [0.64, 2.15]1 study · 31 participantsLow
Downgraded: imprecision
Adverse events ‐ Bacterial infections clinical assessment
IV cyclophosphamide vs IV dexamethasone and oral cyclophosphamide
no estimate extracted1 study · 49 participantsLow
Downgraded: imprecision

Chlorambucil versus indomethacin for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 2 questions
OutcomeEffectEvidenceStatus / GRADE
Number with complete remission clinical assessment
Chlorambucil vs indomethacin
RR 1 [0.42, 2.4]1 study · 30 participantsVery low
Number with end stage renal failure clinical assessment
Chlorambucil vs indomethacin
RR 0.2 [0.01, 3.85]1 study · 30 participantsVery low

Azathioprine versus placebo for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 2 questions
OutcomeEffectEvidenceStatus / GRADE
Number of patients with complete remission ‐ All renal pathologies clinical assessment
Azathioprine vs placebo
RR 0.94 [0.15, 5.84]1 study · 31 participantsLow
Downgraded: imprecision
Number of patients with complete or partial remission ‐ All renal pathologies clinical assessment
Azathioprine vs placebo
RR 0.94 [0.28, 3.09]1 study · 31 participantsLow
Downgraded: imprecision

Fosinopril and prednisone versus prednisone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: patients with idiopathic steroid‐resistant nephrotic syndrome in children
CD003594.PUB4 · 4 questions
OutcomeEffectEvidenceStatus / GRADE
Proteinuria ‐ After 12 weeks of treatment
Fosinopril and prednisone vs prednisone
no estimate extracted1 study · 45 participantsLow
Downgraded: imprecision
Serum albumin (end study)
Fosinopril and prednisone vs prednisone
no estimate extracted1 study · 45 participantsLow
Downgraded: imprecision
Creatinine clearance (end study)
Fosinopril and prednisone vs prednisone
no estimate extracted1 study · 45 participantsLow
Downgraded: imprecision
Serum potassium (end study)
Fosinopril and prednisone vs prednisone
no estimate extracted1 study · 45 participantsLow
Downgraded: imprecision