[The effect of cyclosporine A in steroid-dependent nephrotic syndrome in children]
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Published on: November 8, 2015
Anna Wasilewska1, Walentyna Zoch-Zwierz, Barbara Tomaszewska
1I Klinika Chorób Dzieci AM w Białymstoku.
Insights
Cyclosporine A (CyA) effectively treats relapsing nephrotic syndrome in children with minimal change nephrotic syndrome and focal segmental glomerulosclerosis. While CyA can cause transient side effects and alter blood pressure rhythms, its efficacy is notable.
Area of Science:
- Pediatric Nephrology
- Immunosuppressive Therapy
- Glomerular Diseases
Background:
- Steroid-dependent nephrotic syndrome (NS) in children often requires alternative treatments due to steroid resistance or toxicity.
- Relapsing forms of NS, including minimal change nephrotic syndrome (MCSN) and focal segmental glomerulosclerosis (FSGS), present significant management challenges.
Purpose of the Study:
- To evaluate the efficacy and safety of cyclosporine A (CyA) in pediatric patients with steroid-dependent nephrotic syndrome.
- To assess the impact of CyA treatment on proteinuria, renal function, and blood pressure parameters.
Main Methods:
- A cohort of 21 children with relapsing NS (9 MCSN, 12 FSGS) received CyA combined with prednisone and an ACE inhibitor.
- Serum CyA levels, proteinuria, serum creatinine, uric acid, albumin, cholesterol, and creatinine clearance were monitored over 12 months.
- Ambulatory blood pressure monitoring (ABPM) was performed before, during, and after CyA treatment.
Main Results:
- Urinary protein excretion significantly decreased over the 12-month treatment period.
- Serum creatinine and uric acid levels increased, indicating potential renal effects.
- Ambulatory blood pressure monitoring revealed an increase in mean systolic and diastolic pressures and a blunted nocturnal dip, suggesting a disturbed circadian rhythm of blood pressure.
Conclusions:
- Cyclosporine A is an effective therapeutic option for children with relapsing steroid-dependent nephrotic syndrome, including MCSN and FSGS.
- Adverse effects associated with CyA treatment were generally occasional and transient when monitored closely.
- Cyclosporine A administration can disrupt the 24-hour pattern of arterial blood pressure in pediatric patients.
Unlabelled:
The aim of study was the analysis of cyclosporine A (CyA) treatment efficacy in children with steroid-dependent nephrotic syndrome (NS).
Material And Methods:
The examined group consisted of 21 children (F--8, M--16) at the mean age 12.1 +/- 4.6 years with the relapses NS in the course of minimal change nephrotic syndrome (MCSN) in 9 (43%) and focal segmental glomerulosclerosis (FSGS) in 12 (57%) cases. All children were administrated CyA (Sandimun Neoral f. Novartis Pharma AG), together with prednisone (Encorton f. Polfa Pabianice) (0.2 - 0.5 mg/kg b.w./24 h) and ACE inhibitor (Enap f. Krka). The concentration of cyclospornine A in serum was measured by monoclonal antibody fluorescence polarization immunoassay. Serum concentration of creatinine, uric acid, albumin, cholesterol and creatinine clearance (Schwartz method) and proteinuria was analysed: A - before treatment, B--in 3rd day, C--in 3rd month, D--in 6th month, E--in 12th month of CyA treatment and F--after 3-6 months after finishing treatment. Blood pressure was measured by ABPM in examination A, D, E, and F.
Results:
During administration of CyA the urinary protein excretion decreased successively and in 12th month of treatment was 4.5 +/- 3.9 mg/kg bw/24 h. The serum creatinine concentration increased by 33.9%, and uric acid by 52.8% in comparison to the initial level. Mean systolic blood pressure (RRs) in ambulatory blood pressure monitoring (ABPM) during 24 hours was 120.5 +/- 9.2 mmHg before treatment and increased by about 6% after 12 months of treatment. Respectively diastolic blood pressure (RRr) was 66.7 +/- 4.3 mmHg and increased to 71.0 +/- 2.5 mmHg at the end of 12 h month. The nocturnal fall of RRs before treatment was 14.2 +/- 2.8%, and RRr 14.9 +/- 2.1%. During treatment nocturnal fall of both RRs and RRr decreased to 9.2 +/- 2.7% for RRs and 9.8 +/- 2.7% for RRr after 6 months of treatment. After 12 months of treatment and in remission the nocturnal fall of blood pressure was still below 10%.
Conclusions:
CyA is an effective drug in children with relapses of steroid-dependent in the course of MCSN and FSGS. Side effects after CyA treatment, under the control of its concentration in serum and parameters of renal function are occasional and transient. Cyclosporine A disturbs the 24 hours rhythm of arterial blood pressure.
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