[The effect of cyclosporine A in steroid-dependent nephrotic syndrome in children]

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.
Abstract

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