Which therapies are used in the treatment of pediatric nephrotic syndrome?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Corticosteroids

If kidney biopsy is not initially indicated, a trial of corticosteroids is the first step in the treatment of INS.

Diuretics and albumin

Loop diuretics, such as furosemide, are often used to reduce edema. Metolazone may be beneficial in combination with furosemide for resistant edema. Intravenous 25% albumin can be combined with diuretics and may be particularly useful in diuretic-resistant edema and in patients with significant ascites or scrotal, penile, or labial edema. Caution should be used when administering albumin—in addition to pulmonary edema, albumin infusion can result in acute kidney injury and allergic reaction.

Antihypertensive agents

Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) can reduce hypertension and may also contribute to reducing proteinuria. However, because ACE inhibitors and ARBs can cause birth defects, adolescent women who are taking these agents must be counseled regarding the use of birth control, and pregnancy testing should be considered before starting these agents.

Calcium channel blockers and beta-blockers may also be used as first-line agents for hypertension.

Alkylating agents

Alkylating agents (eg, cyclophosphamide [CYP]) are most often used in children with suspected or biopsy-proven MCNS with frequent relapses. CYP offers the benefit of possible sustained remission, although with the possible risks of infertility and other adverse effects.

Calcineurin inhibitors

Calcineurin inhibitors (eg, cyclosporin A [CSA], tacrolimus [TAC]) are most often used in patients with INS due to focal segmental glomerulosclerosis (FSGS), or patients with suspected or biopsy-proven MCNS with frequent relapses who fail to respond to or whose parents refuse other treatments (such as CYP).

Rituximab

Rituximab (a chimeric monoclonal antibody against CD20 that depletes B cells) has been used with increasing frequency in patients with suspected or biopsy-proven MCNS with frequent relapses who fail to respond to other treatments.

Home monitoring

Home monitoring of urine protein and fluid status is an important aspect of management. All patients and parents should be trained to monitor first-morning urine protein at home with urine test strips. Urine testing at home is also useful in monitoring the response (or the lack of a response) to corticosteroid treatment.

See Treatment and Medication for more detail.


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