Pediatric Nephrotic Syndrome Medication

Updated: Oct 02, 2017
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Medication

Medication Summary

Prednisone is the first-line therapy for children with nephrotic syndrome. Other immunosuppressive medications may be useful in those whose symptoms fail to respond to standard corticosteroid therapy or in those who have frequent relapses.

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Glucocorticoids

Class Summary

All glucocorticoids are effective; however, prednisone or prednisolone is used most commonly. Their specific mode of action in nephrotic syndrome is unknown.

Prednisone

Prednisone is a delta1-derivative of naturally occurring adrenocortical steroids. It suppresses key components of the immune system.

Prednisolone (Orapred, Pediapred, Prelone)

Prednisolone is a delta1-derivative of the naturally occurring adrenocortical steroids. It suppresses key components of the immune system.

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Diuretics

Class Summary

Diuretics promote excretion of water and electrolytes by the kidneys. These agents are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.

Furosemide (Lasix)

Furosemide is used when symptomatic edema occurs. It increases excretion of water by interfering with the chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule.

Metolazone (Zaroxolyn)

Metolazone increases excretion of sodium, water, potassium and hydrogen ions by inhibiting reabsorption of sodium in the distal tubules. Metolazone may be used to augment diuretic response during treatment with furosemide.

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Plasma protein

Class Summary

This agent is used to supplement diuresis in patients with edema. It increases oncotic pressure and thereby promotes a fluid shift from interstitial tissues.

Albumin (Albuminar, Buminate, Flexbumin, Plasbumin)

Albumin raises oncotic pressure and thus supplements the diuretic effect of furosemide.

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Immunosuppressive agents

Class Summary

This agent is used to supplement diuresis in patients with edema. It increases oncotic pressure and thereby promotes a fluid shift from interstitial tissues.

Cyclophosphamide

Cyclophosphamide is a cyclic polypeptide that suppresses some humoral activity. It is chemically related to nitrogen mustards. In the liver, this agent is biotransformed by the cytochrome P-450 system to its active metabolite, 4-hydroxycyclophosphamide, which alkylates the target sites in susceptible cells in an all-or-none type reaction. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

The mechanism of action of cyclophosphamide in autoimmune diseases is thought to involve immunosuppression due to destruction of immune cells via DNA cross-linking.

In high doses, cyclophosphamide affects B cells by inhibiting clonal expansion and suppression of production of immunoglobulins. With long-term low-dose therapy, it affects T cell functions.

Cyclophosphamide has been successfully used in conditions that require immunosuppression. It is highly effective for frequently relapsing steroid-sensitive nephrotic syndrome; half of the children enter a prolonged remission. Researchers have formulated various protocols for different renal pathological lesions.

Cyclosporine (Sandimmune, Neoral, Gengraf)

Cyclosporine is an 11-amino acid cyclic peptide and natural product of fungi that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease) for various organs.

This agent is a specific modulator of T-cell replication and activity. It depresses cell-mediated immune responses by inhibiting helper T-cell function. It may produce preferential and reversible inhibition of T lymphocytes in G0 or G1 phase of the cell cycle. Maximum suppression of T-lymphocyte proliferation requires that drug be present during first 24 h of antigenic exposure. This agent has minimal activity against B cells.

Cyclosporine binds to cyclophilin, an intracellular protein, which in turn prevents formation of interleukin 2 and the subsequent recruitment of activated T cells.

The bioavailability of cyclosporine averages about 30%, but this varies markedly between individual patients.

Tacrolimus (Prograf)

Tacrolimus is an immunomodulator produced by the bacterium Streptomyces tsukubaensis. Its mechanism of action is similar to that of cyclosporine. This agent is primarily used in transplant recipients, but is also used in Behçet disease to treat uveitis.

Mycophenolate mofetil (CellCept, Myfortic)

This agent inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. It inhibits antibody production.

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