Approach Considerations

Because of the high prevalence of minimal-change disease (MCD) in children with nephrotic syndrome, an empiric trial of corticosteroids commonly is the first step in therapy. Corticosteroids are the treatment of choice, leading to complete remission of proteinuria in most cases. Approximately 90% of children respond within 2 weeks to prednisone at a dose of 2 mg/kg/day (not to exceed 80 mg/day). After the remission of proteinuria, prednisone is continued for another 6 weeks, at lower doses.

In some children, proteinuria fails to clear by 6-8 weeks, and performing a kidney biopsy may be useful to determine if another process may be present. For example, MCD secondary to Hodgkin lymphoma is frequently resistant to steroids and will remit with cure of the primary disease. Generally, if proteinuria persists after two relapses or courses of steroids, a tissue diagnosis should be made before starting cytotoxic or immunosuppressive therapy.

Adults respond more slowly than children. A response in up to 80%-90% of cases has been recorded in adolescents and adults, but the time to remission is up to 16 weeks. In patients with steroid-resistant MCD or frequent relapses, a trial of immunosuppressants is given.

A Cochrane review of treatments of nephrotic syndrome in adults with MCD reported that calcineurin inhibitors (eg, tacrolimus, cyclosporine) with or without low-dose prednisolone were as effective as prednisolone alone in achieving remission, with lower rates of adverse effects.^[26]

Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs), alone or in combination, should be used with a goal of reducing the proteinuria. Blood pressure and kidney function should be monitored closely in patients on ACE inhibitors and ARBs.

The presence of hypovolemia necessitates immediate volume expansion with purified plasma protein fraction and isotonic saline. Parenteral albumin infusions are not appropriate for long-term management of hypoalbuminemia because they have only a transient effect. Such crises should be avoided with recognition of the earlier signs of hypovolemia, including abdominal pain and increase in hematocrit, and correction of contributing factors (eg, diarrhea, septicemia, diuretic therapy).

Edema should be controlled by dietary sodium restriction. Small amounts of edema have little clinical significance. The use of diuretics should be reserved for patients with severe edema, particularly in the presence of respiratory or gastrointestinal symptoms, and when the condition restricts activity.

Thrombotic episodes should be prevented by mobilization and meticulous attention to venipuncture and intravenous infusion sites. Established episodes should be managed with heparinization.

Infections must be treated aggressively. Cellulitis, peritonitis, otitis, and pneumonia are common infections. Susceptibility to pneumococcal infections warrants the administration of penicillin prophylaxis to patients in relapse. Corticosteroids increase susceptibility to infection.

Consultations

Consultation with a nephrologist generally is needed. The nephrologist has the expertise to perform and interpret the kidney biopsy. A renal pathologist has the expertise to interpret biopsy findings under light microscopy, immunofluorescence, and electron microscopy.

Diet and Activity

An adequate dietary protein intake, in accordance with the recommended daily allowance (RDA) is necessary. No evidence suggests that protein intake that is higher than the RDA will elevate hepatic albumin synthesis.

Dietary sodium restriction helps forestall the progression of edema and also is prudent in the management of hypertension.

Mobilization, rather than bed rest, is indicated to avoid thromboembolic complications.

Long-Term Monitoring

Followup care includes the following:

Carefully monitor medication doses and adverse effects
Monitor vital signs for possible onset of hypertension
Monitor volume status
Monitor for signs of infection

Medication

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Author

Abeera Mansur, MD Consultant Nephrologist, Doctors Hospital and Medical Center, Pakistan; Nephrologist, CHI Health Good Samaritan of Kearney, Nebraska

Abeera Mansur, MD is a member of the following medical societies: American College of Physicians, American Society of Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Eleanor Lederer, MD, FASN Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD, FASN is a member of the following medical societies: American Association for the Advancement of Science, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Society of Nephrology<br/>Received income in an amount equal to or greater than $250 from: Healthcare Quality Strategies, Inc.

Chief Editor

Vecihi Batuman, MD, FASN Professor of Medicine, Section of Nephrology-Hypertension, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

Anil Kumar Mandal, MD Clinical Professor, Department of Internal Medicine, Division of Nephrology, University of Florida College of Medicine

Anil Kumar Mandal, MD is a member of the following medical societies: American College of Clinical Pharmacology, American College of Physicians, American Society of Nephrology, Central Society for Clinical and Translational Research

Disclosure: Nothing to disclose.

Susie Lew, MD Professor of Medicine, Department of Medicine, Division of Renal Diseases and Hypertension, George Washington Unversity School of Medicine and Health Sciences; Medical Director, Peritoneal Dialysis Unit, George Washington University Medical Center, Gambro Healthcare/DaVita

Susie Lew, MD is a member of the following medical societies: American College of Physicians, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation

Disclosure: Received grant/research funds from Amgen for investigator; Received consulting fee from Gambro for consulting; Received grant/research funds from Questcor for investigator; Received grant/research funds from Bristol Meyers Squibb for investigator; Received grant/research funds from CMS for investigator.

Florin Georgescu, MD Consulting Staff, Kidney Specialists of Savannah

Florin Georgescu, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology

Disclosure: Nothing to disclose.

Minimal-Change Disease Treatment & Management

Approach Considerations

Consultations

Diet and Activity

Long-Term Monitoring

References

Tables

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