Minimal-Change Disease Treatment & Management
- Author: Abeera Mansur, MD; Chief Editor: Vecihi Batuman, MD, FACP, FASN more...
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 renal 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% has been recorded in adolescents and adults. However, the time to remission is up to 16 weeks. If patients are steroid-resistant or they relapse frequently, a trial of immunosuppressants is given.
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 renal 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 sodium chloride solution. 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, increase in hematocrit, and response to contributing factors (eg, diarrhea, septicemia, diuretic therapy).
Edema should be controlled by dietary sodium restriction. Small amounts of edema are not of much 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.
Consultation with a nephrologist generally is needed. The nephrologist has the expertise to perform and interpret the renal 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 hepatic albumin synthesis is elevated with protein intake that is higher than the RDA.
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.
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