How is minimal-change disease (MCD) treated?

Updated: Jan 05, 2021
  • Author: Abeera Mansur, MD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

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% of cases 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 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 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.


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