Minimal change disease – Interesting Facts

What is minimal change disease (MCD; minimal change nephrotic syndrome)?

Minimal change disease is a disorder of glomeruli that leads to heavy proteinuria.

Kidney biopsy shows normal glomeruli by light microscopy but shows effacement of the podocyte foot processes on electron microscopy. Immunofluorescent microscopy typically is negative, although some patients may show staining for immunoglobulin M (IgM) in the mesangial regions of the glomeruli.

Technically, a patient cannot be said to have MCD with certainty without a kidney biopsy.

However, so many young children with nephrotic syndrome have MCD that kidney biopsies are performed only in children with atypical findings or after failure of a trial of glucocorticoids.

Older adolescents and adults are diagnosed with MCD after a kidney biopsy is performed.

5 Interesting Facts of Minimal change disease

1. Minimal change disease (MCD) is the cause of nephrotic syndrome in approximately 90% of children younger than age 6, in approximately 65% of older children, and in approximately 20% to 30% of adolescents. In adults, only approximately 10% to 25% of nephrotic syndrome results from MCD, but it represents the third most common cause of nephrotic syndrome in adults after membranous nephropathy and focal, segmental glomerulosclerosis.

2. MCD is an immune-mediated disease, thought to be mediated by a circulating factor capable of inducing proteinuria. Presumably the circulating factor is secreted by lymphoid cells and functions as a vascular permeability factor that directly affects the function of the podocytes.

3. Patients with MCD typically present with mild to severe edema. Because the onset with periorbital edema commonly follows an upper respiratory infection in young children, nephrotic syndrome may sometimes be confused with an allergic reaction until a more thorough evaluation is performed.

4. The cornerstone of therapy of typical nephrotic syndrome in young children is high-dose glucocorticoids. Children treated with a longer initial course of prednisone are less likely to experience frequent relapses than those children treated with a more abbreviated course of steroid therapy. Prednisone is also the standard initial therapy for adults with MCD. Adults with MCD tend to require a longer course of prednisone before remission is attained.

5. The vast majority of children with MCD have a very favorable prognosis. In children a prompt remission within 7 to 9 days of steroid therapy, the absence of microhematuria, and age greater than 4 years at presentation predict fewer relapses.

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