Minimal change disease

Adult minimal change disease is rare and seen in 10-15% of biopsy specimens from patients with nephrotic-range proteinuria

Minimal-change disease (MCD) counts for 10 to 15% of cases of primary nephrotic syndrome in adults.

There were no differences in remissions, time to remission, relapse rate, or time to relapse between daily- and alternate-day–treated patients.

More than one quarter of patients were steroid resistant.

At least one relapse occurred in 73% of patients; 28% were frequently relapsing.

A significant proportion of frequently relapsing patients became steroid dependent.

Second-line agents are used for steroid dependence, steroid resistance, or frequent relapsed patients.

Acute renal failure occurs in a significant number of adult MCD patients.

Only a few patients with adult MCD progress to ESRD.

It is the most common cause of the nephrotic syndrome in children.

70-90% of children younger than 10 years with nephrotic syndrome have MCD.

50% of those in older children suffering with nephrotic syndrome have MCD.

Remission achieved in about 75% of patients with steroids.

More than 25% patients are resistant to an initial trial of steroids.

Patients frequently experience more than one relapse.

Second-line agents including cyclosporine, cyclophosphamide, mycophenolate mofetil and tacrolimus are prescribed for indications that included steroid resistance or dependence, partial response to steroids, frequent relapses, and toxicity or contraindications to steroids.

No single agent is superior in achieving remission, however there are significantly more remissions in steroid-dependent patients compared with steroid-resistant patients.

Patients who responded to initial steroid therapy were more likely to have better outcomes compared with those who did not have any response to steroids.

Steroid responders had higher serum albumin than steroid nonresponders.

The pathogenesis remains unknown.

T cell activation and resultant cytokine mediated injury to the glomerular foot processes has been proposed as a major contributor to disease pathogenesis

Association of disease onset with viral infections, allergies, malignancy, and numerous medications.

Some patients respond quickly to steroids and never experience relapse.

Some patients relapse frequently, and become steroid dependent.

Some patients become resistant to steroids

The main characteristics of MCD in children is abrupt onset of proteinuria with the development of the nephrotic syndrome.

Hematuria, hypertension, and renal insufficiency are seen in adults but are unusual in children.

Steroids are the first-line therapy with an overall rate of steroid responsiveness of cohort 75% by 13 wk.

Adults respond more slowly to therapy than children, 50% of whom respond within 2 wk and almost all within 8 wk.

In general, adults will require longer duration of steroid treatment and are not considered steroid resistant until after 4 mo of therapy.

Alternate-day steroids may represent a reasonable option for treatment.

Many patients experience a relapse in the nephrotic syndrome.

In childhood disease age of onset is related to frequency of relapses, with younger patients showing a tendency toward more relapses.

Limited data on effectiveness in adults with steroid-dependent or steroid-resistant MCD: Cyclophosphamide has been reported to induce and maintain remission in 25 to 60% of patients for up to 5 yr .

Cyclosporine has been reported to induce remission in approximately 60% of patients, but have a high rate of relapse.

Acute renal failure can occur in the setting of MCD.

Compared with patients without ARF, those with ARF did have higher protein excretion and lower serum albumin, were older and were more likely to be hypertensive at presentation.

ARF that accompanies MCD is reversible, with the vast majority achieving complete recovery of renal function.

The risk for thromboembolism is increased in MCD as in all patients with the nephrotic syndrome.

The prothrombotic state probably arises from abnormalities, including increased plasma viscosity, low plasminogen, low antithrombin III and the administration of steroids.

Patients have increased susceptibility to infection possibly related to accumulation of edema and ascites, urinary losses of IgG, impaired ability to generate specific antibodies and decreased serum levels of the alternative complement factor B and hemolytic factor D, and use of immunosuppressive medications during relapses.

ARF during the course of the nephrotic syndrome increases the risk for infection.

Patients with steroid responsiveness have more favorable outcomes.

Prevalence of adult minimal change disease is increased in Caucasian patients who are 80 years of age or older.

Adult patients with minimal change disease may present with rapid onset of classic nephrotic syndrome, with rapid development of hypoalbuminemia, edema and possibly even acute kidney injury due to intravascular volume depletion.

Overt hematuria is uncommon.

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