Minimal change disease

Minimal change disease or nil disease (lipoid nephrosis) is a disease of the kidney which causes nephrotic syndrome and usually affects children (peak incidence at 2-3 years of age).^[1]

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Symptoms

The symptoms are proteinuria (leakage of protein into the urine) and water retention. There are other kidney diseases that have these same symptoms but a needle biopsy shows change in the kidney tissue if these other diseases are present.

Causes

Minimal change disease can be associated with food allergies, medications, or hematologic malignancies, or it can occur idiopathically. The pathology does not appear to involve complement, immunoglobulins, or immune complex deposition. Rather, an altered cell-mediated immunologic response with abnormal secretion of lymphokines by T cells is thought to reduce the production of anions in the glomerular basement membrane, thereby increasing the glomerular permeability to serum albumin through a reduction of electrostatic repulsion.^[2] The loss of anionic charges is also thought to favor foot process fusion. With minimal change disease the kidney tissue appears normal under a light microscope, but shows podocyte foot process effacement under an electron microscope.^[1]

Treatment

Prednisone is prescribed along with a blood pressure medication, typically an ACE inhibitor such as lisinopril. Often the liver is overactive with minimal change disease and over produces cholesterol. Therefore a statin drug is often prescribed for the duration of the treatment. When the urine is clear of protein, the drugs can be discontinued. 50% of patients will relapse and need further treatment.

Other notes

80% of those who get minimal change disease have a recurrence with 20% never realizing another occurrence. Some authors have noted that other conditions associated with T-cell abnormalities, such as Hodgkin's disease and T-cell lymphoma, are sometimes associated with minimal change disease.

References