Minimal change disease is a kidney disorder that can lead to nephrotic syndrome, although the nephrons of the kidney look normal under a regular microscope.
Minimal change nephrotic syndrome; Nil disease; Lipoid nephrosis; Idiopathic nephrotic syndrome of childhood
Each kidney is made of more than a million units called nephrons, which filter blood and produce urine.
In minimal change disease, there is damage to the glomeruli -- the tiny blood vessels inside the nephron where blood is filtered to make urine and waste is removed. The disease gets its name because this damage is not visible under a regular microscope. It can only be seen under an electron microscope.
Minimal change disease is the most common cause of nephrotic syndrome in children. It causes about 80% of cases in young children. It is also seen in adults, but makes up only 10 to 15% of nephrotic syndrome cases.
The cause is unknown, but the disease may occur after or be related to:
There may be symptoms of nephrotic syndrome:
Minimal change disease does not reduce the amount of urine produced. It rarely progresses to kidney failure.
The doctor may not be able to see any signs of the disease, other than swelling. Blood and urine tests reveal signs of nephrotic syndrome, including:
A kidney biopsy and examination of the tissue with an electron microscope can show signs of minimal change disease. An immunofluorescence exam of the biopsied kidney tissue will be negative.
Corticosteroids can cure minimal change disease in most children. Some patients may need to stay on steroids to keep the disease in remission.
Adults do not respond to steroids as well as children, but many still find steroids effective. Adults may have more frequent relapses and become dependent on steroids.
Patients who have three or more relapses may do better with cytotoxic therapy instead of steroids. In most cases, this involves a medication called cyclophosphamide. Other medicines that have been used include cyclosporine and chlorambucil.
Swelling may be treated with:
You may also be told to reduce the amount of salt in your diet.
Children with minimal change disease usually respond better to corticosteroids than adults. Children often respond within the first month.
A relapse can occur. However, patients may improve after long-term treatment with corticosteroids and medications that suppress the immune system (immunosuppressive medications).
Call for an appointment with your health care provider if:
There is no known prevention.
Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.
Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Brenner BM, ed. Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 30.
Pais P, Avner ED. Nephrotic syndrome. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 521.